Pediatric Cardiology Featured Articles of April 2017

Pediatric Cardiology Reviews of April 2017 Manuscripts

 

Long-Term Outcomes of Children with Trisomy 13 and 18 After Congenital Heart Disease Interventions.

Peterson JK, Kochilas LK, Catton KG, Moller JH, Setty SP.

Ann Thorac Surg. 2017 Apr 26. pii: S0003-4975(17)30375-2. doi: 10.1016/j.athoracsur.2017.02.068. [Epub ahead of print]

PMID: 28456396

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Select item 28445485

 

Take Home Points:

 

  • Greater than 10-year survival is possible for selected patients with trisomy 13 or 18 who undergo complete repair of their congenital heart disease.
  • Due to other complex needs and their severe long term developmental disabilities, an interdisciplinary team approach, along with parental involvement and program level administrative input, will be necessary to decide whether intervention should be recommended for these patients.

Hershenson, JaredCommentary from Dr. Jared Hershenson (Greater Washington DC), section editor of Pediatric Cardiology Journal Watch:  Historically, limited intervention has been recommended for patients with trisomy 13 or 18 due to an expected death in most occurring before 1 year of life. A few more recent studies have suggested a higher survival rate, especially among those who underwent intervention to repair the congenital heart disease (CHD). This retrospective review represents the largest cohort and longer follow up than previously reported.  Of the intent-to-treat cohort, 29 patients with T13 and 69 with T18 were studied from 3 large centers in the US and assigned to either corrective/complete CHD repair, palliative repair, or single ventricle palliation. The other T13 and T18 patients during that 36-year time period represented the non-intervention cohort. Patient characteristics, including type of CHD based on STAT scoring, non-cardiac morbidities, era of repair, type of surgical repair, mortality (in-hospital) and length of stay were recorded. Linkage to the National Death Index was done to obtain long-term mortality data. Most patients not offered intervention were from an earlier era, more likely to have a single ventricle CHD, and have more comorbidities. Pre-operative comorbidities were common and included tracheostomy and gastrostomy. 62% of those patients treated underwent single-stage corrective repair, 27.5% underwent palliation (PA band, PDA ligation or shunt) and the few remaining had single ventricle palliation. 66% had STAT 1 or 2 procedure complexity, and 33.7% had STAT 3 or 4. There were no STAT 5 surgeries. The median survival for T13 and T18 with any type of cardiac surgery were summarized in figure 1, with a median survival of 14.8 and 16.2 years respectively. When comparing the corrective and palliative pathways, a significant increase on length of survival was shown with definitive repair, 32.2 years vs. 10.1 years and an 80% 10-year survival (figure 2). As expected though, the mortality rates, even for the lower risk surgeries, were significantly higher for the trisomies than reported in the general CHD population.

Peds 1 april

Ped 2 May

 

 

 

Right ventricular systolic function in hypoplastic left heart syndrome: A comparison of manual and automated software to measure fractional area change.

Ruotsalainen HK, Bellsham-Revell HR, Bell AJ, Pihkala JI, Ojala TH, Simpson JM.

Echocardiography. 2017 Apr;34(4):587-593. doi: 10.1111/echo.13470. Epub 2017 Feb 13.

PMID: 28191731

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Select item 27099276

 

Take Home Points:

 

  • Automated fractional area change of the single right ventricle correlates better than manual derivation with MRI-derived EF and has good intra-observer and inter-observer variability.
  • Slight differences in FAC were noted between the automated measurement programs, so they should not be used interchangeably.

Commentary from Dr. Jared Hershenson (Greater Washington DC), section editor of Pediatric Cardiology Journal Watch:  Quantification of right ventricular (RV) function in HLHS is difficult due to the intrinsic anatomic nature of the RV as well as the variability of the size and morphology of the hypoplastic left ventricle in HLHS, which is known to affect RV function due to ventricular-ventricular interactions. MRI is considered the gold standard but is often less available and requires sedation in younger patients.  Many investigators have looked at novel functional assessment including tissue Doppler imaging, speckle tracking and 3D echo. Previous studies have looked at fractional area change (FAC) of the RV showing possibly better correlation with MRI derived EF. This study compared 2 relatively new automated measures of FAC (VVI – Siemens and Q-lab – Philips) with manual measurement of FAC and with MRI derived ejection fraction. 51 patients at various stages of repair were studied. Mean age was 2 years old. Most had normal systolic function by MRI (mean 58.9 +/- 9.2%). Correlation of VVI FAC was 0.7 and Q-lab was 0.6 using Pearson’s correlation. Intra-observer and inter-observer repeatability was better for both automated measures. Inter-observer repeatability was worst with the manually derived FAC, likely since the shape of the RV is unusual and the endocardial border difficult to define. Since there were few patients with decreased RV function, further studies will be necessary to determine how well FAC correlates with MRI in that scenario. A limitation of this study with regards to office-based follow up and assessment was that all echocardiograms were done under anesthesia.

Peds 3 april

Penetrance of Hypertrophic Cardiomyopathy in Children Who Are Mutation Positive.

Vermeer AM, Clur SB, Blom NA, Wilde AA, Christiaans I.

J Pediatr. 2017 Apr 7. pii: S0022-3476(17)30382-7. doi: 10.1016/j.jpeds.2017.03.033. [Epub ahead of print]

PMID: 28396031

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Select item 28396253

 

Take Home Points:

 

  • Genotype positive, phenotype negative patients for hypertrophic cardiomyopathy (primarily MYBC3) have a seemingly low risk of developing hypertrophy or having cardiac events during childhood (< 18 years old) if their initial evaluation is negative.
  • Frequency of screening during childhood could potentially be less often than previously established if future larger studies are consistent with these results.

 

Commentary from Dr. Jared Hershenson (Greater Washington DC), section editor of Pediatric Cardiology Journal Watch: 19 children with a mean age of 12.1 +/-3.4 years in the Netherlands with gene positive HCM but phenotype negative were enrolled and followed for a mean time period of 6.9 +/- 3.8 years. None h1ad been evaluated prior to genetic testing. European Society of Cardiology guidelines were used for diagnosis and testing modalities. Almost 80% at MYBC3 mutation. 8 patients (6.7%) were diagnosed with HCM at first (n=5) or follow up (n=3) evaluations, and 6 of those (5%) were less than 18 years old. Patients were followed annually, but only 80% had subsequent follow up evaluation. None died and only 1 patient had ICD placement for one risk factor for sudden cardiac death (extreme LVH; 30 mm) and had an appropriate shock 4 years after implantation. There were no other cardiac events in the entire cohort. Overall, they showed a low frequency of hypertrophy overall and a very low frequency if the initial evaluation was negative. This study is consistent with other studies, notably Jensen et al, Circulation 2013, that the majority of patients do not develop hypertrophy in childhood. The high incidence of a single mutation may suggest that these results are not applicable to the other mutations or in those with multiple mutations.  Guidelines for screening could potentially require revision, with less frequency during childhood/adolescence, and more often during early adulthood (20s).

Peds 4 april

 

 

 

 

Delayed Myocardial Enhancement in Pediatric Hypertrophic Cardiomyopathy: Correlation with LV Function, Echocardiography, and Demographic Parameters.

El Saiedi S, Behairy NH, Kharabish A, Esmail R, Seliem ZS, Shafik M, El Mozy W.

Pediatr Cardiol. 2017 Apr 11. doi: 10.1007/s00246-017-1612-y. [Epub ahead of print]

PMID: 28401251

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Take Home Points:

  • Delayed myocardial enhancement (DE) is a common finding in childhood hypertrophic cardiomyopathy.
  • The percentage of DE correlates with the NYHA functional class, the LVOT peak gradient and the left ventricular mass index.

 

Voges IngaCommentary from Dr. Inga Voges (London UK), section editor of Congenital Heart Surgery Journal Watch: Only few data exist on the presence and role of delayed enhancement (DE) in children with hypertrophic cardiomyopathy (HCM). This prospective study evaluated the role of delayed enhancement cardiovascular magnetic resonance imaging (DE-MRI) in pediatric patients with HCM. 40 children with HCM were included and clinical, DE-MRI, echocardiographic and Holter monitoring data were collected.

In 33 patients (82.5%) DE was detected with a midwall patchy and focal enhancement pattern in most patients. The percentage of DE correlated with the NYHA functional class, the LVOT peak gradient measured by echocardiography and the left ventricular mass index.

The authors conclude that their data may help in further patient management.

Peds 5 april

 

 

 

Pediatric Fontan patients are at risk for myocardial fibrotic remodeling and dysfunction.

Kato A, Riesenkampff E, Yim D, Yoo SJ, Seed M, Grosse-Wortmann L.

Int J Cardiol. 2017 Apr 22. pii: S0167-5273(17)30279-6. doi: 10.1016/j.ijcard.2017.04.073. [Epub ahead of print]

PMID: 28461021

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Take Home Points:

  • Fontan patients with a single right ventricle (SRV) have higher markers of diffuse myocardial fibrosis than single left ventricle (SLV) patients and controls.
  • CMR feature tracking derived circumferential and radial strains were reduced in pediatric Fontan patients.
  • Elevated markers of diffuse fibrosis were associated with abnormal strain and strain rate.

 

Comment from Dr. Inga Voges (London), section editor of Congenital Heart Surgery Journal Watch: This interesting retrospective study used T1 mapping by cardiovascular magnetic resonance (CMR) imaging to assess diffuse myocardial fibrosis in SV patients. In addition, CMR feature tracking to measure strain and strain rate was applied.

The authors included 21 SV patients after Fontan completion and compared them with 24 healthy controls. Of the 21 patients, 13 had a SLV and 8 had a SRV.

The main findings were, higher native T1 relaxation times and higher extracellular volumes (ECV), both markers of diffuse fibrosis, in patients with a SRV compared to patients with a SLV and compared to controls (see figure). In both, SRV and SLV patients, global circumferential and radial strains were reduced. Patients with a SRV showed reduced radial and circumferential strains compared to SLV patients. Longitudinal Elevated native T1 times and ECV were associated with decreased ventricular strain and strain rate.

Despite the small number of included patients, these findings suggest that Fontan patients with a SRV may have a higher risk for adverse ventricular structural and functional remodelling.

Peds 6 april

 

  

Pediatric Cardiology April 2017

 

  1. Update on congenital heart disease and sudden infant/perinatal death: from history to future trends.

Ottaviani G, Buja LM.

J Clin Pathol. 2017 Apr 27. pii: jclinpath-2017-204326. doi: 10.1136/jclinpath-2017-204326. [Epub ahead of print] Review.

PMID: 28450386

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  1. Novel missense mutation in DLL4 in a Japanese sporadic case of Adams-Oliver syndrome.

Nagasaka M, Taniguchi-Ikeda M, Inagaki H, Ouchi Y, Kurokawa D, Yamana K, Harada R, Nozu K, Sakai Y, Mishra SK, Yamaguchi Y, Morikoka I, Toda T, Kurahashi H, Iijima K.

J Hum Genet. 2017 Apr 27. doi: 10.1038/jhg.2017.48. [Epub ahead of print]

PMID: 28446798

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Select item 28456396

 

  1. Long-Term Outcomes of Children With Trisomy 13 and 18 After Congenital Heart Disease Interventions.

Peterson JK, Kochilas LK, Catton KG, Moller JH, Setty SP.

Ann Thorac Surg. 2017 Apr 26. pii: S0003-4975(17)30375-2. doi: 10.1016/j.athoracsur.2017.02.068. [Epub ahead of print]

PMID: 28456396

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Select item 28445485

 

  1. Validity and reliability of the Physical Activity Questionnaire for Children (PAQ-C) and Adolescents (PAQ-A) in individuals with congenital heart disease.

Voss C, Dean PH, Gardner RF, Duncombe SL, Harris KC.

PLoS One. 2017 Apr 26;12(4):e0175806. doi: 10.1371/journal.pone.0175806. eCollection 2017.

PMID: 28445485 Free Article

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  1. Whole-exome sequencing identifies SGCD and ACVRL1 mutations associated with total anomalous pulmonary venous return (TAPVR) in Chinese population.

Li J, Yang S, Pu Z, Dai J, Jiang T, Du F, Jiang Z, Cheng Y, Dai G, Wang J, Qi J, Cao L, Cheng X, Ren C, Li X, Qin Y.

Oncotarget. 2017 Apr 25;8(17):27812-27819. doi: 10.18632/oncotarget.15434.

PMID: 28412737 Free Article

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  1. Asian consortium on radiation dose of pediatric cardiac CT (ASCI-REDCARD).

Hui PKT, Goo HW, Du J, Ip JJK, Kanzaki S, Kim YJ, Kritsaneepaiboon S, Lilyasari O, Siripornpitak S.

Pediatr Radiol. 2017 Apr 24. doi: 10.1007/s00247-017-3847-4. [Epub ahead of print]

PMID: 28435986

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  1. Predictors of missed appointments in patients referred for congenital or pediatric cardiac magnetic resonance.

Lu JC, Lowery R, Yu S, Ghadimi Mahani M, Agarwal PP, Dorfman AL.

Pediatr Radiol. 2017 Apr 21. doi: 10.1007/s00247-017-3851-8. [Epub ahead of print]

PMID: 28432402

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Select item 28434627

 

  1. [Sports in children with congenital heart diseases].

Bosser G, Moulin-Zinsch A, Fischer-Atalla R.

Presse Med. 2017 Apr 21. pii: S0755-4982(17)30110-0. doi: 10.1016/j.lpm.2017.03.005. [Epub ahead of print] French.

PMID: 28434627

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Select item 28430286

 

  1. Clinical presentation and outcomes of patients with acute rheumatic fever and rheumatic heart disease seen at a tertiary hospital setting in Port Elizabeth, South Africa.

Makrexeni ZM, Pepeta L.

Cardiovasc J Afr. 2017 Apr 20;28:1-7. doi: 10.5830/CVJA-2017-019. [Epub ahead of print]

PMID: 28430286

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  1. Echocardiographic findings in infants with presumed congenital Zika syndrome: Retrospective case series study.

Cavalcanti DD, Alves LV, Furtado GJ, Santos CC, Feitosa FG, Ribeiro MC, Menge P, Lira IM, Alves JG.

PLoS One. 2017 Apr 20;12(4):e0175065. doi: 10.1371/journal.pone.0175065. eCollection 2017.

PMID: 28426680 Free PMC Article

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  1. Recurrence of congenital heart defects among siblings-a nationwide study.

Brodwall K, Greve G, Leirgul E, Tell GS, Vollset SE, Øyen N.

Am J Med Genet A. 2017 Apr 19. doi: 10.1002/ajmg.a.38237. [Epub ahead of print]

PMID: 28425218

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Select item 28422456

 

  1. Identification of a novel and functional mutation in the TBX5 gene in a patient by screening from 354 patients with isolated ventricular septal defect.

Chen HX, Zhang X, Hou HT, Wang J, Yang Q, Wang XL, He GW.

Eur J Med Genet. 2017 Apr 18. pii: S1769-7212(16)30323-8. doi: 10.1016/j.ejmg.2017.04.011. [Epub ahead of print]

PMID: 28434921

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Select item 28431884

 

  1. Additive effect of congenital heart disease and early developmental disorders on attention-deficit/hyperactivity disorder and autism spectrum disorder: a nationwide population-based longitudinal study.

Tsao PC, Lee YS, Jeng MJ, Hsu JW, Huang KL, Tsai SJ, Chen MH, Soong WJ, Kou YR.

Eur Child Adolesc Psychiatry. 2017 Apr 17. doi: 10.1007/s00787-017-0989-8. [Epub ahead of print]

PMID: 28417257

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  1. Outcomes from anomalous origin of the left coronary artery from the pulmonary artery repair: Long-term complications in relation to residual myocardial abnormalities.

Kanoh M, Inai K, Shinohara T, Tomimatsu H, Nakanishi T.

J Cardiol. 2017 Apr 17. pii: S0914-5087(17)30095-3. doi: 10.1016/j.jjcc.2017.03.008. [Epub ahead of print]

PMID: 28427867

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  1. Congenital Lung Agenesis: Incidence and Outcome in the North of England.

Thomas M, Robertson N, Miller N, Rankin J, McKean M, Brodlie M.

Birth Defects Res. 2017 Apr 12. doi: 10.1002/bdr2.1011. [Epub ahead of print]

PMID: 28402072

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Select item 28403448

 

  1. EuroEcho-Imaging 2016: highlights.

Magne J, Popescu BA, Bucciarelli-Ducci C, Cosyns B, Donal E, Gimelli A, Miller O, Badano L, Habib G.

Eur Heart J Cardiovasc Imaging. 2017 Apr 11. doi: 10.1093/ehjci/jex063. [Epub ahead of print]

PMID: 28407053

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Select item 28430115

 

  1. Clinical utility and diagnostic accuracy of palm-held, mini-sized ultrasonocardiographic scanner in congenital heart disease.

Lo MH, Huang CF, Lin IC, Lin YJ, Kuo HC, Hsieh KS.

J Formos Med Assoc. 2017 Apr 9. pii: S0929-6646(16)30358-8. doi: 10.1016/j.jfma.2017.02.018. [Epub ahead of print]

PMID: 28404481 Free Article

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  1. Vertebral artery from descending thoracic aorta: rare anatomic variant with diagnostic implication.

Sharma A, Kumar S, Sharma S.

Acta Neurochir (Wien). 2017 Apr 8. doi: 10.1007/s00701-017-3175-3. [Epub ahead of print]

PMID: 28391445

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Select item 28390064

 

  1. A recognizable systemic connective tissue disorder with polyvalvular heart dystrophy and dysmorphism associated with TAB2 mutations.

Ritelli M, Morlino S, Giacopuzzi E, Bernardini L, Torres B, Santoro G, Ravasio V, Chiarelli N, D’Angelantonio D, Novelli A, Grammatico P, Colombi M, Castori M.

Clin Genet. 2017 Apr 6. doi: 10.1111/cge.13032. [Epub ahead of print]

PMID: 28386937

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Select item 28391001

 

  1. Three-Dimensional Mitral Valve Morphology and Age-Related Trends Children and Young Adults with Structurally Normal Hearts Using Transthoracic Echocardiography.

Jolley MA, Ghelani SJ, Adar A, Harrild DM.

J Am Soc Echocardiogr. 2017 Apr 6. pii: S0894-7317(17)30048-2. doi: 10.1016/j.echo.2017.01.018. [Epub ahead of print]

PMID: 28391001

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Select item 28382967

 

  1. MicroRNAs Association in the Cardiac Hypertrophy Secondary to Complex Congenital Heart Disease in Children.

Sánchez-Gómez MC, García-Mejía KA, Pérez-Díaz Conti M, Díaz-Rosas G, Palma-Lara I, Sánchez-Urbina R, Klünder-Klünder M, Botello-Flores JA, Balderrábano-Saucedo NA, Contreras-Ramos A.

Pediatr Cardiol. 2017 Apr 5. doi: 10.1007/s00246-017-1607-8. [Epub ahead of print]

PMID: 28382463

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Select item 28375677

 

  1. Autosomal Recessive Cardiomyopathy Presenting as Acute Myocarditis.

Belkaya S, Kontorovich AR, Byun M, Mulero-Navarro S, Bajolle F, Cobat A, Josowitz R, Itan Y, Quint R, Lorenzo L, Boucherit S, Stoven C, Di Filippo S, Abel L, Zhang SY, Bonnet D, Gelb BD, Casanova JL.

J Am Coll Cardiol. 2017 Apr 4;69(13):1653-1665. doi: 10.1016/j.jacc.2017.01.043.

PMID: 28359509

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Select item 28374449

 

  1. 4D MUSIC CMR: value-based imaging of neonates and infants with congenital heart disease.

Nguyen KL, Han F, Zhou Z, Brunengraber DZ, Ayad I, Levi DS, Satou GM, Reemtsen BL, Hu P, Finn JP.

J Cardiovasc Magn Reson. 2017 Apr 3;19(1):40. doi: 10.1186/s12968-017-0352-8.

PMID: 28366171 Free PMC Article

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Select item 28370066

 

  1. Isolated right ventricular apical hypoplasia characterized by computed tomography and echocardiography.

Zhou D, Liao HQ, Ouyang MZ, Shang QL, Zhang M.

J Clin Ultrasound. 2017 Apr 3. doi: 10.1002/jcu.22479. [Epub ahead of print]

PMID: 28370066

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Select item 28320222

 

  1. Child and Adolescent Health From 1990 to 2015: Findings From the Global Burden of Diseases, Injuries, and Risk Factors 2015 Study.

Global Burden of Disease Child and Adolescent Health Collaboration., Kassebaum N, Kyu HH, Zoeckler L, Olsen HE, Thomas K, Pinho C, Bhutta ZA, Dandona L, Ferrari A, Ghiwot TT, Hay SI, Kinfu Y, Liang X, Lopez A, Malta DC, Mokdad AH, Naghavi M, Patton GC, Salomon J, Sartorius B, Topor-Madry R, Vollset SE, Werdecker A, Whiteford HA, Abate KH, Abbas K, Abreha Damtew S, Ahmed MB, Akseer N, Al-Raddadi R, Alemayohu MA, Altirkawi K, Abajobir AA, Amare AT, Antonio CA, Arnlov J, Artaman A, Asayesh H, Avokpaho EF, Awasthi A, Ayala Quintanilla BP, Bacha U, Balem D, Barac A, Bärnighausen TW, Baye E, Bedi N, Bensenor IM, Berhane A, Bernabe E, Bernal OA, Beyene AS, Biadgilign S, Bikbov B, Boyce CA, Brazinova A, Hailu GB, Carter A, Castañeda-Orjuela CA, Catalá-López F, Charlson FJ, Chitheer AA, Choi JJ, Ciobanu LG, Crump J, Dandona R, Dellavalle RP, Deribew A, deVeber G, Dicker D, Betsu BB, Ding EL, Dubey M, Endries AY, Erskine HE, Faraon EJ, Faro A, Farzadfar F, Fernandes JC, Fijabi DO, Fitzmaurice C, Fleming TD, Flor LS, Foreman KJ, Franklin RC, Fraser MS, Frostad JJ, Fullman N, Gebregergs GB, Gebru AA, Geleijnse JM, Gibney KB, Gidey Yihdego M, Ginawi IA, Gishu MD, Gizachew TA, Glaser E, Gold AL, Goldberg E, Gona P, Goto A, Gugnani HC, Jiang G, Gupta R, Tesfay FH, Hankey GJ, Havmoeller R, Hijar M, Horino M, Hosgood HD, Hu G, Jacobsen KH, Jakovljevic MB, Jayaraman SP, Jha V, Jibat T, Johnson CO, Jonas J, Kasaeian A, Kawakami N, Keiyoro PN, Khalil I, Khang YH, Khubchandani J, Ahmad Kiadaliri AA, Kieling C, Kim D, Kissoon N, Knibbs LD, Koyanagi A, Krohn KJ, Kuate Defo B, Kucuk Bicer B, Kulikoff R, Kumar GA, Lal DK, Lam HY, Larson HJ, Larsson A, Laryea DO, Leung J, Lim SS, Lo LT, Lo WD, Looker KJ, Lotufo PA, Magdy Abd H, El Razek, Malekzadeh R, Markos Shifti D, Mazidi M, Meaney PA, Meles KG, Memiah P, Mendoza W, Abera Mengistie M, Mengistu GW, Mensah GA, Miller TR, Mock C, Mohammadi A, Mohammed S, Monasta L, Mueller U, Nagata C, Naheed A, Nguyen G, Nguyen QL, Nsoesie E, Oh IH, Okoro A, Olusanya JO, Olusanya BO, Ortiz A, Paudel D, Pereira DM, Perico N, Petzold M, Phillips MR, Polanczyk GV, Pourmalek F, Qorbani M, Rafay A, Rahimi-Movaghar V, Rahman M, Rai RK, Ram U, Rankin Z, Remuzzi G, Renzaho AM, Roba HS, Rojas-Rueda D, Ronfani L, Sagar R, Sanabria JR, Kedir Mohammed MS, Santos IS, Satpathy M, Sawhney M, Schöttker B, Schwebel DC, Scott JG, Sepanlou SG, Shaheen A, Shaikh MA, She J, Shiri R, Shiue I, Sigfusdottir ID, Singh J, Slipakit N, Smith A, Sreeramareddy C, Stanaway JD, Stein DJ, Steiner C, Sufiyan MB, Swaminathan S, Tabarés-Seisdedos R, Tabb KM, Tadese F, Tavakkoli M, Taye B, Teeple S, Tegegne TK, Temam Shifa G, Terkawi AS, Thomas B, Thomson AJ, Tobe-Gai R, Tonelli M, Tran BX, Troeger C, Ukwaja KN, Uthman O, Vasankari T, Venketasubramanian N, Vlassov VV, Weiderpass E, Weintraub R, Gebrehiwot SW, Westerman R, Williams HC, Wolfe CD, Woodbrook R, Yano Y, Yonemoto N, Yoon SJ, Younis MZ, Yu C, Zaki ME, Zegeye EA, Zuhlke LJ, Murray CJ, Vos T.

JAMA Pediatr. 2017 Apr 3. doi: 10.1001/jamapediatrics.2017.0250. [Epub ahead of print]

PMID: 28384795

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Select item 28370252

 

  1. Chromosome 13q deletion syndrome involving 13q31‑qter: A case report.

Wang YP, Wang DJ, Niu ZB, Cui WT.

Mol Med Rep. 2017 Apr 3. doi: 10.3892/mmr.2017.6425. [Epub ahead of print]

PMID: 28393221

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Select item 28393185

 

  1. [Importance of neonatal screening for congenital heart disease in China].

Huang GY.

Zhonghua Er Ke Za Zhi. 2017 Apr 2;55(4):241-243. doi: 10.3760/cma.j.issn.0578-1310.2017.04.001. Chinese. No abstract available.

PMID: 28441817

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Select item 27941485

 

  1. 22q11.2 deletion syndrome in diverse populations.

Kruszka P, Addissie YA, McGinn DE, Porras AR, Biggs E, Share M, Crowley TB, Chung BH, Mok GT, Mak CC, Muthukumarasamy P, Thong MK, Sirisena ND, Dissanayake VH, Paththinige CS, Prabodha LB, Mishra R, Shotelersuk V, Ekure EN, Sokunbi OJ, Kalu N, Ferreira CR, Duncan JM, Patil SJ, Jones KL, Kaplan JD, Abdul-Rahman OA, Uwineza A, Mutesa L, Moresco A, Obregon MG, Richieri-Costa A, Gil-da-Silva-Lopes VL, Adeyemo AA, Summar M, Zackai EH, McDonald-McGinn DM, Linguraru MG, Muenke M.

Am J Med Genet A. 2017 Apr;173(4):879-888. doi: 10.1002/ajmg.a.38199.

PMID: 28328118

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  1. Noonan syndrome, PTPN11 mutations, and brain tumors. A clinical report and review of the literature.

Siegfried A, Cances C, Denuelle M, Loukh N, Tauber M, Cavé H, Delisle MB.

Am J Med Genet A. 2017 Apr;173(4):1061-1065. doi: 10.1002/ajmg.a.38108.

PMID: 28328117

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Select item 28190295

 

  1. Neonatal lupus: Follow-up in infants with anti-SSA/Ro antibodies and review of the literature.

Zuppa AA, Riccardi R, Frezza S, Gallini F, Luciano RM, Alighieri G, Romagnoli C, De Carolis S.

Autoimmun Rev. 2017 Apr;16(4):427-432. doi: 10.1016/j.autrev.2017.02.010. Epub 2017 Feb 14. Review.

PMID: 28212920

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Select item 27807680

 

  1. Managing a “New” Murmur in Healthy Children and Teens.

Gupta LJ, May JW.

Clin Pediatr (Phila). 2017 Apr;56(4):357-362. doi: 10.1177/0009922816656623. Epub 2016 Jul 19.

PMID: 27356630

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Select item 28365652

 

  1. Interstage Home Monitoring After Newborn First-Stage Palliation for Hypoplastic Left Heart Syndrome: Family Education Strategies.

Nieves JA, Uzark K, Rudd NA, Strawn J, Schmelzer A, Dobrolet N.

Crit Care Nurse. 2017 Apr;37(2):72-88. doi: 10.4037/ccn2017763.

PMID: 28365652

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Select item 26889873

 

  1. Significant motor improvement in an infant with congenital heart disease and a rolandic stroke: The impact of early intervention.

Gallagher A, Dagenais L, Doussau A, Décarie JC, Materassi M, Gagnon K, Prud’homme J, Vobecky S, Poirier N, Carmant L.

Dev Neurorehabil. 2017 Apr;20(3):165-168. doi: 10.3109/17518423.2015.1132280. Epub 2016 Feb 18.

PMID: 26889873

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Select item 28266734

 

  1. Cardiac response to enzyme replacement therapy in infantile Pompe disease with severe hypertrophic cardiomyopathy.

Avula S, Nguyen TM, Marble M, Lilje C.

Echocardiography. 2017 Apr;34(4):621-624. doi: 10.1111/echo.13490. Epub 2017 Mar 7.

PMID: 28266734

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  1. Right ventricular systolic function in hypoplastic left heart syndrome: A comparison of manual and automated software to measure fractional area change.

Ruotsalainen HK, Bellsham-Revell HR, Bell AJ, Pihkala JI, Ojala TH, Simpson JM.

Echocardiography. 2017 Apr;34(4):587-593. doi: 10.1111/echo.13470. Epub 2017 Feb 13.

PMID: 28191731

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  1. Structural cerebral abnormalities and neurodevelopmental status in single ventricle congenital heart disease before Fontan procedure.

Knirsch W, Mayer KN, Scheer I, Tuura R, Schranz D, Hahn A, Wetterling K, Beck I, Latal B, Reich B.

Eur J Cardiothorac Surg. 2017 Apr 1;51(4):740-746. doi: 10.1093/ejcts/ezw399.

PMID: 28013288

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  1. Resilience and personal growth: A potential resource for therapeutic programmes in people with congenital heart disease.

Dorka R.

Eur J Cardiovasc Nurs. 2017 Apr;16(4):270-271. doi: 10.1177/1474515116687223. Epub 2017 Jan 10. No abstract available.

PMID: 28071940

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  1. Hippocampal damage and memory impairment in congenital cyanotic heart disease.

Muñoz-López M, Hoskote A, Chadwick MJ, Dzieciol AM, Gadian DG, Chong K, Banks T, de Haan M, Baldeweg T, Mishkin M, Vargha-Khadem F.

Hippocampus. 2017 Apr;27(4):417-424. doi: 10.1002/hipo.22700. Epub 2017 Jan 31.

PMID: 28032672 Free PMC Article

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  1. Making the Quick Diagnosis: A Case of Neonatal Shock.

Gardiner M, Ruttan TK, Kienstra AJ, Wilkinson M.

J Emerg Med. 2017 Apr;52(4):e139-e144. doi: 10.1016/j.jemermed.2016.11.003. Epub 2016 Dec 13.

PMID: 27986330

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40.The effects of corrective surgery on endothelial biomarkers and anthropometric data in children with congenital heart disease.

Chung HT, Chang YS, Liao SL, Lai SH.

J Int Med Res. 2017 Apr;45(2):493-503. doi: 10.1177/0300060516685659. Epub 2017 Jan 1.

PMID: 28415932

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  1. Clinical Factors Associated with Cerebral Metabolism in Term Neonates with Congenital Heart Disease.

Harbison AL, Votava-Smith JK, Del Castillo S, Kumar SR, Lee V, Schmithorst V, Lai HA, O’Neil S, Bluml S, Paquette L, Panigrahy A.

J Pediatr. 2017 Apr;183:67-73.e1. doi: 10.1016/j.jpeds.2016.12.061. Epub 2017 Jan 19.

PMID: 28109537

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Select item 28081891

 

  1. Preschool Neurodevelopmental Outcomes in Children with Congenital Heart Disease.

Brosig CL, Bear L, Allen S, Hoffmann RG, Pan A, Frommelt M, Mussatto KA.

J Pediatr. 2017 Apr;183:80-86.e1. doi: 10.1016/j.jpeds.2016.12.044. Epub 2017 Jan 9.

PMID: 28081891

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  1. Social determinants of health: integral to developmental risk assessment in congenital heart disease.

Wong P, Denburg A, Moore A, Ford-Jones E.

J Pediatr. 2017 Apr;183:201. doi: 10.1016/j.jpeds.2016.11.058. Epub 2016 Dec 14. No abstract available.

PMID: 27986271

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  1. “I was so worried about every drop of milk” – feeding problems at home are a significant concern for parents after major heart surgery in infancy.

Tregay J, Brown K, Crowe S, Bull C, Knowles R, Wray J.

Matern Child Nutr. 2017 Apr;13(2). doi: 10.1111/mcn.12302. Epub 2016 Feb 19.

PMID: 26891904

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  1. Renal dysplasia characterized by prominent cartilaginous metaplasia lesions in VACTERL association: A case report.

Nakaya T, Hyuga T, Tanaka Y, Kawai S, Nakai H, Niki T, Tanaka A.

Medicine (Baltimore). 2017 Apr;96(15):e6499. doi: 10.1097/MD.0000000000006499.

PMID: 28403078 Free PMC Article

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  1. Germline mutations in ABL1 cause an autosomal dominant syndrome characterized by congenital heart defects and skeletal malformations.

Wang X, Charng WL, Chen CA, Rosenfeld JA, Al Shamsi A, Al-Gazali L, McGuire M, Mew NA, Arnold GL, Qu C, Ding Y, Muzny DM, Gibbs RA, Eng CM, Walkiewicz M, Xia F, Plon SE, Lupski JR, Schaaf CP, Yang Y.

Nat Genet. 2017 Apr;49(4):613-617. doi: 10.1038/ng.3815. Epub 2017 Mar 13.

PMID: 28288113

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  1. Six-Minute Walk Test in Evaluation of Children with Pulmonary Arterial Hypertension.

Zuk M, Migdal A, Jagiellowicz-Kowalska D, Mazurkiewicz K, Sadel-Wieczorek A, Brzezinska-Rajszys G.

Pediatr Cardiol. 2017 Apr;38(4):754-761. doi: 10.1007/s00246-017-1575-z. Epub 2017 Feb 27.

PMID: 28239753 Free PMC Article

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  1. Piloting the Use of Patient-Specific Cardiac Models as a Novel Tool to Facilitate Communication During Cinical Consultations.

Biglino G, Koniordou D, Gasparini M, Capelli C, Leaver LK, Khambadkone S, Schievano S, Taylor AM, Wray J.

Pediatr Cardiol. 2017 Apr;38(4):813-818. doi: 10.1007/s00246-017-1586-9. Epub 2017 Feb 18.

PMID: 28214968 Free PMC Article

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  1. Alkaline Phosphatase: A Biomarker of Cardiac Function in Pediatric Patients.

Makil ES, Tang X, Frazier EA, Collins RT 2nd.

Pediatr Cardiol. 2017 Apr;38(4):762-769. doi: 10.1007/s00246-017-1577-x. Epub 2017 Feb 9.

PMID: 28184975

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  1. Factors Associated with Readmission of Patients with Congenital Heart Disease in a Swiss University Hospital.

Chave M, Marques-Vidal P.

Pediatr Cardiol. 2017 Apr;38(4):650-655. doi: 10.1007/s00246-016-1562-9. Epub 2017 Feb 2.

PMID: 28154912

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  1. Initial Field Test of a Cloud-Based Cardiac Auscultation System to Determine Murmur Etiology in Rural China.

Pyles L, Hemmati P, Pan J, Yu X, Liu K, Wang J, Tsakistos A, Zheleva B, Shao W, Ni Q.

Pediatr Cardiol. 2017 Apr;38(4):656-662. doi: 10.1007/s00246-016-1563-8. Epub 2017 Feb 2.

PMID: 28150025

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  1. Left Ventricular Noncompaction Cardiomyopathy in Pediatric Patients: A Case Series of a Clinically Heterogeneous Disease.

Gupta U, Makhija P.

Pediatr Cardiol. 2017 Apr;38(4):681-690. doi: 10.1007/s00246-016-1566-5. Epub 2017 Jan 21.

PMID: 28108756

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  1. Risk Factors for Necrotizing Enterocolitis in Neonates: A Retrospective Case-Control Study.

Lu Q, Cheng S, Zhou M, Yu J.

Pediatr Neonatol. 2017 Apr;58(2):165-170. doi: 10.1016/j.pedneo.2016.04.002. Epub 2016 Jun 22.

PMID: 27543379 Free Article

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  1. Current and future therapeutic approaches to the congenital myopathies.

Jungbluth H, Ochala J, Treves S, Gautel M.

Semin Cell Dev Biol. 2017 Apr;64:191-200. doi: 10.1016/j.semcdb.2016.08.004. Epub 2016 Aug 8. Review.

PMID: 27515125

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Select item 28142228

 

  1. NOTCH1-Dependent Nitric Oxide Signaling Deficiency in Hypoplastic Left Heart Syndrome Revealed Through Patient-Specific Phenotypes Detected in Bioengineered Cardiogenesis.

Hrstka SC, Li X, Nelson TJ; Wanek Program Genetics Pipeline Group..

Stem Cells. 2017 Apr;35(4):1106-1119. doi: 10.1002/stem.2582. Epub 2017 Mar 5.

PMID: 28142228

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  1. Comorbidities in Down syndrome livebirths and health care intervention: an initial experience from the birth defects registry in Southern Thailand.

Jaruratanasirikul S, Limpitikul W, Dissaneevate P, Booncharoen P, Tantichantakarun P.

World J Pediatr. 2017 Apr;13(2):152-157. doi: 10.1007/s12519-016-0093-z. Epub 2017 Jan 15.

PMID: 28101777

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  1. Elevated serum levels of ghrelin and TNF-α in patients with cyanotic and acyanotic congenital heart disease.

Zhang S, Guo GL, Yang LL, Sun LQ.

World J Pediatr. 2017 Apr;13(2):122-128. doi: 10.1007/s12519-016-0068-0. Epub 2016 Nov 15.

PMID: 27878778

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  1. Z-score of Mitral Annular Plane Systolic Excursion is a Useful Indicator of Evaluation of Left Ventricular Function in Patients with Acute-Phase Kawasaki Disease.

Hashimoto I, Watanabe K.

Pediatr Cardiol. 2017 Apr 29. doi: 10.1007/s00246-017-1619-4. [Epub ahead of print]

PMID: 28456832

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  1. Erratum to: Decreased Diastolic Ventricular Kinetic Energy in Young Patients with Fontan Circulation Demonstrated by Four-Dimensional Cardiac Magnetic Resonance Imaging.

Sjöberg P, Heiberg E, Wingren P, Johansson JR, Malm T, Arheden H, Liuba P, Carlsson M.

Pediatr Cardiol. 2017 Apr 29. doi: 10.1007/s00246-017-1617-6. [Epub ahead of print] No abstract available.

PMID: 28456830

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  1. Cardiotoxicity and cardiomyopathy in children and young adult survivors of hematopoietic stem cell transplant.

Rotz SJ, Ryan TD, Hlavaty J, George SA, El-Bietar J, Dandoy CE.

Pediatr Blood Cancer. 2017 Apr 28. doi: 10.1002/pbc.26600. [Epub ahead of print] Review.

PMID: 28453909

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  1. Administration of Dexrazoxane Improves Cardiac Indices in Children and Young Adults With Acute Myeloid Leukemia (AML) While Maintaining Survival Outcomes.

Schloemer NJ, Brickler M, Hoffmann R, Pan A, Simpson P, McFadden V, Block J, Tower RL 2nd, Burke MJ.

J Pediatr Hematol Oncol. 2017 Apr 27. doi: 10.1097/MPH.0000000000000838. [Epub ahead of print]

PMID: 28452856

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  1. Training Pediatric Fellows in Palliative Care: A Pilot Comparison of Simulation Training and Didactic Education.

Brock KE, Cohen HJ, Sourkes BM, Good JJ, Halamek LP.

J Palliat Med. 2017 Apr 24. doi: 10.1089/jpm.2016.0556. [Epub ahead of print]

PMID: 28436742

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Select item 28365261

 

  1. Pediatric Fontan patients are at risk for myocardial fibrotic remodeling and dysfunction.

Kato A, Riesenkampff E, Yim D, Yoo SJ, Seed M, Grosse-Wortmann L.

Int J Cardiol. 2017 Apr 22. pii: S0167-5273(17)30279-6. doi: 10.1016/j.ijcard.2017.04.073. [Epub ahead of print]

PMID: 28461021

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  1. Hypertrophic Cardiomyopathy Cardiac Troponin C Mutations Differentially Affect Slow Skeletal and Cardiac Muscle Regulation.

Veltri T, Landim-Vieira M, Parvatiyar MS, Gonzalez-Martinez D, Dieseldorff Jones KM, Michell CA, Dweck D, Landstrom AP, Chase PB, Pinto JR.

Front Physiol. 2017 Apr 20;8:221. doi: 10.3389/fphys.2017.00221. eCollection 2017.

PMID: 28473771

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  1. How often an isolated cardiac disproportion predicts a coarctation of the aorta? Single center experience and systematic review of the literature.

Ghi T, Dall’Asta A, Cavalli C, Galli L, Weiss A, Pedrazzi G, Kaihura CT, Volpe N, Agnetti A, Frusca T.

J Matern Fetal Neonatal Med. 2017 Apr 20:1-8. doi: 10.1080/14767058.2017.1314459. [Epub ahead of print]

PMID: 28366040

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Select item 28429248

 

  1. Time in therapeutic range as a marker for thrombotic and bleeding outcomes in Fontan patients.

Faircloth JM, Miner KM, Alsaied T, Nelson N, Ciambarella J, Mizuno T, Palumbo JS, Vinks AA, Veldtman GR.

J Thromb Thrombolysis. 2017 Apr 20. doi: 10.1007/s11239-017-1499-8. [Epub ahead of print]

PMID: 28429248

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Select item 28426680

 

  1. A review of pediatric pulmonary hypertension with new guidelines.

Kula S, Pektaş A.

Turk J Med Sci. 2017 Apr 18;47(2):375-380. doi: 10.3906/sag-1605-172.

PMID: 28425226

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Select item 28420661

 

  1. Exercise capacity following pediatric heart transplantation: A systematic review.

Peterson S, Su JA, Szmuszkovicz JR, Johnson R, Sargent B.

Pediatr Transplant. 2017 Apr 17. doi: 10.1111/petr.12922. [Epub ahead of print]

PMID: 28419703

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  1. Evaluation of Ventricular Septal Defect with Special Reference to the Spontaneous Closure Rate, Subaortic Ridge, and Aortic Valve Prolapse II.

Eroglu AG, Atik SU, Sengenc E, Cig G, Saltik IL, Oztunc F.

Pediatr Cardiol. 2017 Apr 12. doi: 10.1007/s00246-017-1597-6. [Epub ahead of print]

PMID: 28401252

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  1. Right Atrial Dysfunction in the Fetus with Severely Regurgitant Tricuspid Valve Disease: A Potential Source of Cardiovascular Compromise.

Howley LW, Khoo NS, Moon-Grady AJ, Patel SS, Alrais F, Tworetzky W, Colen T, Brooks P, Trines J, Ojala T, Hornberger LK.

J Am Soc Echocardiogr. 2017 Apr 11. pii: S0894-7317(17)30002-0. doi: 10.1016/j.echo.2017.01.002. [Epub ahead of print]

PMID: 28410946

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  1. Cardiac pathology in spinal muscular atrophy: a systematic review.

Wijngaarde CA, Blank AC, Stam M, Wadman RI, van den Berg LH, van der Pol WL.

Orphanet J Rare Dis. 2017 Apr 11;12(1):67. doi: 10.1186/s13023-017-0613-5. Review.

PMID: 28399889 Free PMC Article

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Select item 28401251

 

  1. Delayed Myocardial Enhancement in Pediatric Hypertrophic Cardiomyopathy: Correlation with LV Function, Echocardiography, and Demographic Parameters.

El Saiedi S, Behairy NH, Kharabish A, Esmail R, Seliem ZS, Shafik M, El Mozy W.

Pediatr Cardiol. 2017 Apr 11. doi: 10.1007/s00246-017-1612-y. [Epub ahead of print]

PMID: 28401251

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Select item 28394409

 

  1. Severe cardiac involvement in Gaucher type IIIC: a case report and review of the literature.

Kör Y, Keskin M, Başpınar O.

Cardiol Young. 2017 Apr 10:1-4. doi: 10.1017/S1047951117000579. [Epub ahead of print]

PMID: 28393750

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  1. Clinical and Molecular Characterization of Infantile-Onset Pompe Disease in Mainland Chinese Patients: Identification of Two Common Mutations.

Chen X, Liu T, Huang M, Wu J, Zhu J, Guo Y, Xu X, Li F, Wang J, Fu L.

Genet Test Mol Biomarkers. 2017 Apr 10. doi: 10.1089/gtmb.2016.0424. [Epub ahead of print]

PMID: 28394184

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  1. Multiple variations of the cerebral arteries associated with tetralogy of Fallot: a case report.

Uchino A, Saito N, Kozawa E, Masutani S.

Surg Radiol Anat. 2017 Apr 10. doi: 10.1007/s00276-017-1848-5. [Epub ahead of print]

PMID: 28396981

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  1. A child with resistant Kawasaki disease successfully treated with anakinra: a case report.

Sánchez-Manubens J, Gelman A, Franch N, Teodoro S, Palacios JR, Rudi N, Rivera J, Antón J.

BMC Pediatr. 2017 Apr 8;17(1):102. doi: 10.1186/s12887-017-0852-6.

PMID: 28390409 Free PMC Article

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Select item 28391520

 

  1. Novel nesprin-1 mutations associated with dilated cardiomyopathy cause nuclear envelope disruption and defects in myogenesis.

Zhou C, Li C, Zhou B, Sun H, Koullourou V, Holt I, Puckelwartz MJ, Warren DT, Hayward R, Lin Z, Zhang L, Morris GE, McNally EM, Shackleton S, Rao L, Shanahan CM, Zhang Q.

Hum Mol Genet. 2017 Apr 7. doi: 10.1093/hmg/ddx116. [Epub ahead of print]

PMID: 28398466

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  1. Penetrance of Hypertrophic Cardiomyopathy in Children Who Are Mutation Positive.

Vermeer AM, Clur SB, Blom NA, Wilde AA, Christiaans I.

J Pediatr. 2017 Apr 7. pii: S0022-3476(17)30382-7. doi: 10.1016/j.jpeds.2017.03.033. [Epub ahead of print]

PMID: 28396031

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  1. Moderate therapeutic hypothermia induces multimodal protective effects in oxygen-glucose deprivation/reperfusion injured cardiomyocytes.

Krech J, Tong G, Wowro S, Walker C, Rosenthal LM, Berger F, Schmitt KR.

Mitochondrion. 2017 Apr 7. pii: S1567-7249(16)30233-1. doi: 10.1016/j.mito.2017.04.001. [Epub ahead of print]

PMID: 28396253

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  1. Mutations in TMEM260 Cause a Pediatric Neurodevelopmental, Cardiac, and Renal Syndrome.

Ta-Shma A, Khan TN, Vivante A, Willer JR, Matak P, Jalas C, Pode-Shakked B, Salem Y, Anikster Y, Hildebrandt F, Katsanis N, Elpeleg O, Davis EE.

Am J Hum Genet. 2017 Apr 6;100(4):666-675. doi: 10.1016/j.ajhg.2017.02.007. Epub 2017 Mar 16.

PMID: 28318500

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  1. Pericardial Effusion Following Hematopoietic Cell Transplantation in Children is Associated with Increased Risk of Mortality.

Cox K, Punn R, Schnorr E, Pinsky BA, Kharbanda S.

Biol Blood Marrow Transplant. 2017 Apr 5. pii: S1083-8791(17)30363-4. doi: 10.1016/j.bbmt.2017.03.028. [Epub ahead of print]

PMID: 28390986

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  1. Evaluation of myocardial deformation in patients with Kawasaki disease using speckle-tracking echocardiography during mid-term follow-up.

Dedeoglu R, Barut K, Oztunc F, Atik S, Adrovic A, Sahin S, Cengiz D, Kasapcopur O.

Cardiol Young. 2017 Apr 5:1-9. doi: 10.1017/S1047951117000580. [Epub ahead of print]

PMID: 28376935

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  1. Plasma exchange for the patients with dilated cardiomyopathy in children is safe and effective in improving both cardiac function and daily activities.

Moriguchi T, Koizumi K, Matsuda K, Harii N, Goto J, Harada D, Sugawara H, Hoshiai M, Kise H, Baba A.

J Artif Organs. 2017 Apr 5. doi: 10.1007/s10047-017-0956-7. [Epub ahead of print]

PMID: 28382424

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  1. Using the Electronic Medical Record to Correlate Kawasaki Disease Phenotypes With Clinical Outcomes.

Jaggi P, Grcic M, Kovalchin J, Wilhelm CM, Yildirim-Toruner C, Texter K.

J Pediatric Infect Dis Soc. 2017 Apr 5. doi: 10.1093/jpids/pix016. [Epub ahead of print]

PMID: 28383697

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  1. Relationship between perfusion index and patent ductus arteriosus in preterm infants.

Gomez-Pomar E, Makhoul M, Westgate PM, Ibonia KT, Patwardhan A, Giannone PJ, Bada HS, Abu Jawdeh EG.

Pediatr Res. 2017 Apr 5. doi: 10.1038/pr.2017.10. [Epub ahead of print]

PMID: 28099422

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Select item 28376547

 

  1. Changing Management of the Patent Ductus Arteriosus: Effect on Neonatal Outcomes and Resource Utilization.

Chock VY, Goel VV, Palma JP, Luh TM, Wang NA, Gaskari S, Punn R, Silverman NH, Benitz WE.

Am J Perinatol. 2017 Apr 4. doi: 10.1055/s-0037-1601442. [Epub ahead of print]

PMID: 28376547

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Select item 28375639

 

  1. Prognostic Significance of Reduced Blood Pressure Response to Exercise in Pediatric Pulmonary Arterial Hypertension.

Zhang HD, Lv ZC, Wang LT, Rothman A, Lian TY, He YY, Wu Y, Lawrie A, Beghetti M, Jing ZC.

Am J Respir Crit Care Med. 2017 Apr 4. doi: 10.1164/rccm.201701-0131LE. [Epub ahead of print] No abstract available.

PMID: 28375639

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Select item 28375677

 

  1. Segregating bodily isomerism or heterotaxy: potential echocardiographic correlations of morphological findings.

Tremblay C, Loomba RS, Frommelt PC, Perrin D, Spicer DE, Backer C, Anderson RH.

Cardiol Young. 2017 Apr 3:1-11. doi: 10.1017/S104795111700049X. [Epub ahead of print]

PMID: 28367761

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Select item 28421174

 

  1. Utility of Cardiovascular Magnetic Resonance-Derived Wave Intensity Analysis As a Marker of Ventricular Function in Children with Heart Failure and Normal Ejection Fraction.

Ntsinjana HN, Chung R, Ciliberti P, Muthurangu V, Schievano S, Marek J, Parker KH, Taylor AM, Biglino G.

Front Pediatr. 2017 Apr 3;5:65. doi: 10.3389/fped.2017.00065. eCollection 2017.

PMID: 28421174 Free PMC Article

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Select item 28408091

 

  1. Retrocardiac lung lobe in a neonate with Scimitar syndrome.

Rivero Jiménez N, Labrandero de Lera C, Bret-Zurita M.

Pediatr Pulmonol. 2017 Apr 3. doi: 10.1002/ppul.23701. [Epub ahead of print]

PMID: 28371509

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Select item 28288537

 

  1. Exosomes from pediatric dilated cardiomyopathy patients modulate a pathological response in cardiomyocytes.

Jiang X, Sucharov J, Stauffer BL, Miyamoto SD, Sucharov CC.

Am J Physiol Heart Circ Physiol. 2017 Apr 1;312(4):H818-H826. doi: 10.1152/ajpheart.00673.2016. Epub 2017 Jan 27.

PMID: 28130338

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Select item 28359493

 

  1. Practices and Attitudes of Canadian Cardiologists Caring for Patients With Trisomy 18.

Young AA, Simpson C, Warren AE.

Can J Cardiol. 2017 Apr;33(4):548-551. doi: 10.1016/j.cjca.2016.11.007. Epub 2016 Nov 17.

PMID: 28259368

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Select item 27873570

 

  1. Right aortic arch with isolation of the left subclavian artery: a rare association with airway obstruction.

Yubbu P, Latiff HA, Adam Abbaker AM.

Cardiol Young. 2017 Apr;27(3):613-616. doi: 10.1017/S1047951116001840. Epub 2016 Nov 7.

PMID: 27817752

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Select item 27680744

 

  1. Tricuspid atresia associated with aortopulmonary window: diagnostic and therapeutic dilemmas.

Milovanovic V, Stefanovic I, Ilic S.

Cardiol Young. 2017 Apr;27(3):580-583. doi: 10.1017/S1047951116001499. Epub 2016 Sep 29.

PMID: 27680574

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Select item 27322729

 

  1. Understanding the spectrum of sinus venosus interatrial communications.

Tretter JT, Chikkabyrappa S, Spicer DE, Backer CL, Mosca RS, Anderson RH, Bhatla P.

Cardiol Young. 2017 Apr;27(3):418-426. doi: 10.1017/S1047951116000664. Epub 2016 May 10.

PMID: 27161562

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Select item 27416545

 

  1. Regional right ventricular remodeling and function in children with idiopathic pulmonary arterial hypertension vs those with pulmonary valve stenosis: Insights into mechanics of right ventricular dysfunction.

Driessen MM, Meijboom FJ, Hui W, Dragulescu A, Mertens L, Friedberg MK.

Echocardiography. 2017 Apr 1. doi: 10.1111/echo.13529. [Epub ahead of print]

PMID: 28370259

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Select item 28191731

 

  1. Cardio-oncology Related to Heart Failure: Pediatric Considerations for Cardiac Dysfunction.

Rose-Felker K, Border WL, Hong BJ, Chow EJ.

Heart Fail Clin. 2017 Apr;13(2):311-325. doi: 10.1016/j.hfc.2016.12.007. Review.

PMID: 28279417

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Select item 28139301

 

  1. Comparison of stroke volumes assessed by three-dimensional echocardiography and transpulmonary thermodilution in a pediatric animal model.

Linden K, Ladage D, Dewald O, Gatzweiler E, Pieper A, Seehase M, Duerr GD, Breuer J, Herberg U.

J Clin Monit Comput. 2017 Apr;31(2):353-360. doi: 10.1007/s10877-016-9843-7. Epub 2016 Feb 17.

PMID: 26886899

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Select item 27865735

 

  1. Quantifying right atrial filling and emptying: A 4D-flow MRI study.

Callaghan FM, Arnott C, Figtree GA, Kutty S, Celermajer DS, Grieve SM.

J Magn Reson Imaging. 2017 Apr;45(4):1046-1054. doi: 10.1002/jmri.25457. Epub 2016 Sep 14.

PMID: 27626621

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Select item 27799408

 

  1. Ischemia-induced Drp1 and Fis1-mediated mitochondrial fission and right ventricular dysfunction in pulmonary hypertension.

Tian L, Neuber-Hess M, Mewburn J, Dasgupta A, Dunham-Snary K, Wu D, Chen KH, Hong Z, Sharp WW, Kutty S, Archer SL.

J Mol Med (Berl). 2017 Apr;95(4):381-393. doi: 10.1007/s00109-017-1522-8. Epub 2017 Mar 6.

PMID: 28265681

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Select item 28153478

 

  1. Perfusion Index and Pulse Oximetry Screening for Congenital Heart Defects.

Schena F, Picciolli I, Agosti M, Zuppa AA, Zuccotti G, Parola L, Pomero G, Stival G, Markart M, Graziani S, Gagliardi L, Bellan C, La Placa S, Limoli G, Calzetti G, Guala A, Bonello E, Mosca F; Neonatal Cardiology Study Group of the Italian Society of Neonatology..

J Pediatr. 2017 Apr;183:74-79.e1. doi: 10.1016/j.jpeds.2016.12.076. Epub 2017 Jan 30.

PMID: 28153478

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Select item 28109537

 

  1. Assessment of central venous physiology of Fontan circulation using peripheral venous pressure.

Masutani S, Kurishima C, Yana A, Kuwata S, Iwamoto Y, Saiki H, Ishido H, Senzaki H.

J Thorac Cardiovasc Surg. 2017 Apr;153(4):912-920. doi: 10.1016/j.jtcvs.2016.11.061. Epub 2016 Dec 19.

PMID: 28108065

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Select item 27938905

 

  1. An adolescent with chest pain and cardiac hemangioma.

Shaner ML, Pauliks LB, Leach D, Myers JL, Chang D.

JAAPA. 2017 Apr;30(4):25-28. doi: 10.1097/01.JAA.0000513348.07406.4e.

PMID: 28350727

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Select item 28241245

 

  1. Pediatric Development of Bosentan Facilitated by Modeling and Simulation.

Zisowsky J, Géhin M, Kusic-Pajic A, Krause A, Beghetti M, Dingemanse J.

Paediatr Drugs. 2017 Apr;19(2):121-130. doi: 10.1007/s40272-016-0206-0.

PMID: 28078552

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Select item 28367598

 

  1. Forgotten Right Ventricle in Pediatric Dilated Cardiomyopathy.

Agha HM, Ibrahim H, El Satar IA, El Rahman NA, El Aziz DA, Salah Z, El Saeidi S, Mostafa F, Attia W, El Rahman MA, El Mohsen GA.

Pediatr Cardiol. 2017 Apr;38(4):819-827. doi: 10.1007/s00246-017-1588-7. Epub 2017 Mar 18.

PMID: 28315942

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Select item 28289784

 

  1. High Blood Pressure States in Children, Adolescents, and Young Adults Associate Accelerated Vascular Aging, with a Higher Impact in Females’ Arterial Properties.

Curcio S, García-Espinosa V, Castro JM, Peluso G, Marotta M, Arana M, Chiesa P, Giachetto G, Bia D, Zócalo Y.

Pediatr Cardiol. 2017 Apr;38(4):840-852. doi: 10.1007/s00246-017-1591-z. Epub 2017 Mar 13.

PMID: 28289784

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Select item 28271152

 

  1. Six-Minute Walk Test in Evaluation of Children with Pulmonary Arterial Hypertension.

Zuk M, Migdal A, Jagiellowicz-Kowalska D, Mazurkiewicz K, Sadel-Wieczorek A, Brzezinska-Rajszys G.

Pediatr Cardiol. 2017 Apr;38(4):754-761. doi: 10.1007/s00246-017-1575-z. Epub 2017 Feb 27.

PMID: 28239753 Free PMC Article

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Select item 28239752

 

  1. Potential and Limitations of Cochrane Reviews in Pediatric Cardiology: A Systematic Analysis.

Poryo M, Khosrawikatoli S, Abdul-Khaliq H, Meyer S.

Pediatr Cardiol. 2017 Apr;38(4):719-733. doi: 10.1007/s00246-017-1572-2. Epub 2017 Feb 27.

PMID: 28239752

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Select item 28236162

 

  1. Regression and Complications of z-score-Based Giant Aneurysms in a Dutch Cohort of Kawasaki Disease Patients.

Dietz SM, Kuipers IM, Koole JCD, Breur JMPJ, Fejzic Z, Frerich S, Dalinghaus M, Roest AAW, Hutten BA, Kuijpers TW.

Pediatr Cardiol. 2017 Apr;38(4):833-839. doi: 10.1007/s00246-017-1590-0. Epub 2017 Feb 24.

PMID: 28236162 Free PMC Article

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Select item 28224170

 

  1. Speckle Tracking in ALCAPA Patients After Surgical Repair as Predictor of Residual Coronary Disease.

Castaldi B, Vida V, Reffo E, Padalino M, Daniels Q, Stellin G, Milanesi O.

Pediatr Cardiol. 2017 Apr;38(4):794-800. doi: 10.1007/s00246-017-1583-z. Epub 2017 Feb 18.

PMID: 28214964

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Select item 28210769

 

  1. Right Ventricle-to-Pulmonary Artery Shunt in Pulmonary Atresia with a Ventricular Septal Defect: A Word of Caution.

Choi KH, Sung SC, Kim H, Lee HD, Ban GH, Kim G, Ko H.

Pediatr Cardiol. 2017 Apr;38(4):707-711. doi: 10.1007/s00246-017-1570-4. Epub 2017 Feb 3.

PMID: 28154913

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Select item 28154912

 

  1. Twelve Years of Kawasaki Disease in Portugal: Epidemiology in Hospitalized Children.

Pinto FF, Laranjo S, Mota Carmo M, Brito MJ, Cruz Ferreira R.

Pediatr Infect Dis J. 2017 Apr;36(4):364-368. doi: 10.1097/INF.0000000000001444.

PMID: 27918384

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Select item 28184962

 

  1. NOTCH1-Dependent Nitric Oxide Signaling Deficiency in Hypoplastic Left Heart Syndrome Revealed Through Patient-Specific Phenotypes Detected in Bioengineered Cardiogenesis.

Hrstka SC, Li X, Nelson TJ; Wanek Program Genetics Pipeline Group..

Stem Cells. 2017 Apr;35(4):1106-1119. doi: 10.1002/stem.2582. Epub 2017 Mar 5.

PMID: 28142228

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Select item 28429690

 

  1. Levoatrial Cardinal Vein in a Series of Five Prenatal Cases with Hypoplastic Left Heart Syndrome and Intact Atrial Septum.

Hellmund A, Berg C, Herberg U, Geipel A, Kempe A, Gembruch U.

Ultraschall Med. 2017 Apr;38(2):206-211. doi: 10.1055/s-0034-1399695. Epub 2015 Jun 17.

PMID: 26085459

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Select item 28095749

 

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Fetal Cardiology Featured Articles of April 2017

 

Fetal Cardiology Reviews of April 2017 Manuscripts

 

Fetal cardiac cine imaging using highly accelerated dynamic MRI with retrospective motion correction and outlier rejection.

van Amerom JF, Lloyd DF, Price AN, Kuklisova Murgasova M, Aljabar P, Malik SJ, Lohezic M, Rutherford MA, Pushparajah K, Razavi R, Hajnal JV.

Magn Reson Med. 2017 Apr 3. doi: 10.1002/mrm.26686. [Epub ahead of print]

PMID: 28370252

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Select item 28393221

 

Take Home Points:

  • A multi-step strategy for reconstruction of 2D fetal cardiac MRI cine images including acquisition of highly accelerated MRI data, cardiac synchronization, motion correction, outlier rejection and cardiac cine reconstruction is demonstrated
  • The researchers could show that this method is able to improve fetal cardiac MRI cine imaging.

 

Voges IngaCommentary from Dr. Inga Voges (London UK), section editor of Congenital Heart Surgery Journal Watch: Fetal cardiac magnetic resonance imaging (MRI) has the potential to complement fetal echocardiography and is increasingly being used. However, the MRI exam is challenging because of various fetal movements. This nice study proposes a multi-step strategy involving acquisition of highly accelerated MRI data and image reconstruction, cardiac synchronization, motion correction, outlier rejection and cardiac cine reconstruction. 30 pregnant women were scanned and 36 cine cardiac MRI data sets were successfully reconstructed using the proposed method (see figures).  Image quality and each step of the described strategy were evaluated. The authors show that the combination of the post-processing steps improved the image quality (see figures) and that this approach seems to be promising for further fetal cardiac MRI studies.

Fetal 1 april

Fetasl 2 april

Right Atrial Dysfunction in the Fetus with Severely Regurgitant Tricuspid Valve Disease: A Potential Source of Cardiovascular Compromise.

Howley LW, Khoo NS, Moon-Grady AJ, Patel SS, Alrais F, Tworetzky W, Colen T, Brooks P, Trines J, Ojala T, Hornberger LK.

J Am Soc Echocardiogr. 2017 Apr 11. pii: S0894-7317(17)30002-0. doi: 10.1016/j.echo.2017.01.002. [Epub ahead of print]

PMID: 28410946

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Select item 28399889

 

Take Home Points:

 

  • Fetal tricuspid valve disease has more severe right atrial dilation and dysfunction than compared to right heart obstruction only and control groups.
  • RA dysfunction may have a critical influence in the worsened outcomes and higher incidence of hydrops in these patients.

Hershenson, JaredCommentary from Dr. Jared Hershenson (Greater Washington DC), section editor of Fetal Cardiology Journal Watch:  Fetal tricuspid valve disease with significant tricuspid regurgitation (rTVD) is associated with hydrops, sudden fetal demise, or hemodynamic instability after birth, but the pathophysiology has not been completely defined. As most structural heart defects are well tolerated in fetal life, atrial dysfunction was hypothesized to have a significant effect due to potential decreases in left ventricular filling and output and increased right atrial and central venous pressure. This study compared RA function of those with rTVD vs. those with right heart obstruction (RHO) and control patients over a 10-year period. The groups used velocity vector imaging to measure some novel measures of atrial function, including RA emptying fraction (measure of global atrial function) and atrial volume expansion index (measure of atrial reservoir function), as well as other standard fetal echo measurements. Groups were compared using standard statistical measures and univariable logistic regression was performed to identify the factors associated with death. rTVD patients had increased RA volume, reduced emptying, decreased emptying fraction and decreased fractional area change than those with RHO alone. LV Tei index and umbilical artery pulsatility index were greatest in rTVD as compared to RHO and control groups. ~ 27% of fetuses with rTVD died during fetal life or within the neonatal period, with those with functional pulmonary atresia having the worse outcomes. This was in contrast with a 6.5% mortality in the RHO group. 11.8% of rTVD developed hydrops vs. 5% in RHO. Three RA function parameters were associated with demise, including RA indexed emptying rate, RA maximum volume, and RA minimum area. Based on the data, the authors suspect that decreased atrial contraction and reservoir functioning in the rTVD group may lead to decreased flow across the PFO resulting in less LV filling and a drop in cardiac output which could explain the higher incidence of hydrops and fetal demise.

Fetal 3 april

Fetal 4 april

fetal 5 april

Fetal Cardiology April 2017

 

  1. Disruption of spatiotemporal hypoxic signaling causes congenital heart disease in mice.

Yuan X, Qi H, Li X, Wu F, Fang J, Bober E, Dobreva G, Zhou Y, Braun T.

J Clin Invest. 2017 Apr 24. pii: 88725. doi: 10.1172/JCI88725. [Epub ahead of print]

PMID: 28436940 Free Article

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Select item 28436549

 

  1. Human fetal heart specific coexpression network involves congenital heart disease/defect candidate genes.

Wang B, You G, Fu Q.

Sci Rep. 2017 Apr 24;7:46760. doi: 10.1038/srep46760.

PMID: 28436429 Free PMC Article

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Select item 28432402

 

  1. Variable phenotype in a novel mutation in PHOX2B.

Lombardo RC, Kramer E, Cnota JF, Sawnani H, Hopkin RJ.

Am J Med Genet A. 2017 Apr 19. doi: 10.1002/ajmg.a.38218. [Epub ahead of print]

PMID: 28422456

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Select item 28422173

 

  1. Demographic and perinatal outcome data of fetuses with SUA/PRUV.

Sun L, Wang Y.

J Matern Fetal Neonatal Med. 2017 Apr 3:1-6. doi: 10.1080/14767058.2017.1309384. [Epub ahead of print]

PMID: 28320222

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Select item 28372585

 

  1. Multiple gene variations contributed to congenital heart disease via GATA family transcriptional regulation.

Qian Y, Xiao D, Guo X, Chen H, Hao L, Ma X, Huang G, Ma D, Wang H.

J Transl Med. 2017 Apr 3;15(1):69. doi: 10.1186/s12967-017-1173-0.

PMID: 28372585 Free PMC Article

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Select item 28370096

 

  1. Recipient Twin Circular Shunt Physiology Before Fetal Laser Surgery: Survival and Risks for Postnatal Right Ventricular Outflow Tract Obstruction.

Pruetz JD, Votava-Smith JK, Chmait HR, Korst LM, Llanes A, Chmait RH.

J Ultrasound Med. 2017 Apr 3. doi: 10.7863/ultra.16.08038. [Epub ahead of print]

PMID: 28370096

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Select item 28370380

 

  1. Fetal cardiac cine imaging using highly accelerated dynamic MRI with retrospective motion correction and outlier rejection.

van Amerom JF, Lloyd DF, Price AN, Kuklisova Murgasova M, Aljabar P, Malik SJ, Lohezic M, Rutherford MA, Pushparajah K, Razavi R, Hajnal JV.

Magn Reson Med. 2017 Apr 3. doi: 10.1002/mrm.26686. [Epub ahead of print]

PMID: 28370252

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Select item 28393221

 

  1. Prenatal diagnosis of congenital heart diseases by fetal echocardiography in second trimester: a Chinese multicenter study.

Chu C, Yan Y, Ren Y, Li X, Gui Y.

Acta Obstet Gynecol Scand. 2017 Apr;96(4):454-463. doi: 10.1111/aogs.13085. Epub 2017 Feb 13.

PMID: 28029179

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Select item 28242012

 

  1. Genetics of Congenital Heart Disease: Past and Present.

Muntean I, Togănel R, Benedek T.

Biochem Genet. 2017 Apr;55(2):105-123. doi: 10.1007/s10528-016-9780-7. Epub 2016 Nov 2. Review.

PMID: 27807680

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Select item 27938448

 

  1. An update on the molecular diagnosis of congenital heart disease: focus on loss-of-function mutations.

Li YJ, Yang YQ.

Expert Rev Mol Diagn. 2017 Apr;17(4):393-401. doi: 10.1080/14737159.2017.1300062. Epub 2017 Mar 8.

PMID: 28274167

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Select item 26572766

 

  1. Congenital heart defect causing mutation in Nkx2.5 displays in vivo functional deficit.

Zakariyah AF, Rajgara RF, Veinot JP, Skerjanc IS, Burgon PG.

J Mol Cell Cardiol. 2017 Apr;105:89-98. doi: 10.1016/j.yjmcc.2017.03.003. Epub 2017 Mar 14.

PMID: 28302382

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Select item 28109537

 

  1. CITED2 Mutations in Conserved Regions Contribute to Conotruncal Heart Defects in Chinese Children.

Li B, Pu T, Liu Y, Xu Y, Xu R.

DNA Cell Biol. 2017 Apr 24. doi: 10.1089/dna.2017.3701. [Epub ahead of print]

PMID: 28436679

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Select item 28436859

 

  1. The 24-Segment Sphericity Index: A New Technique to Evaluate Fetal Cardiac Diastolic Shape.

DeVore GR, Klas B, Satou G, Sklansky M.

Ultrasound Obstet Gynecol. 2017 Apr 24. doi: 10.1002/uog.17505. [Epub ahead of print]

PMID: 28437575

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Select item 28461021

 

  1. EED orchestration of heart maturation through interaction with HDACs is H3K27me3-independent.

Ai S, Peng Y, Li C, Gu F, Yu X, Yue Y, Ma Q, Chen J, Lin Z, Zhou P, Xie H, Prendiville TW, Zheng W, Liu Y, Orkin SH, Wang DZ, Yu J, Pu WT, He A.

Elife. 2017 Apr 10;6. pii: e24570. doi: 10.7554/eLife.24570.

PMID: 28394251 Free PMC Article

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Select item 28443268

 

  1. EXIT procedure for fetal mediastinal teratoma with large pericardial effusion: a case report with review of literature.

Agarwal A, Rosenkranz E, Yasin S, Swaminathan S.

J Matern Fetal Neonatal Med. 2017 Apr 2:1-5. doi: 10.1080/14767058.2017.1306851. [Epub ahead of print]

PMID: 28288537

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Select item 28441822

 

  1. Microarray Analysis of Differential Gene Expression Profile Between Human Fetal and Adult Heart.

Geng Z, Wang J, Pan L, Li M, Zhang J, Cai X, Chu M.

Pediatr Cardiol. 2017 Apr;38(4):700-706. doi: 10.1007/s00246-017-1569-x. Epub 2017 Mar 22.

PMID: 28331934

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Select item 28315942

 

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CHD Intervention Featured Articles of April 2017

Cardiac Catheterization Reviews of April 2017 Manuscripts

 

Tricuspid Regurgitation Does Not Impact Right Ventricular Remodeling After Percutaneous Pulmonary Valve Implantation.

Tanase D, Ewert P, Georgiev S, Meierhofer C, Pabst von Ohain J, McElhinney DB, Hager A, Kühn A, Eicken A.

JACC Cardiovasc Interv. 2017 Apr 10;10(7):701-708. doi: 10.1016/j.jcin.2017.01.036.

 

Take Home Points:

 

  • Percutaneous pulmonary valve implantation (PPVI) improves right ventricular (RV) size and exercise performance regardless of the severity of tricuspid regurgitation (TR).
  • Severity of TR improves following PPVI, and therefore the presence of significant TR should not preclude attempts at PPVI.

 

Whiteside, WendyComment from Dr. Wendy Whiteside (Cincinnati), section editor of Congenital Heart Disease Interventions Journal Watch:  Percutaneous pulmonary valve implantation (PPVI) has become the therapy of choice for treatment of RV outflow tract (RVOT) conduit dysfunction.  In patients presenting for PPVI, TR is a frequent associated finding, occurring in as many as 1/3 of patients.  There are multiple etiologies and mechanisms for TR in these patients, including annular dilation in the setting of a dilated right ventricle, valvar dysplasia, or surgical or pacemaker lead disruption, however the presence of significant TR may impact the decision to perform pulmonary valve replacement via a percutaneous or surgical approach and may affect the ability for the RV to remodel following pulmonary valve replacement.  If significant TR is present and the tricuspid valve is felt to require surgical intervention at the time of valve replacement, these patients may be referred exclusively for surgical intervention without consideration for a less invasive approach.  However, if the TR can be improved just by improving the RVPA conduit dysfunction and subsequent RV remodeling, these patients may be able to have a less invasive percutaneous procedure rather than a surgical one.

 

To investigate the impact of moderate or severe TR on RV remodeling after PPVI, Tanase et al conducted a matched cohort study comparing patients who underwent percutaneous pulmonary valve implantation (PPVI) either with significant TR (moderate or severe) or without significant TR, assessing the outcomes of TR severity by echocardiogram, RV size by cardiac MRI, and exercise performance by objective measures from cardiopulmonary exercise testing.

 

Among the 173 patients at their center who underwent PPVI over an 8-year period, patients with moderate to severe TR by echocardiographic criteria were identified, and represented 13% of their PPVI population.  Ultimately, the study included 18 patients with RVOT dysfunction and significant TR and 18 matched control subjects with RVOT dysfunction but no TR.  Patients were matched to have the same pulmonary valve pathology (primary stenosis, insufficiency, mixed), similar indexed RV end-diastolic volume, and similar NYHA functional classification).  Data were obtained at baseline, 6 months post-PPVI, and at latest follow-up (median of 6.5 years post-PPVI, range 8 months to 9.3 years).  All patients with significant TR were identified by serial echocardiography (looking back at least 8 years prior to PPVI) and found to have gradually increasing TR over time with no patients having a sudden increase in TR after surgery to suggest a primary anatomic valve issue.

 

Median patient age was 22±8 years, majority had NYHA functional class II symptoms, median peak oxygen uptake (VO2 peak) was 28.5 mlO2/kg/min, and median RVEDVi was 100 ml/m2 (range 61-185 ml/m2).  After PPVI, the degree of TR improved in 15 of 18 patients (83%) and was unchanged in the remaining 3 patients at 6-month follow-up.  At latest follow-up, no patient had significant TR (15 trivial and 3 mild).  There was a significant decrease in RVEDVi from pre- to post-PPVI with no difference between TR groups.  Similarly, while VO2 increased following PPVI, there was no difference in this increase between TR groups.  Tanase et al therefore conclude that in most patients with RVOT conduit dysfunction and significant TR, PPVI leads to a reduction in TR.  PPVI also decreases RV volume and improves exercise tolerance without a difference between baseline TR severity groups

 

These findings help to provide insight into a clinically very meaningful question—whether the presence of significant TR in a patient with a dysfunctional RVOT conduit should play into the decision for type and timing of intervention.  While they provide convincing evidence to suggest that secondary TR will improve following PPVI, there some important limitations to consider. The mechanism of TR is an important variable in the ability to improve TR.  Tanase et al attempted to exclude patients with a primary tricuspid valve abnormality (by showing a slow progression of TR over time in the included study patients).  Generalizing these results, therefore, to patients with primary valve abnormalities (dysplastic or surgically manipulated valves) should be done with caution.  Secondly, the median RVEDVi of included patients is small (100 ml/m2) and may not be representative of most PPVI populations.  This may lessen the effect of the additional volume load of TR on RV remodeling following PPVI.  Despite these limitations and small patient size, this study is of interest and should be considered in decision making for patients with RVOT conduit dysfunction and concomitant TR.

 

 

 

 

 

 

Impacts of early cardiac catheterization for children with congenital heart disease supported by extracorporeal membrane oxygenation.

Kato A, Lo Rito M, Lee KJ, Haller C, Guerguerian AM, Sivarajan VB, Honjo O.

Catheter Cardiovasc Interv. 2017 Apr;89(5):898-905. doi: 10.1002/ccd.26632. Epub 2016 Jul 14.

PMID: 27416545

 

Take Home Points:

  • Early catheterization is associated with shorter duration of ECMO support and higher survival probability at 30 days after ECMO cannulation.
  • ECMO-related end-organ dysfunction is a significantly poor prognostic factor for successful decannulation.

 

Averin , KonstantinCommentary from Dr. Konstantin Averin (Edmonton), section editor of Congenital Heart Disease Interventions Journal Watch: Extracorporeal membrane oxygenation (ECMO) has been widely used in the pediatric population for cases of peri-operative hemodynamic instability, failure to wean from cardiopulmonary bypass, and cardiopulmonary resuscitation. Cardiac catheterization in patients on ECMO support poses significant challenges but can potentially be useful in improving clinical outcomes. This single center retrospective cohort study focused on patients with congenital heart disease (CHD) and hypothesized that timing of cardiac catheterization may be a predictor of clinical outcomes. The specific aims of the study were: (1) to analyze the institution’s experience with cardiac catheterization on pediatric patients with CHD supported by cardiac ECMO and (2) to determine factors associated with successful weaning from ECMO and short-term outcomes.

 

Three hundred and forty-two patients required ECMO support between 2000 and 2014. Of these, 47 underwent 49 cardiac catheterizations that met inclusion criteria with a median patient age of 65 days and median weight 4.2kg. ECMO was successfully weaned in 33 patients (70%) after a median support time of 4 days with 51% of patients surviving to hospital discharge. Cardiac catheterizations were performed a median of 1 day after ECMO initiation and during 27 of them an intervention was performed (balloon/stent angioplasty and balloon atrial septostomy). There was no procedure related mortality but 9 (18%) procedure-related serious complications with 8 occurring during interventional catheterization (pulmonary artery rupture, stent dislodgement, arrhythmia, pulmonary hemorrhage, and sheath tip migration). In a multivariate analysis, absence of renal or respiratory complications were prognostic of successful ECMO weaning. Kaplan-Meier analysis demonstrated that patient who underwent earlier catheterization (<48 hours after cannulation) had a higher survival probability at 30 days after ECMO cannulation compared with late catheterization.

 

The authors conclude that early cardiac catheterization in pediatric patients with CHD who require ECMO support may be associated with better short-term survival. Early cardiac catheterization should be considered in this cohort, especially if there are unresolved anatomic or physiologic questions. The absence of ECMO-related complications is a predictor for successful weaning off ECMO.

cath 1 april

 

 

Impact of imaging approach on radiation dose and associated cancer risk in children undergoing cardiac catheterization.

Hill KD, Wang C, Einstein AJ, Januzis N, Nguyen G, Li JS, Fleming GA, Yoshizumi TK.

Catheter Cardiovasc Interv. 2017 Apr;89(5):888-897. doi: 10.1002/ccd.26630. Epub 2016 Jun 17.

PMID: 27315598

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Select item 27473470

 

Take Home Points:

  • Congenital cardiac catheterizations utilize much higher doses of ionizing radiation than other imaging modalities, such as computed tomography, increasing the lifetime cancer risk for patients
  • Traditional measures of radiation exposure (air kerma or dose-area product) do not accurately measure the true tissue exposure to radiation
  • Altering the methods of image acquisition (collimation, magnification, minimizing source to image distance, removal of antiscatter grids, lower acquisition frame rates) lead to marked variation in effective radiation doses and are easily modifiable by the interventional cardiologist

 

Seckler, MikeCommentary from Dr. Michael Seckeler (Tucson), section editor of Congenital Heart Disease Interventions Journal Watch: The authors have undertaken a very important study for any practitioner who utilizes ionizing radiation for imaging pediatric patients. Using two patient phantoms representing a newborn and a 5-year-old child, they systematically compared the relative effects of different alterable parameters of fluoroscopic imaging used in standard congenital catheterization laboratories on effective radiation doses in the phantoms. In addition, they were able to estimate lifetime cancer risks based on the effective radiation doses. This is the first study to look at this in pediatric patients.

 

The modifiable factors included collimation, magnification, minimizing source to image distance, removal of antiscatter grids and lower acquisition frame rates. These were tested in various combinations and effective radiation doses measured. Optimizing the imaging for minimal radiation exposure (lower magnification, maximal collimation, lowest source to image distance, removal of antiscatter grids and lower acquisition frame rates) all led to significant reductions in the effective radiation doses and lower predicted lifetime cancer risks (Figure 1).

 

The authors do report several limitations to their conclusions. First, optimizing the radiation exposure does not always lead to the best image quality, which has the potential to compromise patient safety during a procedure. However, by highlighting how each of several different factors can improve radiation exposure, this data will allow interventional cardiologists to make as many modifications as possible to find the optimal balance between safety and image quality. Second, the radiation exposures were derived from phantoms and mathematical simulations; however, the intention was not to provide exact doses, but the relative changes in exposure with changes in imaging modality.

This is a very important paper for our field and provides tools to help interventional cardiologists minimize the radiation exposure for our patients.

cath 2 april

CHD Interventions April 2017

 

  1. Real-time three dimensional CT and MRI to guide interventions for congenital heart disease and acquired pulmonary vein stenosis.

Suntharos P, Setser RM, Bradley-Skelton S, Prieto LR.

Int J Cardiovasc Imaging. 2017 Apr 28. doi: 10.1007/s10554-017-1151-x. [Epub ahead of print]

PMID: 28455631

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Select item 28457522

 

  1. Clinical Trial Principles and Endpoint Definitions for Paravalvular Leaks in Surgical Prosthesis: An Expert Statement.

Ruiz CE, Hahn RT, Berrebi A, Borer JS, Cutlip DE, Fontana G, Gerosa G, Ibrahim R, Jelnin V, Jilaihawi H, Jolicoeur EM, Kliger C, Kronzon I, Leipsic J, Maisano F, Millan X, Nataf P, O’Gara PT, Pibarot P, Ramee SR, Rihal CS, Rodes-Cabau J, Sorajja P, Suri R, Swain JA, Turi ZG, Tuzcu EM, Weissman NJ, Zamorano JL, Serruys PW, Leon MB; Paravalvular Leak Academic Research Consortium..

J Am Coll Cardiol. 2017 Apr 25;69(16):2067-2087. doi: 10.1016/j.jacc.2017.02.038. Review.

PMID: 28427582

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Select item 28437783

 

  1. In vivo intracardiac vector velocity imaging using phased array transducers for pediatric cardiology.

Fadnes S, Wigen M, Nyrnes SA, Lovstakken L.

IEEE Trans Ultrason Ferroelectr Freq Control. 2017 Apr 24. doi: 10.1109/TUFFC.2017.2689799. [Epub ahead of print]

PMID: 28436859

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Select item 28436940

 

  1. Prediction of adverse events after catheter-based procedures in adolescents and adults with congenital heart disease in the IMPACT registry.

Stefanescu Schmidt AC, Armstrong A, Kennedy KF, Nykanen D, Aboulhosn J, Bhatt AB.

Eur Heart J. 2017 Apr 18. doi: 10.1093/eurheartj/ehx200. [Epub ahead of print]

PMID: 28430913

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Select item 28430909

 

  1. Clinical Trial Principles and Endpoint Definitions for Paravalvular Leaks in Surgical Prosthesis: An Expert Statement.

Ruiz CE, Hahn RT, Berrebi A, Borer JS, Cutlip DE, Fontana G, Gerosa G, Ibrahim R, Jelnin V, Jilaihawi H, Jolicoeur EM, Kliger C, Kronzon I, Leipsic J, Maisano F, Millan X, Nataf P, O’Gara PT, Pibarot P, Ramee SR, Rihal CS, Rodes-Cabau J, Sorajja P, Suri R, Swain JA, Turi ZG, Tuzcu EM, Weissman NJ, Zamorano JL, Serruys PW, Leon MB; of the Paravalvular Leak Academic Research Consortium..

Eur Heart J. 2017 Apr 18. doi: 10.1093/eurheartj/ehx211. [Epub ahead of print]

PMID: 28430909

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Select item 28434921

 

  1. Cell Therapy Trials in Congenital Heart Disease.

Oh H.

Circ Res. 2017 Apr 14;120(8):1353-1366. doi: 10.1161/CIRCRESAHA.117.309697. Review.

PMID: 28408455

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  1. A systematic review of 3-D printing in cardiovascular and cerebrovascular diseases.

Sun Z, Lee SY.

Anatol J Cardiol. 2017 Apr 10. doi: 10.14744/AnatolJCardiol.2017.7464. [Epub ahead of print]

PMID: 28430115 Free Article

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  1. Avoidable costs of stenting for aortic coarctation in the United Kingdom: an economic model.

Salcher M, Mcguire A, Muthurangu V, Kelm M, Kuehne T, Naci H; CARDIOPROOF..

BMC Health Serv Res. 2017 Apr 10;17(1):258. doi: 10.1186/s12913-017-2215-2.

PMID: 28395657 Free PMC Article

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  1. Coincidental Significant Tricuspid Regurgitation at the Time of Right Ventricle-to-Pulmonary Artery Conduit Intervention: Should We Address it, Ignore it, or Take a More Nuanced Approach?

Hebson CL, Ephrem G, Rodriguez FH 3rd.

JACC Cardiovasc Interv. 2017 Apr 10;10(7):709-711. doi: 10.1016/j.jcin.2017.02.032. No abstract available.

PMID: 28385409

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Select item 28396933

 

  1. Coronary Sinus Defect Following Transcatheter Closure of ASD Using Amplatzer Septal Occluder: Potential Erosion by the Device.

Mohammad Nijres B, Al-Kubaisi M, Bokowski J, Abdulla RI, Awad S.

Pediatr Cardiol. 2017 Apr 10. doi: 10.1007/s00246-017-1613-x. [Epub ahead of print]

PMID: 28396933

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  1. Impacts of early cardiac catheterization for children with congenital heart disease supported by extracorporeal membrane oxygenation.

Kato A, Lo Rito M, Lee KJ, Haller C, Guerguerian AM, Sivarajan VB, Honjo O.

Catheter Cardiovasc Interv. 2017 Apr;89(5):898-905. doi: 10.1002/ccd.26632. Epub 2016 Jul 14.

PMID: 27416545

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Select item 26711371

 

  1. Patent foramen ovale with right atrial septal pouch.

Kijima Y, Bokhoor P, Tobis JM.

Catheter Cardiovasc Interv. 2017 Apr;89(5):E169-E171. doi: 10.1002/ccd.26357. Epub 2015 Dec 29.

PMID: 26711371

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Select item 28356308

 

  1. In-hospital cost comparison between percutaneous pulmonary valve implantation and surgery.

Andresen B, Mishra V, Lewandowska M, Andersen JG, Andersen MH, Lindberg H, Døhlen G, Fosse E.

Eur J Cardiothorac Surg. 2017 Apr 1;51(4):747-753. doi: 10.1093/ejcts/ezw378.

PMID: 28007875 Free PMC Article

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Select item 28071940

 

  1. Evolution of hybrid interventions for congenital heart disease.

Agrawal H, Alkashkari W, Kenny D.

Expert Rev Cardiovasc Ther. 2017 Apr;15(4):257-266. doi: 10.1080/14779072.2017.1307733. Epub 2017 Mar 23. Review.

PMID: 28293976

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Select item 28274167

 

  1. Minimally invasive endoscopic surgery versus catheter-based device occlusion for atrial septal defects in adults: reconsideration of the standard of care.

Schneeberger Y, Schaefer A, Conradi L, Brickwedel J, Reichenspurner H, Kozlik-Feldmann R, Detter C.

Interact Cardiovasc Thorac Surg. 2017 Apr 1;24(4):603-608. doi: 10.1093/icvts/ivw366.

PMID: 28040751

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Select item 28040749

 

  1. The Medtronic Micro Vascular Plug™ for Vascular Embolization in Children With Congenital Heart Diseases.

Sathanandam S, Justino H, Waller BR 3rd, Gowda ST, Radtke W, Qureshi AM.

J Interv Cardiol. 2017 Apr;30(2):177-184. doi: 10.1111/joic.12369. Epub 2017 Feb 16.

PMID: 28211168

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Select item 28470155

 

  1. Pulmonary artery dissection following balloon valvuloplasty in a dog with pulmonic stenosis.

Grint KA, Kellihan HB.

J Vet Cardiol. 2017 Apr;19(2):182-189. doi: 10.1016/j.jvc.2016.09.005. Epub 2016 Nov 30.

PMID: 27913078

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Select item 28382264

 

  1. Bronchial artery embolization for the treatment of haemoptysis in pulmonary hypertension.

Rasciti E, Sverzellati N, Silva M, Casadei A, Attinà D, Palazzini M, Galiè N, Zompatori M.

Radiol Med. 2017 Apr;122(4):257-264. doi: 10.1007/s11547-016-0714-6. Epub 2016 Dec 26.

PMID: 28025781

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Select item 28189544

 

  1. Three-dimensional Printed Cardiac Models: Applications in the Field of Medical Education, Cardiovascular Surgery, and Structural Heart Interventions.

Valverde I.

Rev Esp Cardiol (Engl Ed). 2017 Apr;70(4):282-291. doi: 10.1016/j.rec.2017.01.012. Epub 2017 Feb 8. English, Spanish.

PMID: 28189544

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Select item 28137395

 

  1. Seven Coils in 1 Heart: Therapeutic Option for Multiple VSD.

Sabiniewicz R, Weryński P.

JACC Cardiovasc Interv. 2017 Apr 24;10(8):837-838. doi: 10.1016/j.jcin.2017.02.016. Epub 2017 Mar 29. No abstract available.

PMID: 28365261

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  1. Are the AMPLATZER Duct Occluder II Additional Sizes devices dedicated only for smaller children?

Fiszer R, Chojnicki M, Szkutnik M, Haponiuk I, Chodór B, Białkowski J.

EuroIntervention. 2017 Apr 20;12(17):2100-2103. doi: 10.4244/EIJ-D-15-00238.

PMID: 27867138 Free Article

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  1. Targeted endomyocardial biopsy guided by real-time cardiovascular magnetic resonance.

Unterberg-Buchwald C, Ritter CO, Reupke V, Wilke RN, Stadelmann C, Steinmetz M, Schuster A, Hasenfuß G, Lotz J, Uecker M.

J Cardiovasc Magn Reson. 2017 Apr 19;19(1):45. doi: 10.1186/s12968-017-0357-3.

PMID: 28424090 Free PMC Article

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  1. Transcatheter closure of a residual aorto-left ventricular tunnel: report of a case with a 6-year follow-up.

Djukic M, Djordjevic SA, Dähnert I.

Cardiol Young. 2017 Apr 17:1-4. doi: 10.1017/S1047951117000701. [Epub ahead of print]

PMID: 28414001

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  1. Radiation dose reduction in pediatric great vessel stent computed tomography using iterative reconstruction: A phantom study.

den Harder AM, Suchá D, van Doormaal PJ, Budde RPJ, de Jong PA, Schilham AMR, Breur JMPJ, Leiner T.

PLoS One. 2017 Apr 14;12(4):e0175714. doi: 10.1371/journal.pone.0175714. eCollection 2017.

PMID: 28410386 Free PMC Article

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  1. Clinical Impact of Stent Implantation for Coarctation of the Aorta with Associated Hypoplasia of the Transverse Aortic Arch.

Lu WH, Fan CS, Chaturvedi R, Lee KJ, Manlhiot C, Benson L.

Pediatr Cardiol. 2017 Apr 10. doi: 10.1007/s00246-017-1611-z. [Epub ahead of print]

PMID: 28396934

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  1. Percutaneous Mechanical Circulatory Support Using Impella® Devices for Decompensated Cardiogenic Shock: A Pediatric Heart Center Experience.

Parekh D, Jeewa A, Tume SC, Dreyer WJ, Pignatelli R, Horne D, Justino H, Qureshi AM.

ASAIO J. 2017 Apr 6. doi: 10.1097/MAT.0000000000000581. [Epub ahead of print]

PMID: 28394814

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  1. Intractable Back Pain After Coil Embolization of Giant Veno-Venous Collaterals in a Patient With Fontan Circulation.

Okada S, Kamada M, Nakagawa N, Ishiguchi Y, Moritoh Y, Shohi M, Okamoto K, Hasegawa S, Ohga S.

Int Heart J. 2017 Apr 6;58(2):298-301. doi: 10.1536/ihj.16-194. Epub 2017 Mar 21.

PMID: 28320993 Free Article

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  1. Impact of imaging approach on radiation dose and associated cancer risk in children undergoing cardiac catheterization.

Hill KD, Wang C, Einstein AJ, Januzis N, Nguyen G, Li JS, Fleming GA, Yoshizumi TK.

Catheter Cardiovasc Interv. 2017 Apr;89(5):888-897. doi: 10.1002/ccd.26630. Epub 2016 Jun 17.

PMID: 27315598

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  1. Early Cardiac Catheterization Leads to Shortened Pediatric Extracorporeal Membrane Oxygenation Run Duration.

Burke CR, Chan T, Rubio AE, McMullan DM.

J Interv Cardiol. 2017 Apr;30(2):170-176. doi: 10.1111/joic.12368. Epub 2017 Mar 8.

PMID: 28271557

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  1. Percutaneous Coronary Intervention With Bioresorbable Scaffolds in a Young Child.

Nazif TM, Kalra S, Ali ZA, Karmpaliotis D, Turner ME, Starc TJ, Cao Y, Marboe CC, Collins MB, Leon MB, Kirtane AJ.

JAMA Cardiol. 2017 Apr 1;2(4):430-434. doi: 10.1001/jamacardio.2016.4954.

PMID: 28030655

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  1. Multicenter Off-Label Use of Nit-Occlud Coil in Retrograde Closure of Small Patent Ductus Arteriosus.

Zanjani KS, Sobhy R, El-Kaffas R, El-Sisi A.

Pediatr Cardiol. 2017 Apr;38(4):828-832. doi: 10.1007/s00246-017-1589-6. Epub 2017 Feb 21.

PMID: 28224170

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  1. Preprocedural Risk Assessment Prior to PPVI with CMR and Cardiac CT.

Malone L, Fonseca B, Fagan T, Gralla J, Wilson N, DiMaria M, Truong U, Browne LP.

Pediatr Cardiol. 2017 Apr;38(4):746-753. doi: 10.1007/s00246-017-1574-0. Epub 2017 Feb 16.

PMID: 28210769

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Pediatric Cardiology Featured Articles of March 2017

Pediatric Cardiology Featured Reviews of February-March 2017 Publications

 

Altered aortic 3D hemodynamics and geometry in pediatric Marfan syndrome patients.

van der Palen RL, Barker AJ, Bollache E, Garcia J, Rose MJ, van Ooij P, Young LT, Roest AA, Markl M, Robinson JD, Rigsby CK.

J Cardiovasc Magn Reson. 2017 Mar 17;19(1):30. doi: 10.1186/s12968-017-0345-7.

PMID: 28302143

Free PMC Article

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Select item 28318851 128.

 

Take Home Points:

 

  • Marfan syndrome patients have lower mean wall shear stress and altered blood flow patterns in the proximal ascending and proximal descending aorta, both areas at risk for aortic dissection and rupture.
  • 4D flow CMR derived hemodynamic parameters may be helpful to monitor disease progression and severity in Marfan syndrome patients.

 
Voges IngaCommentary from Dr. Inga Voges (London UK) section editor of Congenital Heart Surgery Journal Watch:
 This interesting prospective study examined aortic blood flow patterns and geometry in 25 patients with Marfan syndrome (MFS) and 21 healthy controls by using cardiovascular magnetic resonance (CMR) imaging including 4D flow CMR. CMR cine imaging and contrast-enhanced magnetic resonance angiographic data were used to assess thoracic aortic diameters. After postprocessing of 4D flow datasets, blood flow patterns and regional thoracic aortic wall shear stress (in 10 aortic segments) were additionally analyzed.

The authors found abnormal flow patterns and reduced wall shear stress in the aortic root/proximal ascending aorta and the proximal descending aorta (see figure below), both areas that were dilated in MFS patients compared to the controls.  Mean wall shear stress was inversely related with aortic diameters and aortic size change in both MFS patients and controls.

The authors conclude that the hemodynamic parameters, aortic flow patterns and wall shear stress, may serve as additional valuable markers of disease severity.

Pediatric media 1

 

Rates of autism and potential risk factors in children with congenital heart defects.

Bean Jaworski JL, Flynn T, Burnham N, Chittams JL, Sammarco T, Gerdes M, Bernbaum JC, Clancy RR, Solot CB, Zackai EH, McDonald-McGinn DM, Gaynor JW.

Congenit Heart Dis. 2017 Mar 16. doi: 10.1111/chd.12461. [Epub ahead of print]

PMID:28299880

Similar articles

Select item 28306681 135.

 

Take Home Points:

 

  • Children with CHD are at increased risk for autism spectrum disorders compared to the national average. 1 in 30.9 (3.2%) children with serious CHD were diagnosed with autism spectrum disorder compared to the national rate of 1 in 68 (1.47%).
  • Abnormalities noted on neurodevelopmental testing at age 4 were the only independent predictor of a diagnosis of autism spectrum disorder in this sample of children with CHD.
  • These findings emphasize the importance of neurodevelopmental testing in children with CHD.

 

Abarbanell picture smallCommentary from Dr. Ginnie Abarbanell (Atlanta), section editor of Pediatric Cardiology Journal Watch: Recent research has indicated the children with congenital heart disease (CHD) are at risk for neurodevelopmental delays and are more likely to need special education services.  This study from Philadelphia evaluated whether children with CHD are also at increased risk for autism spectrum disorders.  Children included in this study were part of a larger prospective study on the “Neurodevelopmental outcomes in children with apo-lipoprotein E polymorphisms after infant cardiac surgery.”  195 children with serious CHD requiring cardiac surgery within first 6 months of life were included in this study and researchers in particular focused on the results of neurodevelopmental testing performed at age 4 years and the social communication questionnaire (SCQ).  The SCQ is a parent reported questionnaire used as a screening tool for autistic spectrum disorders.  Researchers found that among their sample that children with CHD were more likely to either have a parent report a diagnosis of autism spectrum disorder or screen positive using the SCQ for autism spectrum disorder compared to a national rate of autism spectrum diagnoses.  1 in 30.9 (3.2%) children with CHD were reported to have autism spectrum disorder compared to the national rate of 1 in 68 (1.47%).  Univariate analysis suggested that medical factors such as delayed sternal closure, presence of a genetic abnormality and parental report of developmental problems and symptoms of ADHD were associated with increased odds of autism septum disorder.  The only independent predictor on multivariable analysis for autism spectrum disorder in this sample of children with CHD was abnormal neurodevelopmental testing with particular deficits in social skills, intellectual function and pervasive developmental problems.  These results would confer that children with CHD are at risk for neurodevelopmental delays and in particular at increased risk for autism spectrum disorder and underscores the importance of neurodevelopmental screening in children with CHD.

pediatric media 2

 

Right-Dominant Unbalanced Atrioventricular Septal Defect: Echocardiography in Surgical Decision Making.

Arunamata A, Balasubramanian S, Mainwaring R, Maeda K, Selamet Tierney ES.

J Am Soc Echocardiogr. 2017 Mar;30(3):216-226. doi: 10.1016/j.echo.2016.10.017. Epub 2016 Dec 6.

PMID: 27939051

Similar articles

Select item 27909838 269.

 

Take Home Points:

 

  • In this small study (n=46) of infants with right dominant AVSD over 60% underwent single ventricle palliation.
  • Survival among infants with right dominant AVSD in this study was 76% at 7 years.
  • Single ventricle palliation may need to be considered in infants with right dominant AVSD and echocardiographic findings of an atrioventricular valve index (AVV) £7 and a right ventricle/left ventricle inflow angle of £ 114°.

 

Commentary from Dr. Ginnie Abarbanell (Atlanta), section editor of Pediatric Cardiology Journal Watch: It can sometimes be a surgical dilemma as to when to purse a biventricular vs a single ventricle surgical repair in the setting of unbalanced atrioventricular septal defect (AVSD).  Researchers in this study evaluated echocardiographic markers that maybe helpful in assessing surgical planning in particular whether single ventricle palliation should be pursed in patients with right-dominant AVSD.  46 patients with right dominant AVSD were included in this study.  Survival was 76% at 7 years and 61% (n=28) of patients underwent single ventricle palliation.  Of the infants who underwent single ventricle palliation they had small left ventricular dimensions and volumes as well as a decreased atrioventricular valve index (AVVI) and right ventricle(RV)/left ventricle (LV) inflow angle. A left ventricular end-diastolic volume (LVEDV) Z score £ -2.5, AVVI £ 0.7 and a RV/LV inflow angle of £ 114° was found to have a moderate association with single ventricle palliation.  See Figure 2.  Researchers validated their results on 12 additional infants with right dominant AVSD and found that a RV/LV inflow angle £114° had a sensitivity of 100% and AVVI £ 0.70 had a sensitivity of 88% for predicting single ventricle palliation.  This study would suggest that echocardiographic measurements of LVEDV, AVVI and RV/LV inflow angle could be helpful tools to assess whether single ventricle palliation vs biventricular palliation in right dominant AVSD is needed.

Pediatric media 3 Pediatric media 4

 

 

Surgical rescue after transcatheter interventional procedures in congenital heart disease patients: an existing problem.

Varrica A, Lo Rito M, Generali T, Satriano A, D’Oria V, Conforti E, Pluchinotta F, Chessa M, Butera G, Frigiola A, Carminati M, Giamberti A.

EuroIntervention. 2017 Feb 20;12(14):1724-1729. doi: 10.4244/EIJ-D-16-00031.

PMID:27773863

Free Article

Similar articles

 

Take Home Points:

 

  • Despite improvements in catheterization equipment and techniques, there is still the potential need for surgical intervention after cath-based intervention in immediate and short-term follow-up.
  • This study stresses the importance of the availability of surgical colleagues, particularly during challenging cath-based interventions, as well as long term follow-up to look for future complications.

Commentary from Dr. Ginnie Abarbanell (Atlanta), section editor of Pediatric Cardiology Journal Watch: Varrica and colleagues present a retrospective review of their single-center experience with interventional cardiac catheterizations and assess the rate of surgical interventions following catheterization. They divided the cases into early complications – during the catheterization or prior to hospital discharge, and latecomplications – surgery after hospital discharge. Of nearly 7,000 catheterizations during the review period, 3,205 involved an intervention (Table 1). A total of 40 patients (1.2%) required surgical intervention and 55% of interventions were early.pediatric media v42

 

Atrial septal defect (ASD) closure required the highest number of surgical interventions, but was also the most commonly performed cath-based intervention. Of the early ASD surgical interventions, 79% were related to device embolization or erosion. The highest need for surgical intervention occurred in the percutaneous pulmonary valve implantation patients – early interventions were for coronary artery compression and device embolization and later interventions were for infective endocarditis involving the implanted valve. Of all late interventions, ASD device complications were the most common cause leading to 72% of surgeries.

 

Despite the limitation of being a single-center study, the large number of patients and long follow-up period provide additional insight into the risks of cath-based interventions in the current era as well as the importance of continued follow-up after interventions are performed.

Cardiac 3D Printing and its Future Directions.

Vukicevic M, Mosadegh B, Min JK, Little SH.

JACC Cardiovasc Imaging. 2017 Feb;10(2):171-184. doi: 10.1016/j.jcmg.2016.12.001. Review.

PMID: 28183437

Similar articles

 

Take Home Points:

 

  • 3-D printing is being increasingly utilized in congenital and structural cardiac interventions.
  • Methods are expanding beyond simple anatomic visualization and now include the potential to physically model and assess hemodynamics in vitro to more accurately guide appropriate interventions and development of new interventional devices.

 

Commentary from Dr. Ginnie Abarbanell (Atlanta), section editor of Pediatric Cardiology Journal Watch: The authors provide an overview of the available 3-D printing technologies and their different capabilities in regards to model resolution and material availability. They also outline the steps of generating a 3-D model from initial image acquisition of a 3-D volumetric dataset (typically CT or MR data), through image

pediatric media v6 1

segmentation (the process of highlighting the pertinent anatomical structures), 3-D reconstruction, generating a digital 3-D model and finally printing the physical model.

 

The multiple uses of 3-D printed models that have been developed so far are also presented. These include:

  1. Trainee and patient education, particularly of complex congenital heart defects
  2. Functional flow models which have been used to model aortic stenosis and coronary artery flow
  3. Surgical and catheterization procedural planning, including double outlet right ventricle repair, complex percutaneous pulmonary valve implantation and aortic coarctation stenting
  4. Development of new devices that are more appropriate for human anatomy rather than using animal models

 

They conclude with a discussion of currently available printing materials and their different mechanical properties followed by a review of critical developments in the literature for different aspects of creating 3-D printed models. Importantly, limitations of the currently available technology are presented, including accuracy of mechanical properties of the materials to simulate cardiac muscle and a lack of validation of model accuracy across a variety of generation methods and 3-D printing hardware.

 

Pediatric cardiology March 2017

 

  1. The United States Pediatric Cardiology 2015 Workforce Assessment: A Survey of Current Training and Employment Patterns: A Report of the American College of Cardiology, American Heart Association, American Academy of Pediatrics Section on Cardiology and Cardiac Surgery, and Society for Pediatric Cardiology Training Program Directors.

Ross RD, Srivastava S, Cabrera AG, Ruch-Ross HS, Radabaugh CL, Minich LL, Mahle WT, Brown DW.

J Am Coll Cardiol. 2017 Mar 14;69(10):1347-1352. doi: 10.1016/j.jacc.2016.09.921. Epub 2016 Nov 28. No abstract available.

PMID:

 

27908576

Similar articles

 

  1. Clinical Progress in Cell Therapy for Single VentricleCongenital Heart Disease.

Bittle GJ, Wehman B, Karathanasis SK, Kaushal S.

Circ Res. 2017 Mar 31;120(7):1060-1062. doi: 10.1161/CIRCRESAHA.117.310702. No abstract available.

PMID:

 

28360342

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Select item 28052915 83.

 

  1. Trends in National Institutes of Health-FundedCongenital Heart DiseaseResearch from 2005 to 2015.

Burns KM, Pemberton VL, Schramm CA, Pearson GD, Kaltman JR.

Pediatr Cardiol. 2017 Mar 27. doi: 10.1007/s00246-017-1605-x. [Epub ahead of print]

PMID:

 

28349207

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Select item 28350721 96.

 

  1. A Rare Case of Cor Triatriatum Leading to Respiratory Failure and Shock in an 8-Month-Old.

Burger C.

Pediatr Emerg Care. 2017 Mar 27. doi: 10.1097/PEC.0000000000001113. [Epub ahead of print]

PMID:

 

28350721

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Select item 28337699 97.

 

  1. Lung Ultrasound is Comparable with Chest Roentgenogram for Diagnosis of Community-Acquired Pneumonia in Hospitalised Children.

Yadav KK, Awasthi S, Parihar A.

Indian J Pediatr. 2017 Mar 24. doi: 10.1007/s12098-017-2333-1. [Epub ahead of print]

PMID:

 

28337699

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Select item 28337558 98.

 

  1. Improving on the diagnostic characteristics of echocardiography for pulmonary hypertension.

Broderick-Forsgren K, Davenport CA, Sivak JA, Hargett CW, Foster MC, Monteagudo A, Armour A, Rajagopal S, Arges K, Velazquez EJ, Samad Z.

Int J Cardiovasc Imaging. 2017 Mar 24. doi: 10.1007/s10554-017-1114-2. [Epub ahead of print]

PMID:

 

28337558

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Select item 28341902 99.

 

  1. PUF60 variants cause a syndrome of ID, short stature, microcephaly, coloboma, craniofacial, cardiac, renal and spinal features.

Low KJ, Ansari M, Abou Jamra R, Clarke A, El Chehadeh S, FitzPatrick DR, Greenslade M, Henderson A, Hurst J, Keller K, Kuentz P, Prescott T, Roessler F, Selmer KK, Schneider MC, Stewart F, Tatton-Brown K, Thevenon J, Vigeland MD, Vogt J, Willems M, Zonana J, Study DD, Smithson SF.

Eur J Hum Genet. 2017 Mar 22. doi: 10.1038/ejhg.2017.27. [Epub ahead of print]

PMID:

 

28327570

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Select item 28327193 105.

 

  1. School-related adjustment in children and adolescents with CHD.

Im YM, Lee S, Yun TJ, Choi JY.

Cardiol Young. 2017 Mar 20:1-7. doi: 10.1017/S1047951117000324. [Epub ahead of print]

PMID:

 

28318457

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Select item 28336264 112.

 

  1. Genomic imbalances in syndromiccongenital heart disease.

Molck MC, Simioni M, Vieira TP, Sgardioli IC, Monteiro FP, Souza J, Fett-Conte AC, Félix TM, Monlléo IL, Gil-da-Silva-Lopes VL.

J Pediatr (Rio J). 2017 Mar 20. pii: S0021-7557(17)30218-8. doi: 10.1016/j.jped.2016.11.007. [Epub ahead of print]

PMID:

 

28336264

Free Article

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Select item 28317159 113.

 

  1. N-Terminal Pro-B-Type Natriuretic Peptide and Phonocardiography in Differentiating Innocent Cardiac Murmurs fromCongenitalCardiac Anomalies in Asymptomatic Puppies.

Marinus SM, van Engelen H, Szatmári V.

J Vet Intern Med. 2017 Mar 18. doi: 10.1111/jvim.14667. [Epub ahead of print]

PMID:

 

28316101

Free Article

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Select item 28328585 115.

 

  1. HeartFailure in Pediatric Patients WithCongenital Heart Disease.

Hinton RB, Ware SM.

Circ Res. 2017 Mar 17;120(6):978-994. doi: 10.1161/CIRCRESAHA.116.308996.

PMID:

 

28302743

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Select item 28302742 121.

 

  1. Neurodevelopmental Abnormalities andCongenital Heart Disease: Insights Into Altered Brain Maturation.

Morton PD, Ishibashi N, Jonas RA.

Circ Res. 2017 Mar 17;120(6):960-977. doi: 10.1161/CIRCRESAHA.116.309048.

PMID:

 

28302742

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Select item 28302741 122.

 

  1. Has the Congenitally MalformedHeartChanged Its Face? Journey From Understanding Morphology to Surgical Cure in Congenital Heart Disease.

Anderson RH.

Circ Res. 2017 Mar 17;120(6):901-903. doi: 10.1161/CIRCRESAHA.116.310229. No abstract available.

PMID:

 

28302737

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Select item 28302736 127.

 

  1. Perspective onCongenital Heart DiseaseResearch.

Kaltman JR, Burns KM, Pearson GD.

Circ Res. 2017 Mar 17;120(6):898-900. doi: 10.1161/CIRCRESAHA.116.310334. No abstract available.

PMID:

 

28302736

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Select item 28302735 128.

 

  1. Evaluating the Performance of Ultrasound Screening forCongenital Heart Disease: A Descriptive Cohort Study.

Froehlich RJ, Maggio L, Has P, Werner EF, Rouse DJ.

Am J Perinatol. 2017 Mar 16. doi: 10.1055/s-0037-1601309. [Epub ahead of print]

PMID:

 

28301892

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Select item 28302079 131.

 

  1. Lower Circulating Folate Induced by a Fidgetin Intronic Variant is Associated with ReducedCongenital Heart DiseaseSusceptibility.

Wang D, Wang F, Shi KH, Tao H, Li Y, Zhao R, Lu H, Duan W, Qiao B, Zhao SM, Wang H, Zhao JY.

Circulation. 2017 Mar 16. pii: CIRCULATIONAHA.116.025164. doi: 10.1161/CIRCULATIONAHA.116.025164. [Epub ahead of print]

PMID:

 

28302752

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Select item 28299882 133.

 

  1. Rates of autism and potential risk factors in children withcongenitalheart defects.

Bean Jaworski JL, Flynn T, Burnham N, Chittams JL, Sammarco T, Gerdes M, Bernbaum JC, Clancy RR, Solot CB, Zackai EH, McDonald-McGinn DM, Gaynor JW.

Congenit Heart Dis. 2017 Mar 16. doi: 10.1111/chd.12461. [Epub ahead of print]

PMID:

 

28299880

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Select item 28306681 135.

 

  1. Pulmonary hypertension after timely arterial switch operation in children with simple transposition of the great arteries: a newdiseaseentity?

Lammers AE, Bauer UM.

Heart. 2017 Mar 16. pii: heartjnl-2016-311105. doi: 10.1136/heartjnl-2016-311105. [Epub ahead of print] No abstract available.

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  1. Body mass index (BMI) and weight loss in patients withcongenital heart disease.

Burden AF.

Heart. 2017 Mar 16. pii: heartjnl-2017-311219. doi: 10.1136/heartjnl-2017-311219. [Epub ahead of print] No abstract available.

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  1. Comparison of MR flow quantification in peripheral and main pulmonary arteries in patients after right ventricular outflow tract surgery: A retrospective study.

Rutz T, Meierhofer C, Naumann S, Martinoff S, Ewert P, Stern HC, Fratz S.

J Magn Reson Imaging. 2017 Mar 16. doi: 10.1002/jmri.25701. [Epub ahead of print]

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  1. Gluten Sensitivity among Egyptian Infants withCongenital Heart Disease.

El-Alameey IR, Ahmed HH, Tawfik SM, Hassaballa F, Gawad AM, Eltahlawy E.

Open Access Maced J Med Sci. 2017 Mar 15;5(1):58-63. doi: 10.3889/oamjms.2017.026. Epub 2017 Feb 12.

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  1. Telemedicine in Pediatric Cardiology: A Scientific Statement From the AmericanHeartAssociation.

Satou GM, Rheuban K, Alverson D, Lewin M, Mahnke C, Marcin J, Martin GR, Mazur LS, Sahn DJ, Shah S, Tuckson R, Webb CL, Sable CA; American Heart Association Congenital Cardiac Disease Committee of the Council on Cardiovascular Disease in the Young and Council on Quality Care and Outcomes Research..

Circulation. 2017 Mar 14;135(11):e648-e678. doi: 10.1161/CIR.0000000000000478. Epub 2017 Feb 13. Review. No abstract available.

PMID:

 

28193604

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  1. Acute Decompensation in Pediatric Cardiac Patients: Outcomes After Rapid Response Events.

Bavare AC, Rafie KS, Bastero PX, Hagan JL, Checchia PA.

Pediatr Crit Care Med. 2017 Mar 14. doi: 10.1097/PCC.0000000000001117. [Epub ahead of print]

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28296663

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  1. Fatal severe coronary artery stenosis in Williams syndrome: decision making using late gadolinium enhancement cardiovascular MRI.

Voges I, Franklin RC, Wage R, Babu-Narayan SV.

Cardiol Young. 2017 Mar 13:1-4. doi: 10.1017/S1047951117000312. [Epub ahead of print]

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28287063

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  1. Usage of 3D models of tetralogy of Fallot for medical education: impact on learningcongenital heart disease.

Loke YH, Harahsheh AS, Krieger A, Olivieri LJ.

BMC Med Educ. 2017 Mar 11;17(1):54. doi: 10.1186/s12909-017-0889-0.

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28284205

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  1. [Air pollution and adverse birth outcome in China: a comprehensive review].

Zhu PF, Zhang Y, Ban J, Li TT, Shi XM.

Zhonghua Liu Xing Bing Xue Za Zhi. 2017 Mar 10;38(3):393-399. doi: 10.3760/cma.j.issn.0254-6450.2017.03.024. Chinese.

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28329947

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  1. Maternal acceptability of pulse oximetry screening at home after home birth or very early discharge.

Narayen IC, Kaptein AA, Hogewoning JA, Blom NA, Te Pas AB.

Eur J Pediatr. 2017 Mar 9. doi: 10.1007/s00431-017-2883-2. [Epub ahead of print]

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  1. Obstructed Infradiaphragmatic Total Anomalous Pulmonary Venous Return in a 13-Day-Old Infant Presenting Acutely to the Emergency Department: A Case Report.

Siacunco EA, Pacheco GS, Woolridge DP.

J Emerg Med. 2017 Mar 9. pii: S0736-4679(17)30120-8. doi: 10.1016/j.jemermed.2017.01.051. [Epub ahead of print]

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28285866

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  1. Teachingcongenital heart disease: A new era?

Beckerman Z, Mery CM.

J Thorac Cardiovasc Surg. 2017 Mar 9. pii: S0022-5223(17)30392-6. doi: 10.1016/j.jtcvs.2017.02.045. [Epub ahead of print] No abstract available.

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28359576

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  1. Cardiac response to enzyme replacement therapy in infantile Pompediseasewith severe hypertrophic cardiomyopathy.

Avula S, Nguyen TM, Marble M, Lilje C.

Echocardiography. 2017 Mar 7. doi: 10.1111/echo.13490. [Epub ahead of print]

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28266734

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  1. Texas Pulse Oximetry Project: A Multicenter Educational and Quality Improvement Project for Implementation of CriticalCongenital Heart DiseaseScreening Using Pulse Oximetry.

Guillory C, Gong A, Livingston J, Creel L, Ocampo E, McKee-Garrett T.

Am J Perinatol. 2017 Mar 6. doi: 10.1055/s-0037-1599214. [Epub ahead of print]

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  1. Maternal and infant genetic variants, maternal periconceptional use of selective serotonin reuptake inhibitors, and risk ofcongenitalheart defects in offspring: population based study.

Nembhard WN, Tang X, Hu Z, MacLeod S, Stowe Z, Webber D; National Birth Defects Prevention Study..

BMJ. 2017 Mar 6;356:j832. doi: 10.1136/bmj.j832.

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  1. Pulmonary arterial stiffness indices assessed by intravascular ultrasound in children with early pulmonary vasculardisease: prediction of advanceddisease and mortality during 20-year follow-up.

Ploegstra MJ, Brokelman JG, Roos-Hesselink JW, Douwes JM, van Osch-Gevers LM, Hoendermis ES, van den Bosch AE, Witsenburg M, Bartelds B, Hillege HL, Berger RM.

Eur Heart J Cardiovasc Imaging. 2017 Mar 6. doi: 10.1093/ehjci/jex015. [Epub ahead of print]

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28329155

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Voss C, Duncombe SL, Dean PH, de Souza AM, Harris KC.

J Am Heart Assoc. 2017 Mar 6;6(3). pii: e004665. doi: 10.1161/JAHA.116.004665.

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  1. Prospective ECG-triggering cardiac CT for infants with complexcongenital heart diseaseusing low-dose contrast medium, low tube voltage, and adaptive statistical iterative reconstruction.

Wang SY, Gao W, Zhong YM, Sun AM, Wang Q, Hu LW, Qiu HS, Li JY.

Clin Radiol. 2017 Mar 4. pii: S0009-9260(17)30058-2. doi: 10.1016/j.crad.2017.01.017. [Epub ahead of print]

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  1. A comparison of the incidence of undiagnosedcongenital heart diseasein hospital born and home born children.

Reich JD, Haight D, Reich ZS.

J Neonatal Perinatal Med. 2017 Mar 4. doi: 10.3233/NPM-1651. [Epub ahead of print]

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28282821

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  1. Diminished liver microperfusion in Fontan patients: A biexponential DWI study.

Dijkstra H, Wolff D, van Melle JP, Bartelds B, Willems TP, Oudkerk M, Hillege H, van den Berg AP, Ebels T, Berger RM, Sijens PE.

PLoS One. 2017 Mar 3;12(3):e0173149. doi: 10.1371/journal.pone.0173149. eCollection 2017.

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  1. Respiratory syncytial virus hospitalization risk in the second year of life by specificcongenital heart diseasediagnoses.

Friedman D, Fryzek J, Jiang X, Bloomfield A, Ambrose CS, Wong PC.

PLoS One. 2017 Mar 2;12(3):e0172512. doi: 10.1371/journal.pone.0172512. eCollection 2017.

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  1. Maternal Stress and Anxiety in the Pediatric Cardiac Intensive Care Unit.

Lisanti AJ, Allen LR, Kelly L, Medoff-Cooper B.

Am J Crit Care. 2017 Mar;26(2):118-125. doi: 10.4037/ajcc2017266.

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  1. Ectopia cordis: a novel palliative care technique.

Berry MJ, Saito-Benz M, Klein T, Bowkett B, Richardson VF.

BMJ Support Palliat Care. 2017 Mar;7(1):102-104. doi: 10.1136/bmjspcare-2015-000946. Epub 2016 Apr 21.

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  1. Noncompaction cardiomyopathy in Hirschsprung’sdisease: a case report.

Visonà SD, Thiene G, Mannarino S, Corana G, Osculati A, Angelini A, Rizzo S.

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  1. Pentraxin 3 in neonates with and without diagnosis of pulmonary hypertension.

Farhadi R, Rafiei A, Hamdamian S, Zamani H, Yazdani J.

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  1. Congenitalheartdiseases and their association with the variant distribution features on susceptibility genes.

Su W, Zhu P, Wang R, Wu Q, Wang M, Zhang X, Mei L, Tang J, Kumar M, Wang X, Su L, Dong N.

Clin Genet. 2017 Mar;91(3):349-354. doi: 10.1111/cge.12835. Epub 2016 Sep 5. Review.

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  1. Ciliopathies.

Braun DA, Hildebrandt F.

Cold Spring Harb Perspect Biol. 2017 Mar 1;9(3). pii: a028191. doi: 10.1101/cshperspect.a028191. Review.

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  1. Appropriate Use of Genetic Testing inCongenital Heart DiseasePatients.

Ito S, Chapman KA, Kisling M, John AS.

Curr Cardiol Rep. 2017 Mar;19(3):24. doi: 10.1007/s11886-017-0834-1. Review.

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  1. Congenitaluni-leaflet mitral valve with severe stenosis: A case report with literature review.

Zhang W, Wang Y, Ma C, Zhang Z, Yang J.

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  1. Persistent left superior vena cava accompanyingcongenital heart diseasein children: Experience of a tertiary care center.

Ari ME, Doğan V, Özgür S, Ceylan Ö, Ertuğrul İ, Kayalı Ş, Yoldaş T, Örün UA, Kaya Ö, Karademir S.

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  1. Left Ventricular Non-Compaction: A Cardiomyopathy With Acceptable Prognosis in Children.

Tian T, Yang Y, Zhou L, Luo F, Li Y, Fan P, Dong X, Liu Y, Cui J, Zhou X.

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  1. Compound heterozygous GATA5 mutations in a girl with hydrops fetalis,congenitalheartdefects and genital anomalies.

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  1. Defining the Risk and Associated Morbidity and Mortality of Severe Respiratory Syncytial Virus Infection Among Infants withCongenital Heart Disease.

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Wong J, Pushparajah K, de Vecchi A, Ruijsink B, Greil GF, Hussain T, Razavi R.

Int J Cardiol. 2017 Mar 1;230:439-446. doi: 10.1016/j.ijcard.2016.12.087. Epub 2016 Dec 22.

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  1. The promises and challenges of exome sequencing in familial, non-syndromiccongenital heart disease.

Blue GM, Humphreys D, Szot J, Major J, Chapman G, Bosman A, Kirk EP, Sholler GF, Harvey RP, Dunwoodie SL, Winlaw DS.

Int J Cardiol. 2017 Mar 1;230:155-163. doi: 10.1016/j.ijcard.2016.12.024. Epub 2016 Dec 13.

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  1. Normative Values of Aortic Arch Structures in Premature Infants.

Dijkema EJ, Molenschot MC, Breur JM, de Vries WB, Slieker MG.

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  1. Infundibular Pulmonic Stenosis in a Moluccan Cockatoo (Cacatua moluccensis).

Zoller G, Guzman DS, Summa N, Keller KA, Silverman SJ, Stern JA.

J Avian Med Surg. 2017 Mar;31(1):53-61. doi: 10.1647/2015-126.

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28358604

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  1. Is maternal smoking during pregnancy associated with an increased risk ofcongenitalheartdefects among offspring? A systematic review and meta-analysis of observational studies.

Zhang D, Cui H, Zhang L, Huang Y, Zhu J, Li X.

J Matern Fetal Neonatal Med. 2017 Mar;30(6):645-657. doi: 10.1080/14767058.2016.1183640. Epub 2016 May 16.

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27126055

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  1. A novel, more efficient, staged approach for criticalcongenital heart diseasescreening.

Mouledoux J, Guerra S, Ballweg J, Li Y, Walsh W.

J Perinatol. 2017 Mar;37(3):288-290. doi: 10.1038/jp.2016.204. Epub 2016 Nov 10.

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  1. Pathology and molecular mechanisms of coarctation of the aorta and its association with the ductus arteriosus.

Yokoyama U, Ichikawa Y, Minamisawa S, Ishikawa Y.

J Physiol Sci. 2017 Mar;67(2):259-270. doi: 10.1007/s12576-016-0512-x. Epub 2016 Dec 20. Review.

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28000176

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  1. Use of Model-based Iterative Reconstruction to Improve Detection ofCongenitalCardiovascular Anomalies in Infants Undergoing Free-breathing Computed Tomographic Angiography.

Kligerman S, Bolster F, Mitchell J, Henry T, Jeudy J, White CS.

J Thorac Imaging. 2017 Mar;32(2):127-135. doi: 10.1097/RTI.0000000000000257.

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  1. Down syndrome and the complexity of genome dosage imbalance.

Antonarakis SE.

Nat Rev Genet. 2017 Mar;18(3):147-163. doi: 10.1038/nrg.2016.154. Epub 2016 Dec 28. Review.

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  1. FDG-PET Identification of Infected Pulmonary Artery Conduit Following Tetralogy of Fallot (TOF) Repair.

Zhang Y, Williams H, Pucar D.

Nucl Med Mol Imaging. 2017 Mar;51(1):86-87. doi: 10.1007/s13139-016-0424-y. Epub 2016 Jun 8.

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  1. Cardiorespiratory Interactions in Paediatrics: ‘It’s (almost always) the circulation stupid!’

Rigby ML, Rosenthal M.

Paediatr Respir Rev. 2017 Mar;22:60-65. doi: 10.1016/j.prrv.2016.08.003. Epub 2016 Aug 12. Review.

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  1. The Impact of the Right Ventricular Outflow Tract Patch on Right Ventricular Strain in Tetralogy of Fallot: A Comparison with Valvar Pulmonary Stenosis Utilizing Cardiac Magnetic Resonance.

Anwar S, Harris MA, Whitehead KK, Keller MS, Goldmuntz E, Fogel MA, Mercer-Rosa L.

Pediatr Cardiol. 2017 Mar;38(3):617-623. doi: 10.1007/s00246-016-1558-5. Epub 2017 Jan 31.

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  1. Transposition of Great Arteries with Complex Coronary Artery Variants: Time-Related Events Following Arterial Switch Operation.

Al Anani S, Fughhi I, Taqatqa A, Elzein C, Ilbawi MN, Polimenakos AC.

Pediatr Cardiol. 2017 Mar;38(3):513-524. doi: 10.1007/s00246-016-1543-z. Epub 2016 Dec 19.

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  1. Tricuspid Atresia with Non-compaction: An Early Experience with Implications for Surgical Palliation.

Nguyen HH, Khan R, Silverman NH, Singh GK.

Pediatr Cardiol. 2017 Mar;38(3):495-505. doi: 10.1007/s00246-016-1541-1. Epub 2016 Dec 10.

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  1. Relationship Between Habitual Exercise and Performance on Cardiopulmonary Exercise Testing Differs Between Children With Single and Biventricular Circulations.

O’Byrne ML, Desai S, Lane M, McBride M, Paridon S, Goldmuntz E.

Pediatr Cardiol. 2017 Mar;38(3):472-483. doi: 10.1007/s00246-016-1537-x. Epub 2016 Nov 23.

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  1. Persistent Left Superior Vena Cava.

Nair V, Yusuf K, Yu W, AlAwad H, Paul K, Al Awad E.

Pediatr Dev Pathol. 2017 Mar-Apr;20(2):182-185. doi: 10.1177/1093526616686008. Epub 2017 Jan 25.

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  1. Congenital heart diseaseat Laos Children’s Hospital: Two year experience.

Hwang IC, Sisavanh M, Billamay S, Phangmanixay S, Oudavong B, Kang J, Kwon BS, Kim GB, Bae EJ, Noh CI, Choi JY.

Pediatr Int. 2017 Mar;59(3):271-279. doi: 10.1111/ped.13156. Epub 2017 Jan 26.

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27589674

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  1. Myocardial stress perfusion magnetic resonance: initial experience in a pediatric and young adult population using regadenoson.

Noel CV, Krishnamurthy R, Moffett B, Krishnamurthy R.

Pediatr Radiol. 2017 Mar;47(3):280-289. doi: 10.1007/s00247-016-3762-0. Epub 2016 Dec 29.

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28035425

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  1. Prevalence of non-cardiovascular findings on CT angiography in children withcongenital heart disease.

Malik A, Hellinger JC, Servaes S, Schwartz MC, Keller MS, Epelman M.

Pediatr Radiol. 2017 Mar;47(3):267-279. doi: 10.1007/s00247-016-3742-4. Epub 2016 Dec 6.

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27924361

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  1. Psychiatric Disorders in Adolescents With Single VentricleCongenital Heart Disease.

DeMaso DR, Calderon J, Taylor GA, Holland JE, Stopp C, White MT, Bellinger DC, Rivkin MJ, Wypij D, Newburger JW.

Pediatrics. 2017 Mar;139(3). pii: e20162241. doi: 10.1542/peds.2016-2241. Epub 2017 Feb 1.

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  1. Nuclear Medicine in Pediatric Cardiology.

Milanesi O, Stellin G, Zucchetta P.

Semin Nucl Med. 2017 Mar;47(2):158-169. doi: 10.1053/j.semnuclmed.2016.10.008. Epub 2017 Jan 10. Review.

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28237004

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  1. Type IV Total Anomalous Pulmonary Venous Connection.

St Louis JD, Turk EM, Jacobs JP, O’Brien JE Jr.

World J Pediatr Congenit Heart Surg. 2017 Mar;8(2):142-147. doi: 10.1177/2150135116682456.

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  1. Public Views on Pulse Oximetry Screening for CriticalCongenital Heart Disease.

Kumar P, Iyengar H, Kumar P.

World J Pediatr Congenit Heart Surg. 2017 Mar;8(2):130-134. doi: 10.1177/2150135116674468.

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28329450

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  1. Clinical Genetic Testing in PediatricCardiomyopathy: Is Bigger Better?

Ouellette AC, Mathew J, Manickaraj AK, Manase G, Zahavich L, Wilson J, George K, Benson L, Bowdin S, Mital S.

Clin Genet. 2017 Mar 30. doi: 10.1111/cge.13024. [Epub ahead of print]

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28369760

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  1. Application of the PediatricAppropriate Use Criteria for Chest Pain.

Nguyen T, Fundora MP, Welch E, Douglas PS, Eidem BW, Campbell RM, Weiner RB, Stern KW, Benavidez OJ, Lai WW, Sachdeva R, Lopez L.

J Pediatr. 2017 Mar 30. pii: S0022-3476(17)30339-6. doi: 10.1016/j.jpeds.2017.02.073. [Epub ahead of print]

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28366354

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  1. Effects of L-thyroxine treatment on heart functions in infants with congenital hypothyroidism.

Arslan A, Baş VN, Uytun S, Poyrazoğlu HG.

J Pediatr Endocrinol Metab. 2017 Mar 30. pii: /j/jpem.ahead-of-print/jpem-2016-0393/jpem-2016-0393.xml. doi: 10.1515/jpem-2016-0393. [Epub ahead of print]

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28358714

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  1. Serum Biomarkers of Myocardial Remodeling and Coronary Dysfunction in Early Stages of Hypertrophic Cardiomyopathy in the Young.

Fernlund E, Gyllenhammar T, Jablonowski R, Carlsson M, Larsson A, Ärnlöv J, Liuba P.

Pediatr Cardiol. 2017 Mar 30. doi: 10.1007/s00246-017-1593-x. [Epub ahead of print]

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28361263

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  1. Dopamine as a potential rescue therapy for refractory protein-losing enteropathy in Fontan-palliated patients.

Friedland-Little JM, Gajarski RJ, Schumacher KR.

Pediatr Transplant. 2017 Mar 30. doi: 10.1111/petr.12925. [Epub ahead of print]

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28370952

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  1. Statin-Associated Myopathy in a PediatricPreventive CardiologyPractice.

Johnson PK, Mendelson MM, Baker A, Ryan HH, Warren S, Graham D, Griggs SS, Desai NK, Yellen E, Buckley L, Zachariah JP, de Ferranti SD.

J Pediatr. 2017 Mar 29. pii: S0022-3476(17)30297-4. doi: 10.1016/j.jpeds.2017.02.047. [Epub ahead of print]

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  1. Peripheral Endothelial Function After Arterial Switch Operation for D-looped Transposition of the Great Arteries.

Sun HY, Stauffer KJ, Nourse SE, Vu C, Selamet Tierney ES.

Pediatr Cardiol. 2017 Mar 27. doi: 10.1007/s00246-017-1609-6. [Epub ahead of print]

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28345114

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  1. Differentiation of Impaired From Preserved Hemodynamics in Patients With Fontan Circulation Using Real-time Phase-velocity Cardiovascular Magnetic Resonance.

Körperich H, Müller K, Barth P, Gieseke J, Haas N, Schulze-Neick I, Burchert W, Kececioglu D, Laser KT.

J Thorac Imaging. 2017 Mar 24. doi: 10.1097/RTI.0000000000000261. [Epub ahead of print]

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28346330

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  1. Adult Chest Pain in the PediatricEmergency Department: Treatment and Timeliness From Door In To Door Out.

Sacks JH, Flueckiger PB, Spandorfer PR, Mahle WT, Costello BE.

Pediatr Emerg Care. 2017 Mar 21. doi: 10.1097/PEC.0000000000001081. [Epub ahead of print]

PMID:

 

28328689

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Select item 28318457 112.

 

  1. School-related adjustment in children and adolescents with CHD.

Im YM, Lee S, Yun TJ, Choi JY.

Cardiol Young. 2017 Mar 20:1-7. doi: 10.1017/S1047951117000324. [Epub ahead of print]

PMID:

 

28318457

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Select item 28321935 113.

 

  1. Sets of serum exosomal microRNAs as candidate diagnostic biomarkers for Kawasaki disease.

Jia HL, Liu CW, Zhang L, Xu WJ, Gao XJ, Bai J, Xu YF, Xu MG, Zhang G.

Sci Rep. 2017 Mar 20;7:44706. doi: 10.1038/srep44706.

PMID:

 

28317854

Free PMC Article

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Select item 28317142 117.

 

  1. Pharmacogenomic screening for anthracycline-induced cardiotoxicity in childhood cancer.

Aminkeng F, Ross CJ, Rassekh SR, Rieder MJ, Bhavsar AP, Sanatani S, Bernstein D, Hayden MR, Amstutz U, Carleton BC.

Br J Clin Pharmacol. 2017 Mar 19. doi: 10.1111/bcp.13218. [Epub ahead of print] No abstract available.

PMID:

 

28317142

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Select item 28317159 118.

 

  1. Hyperoxia Reduces Oxygen Consumption in Children with Pulmonary Hypertension.

Guo L, Bobhate P, Kumar S, Vadlamudi K, Kaddoura T, Elgendi M, Holinski P, Coe JY, Rutledge J, Adatia I.

Pediatr Cardiol. 2017 Mar 18. doi: 10.1007/s00246-017-1602-0. [Epub ahead of print]

PMID:

 

28315943

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Select item 28315942 121.

 

  1. Forgotten Right Ventricle in PediatricDilated Cardiomyopathy.

Agha HM, Ibrahim H, El Satar IA, El Rahman NA, El Aziz DA, Salah Z, El Saeidi S, Mostafa F, Attia W, El Rahman MA, El Mohsen GA.

Pediatr Cardiol. 2017 Mar 18. doi: 10.1007/s00246-017-1588-7. [Epub ahead of print]

PMID:

 

28315942

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Select item 28303658 122.

 

  1. Infective endocarditis in children: an update.

Dixon G, Christov G.

Curr Opin Infect Dis. 2017 Mar 17. doi: 10.1097/QCO.0000000000000370. [Epub ahead of print]

PMID:

 

28319472

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Select item 28302143 127.

 

  1. Altered aortic 3D hemodynamics and geometry in pediatricMarfan syndrome patients.

van der Palen RL, Barker AJ, Bollache E, Garcia J, Rose MJ, van Ooij P, Young LT, Roest AA, Markl M, Robinson JD, Rigsby CK.

J Cardiovasc Magn Reson. 2017 Mar 17;19(1):30. doi: 10.1186/s12968-017-0345-7.

PMID:

 

28302143

Free PMC Article

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Select item 28318851 128.

 

  1. A Pilot Study: Cardiac Parameters in Children Receiving New-Generation Antidepressants.

Uchida M, Spencer AE, Kenworthy T, Chan J, Fitzgerald M, Rosales AM, Kagan E, Saunders A, Biederman J.

J Clin Psychopharmacol. 2017 Mar 15. doi: 10.1097/JCP.0000000000000683. [Epub ahead of print]

PMID:

 

28301398

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Select item 28299430 145.

 

  1. Parent reports of health-related quality of life and heart failure severity score independently predict outcome in children with dilated cardiomyopathy.

den Boer SL, Baart SJ, van der Meulen MH, van Iperen GG, Backx AP, Ten Harkel AD, Rammeloo LA, du Marchie Sarvaas GJ, Tanke RB, Helbing WA, Utens EM, Dalinghaus M.

Cardiol Young. 2017 Mar 14:1-9. doi: 10.1017/S1047951116002833. [Epub ahead of print]

PMID:

 

28290258

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Select item 28193604 150.

 

  1. Mutations in TMEM260 Cause a PediatricNeurodevelopmental, Cardiac, and Renal Syndrome.

Ta-Shma A, Khan TN, Vivante A, Willer JR, Matak P, Jalas C, Pode-Shakked B, Salem Y, Anikster Y, Hildebrandt F, Katsanis N, Elpeleg O, Davis EE.

Am J Hum Genet. 2017 Mar 11. pii: S0002-9297(17)30074-5. doi: 10.1016/j.ajhg.2017.02.007. [Epub ahead of print]

PMID:

 

28318500

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  1. Elucidation of MRAS-mediated Noonan syndrome with cardiac hypertrophy.

Higgins EM, Bos JM, Mason-Suares H, Tester DJ, Ackerman JP, MacRae CA, Sol-Church K, Gripp KW, Urrutia R, Ackerman MJ.

JCI Insight. 2017 Mar 9;2(5):e91225. doi: 10.1172/jci.insight.91225.

PMID:

 

28289718

Free PMC Article

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Select item 28274955 174.

 

  1. Low Iron Stores in Otherwise Healthy Children Affect Electrocardiographic Markers of Important Cardiac Events.

Karadeniz C, Özdemir R, Demirol M, Katipoğlu N, Yozgat Y, Meşe T, Ünal N.

Pediatr Cardiol. 2017 Mar 7. doi: 10.1007/s00246-017-1596-7. [Epub ahead of print]

PMID:

 

28271153

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  1. Texas Pulse Oximetry Project: A Multicenter Educational and Quality Improvement Project for Implementation of Critical Congenital Heart Disease Screening Using Pulse Oximetry.

Guillory C, Gong A, Livingston J, Creel L, Ocampo E, McKee-Garrett T.

Am J Perinatol. 2017 Mar 6. doi: 10.1055/s-0037-1599214. [Epub ahead of print]

PMID:

 

28264208

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Select item 28260544 184.

 

  1. Health-related quality of life in children and adolescents living in the north-east of China before and after cardiac catheter interventional treatment.

Li L, Lin P, Gao X.

Cardiol Young. 2017 Mar 6:1-5. doi: 10.1017/S104795111600247X. [Epub ahead of print]

PMID:

 

28260544

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Select item 28262914 185.

 

  1. Impaired Single Right Ventricular Function Compared to Single Left Ventricles during the Early Stages of Palliation: A Longitudinal Study.

Suntratonpipat S, Khoo NS, Colen T, Alhabdan M, Troung D, Zahari N, Kutty S, Smallhorn JF, Tham EB.

J Am Soc Echocardiogr. 2017 Mar 3. pii: S0894-7317(17)30001-9. doi: 10.1016/j.echo.2017.01.001. [Epub ahead of print]

PMID:

 

28268110

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Select item 28257439 202.

 

  1. Estimating Effective Dose of Radiation From PediatricCardiac CT Angiography Using a 64-MDCT Scanner: New Conversion Factors Relating Dose-Length Product to Effective Dose.

Trattner S, Chelliah A, Prinsen P, Ruzal-Shapiro CB, Xu Y, Jambawalikar S, Amurao M, Einstein AJ.

AJR Am J Roentgenol. 2017 Mar;208(3):585-594. doi: 10.2214/AJR.15.15908. Epub 2017 Jan 17.

PMID:

 

28095022

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Select item 28363353 209.

 

  1. Translating Research into Improved Patient Care in Pulmonary Arterial Hypertension.

Bonnet S, Provencher S, Guignabert C, Perros F, Boucherat O, Schermuly RT, Hassoun PM, Rabinovitch M, Nicolls MR, Humbert M.

Am J Respir Crit Care Med. 2017 Mar 1;195(5):583-595. doi: 10.1164/rccm.201607-1515PP. No abstract available.

PMID:

 

27649290

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Select item 28251816 212.

 

  1. Comparison of echocardiography with tissue Doppler imaging and magnetic resonance imaging with delayed enhancement in the assessment of children with hypertrophic cardiomyopathy.

Ziółkowska L, Petryka J, Boruc A, Kawalec W.

Arch Med Sci. 2017 Mar 1;13(2):328-336. doi: 10.5114/aoms.2016.60404. Epub 2016 Jun 7.

PMID:

 

28261285

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  1. The role of inflammatory biomarkers in CHD-associated pulmonary hypertension in children.

Oz-Tuncer G, Olgunturk R, Pektas A, Cilsal E, Kula S, Oguz DA, Tunaoglu SF, Pasaoglu OT, Pasaoglu H.

Cardiol Young. 2017 Mar;27(2):255-260. doi: 10.1017/S1047951116000408.

PMID:

 

28366184

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  1. Altering management decisions with gained anatomical insight from a 3D printed model of a complex ventricular septal defect.

Bhatla P, Mosca RS, Tretter JT.

Cardiol Young. 2017 Mar;27(2):377-380. doi: 10.1017/S104795111600202X. Epub 2016 Nov 21.

PMID:

 

27869052

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  1. Do toxic metals and trace elements have a role in the pathogenesis of conotruncal heart malformations?

Kundak AA, Pektas A, Zenciroglu A, Ozdemir S, Barutcu UB, Orun UA, Okumus N.

Cardiol Young. 2017 Mar;27(2):312-317. doi: 10.1017/S1047951116000536. Epub 2016 Oct 18.

PMID:

 

27751200

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Select item 27573031 224.

 

  1. Pulmonary haemodynamics in Fontan physiology after lobectomy in a patient with a single ventricle associated with pulmonary sequestration.

Mii S, Yasuda K, Murayama H.

Cardiol Young. 2017 Mar;27(2):381-384. doi: 10.1017/S1047951116001153. Epub 2016 Aug 30.

PMID:

 

27572898

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Select item 27225489 226.

 

  1. The comparative relationships between locations of the papillary muscles and electrophysiologic QRS axis in patients with atrioventricular septal defect and common as opposed to separate orifices in the valve guarding the common atrioventricular junction.

Low L, Idriss SF, Anderson RH, Maynard C, Wagner G, Hakacova N.

Cardiol Young. 2017 Mar;27(2):261-266. doi: 10.1017/S104795111600041X. Epub 2016 Apr 18.

PMID:

 

27087659

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Select item 27064196 230.

 

  1. Prevalence of rheumatic valvular heart disease in Rwandan school children: echocardiographic evaluation using the World Heart Federation criteria.

Mucumbitsi J, Bulwer B, Mutesa L, Ndahindwa V, Semakula M, Rusingiza E, Arya P, Breakey S, Patton-Bolman C, Kaplan EL.

Cardiovasc J Afr. 2017 Mar 1;28:1-8. doi: 10.5830/CVJA-2017-007. [Epub ahead of print]

PMID:

 

28252675

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Select item 28081513 232.

 

  1. Assessment of Diffuse Ventricular Myocardial Fibrosis Using Native T1 in Children With Repaired Tetralogy of Fallot.

Yim D, Riesenkampff E, Caro-Dominguez P, Yoo SJ, Seed M, Grosse-Wortmann L.

Circ Cardiovasc Imaging. 2017 Mar;10(3). pii: e005695. doi: 10.1161/CIRCIMAGING.116.005695.

PMID:

 

28292861

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Select item 28273405 238.

 

  1. Appropriate Use of Genetic Testing in Congenital Heart Disease Patients.

Ito S, Chapman KA, Kisling M, John AS.

Curr Cardiol Rep. 2017 Mar;19(3):24. doi: 10.1007/s11886-017-0834-1. Review.

PMID:

 

28224467

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Select item 27988166 242.

 

  1. Application of pediatricappropriate use criteria for initial outpatient evaluation of syncope.

Phelps HM, Kelleman MS, McCracken CE, Benavidez OJ, Campbell RM, Douglas PS, Eidem BW, Lai WW, Lopez L, Stern KW, Welch E, Sachdeva R.

Echocardiography. 2017 Mar;34(3):441-445. doi: 10.1111/echo.13475. Epub 2017 Feb 8.

PMID:

 

28177138

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Select item 28168740 244.

 

  1. Longitudinal changes in adolescents with TOF: implications for care.

Bhat M, Mercer-Rosa L, Fogel MA, Harris MA, Paridon SM, McBride MG, Shults J, Zhang X, Goldmuntz E.

Eur Heart J Cardiovasc Imaging. 2017 Mar 1;18(3):356-363. doi: 10.1093/ehjci/jew272.

PMID:

 

28363199

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Select item 27825557 250.

 

  1. Temporal Trends in Utilization of Transthoracic Echocardiography for Common Outpatient Pediatric CardiologyDiagnoses over the Past 15 Years.

Sachdeva R, Travers CD, McCracken CE, Samai C, Campbell RM, Slesnick TC, Border WL.

J Am Soc Echocardiogr. 2017 Mar;30(3):201-208. doi: 10.1016/j.echo.2016.12.008.

PMID:

 

28259302

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Select item 28139441 267.

 

  1. Right-Dominant Unbalanced Atrioventricular Septal Defect: Echocardiography in Surgical Decision Making.

Arunamata A, Balasubramanian S, Mainwaring R, Maeda K, Selamet Tierney ES.

J Am Soc Echocardiogr. 2017 Mar;30(3):216-226. doi: 10.1016/j.echo.2016.10.017. Epub 2016 Dec 6.

PMID:

 

27939051

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Select item 27909838 269.

 

  1. ECG-derived spatial QRS-T angle is strongly associated with hypertrophic cardiomyopathy.

Cortez D, Schlegel TT, Ackerman MJ, Bos JM.

J Electrocardiol. 2017 Mar – Apr;50(2):195-202. doi: 10.1016/j.jelectrocard.2016.10.001. Epub 2016 Oct 14.

PMID:

 

27839835

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Select item 27839834 277.

 

  1. Mortality after hematopoietic stem cell transplantation for severe mucopolysaccharidosis type I: the 30-year University of Minnesota experience.

Rodgers NJ, Kaizer AM, Miller WP, Rudser KD, Orchard PJ, Braunlin EA.

J Inherit Metab Dis. 2017 Mar;40(2):271-280. doi: 10.1007/s10545-016-0006-2. Epub 2017 Jan 4.

PMID:

 

28054207

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Select item 27943113 279.

 

  1. Advances in Pediatric CardiologyBoot Camp: Boot Camp Training Promotes Fellowship Readiness and Enables Retention of Knowledge.

Ceresnak SR, Axelrod DM, Sacks LD, Motonaga KS, Johnson ER, Krawczeski CD.

Pediatr Cardiol. 2017 Mar;38(3):631-640. doi: 10.1007/s00246-016-1560-y. Epub 2017 Feb 4.

PMID:

 

28161811

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Select item 28161810 300.

 

  1. PocketECG: A New Noninvasive Method for Continuous and Real-Time ECG Monitoring-Initial Results in Children and Adolescents.

Bieganowska K, Kaszuba A, Bieganowski M, Kaczmarek K.

Pediatr Cardiol. 2017 Mar;38(3):448-455. doi: 10.1007/s00246-016-1534-0. Epub 2017 Jan 18.

PMID:

 

28101660

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Select item 28078382 304.

 

  1. Assessing Myocardial Function in Infants with Pulmonary Hypertension: The Role of Tissue Doppler Imaging and Tricuspid Annular Plane Systolic Excursion.

Richardson C, Amirtharaj C, Gruber D, Hayes DA.

Pediatr Cardiol. 2017 Mar;38(3):558-565. doi: 10.1007/s00246-016-1548-7. Epub 2016 Dec 19.

PMID:

 

27995291

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Select item 27995290 310.

 

  1. HAND1 Loss-of-Function Mutation Causes Tetralogy of Fallot.

Wang J, Hu XQ, Guo YH, Gu JY, Xu JH, Li YJ, Li N, Yang XX, Yang YQ.

Pediatr Cardiol. 2017 Mar;38(3):547-557. doi: 10.1007/s00246-016-1547-8. Epub 2016 Dec 10.

PMID:

 

27942761

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Select item 27909753 315.

 

  1. Six-Minute Walk Test as a Predictor for Outcome in Children with Dilated Cardiomyopathy and Chronic Stable Heart Failure.

den Boer SL, Flipse DH, van der Meulen MH, Backx AP, du Marchie Sarvaas GJ, Ten Harkel AD, van Iperen GG, Rammeloo LA, Tanke RB, Helbing WA, Takken T, Dalinghaus M.

Pediatr Cardiol. 2017 Mar;38(3):465-471. doi: 10.1007/s00246-016-1536-y. Epub 2016 Dec 1.

PMID:

 

27909753

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Select item 27900408 316.

 

  1. N-terminal pro-B-type natriuretic peptide diagnostic algorithm versus American Heart Association algorithm for Kawasaki disease.

Dionne A, Meloche-Dumas L, Desjardins L, Turgeon J, Saint-Cyr C, Autmizguine J, Spigelblatt L, Fournier A, Dahdah N.

Pediatr Int. 2017 Mar;59(3):265-270. doi: 10.1111/ped.13154. Epub 2016 Dec 2.

PMID:

 

27589358

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Select item 27740725 326.

 

  1. Neonatal dilated cardiomyopathy.

Soares P, Rocha G, Pissarra S, Soares H, Flôr-de-Lima F, Costa S, Moura C, Dória S, Guimarães H.

Rev Port Cardiol. 2017 Mar;36(3):201-214. doi: 10.1016/j.repc.2016.10.007. Epub 2017 Feb 28. English, Portuguese.

PMID:

 

28256370

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Select item 28214152 338.

 

  1. Clinical and genetic diagnosis of familial hypertrophic cardiomyopathy: Results in pediatric cardiology.

Cardoso B, Gomes I, Loureiro P, Trigo C, Ferreira Pinto F.

Rev Port Cardiol. 2017 Mar;36(3):155-165. doi: 10.1016/j.repc.2016.09.009. Epub 2017 Feb 15. English, Portuguese.

PMID:

 

28214152

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Select item 28237004 339.

 

  1. Cardiac Function After Tetralogy of Fallot/Complete Atrioventricular Canal Repair.

Stephens EH, Tingo J, Najjar M, Yilmaz B, Levasseur S, Dayton JD, Mosca RS, Chai P, Quaegebeur JM, Bacha EA.

World J Pediatr Congenit Heart Surg. 2017 Mar;8(2):189-195. doi: 10.1177/2150135116682719.

PMID:

 

28329461

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Select item 28329452 346

 

  1. Passive range of motion exercise to enhance growth in infants following the Norwood procedure: a safety and feasibility trial.

Lambert LM, Trachtenberg FL, Pemberton VL, Wood J, Andreas S, Schlosser R, Barnard T, Daniels K, Harrington AT, Dagincourt N, Miller TA; Pediatric Heart Network Investigators..

Cardiol Young. 2017 Mar 23:1-8. doi: 10.1017/S1047951117000427. [Epub ahead of print]

PMID:

 

28330522

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Select item 28330516 4.

 

  1. Response to Commentary: “How badly do we need instruction in paediatric cardiac anatomy?”

Rogers LS, Klein M, James J, FitzGerald M.

Cardiol Young. 2017 Mar 22:1-2. doi: 10.1017/S1047951117000336. [Epub ahead of print] No abstract available.

PMID:

 

28327207

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Select item 28322179 6.

 

  1. How badly do we need instruction in paediatric cardiac anatomy?

Anderson RH.

Cardiol Young. 2017 Mar 21:1-4. doi: 10.1017/S1047951117000117. [Epub ahead of print] No abstract available.

PMID:

 

28322175

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Select item 28318481 8.

 

  1. Bilateral coronary artery to left ventricular fistula: CT demonstration of drainage via a single, common channel.

Lee HN, Kim JI, Cho JM.

Cardiol Young. 2017 Mar 10:1-2. doi: 10.1017/S1047951117000294. [Epub ahead of print]

PMID:

 

28279232

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Select item 28270260 18.

 

  1. What is the diagnostic value of the paediatric exercise tolerance test? Results from a UK centre.

Sajnach-Menke MA, Walpole SC.

Cardiol Young. 2017 Mar 8:1-5. doi: 10.1017/S1047951117000233. [Epub ahead of print]

PMID:

 

28270260

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Select item 28270245 19.

 

  1. Extremely refractory Kawasaki disease with disseminated intravascular coagulation.

Koh YK, Lee JH, Park YB.

Cardiol Young. 2017 Mar 7:1-4. doi: 10.1017/S1047951116002742. [Epub ahead of print]

PMID:

 

28264746

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Select item 28260555 21.

 

  1. Cor triatriatum sinister with an intact interatrial septum and a decompressing vein in a toddler.

Binsalamah ZM, De León LE, Heinle JS.

Cardiol Young. 2017 Mar 6:1-4. doi: 10.1017/S1047951117000130. [Epub ahead of print]

PMID:

 

28260553

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Select item 28260552 23.

 

  1. Indomethacin induced and prostaglandin relieved coarctation of the aorta in right aortic arch with left arterial duct: a case report.

Golden-Plotnik S, Rosenberg HC, Altamirano-Diaz LA.

Cardiol Young. 2017 Mar 6:1-4. doi: 10.1017/S1047951117000075. [Epub ahead of print]

PMID:

 

28260552

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Select item 28260551 24.

 

  1. Quality of life of Malaysian children with CHD.

Ong LC, Teh CS, Darshinee J, Omar A, Ang HL.

Cardiol Young. 2017 Mar 6:1-8. doi: 10.1017/S1047951117000166. [Epub ahead of print]

PMID:

 

28260550

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Select item 28260549 26.

 

  1. Reply on “Constriction of the ductus arteriosus, severe right ventricular hypertension, and a right ventricular aneurysm in a fetus after maternal use of a topical treatment for striae gravidarum”.

Rawlings AV, Dweck A, Dugmore P.

Cardiol Young. 2017 Mar 6:1-2. doi: 10.1017/S1047951117000129. [Epub ahead of print] No abstract available.

PMID:

 

28260549

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Select item 28260546 27.

 

  1. Clinical guidelines for the management of patients with transposition of the great arteries with intact ventricular septum.
[No authors listed]

Cardiol Young. 2017 Mar 2:1-40. doi: 10.1017/S1047951117000014. [Epub ahead of print] No abstract available.

PMID:

 

28249633

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Select item 28366186 33.

 

  1. MRI evaluation of mitral-aortic intervalvular fibrosa aneurysm in a boy.

Caro-Dominguez P, Gill N, Yoo SJ.

Cardiol Young. 2017 Mar;27(2):402-403. doi: 10.1017/S1047951116001323.

PMID:

 

28366186

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Select item 28366185 34.

 

  1. Functional near-tricuspid atresia in a patient with absent pulmonary valve and an intact ventricular septum.

Miki Y, Tanaka T, Oshima Y.

Cardiol Young. 2017 Mar;27(2):391-393. doi: 10.1017/S104795111600127X. Epub 2016 Aug 30.

PMID:

 

28300528

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Select item 28181900 37.

 

  1. Extensive coronary and systemic arterial aneurysm development in severe refractory Kawasaki disease.

Johnston N, Coleman D, McMahon CJ.

Cardiol Young. 2017 Mar;27(2):375-376. doi: 10.1017/S1047951116001967. Epub 2016 Nov 10.

PMID:

 

27829480

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Select item 27821206 42.

 

  1. Advances in molecular genetics for pulmonary atresia.

Gao M, He X, Zheng J.

Cardiol Young. 2017 Mar;27(2):207-216. doi: 10.1017/S1047951116001487. Epub 2016 Sep 22.

PMID:

 

27655138

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Select item 27646301 45.

 

  1. Understanding the morphogenesis of the left-sided arterial duct in the setting of a right-sided aortic arch.

Bamforth SD, Anderson RH.

Cardiol Young. 2017 Mar;27(2):369-372. doi: 10.1017/S1047951116001463. Epub 2016 Sep 20. No abstract available.

PMID:

 

27646301

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Select item 27641901 46.

 

  1. Neonatal management and outcomes of prenatally diagnosed CHDs.

Bensemlali M, Bajolle F, Laux D, Parisot P, Ladouceur M, Fermont L, Lévy M, Le Bidois J, Raimondi F, Ville Y, Salomon LJ, Boudjemline Y, Bonnet D.

Cardiol Young. 2017 Mar;27(2):344-353. doi: 10.1017/S1047951116000639. Epub 2016 May 26.

PMID:

 

27225605

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Select item 27225489 52.

 

  1. Current attitudes and clinical practice towards the care of pregnant women with underlying CHD: a paediatric cardiology perspective.

Cribbs MG, Briston DA, Zaidi AN.

Cardiol Young. 2017 Mar;27(2):236-242. doi: 10.1017/S104795111600038X. Epub 2016 Apr 11.

PMID:

 

27064196

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Select item 27056663 63.

 

  1. Pathogenesis of solitary right aortic arch: a mass effect hypothesis based on observations of serial human embryonic sections.

Jin ZW, Yamada T, Kim JH, Rodríguez-Vázquez JF, Murakami G, Arakawa K.

Cardiol Young. 2017 Mar;27(2):359-368. doi: 10.1017/S1047951115002152. Epub 2015 Oct 5.

PMID:

 

26435328

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  1. Type IV Total Anomalous Pulmonary Venous Connection.

St Louis JD, Turk EM, Jacobs JP, O’Brien JE Jr.

World J Pediatr Congenit Heart Surg. 2017 Mar;8(2):142-147. doi: 10.1177/2150135116682456.

PMID:

 

28329458

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ACHD Featured Articles of March 2017

ACHD Featured Reviews of March 2017 Publications

 

Contraceptive Practices of Women with Complex Congenital Heart Disease.

Miner PD, Canobbio MM, Pearson DD, Schlater M, Balon Y, Junge KJ, Bhatt A, Barber D, Nickolaus MJ, Kovacs AH, Moons P, Shaw K, Fernandes SM.

Am J Cardiol. 2017 Mar 15;119(6):911-915. doi: 10.1016/j.amjcard.2016.11.047. Epub 2016 Dec 18.

PMID:28087052

Similar articles

Select item 28318514 142.

Take Home Points:

 

  • A quarter of patients with ACHD had unplanned pregnancies.
  • Contraception counseling provided by the ACHD service only occurred in 43%.
  • Many patients with complex CHD who are at high risk for thromboembolic events are still using estrogen-based oral contraception with a documented risk of events of 4% in the total cohort (28% in the Fontan cohort).


Blanche CupidoCommentary from Dr Blanche Cupido (Cape Town, South Africa), section editor of ACHD Journal Watch:
Women with complex congenital heart disease (CHD) have a greater risk of adverse complications during pregnancy. Contraception and pre-conception counseling plays an important role in trying to reduce the risk of unplanned pregnancies. This study aimed to describe contraceptive practices of women with CHD (aged 18 years or older). It was a cross-sectional survey conducted from 2011-2014 in 9 ACHD centers across North America. The median age of the 505 women who participated was 33 years.  80% of patients were still menstruating.

achd media 1

 

Most patients used barrier methods (87%), with oral contraception (OC) being the next most utilized form of contraception (84%). The use of emergency contraception was 19% and 16% had been sterilized. More patients in the complex CHD group had sterilization (22% vs 12%, p=0.047).

 

The rates of progesterone-only OC use was greater in those patients with more complex CHD (19%, 27% and 39% respectively in those with simple, moderate and complex CHD, p=0.034). Despite current guideline recommendations for the use of progesterone only-based contraception in those patients at high risk of thromboembolic events (mechanical prosthetic valves, cyanotic patients, Fontan circulation patients), many patients with complex CHD (>60%) are receiving an estrogen containing contraception pill. Four percent of the total cohort on OC experienced a thrombotic event. In a high-risk subset of Fontan patients (n=31), 28% had an event.  The prevalence of thrombotic events in those patients with previous OC use was 39% compared to 17% in those with no previous OC use.

 

Although 84% of the total cohort had received some sort of contraception counseling, only 43% received it from their ACHD provider. Patients with greater complexity CHD were more likely to receive counseling (56 vs 45%, p=0.036). Regardless of the complexity of their underlying disease, 25% of patients had an unplanned pregnancy.

 

Mortality Risk Stratification in Fontan Patients Who Underwent Heart Transplantation.

Berg CJ, Bauer BS, Hageman A, Aboulhosn JA, Reardon LC.

Am J Cardiol. 2017 May 15;119(10):1675-1679. doi: 10.1016/j.amjcard.2017.02.005. Epub 2017 Mar 1.

PMID:  28341356

Similar articles

Take Home Point: 

  • The authors propose a risk score to help predict mortality in Fontan patients undergoing cardiac transplantation. This score includes: Age < 18 at time of transplant, Fontan-transplantation interval < 10 years, MELD XI score ≥ 19, systemic ventricle ejection fraction < 20%, moderate to severe atrioventricular valve regurgitation, and pre-transplant advanced mechanical support.

 

jokhadar-maanCommentary from Dr. Maan Jokhadar (Atlanta), section editor of ACHD Journal Watch:   The Fontan procedure and its various modifications have improved the survival of single ventricle patients.  However, Fontan circulation failure and decompensation is a common consequence and referral for cardiac transplantation is increasing in frequency though post-transplant outcomes appear to be worse in this population.  Predicting outcomes in Fontan patients referred for cardiac transplantation would be helpful in candidate selection and potentially in organ allocation.

 

Dr. Berg and colleagues from University of California in Los Angeles conducted a single center, retrospective cohort analysis of all single ventricle patients who underwent cardiac transplantation for failing Fontan between 1991 and 2014.  There was a total of 36 patients with a mean age of 21 years (7 to 48) and a mean follow-up of about 3.5 years.  Two thirds had systemic left ventricle and 2 patients had concurrent heart and liver transplantation. There were a total of 17 deaths during the follow-up that included 8 who died before hospital discharge from heart transplant, 2 with allograft failure, 2 with pulmonary hemorrhage, 2 with septic shock, 1 with superior vena cava syndrome, and 1 with coagulopathy/hemorrhage.  Late causes of death included 3 patients with cardiac allograft vasculopathy, 2 with rejection, and 1 with post-transplant lymphoproliferative disease.  There were 3 patients with unknown cause of death.

 

The patients with the highest risk of death or those under the age of 18 (HR 3.88) with less than a 10-year interval between the Fontan operation and heart transplant (HR 4.38).  Also, transplantation prior to 2004 was associated with increased mortality (HR 4.18).  Interestingly, the number of prior heart surgeries and systemic left ventricle was not associated with increased mortality.  Univariate analysis between preoperative factors and postoperative mortality showed a significant association between severely reduced systemic ventricle ejection fraction < 20% (HR 3.40), moderate to severe atrioventricular valve regurgitation (HR 8.12), advanced mechanical support (renal replacement therapy (HR 72.29) and extracorporeal membrane oxygenation (HR 6.25)), as well as MELD XI score ≥ 19 (HR 4.91).  Fontan pressure > 16.5 mmHg was actually associated with reduced perioperative mortality.  Also of note, elevated trans-pulmonary gradient, chronic renal insufficiency, cirrhosis, and ascites, were not associated with increased postoperative mortality.

 

Though protein-losing enteropathy is generally associated with increased morbidity and mortality, this was not a predictor of post-transplant mortality in this study, which is consistent with prior reports.  It is interesting to note that protein-losing enteropathy is associated with lower bilirubin due to gastrointestinal loss of proteins that include albumin-bound bilirubin.  Thus, lower bilirubin levels result and a lower MELD XI score.

 

Based on these results, the investigators created a scoring system to help predict post-transplant mortality:

 

Age < 18 at time of transplant (1 point)
Fontan-transplantation interval < 10 years (2 points)
MELD XI score ≥ 19 (2 points)
Systemic ventricle ejection fraction < 20% (1 point)
Moderate to severe atrioventricular valve regurgitation (3 points)
Pre-transplant advanced mechanical support (3 points)

Maximum total score 12 points

 

In this study, the scoring system performance was as follows:

 

Score 0-1: No mortality during follow-up period.
Scores of 2-5 points: 50% mortality during follow-up period.
Scores greater than 5: 88% mortality during follow-up period.

 

This scoring system needs prospective validation before it is adopted as part of the evaluation of Fontan patients being considered for transplantation. This study could help answer the question of which Fontan patients are too sick for transplant. However, it should be noted that perioperative and postoperative mortality differs widely from one center to another (4% to 35% with an average of about 22%). The perioperative mortality after heart transplant in this study was about 22%. Transplanted organs are a scarce resource and patients who are listed for transplantation should have a realistic chance of benefit, while reducing morbidity and mortality. Clearly, sicker patients have worse outcomes and are less likely to benefit from cardiac transplantation.

 

Though this study may help answer the question of which Fontan patients may be too sick for transplant, this study does not answer the question of which Fontan patients should be listed and when. There is a fine line between too well for transplant and too sick for transplant, which appears to be more elusive in Fontan patients. Fontan failure patients can remain stable with medical therapy for years without transplant while some have precipitous decline that is often difficult to predict.  Further study is needed to help identify the Fontan patients who are at highest risk of 12 to 24-month mortality and identifying these patients may help refine specific and appropriate transplant criteria, listing status, and organ allocation for this precious gift of life.

 

Adult Congenital Cardiac Care.

Kogon BE, Miller K, Miller P, Alsoufi B, Rosenblum JM.

World J Pediatr Congenit Heart Surg. 2017 Mar;8(2):242-247. doi: 10.1177/2150135117690126.

PMID: 28329454

Similar articles

Take Home Points:

 The Adult Congenital Heart Association has created an online clinic directory of ACHD centers.

  • From 2006 to 2015, there has been an increase in programme access and availability over time in the USA, with more programmes in more states.
  • Access to diagnostic and therapeutic services, as well as ancillary services have expanded in these ACHD programmes.

 

 

Commentary from Dr Leong Ming Chern (Kuala Lumpur, Malaysia), Section editor of ACHD Journal Watch:  The Adult Congenital Heart Association (ACHA) has created an online clinic directory of ACHD centers. This data is self-reported and it incorporates all self-identified ACHD programmes in and out of USA. The authors analyzed the directory in 2006 and 2015, evaluating the growth of the directory in the USA as a whole and the growth of these programme individually.

achd media 2

By the end of 2015, compared to 2006, there were more ACHD programmes reporting to the directory in more states (107 programmes across 42 states vs 57 programmes across 33 states), with higher overall clinical volume (591 vs 164 half-day clinics per week, 96611 vs 34446 patient visits). On average, each programme was busier (5 vs 2 half-day clinics per week per programme). Over the time, the number of reported annual operations performed nearly doubled (4346 operations by 210 surgeons vs 2461 operations by 125 surgeons). There was an increase in per-programme volume for ACHD specialized clinics.

achd media 3

 

Access to diagnostic and therapeutic services such as echocardiography, diagnostic and interventional electrophysiology, cardiac computed tomography and cardiac magnetic resonance were reportedly available in 96%-100% of programmes. Other ancillary services such as high-risk obstetrics, pulmonary hypertension, genetic counseling etc., were reported available in 77%-96% of programmes.

achd media 4 achd media 5

In view of the increased demand and workload in these programmes, efforts have been made to verify the information reported in the clinic directory, streamlining the training of cardiologists and surgeons in the care of patients with ACHD and systemic data collection and quality assessment of certain diagnostic and therapeutic services.

In conclusion, between 2006 and 2015, the clinical directory and the individual programmes have grown in the USA.

 

Am J Cardiol. 2017 Mar 1;119(5):785-789. doi: 10.1016/j.amjcard.2016.11.021. Epub 2016 Dec 2.

Chronobiology of Acute Aortic Dissection in the Marfan Syndrome (from the National Registry of Genetically Triggered Thoracic Aortic Aneurysms and Cardiovascular Conditions and the International Registry of Acute Aortic Dissection).

Siddiqi HK1Luminais SN2Montgomery D3Bossone E4Dietz H5Evangelista A6Isselbacher E7LeMaire S8Manfredini R9Milewicz D10Nienaber CA11Roman M12Sechtem U13Silberbach M14Eagle KA3Pyeritz RE2GenTAC and IRAD investigators.

Take Home Points:

 Marfan Syndrome is associated with acute aortic dissection (AAD).

  • AAD is more likely in winter/spring season (November to April) compared to other half of the year.
  • It is more likely to occur during daytime hours (6am to 6pm) with men more likely to follow such temporal variation than women.

 

Commentary from Dr Leong Ming Chern (Kuala Lumpur, Malaysia), Section editor of ACHD Journal Watch:   Marfan syndrome is associated with acute aortic dissection (AAD). The authors analyzed 2 large registries i.e. the International Registry of Acute Aortic Dissection (IRAD) and the Genetically Trigger Thoracic Aortic Aneurysms and Cardiovascular Conditions (GenTAC) registries, to extract data on 257 patients with Marfan Syndrome who had suffered from an acute aortic dissection from 1980 to 2012, with the aim to study the possible trends in the chronobiology of AAD in this group of patients.

 

Mean age at time of AAD was 38 years and 156 (61%) of subjects were men. 167 (65%) of patients suffered a type A dissection. AAD was more likely in winter/spring season (November to April) than other half of the year (57% vs 43%, p=0.05). No effect of gender, type of dissection or hypertension on the seasonal distribution of AAD.

 

Dissections were significantly more likely to occur during daytime hours, with 66% of dissection occurring from 6am to 6pm (p=0.001). Men were more likely to dissect during the daytime hours than women (74%vs51%, p=0.01).

achd media 6 achd media 7 achd media 8

 

Various studies have described higher incidence of acute coronary events in the mornings and its seasonal pattern, although the exact pathophysiology is not clearly understood. Findings in this study supports the association between AAD in Marfan Syndrome and its temporal and seasonal variation, which can be used to aid treatment and resource planning as well as risk factor modification.

 

 

Outcome of adults with Eisenmenger syndrome treated with drugs specific to pulmonary arterial hypertension: A French multicentre study.

Hascoet S, Fournier E, Jaïs X, Le Gloan L, Dauphin C, Houeijeh A, Godart F, Iriart X, Richard A, Radojevic J, Amedro P, Bosser G, Souletie N, Bernard Y, Moceri P, Bouvaist H, Mauran P, Barre E, Basquin A, Karsenty C, Bonnet D, Iserin L, Sitbon O, Petit J, Fadel E, Humbert M, Ladouceur M.

Arch Cardiovasc Dis. 2017 Mar 9. pii: S1875-2136(17)30045-1. doi: 10.1016/j.acvd.2017.01.006. [Epub ahead of print]

PMID: 28286190

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Select item 28281093 174.

 Take Home Points:

 In tertiary care centers in France, over 80% of patients with Eisenmenger syndrome (ES) are on at least one PAH-specific drug, ranging between 41.7% to 100% at various centers

  • ES Patients who take either 1 or 2 PAH-specific drugs appear to have better clinical outcomes than patients on no PAH-specific drugs or who are on triple therapy
  • Although the onset of PAH in pretricuspid defects tends to occur later than for posttricuspid defects, this study indicates that prognosis of patients with PAH due to pretricuspid defects is fairly poor
  • In patients with ES, use of PAH-specific drugs appears to lower risk of transplantation and mortality; however, drug therapy is still palliative, and overall outcome of ES is still poor.

 

Kay_William_MD.16.CVaCommentary from Dr. W. Aaron Kay (Indianapolis), section editor of ACHD Journal Watch:  This study very nicely demonstrates the “real world” treatment of Eisenmenger syndrome (ES) at a variety of tertiary care centers in France.  Overall, 340 patients from 20 separate centers were included.  Patients were analyzed according to defect type (pretricuspid versus posttricuspid shunt), age at time of analysis, and duration of time from PAH diagnosis to time of analysis.  The overall proportion of ES patients on PAH-specific drugs was 80.9%, varying between ~40% and 100% between centers.  The percentage of patients on PAH-specific drugs was much high for those with pretricuspid shunts than posttricuspid shunts.  In this study, ES was defined as PAH (determined by invasive hemodynamics) including a mean pulmonary artery pressure of > 25 mmHg, a normal pulmonary capillary wedge pressure of < 15 mmHg, and a pulmonary vascular resistance > 3 Woods units, in the presence of a large, non-restrictive intracardiac and/or extracardiac shunt.  Other types of PAH associated with ACHD were not included (i.e. patients with persistent Blalock-Taussig shunts or complex pulmonary artery anatomy).

Endpoints for the study included clinical outcomes, with the primary outcomes being death or the need for lung or heart-lung transplantation.  Other clinical events recorded included heart failure, severe infection, arrhythmias, stroke, hemoptysis and other bleeding events.  Events were analyzed with Kaplan-Meier curves based on two models; one model using patient age as the time scale, and a second model using time since PAH diagnosis the time scale.  Genetic syndrome was also included as a variable, with trisomy 21 by far being the most common syndrome.  Clinical data were available for the clear majority of patients, with 93.8% available at the time of initiation of PAH-specific drug therapy.  Median follow-up after drug initiation was 5.5 years (range 3.0-9.1 years).  During follow-up, 19.7% died, with median age at death 41.8 years.  Of note, age at death was not statistically different between pretricuspid and posttricuspid defects (46.2 vs 39.5 years; P = 0.1).  Nearly all deaths were related to ES.  Overall prognosis was poor; half of patients with posttricuspid defects died or were transplanted prior to 50 years of age; half of patients with pretricuspid defects died or underwent transplantation within 20 years after initial PAH diagnosis.

 

The authors demonstrate that patients have a better prognosis on one or two PAH-specific drugs than if they are on no therapy or triple therapy.  The study was not designed to determine a difference between being on 1 versus 2 drugs, and patients who had a 2nd drug started after initial inclusion were analyzed based on number of drugs they were taking at their most recent clinical evaluation.  Patients on triple therapy (ERA, PDE5 and IV prostanoid) tended to be sickest and have the highest risk of transplantation or death.  A total of 28/340 patients underwent transplantation in this study; 24/28 with heart-lung transplantation, and the remaining 4 with double-lung transplantation combined with percutaneous atrial septal defect closure.

 

The median age of patients was 41.0 years in the pretricuspid group and 34.4 years in the posttricuspid/combined group.  NYHA/WHO functional class was III or IV in 62.7% of patients upon drug initiation, whereas only 2.9% were NYHA/WHO class I.  Arterial oxygen saturation did not change significantly in any group.  However, the 6 minute walk distance increased significantly with PAH-specific drug therapy (from 330 meters to 364 meters), and a higher percentage of patients had improvement in NYHA/WHO FC versus those on no therapy.

 

The most common regimen was monotherapy (46.7%), predominantly with bosentan (64.3% of monotherapy patients), which has been approved for clinical use in ES in France for several years.  Dual therapy (40.9%) was provided mostly with the combination of bosentan and sildenafil.  Median time from PAH diagnosis to initiation of PAH-specific drug therapy was shorter in the pretricuspid group (9.1 months) versus the posttricuspid group (67.0 months).

 

Of note, patients on triple-therapy had a longer duration on medical therapy, a higher proportion of Pretricuspid defects, and a lower proportion of genetic syndromes, as well as a worse initial NYHA/WHO FC at time of drug initiation.  This subset also had the highest incidence of ES-related complications, and these patients were responsible for most transplantations.

achd media 9

Figure 2. Kaplan—Meier curves showing survival without major clinical events (death or transplantation) according to age at follow-up, overall and in subgroups defined by defect location, presence or absence of genetic syndrome and pulmonary arterial hypertension-specific drug therapy (PAH-SDT). Event-free survival. D. Event-free survival in patients with post-tricuspid (post-tric.) or combined defects. PAH < 40 yo: pulmonary arterial hypertension diagnosis before 40 years of age; pre-tric.: pre-tricuspid.

 

 

The Cost Differential Between Warfarin Versus Aspirin Treatment After a Fontan Procedure. Schilling C, Dalziel K, Iyengar AJ, d’Udekem Y.

Heart Lung Circ. 2017 Mar 6. pii: S1443-9506(17)30086-0. doi: 10.1016/j.hlc.2017.02.003. [Epub ahead of print] PMID: 28372885

 

Take Home Points:

 Warfarin patients have an additional annual cost vs. those on aspirin.

  • Aspirin is clinically as effective as warfarin and Fontan patients would benefit from a less expensive and less time-consuming treatment.
  • Clinical consensus regarding this point will be difficult to achieve, but there are overarching socio-economic costs if we as an ACHD community fail to do so.

 

Moe_Tabitha-WEBCommentary from Dr. Tabitha Moe (Phoenix), section editor of ACHD Journal Watch:Management of post-Fontan patients with anti-coagulant vs. anti-platelet management of thrombotic risk is an ongoing debate among ACHD care teams.  This article represents the Australian perspective on this management paradigm.  This paper analyzes the societal costs of placing a patient on long-term warfarin including the patient’s travel, time away from work, and the costs to the health system.  These costs are often underappreciated and are exclusive of the costs associated with adverse events, and bleeding complications.  A randomized trial was unable to prove noninferiority of either aspirin or warfarin secondary to lack of power.  In a review of data from the ESC the model of care varies widely, and there is not consensus regarding the clinical effectiveness of aspirin over warfarin and is left to the cardiologist’s preference. This study is valuable in that they evaluated 475 patients who are status post Fontan, and so by congenital standards, this is a fairly large cohort, consistent with other studies that have come out of the NAZ collaborative. If the data from this one-year retrospective data analysis is extrapolated over a 15-year period then $5.8 million dollars could be saved, and is extrapolated over 30 years $11.6 million dollars could be saved. This type of health economics data is valuable in guiding management decision making.

 

Assessment of the systemic right ventricular function in overweight and obese patients with congenitally corrected transposition of the great arteries. Kowalik E, Klisiewicz A, Biernacka EK, Hoffman P. Kardiol Pol. 2017 Mar 10. doi: 10.5603/KP.a2017.0036. [Epub ahead of print] PMID: 28281729

Take Home Points:

 

  • Increasing weight in the overweight and obese range is associated with impaired systemic right ventricular systolic function in patient with CCTGA.
  • The chronic volume overload secondary to obesity may contribute to premature systemic systolic dysfunction.

  

Commentary from Dr. Tabitha Moe (Phoenix), section editor of ACHD Journal Watch: CCTGA is a rare defect of atrioventricular and ventriculo-arterial discordant connections that may go unidentified even in to late adult hood.  This small cohort paper from Poland evaluates a small cohort of 56 adults with CCTGA and only 22 of whom are considered overweight or obese (BMI >25).  Obesity results in hemodynamic changes that lead to increases in the cardiac output and systemic ventricle enlargement. Obesity may be associated with left ventricular hypertrophy, in patients with structurally normal hearts. Little is known about the influence of obesity on cardiac performance in ACHD, and there isn’t any data on patient with a systemic RV.  Their study excluded patients with hypertension, NYHA Class III/IV functional status, arrhythmia, or single ventricle palliation.  This may exclude a number of the patients about whom this data would be the most valuable, however, it makes for a less complex dataset for analysis. Interestingly, there was no observed correlation between the NT-pro BNP levels and BMI. However, the evidence in noncongenital patients is markedly significant in that obesity is associated with decreased NT-pro BNP levels compared to associated decreased systemic ventricular systolic function.  There was a significant reduction in the systemic RV longitudinal strain in the overweight/obese group.  There is also an inverse correlation between BMI and the e”/a” ratio, and a positive correlation between body mass index and left atrial area. One of the limitations of the study is the inherent limitation of imaging of the RV by echo.  As a rule, the RV remains difficult to adequately assess by echo secondary to complex geometry and proximity to the sternum, as well as increased body habitus and poor echo windows in an obese cohort. What remains unknown with this short-term study is whether or not the amount of time spent within the obese range is associated with premature development of RV dysfunction. Since body weight is a largely modifiable risk factor it may be reasonable to be more aggressive with weight management in this cohort as well as others with complex congenital heart disease.

 

 

ACHD March 2017

 

  1. Anomalous Aortic Origin of a Coronary Artery From the Inappropriate Sinus of Valsalva.

Cheezum MK, Liberthson RR, Shah NR, Villines TC, O’Gara PT, Landzberg MJ, Blankstein R.

J Am Coll Cardiol. 2017 Mar 28;69(12):1592-1608. doi: 10.1016/j.jacc.2017.01.031. Review.

PMID:

 

28335843

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  1. Reply: A Distinct Cardiomyopathy: HCN4 Syndrome Comprising Myocardial Noncompaction, Bradycardia, Mitral Valve Defects, and Aortic Dilation.

Arbustini E, Favalli V, Narula N, Serio A, Grasso M.

J Am Coll Cardiol. 2017 Mar 7;69(9):1210-1211. doi: 10.1016/j.jacc.2016.11.073. No abstract available.

PMID:

 

28254189

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  1. A Distinct Cardiomyopathy: HCN4 Syndrome Comprising Myocardial Noncompaction, Bradycardia, Mitral Valve Defects, and Aortic Dilation.

Schweizer PA, Koenen M, Katus HA, Thomas D.

J Am Coll Cardiol. 2017 Mar 7;69(9):1209-1210. doi: 10.1016/j.jacc.2016.10.085. No abstract available.

PMID:

 

28254188

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  1. The power in being together for young adults who haveheartdisease – the photoshoot experience.

Gallagher R, Potter E, Thomson Mangnall L, Ladak L, Gallagher P, Neubeck L.

Heart Lung. 2017 Mar 30. pii: S0147-9563(17)30073-0. doi: 10.1016/j.hrtlng.2017.02.004. [Epub ahead of print]

PMID:

 

28366291

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Select item 28358329 85.

 

  1. The Changing Landscape of Pulmonary Arterial Hypertension in the Adult withCongenital Heart Disease.

van Dissel AC, Mulder BJ, Bouma BJ.

J Clin Med. 2017 Mar 30;6(4). pii: E40. doi: 10.3390/jcm6040040. Review.

PMID:

 

28358329

Free Article

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Select item 28358424 86.

 

  1. Right ventricular function and N-terminal pro-brain natriuretic peptide levels in adult patients with simple dextro-transposition of the great arteries.

Martínez-Quintana E, Marrero-Negrín N, Gopar-Gopar S, Rodríguez-González F.

Echocardiography. 2017 Mar 29. doi: 10.1111/echo.13526. [Epub ahead of print]

PMID:

 

28370416

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Select item 28369397 89.

 

  1. Association Between Cardiovascular Risk Factors and Aortic Stenosis: The CANHEART Aortic Stenosis Study.

Yan AT, Koh M, Chan KK, Guo H, Alter DA, Austin PC, Tu JV, Wijeysundera HC, Ko DT.

J Am Coll Cardiol. 2017 Mar 28;69(12):1523-1532. doi: 10.1016/j.jacc.2017.01.025.

PMID:

 

28335833

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Select item 28359691 92.

 

  1. Red Flags for Maltese Adults withCongenital Heart Disease: Poorer Dental Care and Less Sports Participation Compared to Other European Patients-An APPROACH-IS Substudy.

Caruana M, Apers S, Kovacs AH, Luyckx K, Thomet C, Budts W, Sluman M, Eriksen K, Dellborg M, Berghammer M, Johansson B, Soufi A, Callus E, Moons P, Grech V; APPROACH-IS consortium and the International Society for Adult Congenital Heart Disease (ISACHD)..

Pediatr Cardiol. 2017 Mar 24. doi: 10.1007/s00246-017-1604-y. [Epub ahead of print]

PMID:

 

28341902

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Select item 28337810 100.

 

  1. Educational needs of adolescents withcongenital heart disease: Impact of a transition intervention programme.

Ladouceur M, Calderon J, Traore M, Cheurfi R, Pagnon C, Khraiche D, Bajolle F, Bonnet D.

Arch Cardiovasc Dis. 2017 Mar 22. pii: S1875-2136(17)30057-8. doi: 10.1016/j.acvd.2017.02.001. [Epub ahead of print]

PMID:

 

28342658

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Select item 28327570 104.

 

  1. Longitudinal Evaluation of Aortic Hemodynamics in Marfan Syndrome: New Insights from a 4D Flow Cardiovascular Magnetic Resonance Multi-Year Follow-Up Study.

Geiger J, Hirtler D, Gottfried K, Rahman O, Bollache E, Barker AJ, Markl M, Stiller B.

J Cardiovasc Magn Reson. 2017 Mar 22;19(1):33. doi: 10.1186/s12968-017-0347-5.

PMID:

 

28327193

Free PMC Article

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Select item 28343629 106.

 

  1. Executive Summary: Trends in U.S. Cardiovascular Care: 2016 Report From 4 ACC National Cardiovascular Data Registries.

Masoudi FA, Ponirakis A, de Lemos JA, Jollis JG, Kremers M, Messenger JC, Moore JW, Moussa I, Oetgen WJ, Varosy PD, Vincent RN, Wei J, Curtis JP, Roe MT, Spertus JA.

J Am Coll Cardiol. 2017 Mar 21;69(11):1424-1426. doi: 10.1016/j.jacc.2016.12.004. Epub 2016 Dec 23. No abstract available.

PMID:

 

28025066

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Select item 28025065 109.

 

  1. Trends in U.S. Cardiovascular Care: 2016 Report From 4 ACC National Cardiovascular Data Registries.

Masoudi FA, Ponirakis A, de Lemos JA, Jollis JG, Kremers M, Messenger JC, Moore JW, Moussa I, Oetgen WJ, Varosy PD, Vincent RN, Wei J, Curtis JP, Roe MT, Spertus JA.

J Am Coll Cardiol. 2017 Mar 21;69(11):1427-1450. doi: 10.1016/j.jacc.2016.12.005. Epub 2016 Dec 23. Review.

PMID:

 

28025065

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Select item 28242061 110.

 

  1. Noninvasive Imaging in AdultCongenital Heart Disease.

Burchill LJ, Huang J, Tretter JT, Khan AM, Crean AM, Veldtman GR, Kaul S, Broberg CS.

Circ Res. 2017 Mar 17;120(6):995-1014. doi: 10.1161/CIRCRESAHA.116.308983.

PMID:

 

28302744

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Select item 28302743 120.

 

  1. Changing Landscape ofCongenital Heart Disease.

Bouma BJ, Mulder BJ.

Circ Res. 2017 Mar 17;120(6):908-922. doi: 10.1161/CIRCRESAHA.116.309302.

PMID:

 

28302739

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Select item 28302738 125.

 

  1. Congenital Heart Disease: The Remarkable Journey From the “Post-Mortem Room” to Adult Clinics.

Marian AJ.

Circ Res. 2017 Mar 17;120(6):895-897. doi: 10.1161/CIRCRESAHA.117.310830. No abstract available.

PMID:

 

28302735

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Select item 28367436 129.

 

  1. Systolic ejection click versus split firstheartsound: Are our ears deceiving us?

Hoeting NM, McCracken CE, McConnell M, Sallee D, Iannucci GJ, Oster ME.

Congenit Heart Dis. 2017 Mar 16. doi: 10.1111/chd.12460. [Epub ahead of print]

PMID:

 

28299882

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Select item 28299880 134.

 

  1. Contraceptive Practices of Women With ComplexCongenital Heart Disease.

Miner PD, Canobbio MM, Pearson DD, Schlater M, Balon Y, Junge KJ, Bhatt A, Barber D, Nickolaus MJ, Kovacs AH, Moons P, Shaw K, Fernandes SM.

Am J Cardiol. 2017 Mar 15;119(6):911-915. doi: 10.1016/j.amjcard.2016.11.047. Epub 2016 Dec 18.

PMID:

 

28087052

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Select item 28318514 142.

 

  1. Three decades later: The fate of the population of patients who underwent the Atriopulmonary Fontan procedure.

Poh CL, Zannino D, Weintraub RG, Winlaw DS, Grigg LE, Cordina R, Hornung T, Bullock A, Justo RN, Gentles TL, Verrall C, du Plessis K, Celermajer DS, d’Udekem Y.

Int J Cardiol. 2017 Mar 15;231:99-104. doi: 10.1016/j.ijcard.2017.01.057. Epub 2017 Jan 7.

PMID:

 

28100430

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Select item 28096041 150.

 

  1. Advance Care Planning in Adults withCongenital Heart Disease: A Patient Priority.

Deng LX, Gleason LP, Khan AM, Drajpuch D, Fuller S, Goldberg LA, Mascio CE, Partington SL, Tobin L, Kim YY, Kovacs AH.

Int J Cardiol. 2017 Mar 15;231:105-109. doi: 10.1016/j.ijcard.2016.12.185. Epub 2017 Jan 4.

PMID:

 

28096041

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Select item 28082094 151.

 

  1. Effects of acute oxygen supplementation on functional capacity andheartrate recovery in Eisenmenger syndrome.

Gonzaga LR, Matos-Garcia BC, Rocco IS, Begot I, Bolzan DW, Tatani SB, Santos VB, Silva CM, Carvalho AC, Arena R, Gomes WJ, Guizilini S.

Int J Cardiol. 2017 Mar 15;231:110-114. doi: 10.1016/j.ijcard.2017.01.025. Epub 2017 Jan 6.

PMID:

 

28082094

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  1. Characteristics and outcomes ofheartfailure-related hospitalization in adults with congenital heart disease.

Moussa NB, Karsenty C, Pontnau F, Malekzadeh-Milani S, Boudjemline Y, Legendre A, Bonnet D, Iserin L, Ladouceur M.

Arch Cardiovasc Dis. 2017 Mar 14. pii: S1875-2136(17)30047-5. doi: 10.1016/j.acvd.2017.01.008. [Epub ahead of print]

PMID:

 

28314706

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  1. [Adultcongenital heart disease: Medical and psychosocial issues].

Ladouceur M, Pontnau F, Iserin L.

Presse Med. 2017 Mar 14. pii: S0755-4982(17)30061-1. doi: 10.1016/j.lpm.2017.02.003. [Epub ahead of print] French.

PMID:

 

28314442

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  1. High sensitivity cardiac troponin T and systemic right ventricular function in adults with congenitally corrected transposition of the great arteries.

Kowalik E, Klisiewicz A, Rybicka J, Biernacka EK, Hoffman P.

Int J Cardiol. 2017 Mar 11. pii: S0167-5273(17)30590-9. doi: 10.1016/j.ijcard.2017.03.043. [Epub ahead of print]

PMID:

 

28302319

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  1. At theHeartof the Pregnancy: What Prenatal and Cardiovascular Genetic Counselors Need to Know about Maternal Heart Disease.

Morales A, Allain DC, Arscott P, James E, MacCarrick G, Murray B, Tichnell C, Shikany AR, Spencer S, Fitzgerald-Butt SM, Kushner JD, Munn C, Smith E, Spoonamore KG, Tandri HS, Kay WA.

J Genet Couns. 2017 Mar 10. doi: 10.1007/s10897-017-0081-z. [Epub ahead of print] Review.

PMID:

 

28283918

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  1. Assessment of the systemic right ventricular function in overweight and obese patients with congenitally corrected transposition of the great arteries.

Kowalik E, Klisiewicz A, Biernacka EK, Hoffman P.

Kardiol Pol. 2017 Mar 10. doi: 10.5603/KP.a2017.0036. [Epub ahead of print]

PMID:

 

28281729

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  1. Outcome of adults with Eisenmenger syndrome treated with drugs specific to pulmonary arterial hypertension: A French multicentre study.

Hascoet S, Fournier E, Jaïs X, Le Gloan L, Dauphin C, Houeijeh A, Godart F, Iriart X, Richard A, Radojevic J, Amedro P, Bosser G, Souletie N, Bernard Y, Moceri P, Bouvaist H, Mauran P, Barre E, Basquin A, Karsenty C, Bonnet D, Iserin L, Sitbon O, Petit J, Fadel E, Humbert M, Ladouceur M.

Arch Cardiovasc Dis. 2017 Mar 9. pii: S1875-2136(17)30045-1. doi: 10.1016/j.acvd.2017.01.006. [Epub ahead of print]

PMID:

 

28286190

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Select item 28281093 174.

 

  1. Impact of advanced medical therapy for the outcome of an adult patient with Eisenmenger syndrome.

Ereminienė E, Kinderytė M, Miliauskas S.

Respir Med Case Rep. 2017 Mar 9;21:16-20. doi: 10.1016/j.rmcr.2017.03.002. eCollection 2017.

PMID:

 

28348949

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  1. Investigating Cardiac Motion Patterns Using Synthetic High-Resolution 3D Cardiovascular Magnetic Resonance Images and Statistical Shape Analysis.

Biffi B, Bruse JL, Zuluaga MA, Ntsinjana HN, Taylor AM, Schievano S.

Front Pediatr. 2017 Mar 8;5:34. doi: 10.3389/fped.2017.00034. eCollection 2017.

PMID:

 

28337429

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  1. Physical activity in adults withcongenital heart diseaseand associations with functional outcomes.

Müller J, Amberger T, Berg A, Goeder D, Remmele J, Oberhoffer R, Ewert P, Hager A.

Heart. 2017 Mar 8. pii: heartjnl-2016-310828. doi: 10.1136/heartjnl-2016-310828. [Epub ahead of print]

PMID:

 

28274955

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  1. Cardiovascular causes of maternal sudden death. Sudden arrhythmic death syndrome is leading cause in UK.

Krexi D, Sheppard MN.

Eur J Obstet Gynecol Reprod Biol. 2017 Mar 7;212:155-159. doi: 10.1016/j.ejogrb.2017.03.006. [Epub ahead of print]

PMID:

 

28371698

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  1. Aortopathies in adultcongenital heart diseaseand genetic aortopathy syndromes: management strategies and indications for surgery.

Kuijpers JM, Mulder BJ.

Heart. 2017 Mar 7. pii: heartjnl-2015-308626. doi: 10.1136/heartjnl-2015-308626. [Epub ahead of print] No abstract available.

PMID:

 

28270426

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Select item 28286131 194.

 

  1. A case of aortic arch coarctation, bicuspid aortic valve and aortic sinus aneurysm in an adult with moderate hypertension.

Chrysohoou C, Katsi V, Trikalinos N, Tzifa A, Alexopoulos N, Brili S, Nihoyiannopoulos P, Tousoulis D.

Hellenic J Cardiol. 2017 Mar 7. pii: S1109-9666(17)30033-7. doi: 10.1016/j.hjc.2017.01.016. [Epub ahead of print] No abstract available.

PMID:

 

28286131

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  1. Real-world, long-term survival of incident patients with pulmonary arterial hypertension.

Marques-Alves P, Baptista R, Marinho da Silva A, Pêgo M, Castro G.

Rev Port Pneumol (2006). 2017 Mar 7. pii: S2173-5115(17)30030-1. doi: 10.1016/j.rppnen.2017.01.006. [Epub ahead of print]

PMID:

 

28283462

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Select item 28264208 197.

 

  1. The Cost Differential Between Warfarin Versus Aspirin Treatment After a Fontan Procedure.

Schilling C, Dalziel K, Iyengar AJ, d’Udekem Y.

Heart Lung Circ. 2017 Mar 6. pii: S1443-9506(17)30086-0. doi: 10.1016/j.hlc.2017.02.003. [Epub ahead of print]

PMID:

 

28372885

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  1. Cyanoticcongenital heart diseaseand atherosclerosis.

Tarp JB, Jensen AS, Engstrøm T, Holstein-Rathlou NH, Søndergaard L.

Heart. 2017 Mar 4. pii: heartjnl-2016-311012. doi: 10.1136/heartjnl-2016-311012. [Epub ahead of print] Review.

PMID:

 

28259844

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  1. Mortality Risk Stratification in Fontan Patients Who UnderwentHeartTransplantation.

Berg CJ, Bauer BS, Hageman A, Aboulhosn JA, Reardon LC.

Am J Cardiol. 2017 Mar 1. pii: S0002-9149(17)30191-1. doi: 10.1016/j.amjcard.2017.02.005. [Epub ahead of print]

PMID:

 

28341356

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  1. Aortic assessment of bicuspid aortic valve patients and their first-degree relatives.

Straneo P, Parma G, Lluberas N, Marichal A, Soca G, Cura L, Paganini JJ, Brusich D, Florio L, Dayan V.

Asian Cardiovasc Thorac Ann. 2017 Mar;25(3):192-198. doi: 10.1177/0218492317696375. Epub 2017 Jan 1.

PMID:

 

28325072

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Select item 28303211 231.

 

  1. Real-Time Three-Dimensional Echocardiography: Characterization of Cardiac Anatomy and Function-Current Clinical Applications and Literature Review Update.

Velasco O, Beckett MQ, James AW, Loehr MN, Lewis TG, Hassan T, Janardhanan R.

Biores Open Access. 2017 Mar 1;6(1):15-18. doi: 10.1089/biores.2016.0033. eCollection 2017. Review.

PMID:

 

28303211

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  1. Leukocytoclastic vasculitis associated with endocarditis in a patient with transposition of the great arteries and mechanical valve replacement.

Riehle C, Scharf GM, Sieweke JT, Zauner F, Flierl U, Treptau J, Zormpas C, Senf J, McCarty NS, Bauersachs J, Sedding DG, Westhoff-Bleck M.

Cardiovasc Pathol. 2017 Mar – Apr;27:68-70. doi: 10.1016/j.carpath.2017.01.005. Epub 2017 Jan 24.

PMID:

 

28171828

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Select item 28131074 241.

 

  1. Thrombosis of a mechanical prosthetic aortic valve in early pregnancy: histopathological findings.

Scheidmann R, Foth R, Sigler M.

Cardiovasc Pathol. 2017 Mar – Apr;27:35-36. doi: 10.1016/j.carpath.2016.12.003. Epub 2017 Jan 2.

PMID:

 

28081513

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  1. Bicuspid aortic valve aortopathy: mechanistic and clinical insights from recent studies.

Guzzardi DG, Verma S, Fedak PW.

Curr Opin Cardiol. 2017 Mar;32(2):111-116. doi: 10.1097/HCO.0000000000000359.

PMID:

 

28182589

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  1. Frontiers of valvularheartdisease: from aortic stenosis to the tricuspid valve and congenital anomalies.

Lüscher TF.

Eur Heart J. 2017 Mar 1;38(9):611-614. doi: 10.1093/eurheartj/ehx083. No abstract available.

PMID:

 

28363226

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Select item 26491108 260.

 

  1. Recommendations on the echocardiographic assessment of aortic valve stenosis: a focused update from the European Association of Cardiovascular Imaging and the American Society of Echocardiography.

Baumgartner H Chair, Hung J Co-Chair, Bermejo J, Chambers JB, Edvardsen T, Goldstein S, Lancellotti P, LeFevre M, Miller F Jr, Otto CM.

Eur Heart J Cardiovasc Imaging. 2017 Mar 1;18(3):254-275. doi: 10.1093/ehjci/jew335.

PMID:

 

28363204

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  1. Evolution of right ventricular size over time after tetralogy of Fallot repair: a longitudinal cardiac magnetic resonance study.

Rutz T, Ghandour F, Meierhofer C, Naumann S, Martinoff S, Lange R, Ewert P, Stern HC, Fratz S.

Eur Heart J Cardiovasc Imaging. 2017 Mar 1;18(3):364-370. doi: 10.1093/ehjci/jew273.

PMID:

 

28363200

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  1. Abnormal Wave Reflections and Left Ventricular Hypertrophy Late After Coarctation of the Aorta Repair.

Quail MA, Short R, Pandya B, Steeden JA, Khushnood A, Taylor AM, Segers P, Muthurangu V.

Hypertension. 2017 Mar;69(3):501-509. doi: 10.1161/HYPERTENSIONAHA.116.08763. Epub 2017 Jan 23.

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28115510

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Select item 28070870 273.

 

  1. Hopelessness among adults withcongenital heart disease: Cause for despair or hope?

Eslami B, Kovacs AH, Moons P, Abbasi K, Jackson JL.

Int J Cardiol. 2017 Mar 1;230:64-69. doi: 10.1016/j.ijcard.2016.12.090. Epub 2016 Dec 21.

PMID:

 

28038812

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Select item 27989580 276.

 

  1. Pregnancy in women with complete transposition of the great arteries following the atrial switch procedure. A study from three of the largest AdultCongenital Heart Diseasecenters in Poland.

Lipczyńska M, Szymański P, Trojnarska O, Tomkiewicz-Pająk L, Pietrzak B, Klisiewicz A, Kumor M, Podolec P, Hoffman P.

J Matern Fetal Neonatal Med. 2017 Mar;30(5):563-567. doi: 10.1080/14767058.2016.1177821. Epub 2016 May 5.

PMID:

 

27072884

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  1. Wish to conceive and concerns to develop cardiovascular complications during pregnancy in patients with Turner syndrome.

van Hagen IM, Duijnhouwer AL, Ten Kate-Booij MJ, Dykgraaf RH, Duvekot JJ, Utens EM, Roos-Hesselink JW.

J Psychosom Obstet Gynaecol. 2017 Mar;38(1):45-52. doi: 10.1080/0167482X.2016.1216961. Epub 2016 Sep 1.

PMID:

 

27584042

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  1. Liver health in adults with Fontan circulation: A multicenter cross-sectional study.

Wu FM, Kogon B, Earing MG, Aboulhosn JA, Broberg CS, John AS, Harmon A, Sainani NI, Hill AJ, Odze RD, Johncilla ME, Ukomadu C, Gauvreau K, Valente AM, Landzberg MJ; Alliance for Adult Research in Congenital Cardiology (AARCC) Investigators..

J Thorac Cardiovasc Surg. 2017 Mar;153(3):656-664. doi: 10.1016/j.jtcvs.2016.10.060. Epub 2016 Nov 12.

PMID:

 

27955914

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Select item 28221263 297.

 

  1. Measurement of Aortic Valve Coaptation and Effective Height Using Echocardiography in Patients with Ventricular Septal Defects and Aortic Valve Prolapse.

Iwashima S, Uchiyama H, Ishikawa T, Takigiku K, Takahashi K, Toyono M, Inoue N, Nii M.

Pediatr Cardiol. 2017 Mar;38(3):608-616. doi: 10.1007/s00246-016-1555-8. Epub 2017 Jan 21.

PMID:

 

28108755

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Select item 28078382 308.

 

  1. Timing and Mode of Delivery in Prenatally DiagnosedCongenital Heart Disease– an Analysis of Practices within the University of California Fetal Consortium (UCfC).

Peyvandi S, Nguyen TA, Almeida-Jones M, Boe N, Rhee L, Anton T, Sklansky M, Tarsa M, Satou G, Moon-Grady AJ; University of California Fetal Consortium (UCfC)..

Pediatr Cardiol. 2017 Mar;38(3):588-595. doi: 10.1007/s00246-016-1552-y. Epub 2017 Jan 11.

PMID:

 

28078382

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Select item 28058479 309.

 

  1. Intra- and inter-reader reproducibility of blood flow measurements on the ascending aorta and pulmonary artery using cardiac magnetic resonance.

Di Leo G, D’Angelo ID, Alì M, Cannaò PM, Mauri G, Secchi F, Sardanelli F.

Radiol Med. 2017 Mar;122(3):179-185. doi: 10.1007/s11547-016-0706-6. Epub 2016 Nov 22.

PMID:

 

27878504

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  1. AdultCongenitalCardiac Care.

Kogon BE, Miller K, Miller P, Alsoufi B, Rosenblum JM.

World J Pediatr Congenit Heart Surg. 2017 Mar;8(2):242-247. doi: 10.1177/2150135117690126.

PMID:

 

28329454

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Select item 28329450 334.

 

  1. A novel FBN1 mutation in a family with inherited Marfan Syndrome: p.Cys2672Arg.

Cetinkaya A, Karaman A, Mutlu MB, Yavuz T.

Congenit Anom (Kyoto). 2017 Mar 20. doi: 10.1111/cga.12220. [Epub ahead of print] No abstract available.

PMID:

 

28321935

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Select item 28316282 114.

 

  1. The importance of the neutrophil-to-lymphocyte ratio in patients with hypertrophic cardiomyopathy.

Ozyilmaz S, Akgul O, Uyarel H, Pusuroglu H, Gul M, Satilmisoglu MH, Bolat I, Ozyilmaz I, Uçar H, Yildirim A, Bakir I.

Rev Port Cardiol. 2017 Mar 17. pii: S0870-2551(17)30126-9. doi: 10.1016/j.repc.2016.09.014. [Epub ahead of print] English, Portuguese.

PMID:

 

28318851

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Select item 28302079 129.

 

  1. Contraceptive Practices of Women With Complex Congenital Heart Disease.

Miner PD, Canobbio MM, Pearson DD, Schlater M, Balon Y, Junge KJ, Bhatt A, Barber D, Nickolaus MJ, Kovacs AH, Moons P, Shaw K, Fernandes SM.

Am J Cardiol. 2017 Mar 15;119(6):911-915. doi: 10.1016/j.amjcard.2016.11.047. Epub 2016 Dec 18.

PMID:

 

28087052

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Select item 28318514 137.

 

  1. Hypertrophic Cardiomyopathy in Youth Athletes: Successful Screening With Point-of-Care Ultrasound by Medical Students.

Fox JC, Lahham S, Maldonado G, Klaus S, Aish B, Sylwanowicz LV, Yanuck J, Wilson SP, Shieh M, Anderson CL, English C, Mayer R, Mohan UR.

J Ultrasound Med. 2017 Mar 4. doi: 10.7863/ultra.16.06044. [Epub ahead of print]

PMID:

 

28258593

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Select item 28285118 198.

 

  1. International criteria for electrocardiographic interpretation in athletes.

Drezner JA, Sharma S, Baggish A, Papadakis M, Wilson MG, Prutkin JM, Gerche A, Ackerman MJ, Borjesson M, Salerno JC, Asif IM, Owens DS, Chung EH, Emery MS, Froelicher VF, Heidbuchel H, Adamuz C, Asplund CA, Cohen G, Harmon KG, Marek JC, Molossi S, Niebauer J, Pelto HF, Perez MV, Riding NR, Saarel T, Schmied CM, Shipon DM, Stein R, Vetter VL, Pelliccia A, Corrado D.

Br J Sports Med. 2017 Mar 3. pii: bjsports-2016-097331. doi: 10.1136/bjsports-2016-097331. [Epub ahead of print]

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28258178

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Select item 28323976 200

  1. Chronobiology of Acute Aortic Dissection in the Marfan Syndrome (from the National Registry of Genetically Triggered Thoracic Aortic Aneurysms and Cardiovascular Conditions and the International Registry of Acute Aortic Dissection).

Siddiqi HK, Luminais SN, Montgomery D, Bossone E, Dietz H, Evangelista A, Isselbacher E, LeMaire S, Manfredini R, Milewicz D, Nienaber CA, Roman M, Sechtem U, Silberbach M, Eagle KA, Pyeritz RE; GenTAC and IRAD investigators..

Am J Cardiol. 2017 Mar 1;119(5):785-789. doi: 10.1016/j.amjcard.2016.11.021. Epub 2016 Dec 2.

PMID:

 

28065489

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Select item 27649290 211.

 

  1. Current attitudes and clinical practice towards the care of pregnant women with underlying CHD: a paediatric cardiologyperspective.

Cribbs MG, Briston DA, Zaidi AN.

Cardiol Young. 2017 Mar;27(2):236-242. doi: 10.1017/S104795111600038X. Epub 2016 Apr 11.

PMID:

 

27064196

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Select item 28252675 231.

 

  1. Thrombosis of a mechanical prosthetic aortic valve in early pregnancy: histopathological findings.

Scheidmann R, Foth R, Sigler M.

Cardiovasc Pathol. 2017 Mar – Apr;27:35-36. doi: 10.1016/j.carpath.2016.12.003. Epub 2017 Jan 2.

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28081513

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Select item 27888578 233.

 

  1. Visualization of perimembranous ventricular septal defect with ruptured sinus of Valsalva aneurysm by three-dimensional transesophageal echocardiography and multidetector three-dimensional computed tomography.

Mahara K, Saito M, Fukumoto R, Tamura H, Kin H, Takanashi S.

J Echocardiogr. 2017 Mar;15(1):37-38. doi: 10.1007/s12574-016-0299-z. Epub 2016 Jun 3. No abstract available.

PMID:

 

27260005

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  1. Bicuspid aortic valve and the specialty clinic: are your patients at risk?

Fedak PW.

Cardiol Young. 2017 Mar 6:1-2. doi: 10.1017/S1047951117000282. [Epub ahead of print] No abstract available.

PMID:

 

28260551

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Select item 28260550 25.

 

  1. Hepatocellular carcinoma in the adult Fontan patient.

Conroy MR, Moe TG.

Cardiol Young. 2017 Mar;27(2):407-409. doi: 10.1017/S1047951116001360. Epub 2016 Sep 19.

PMID:

 

27640301

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