Congenital EP Featured Articles of April 2017

 

Pediatric Cardiac and EP Reviews of April 2017 Manuscripts

 

Pacemaker and Defibrillator Implantation in Patients with Transposition of the Great Arteries

Grubb AF, Shah G, Aziz PF, Krasuski RA

The Journal of Innovations in Cardiac Rhythm Management, 8 (2017), 2658-2664

 

Take Home Points:

 

  • Cardiac implantable device implantation in TGA substrates presents unique challenges both at implant and throughout follow-up.
  • This study does not apply to arterial switch patients.
  • Despite differences in indications for implantable devices and age at device implant between d- and l-TGA patients, both TGA groups unfortunately appear to have similar progressions in the development of heart failure with its associated morbidity and mortality.
  • Utilization of current published guidelines for primary prevention ICD implantation has shortcomings when applied to TGA substrates and would benefit from updated evidence and experience with ICDs in TGA patients to guide and individualize this treatment option in this unique patient population.

 

chang-philip-1780821827Commentary from Dr. Philip Chang (Los Angeles), section editor of Congenital Heart Surgery Journal Watch: Article summary:

Grubb et al presented a retrospective review of their single-center experience with cardiac implantable electronic devices (CIEDs) in d- and l-TGA patients with systemic RVs.  The study looked at all TGA patients with CIEDs cared for at their institution over an 18-year period.  All patients had systemic right ventricles and all patients had undergone biventricular repair approaches, with single-ventricle variants and those with repairs to restore systemic morphologic left ventricular circulation excluded from analysis.  In total, 63 patients were identified (34 d-TGA, 29 l-TGA).  The authors performed detailed chart review for each subject to determine initial device implant timing/age, development of heart failure, and variables associated with ICD follow-up including defibrillation thresholds and shocks.

 

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EP4

 

Study limitations include single-center inclusivity and retrospective design.  The authors did not provide more in-depth detail pertaining to implant approaches, CIED pocket site (left vs. right chest for transvenous devices, which could have implications in ICD defibrillation function), or CIED-related complications.   Several patients reportedly had CRT devices, but no detail was provided in terms of the indications for CRT or implant approach.

 

Reviewer perspective and thoughts for pediatric/CHD EP:

While it is commonly recognized and even expected that TGA patients will require CIED implantation, primarily for pacing indications, it has been quite some time since the topic of CIED utilization and benefit in TGA patients has been studied.  As such, the authors are commended for reviewing their data and presenting their experience, which is quite valuable for our community.  The study definitely highlights the importance of considering multi-institutional pooling of data and experience.  Furthermore, while the growth of the atrial switched d-TGA population is diminishing, there is still a considerable population of these patients, along with a growing number of adult l-TGA patients that should motivate greater awareness of the use of CIEDs in these very unique ACHD subgroups.

There is little debate in the indications for and benefits of pacing in TGA substrates and the authors’ findings of more predominant pacing for sinus node disease in d-TGA and AV block in l-TGA isn’t novel.  It remains unclear as to the role of CRT for wide QRS, heart block, or dyssynchrony in TGA, and more specifically its feasibility and effectiveness in primary systemic RV resynchronization.  With the advent of and interest in His-bundle pacing, the feasibility of this method of pacing in d- vs. l-TGA would be interesting to explore in terms of technical considerations and long-term benefit.  The finding that no primary prevention ICD patients received appropriate shocks was interesting, but not entirely surprising.  This certainly raises the question of the appropriateness of applying standard guidelines for ICD implantation to the TGA population and while some published data exists for risk stratification in d-TGA, greater evidence is needed to guide ICD therapy, particularly for primary prevention indications, in these patients.   Finally, with the subcutaneous ICD as a contemporary implant option, it will also be interesting to see how the balance between implant indication and “ease” of device implant will change.

 

It is sobering to see that both d- and l-TGA patients with systemic RVs progress in very similar fashions to develop heart failure and its associated clinical sequelae and mortality.  This shared finding between d- and l-TGA patients highlights the unique and highly complex substrate in which CIEDs are being applied, as well as the multifactorial process involved in the near-universal fate of the systemic RV in these TGA patients.

 

 

 

Lone Pediatric Atrial Fibrillation in the United States: Analysis of Over 1500 Cases

El-Assaad I, Al-Kindi SG, Saarel EV, Aziz PF

Pediatr Cardiol April 2017 (DOI: 10.1007/s00246-017-1608-7)

 

Take home points:

  • Lone atrial fibrillation is a rare arrhythmia condition among pediatric patients.
  • Increasing age and obesity appear associated with higher incidence of lone atrial fibrillation though evidence indicating direct causality is lacking.
  • While complications such as thromboembolic events are rare in pediatric lone atrial fibrillation, their incidence is not ignorable. However, treatment recommendations and practice patterns are inconsistent.

 

Commentary from Dr. Philip Chang (Los Angeles, CA) section editor of Congenital Heart Surgery Journal WatchThere has been much recent interest in atrial fibrillation in pediatric and young adult patients, particularly as it pertains to risk factors for its development as well as treatment in patients with significant or symptomatic recurrences and in the setting of CHD.  Despite this interest, studies with large sample sizes are lacking in order to better understand its incidence, clinical sequelae, treatment options and response, and long-term outcomes, particularly in otherwise healthy individuals.  The current study from El-Assaad et al provides a descriptive analysis of the largest cohort of young patients with lone atrial fibrillation in the United States.

The authors sought to evaluate risk factors and short-term outcomes of pediatric lone atrial fibrillation, simply defined as atrial fibrillation occurring in the absence of cardiac and systemic diseases.  They utilized a privately managed national healthcare database, Explorys, from which they were able to query and determine an incidence of pediatric lone atrial fibrillation, other variables that may be associated with its diagnosis, and clinical sequelae over a 17-year period (1999-2016).  The database was created from de-identified data provided by 360 hospitals in all 50 US states and over 300,000 providers.

Results of querying the Explorys database yielded nearly 8 million children, with 1910 children linked to an atrial fibrillation diagnosis.  Of these patients, 1750 children met the definition of lone atrial fibrillation.  This resulted in a calculated pediatric lone AF incidence of 7.5 in 100,000 persons at risk.  Nearly 10% of these patients also had concomitant diagnosis of SVT but further characterization of this could not be determined from the dataset variables.  The authors found relatively low percentages of antiarrhythmic use (5%), aspirin or warfarin for anticoagulation (7% and 5%, respectively), and electrical cardioversion (3%).  Older patients tended to be prescribed aspirin or warfarin more frequently compared to younger aged cohorts.

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Multivariate analysis showed increasing age, male gender, and obesity to be associated with risk of lone AF.  Increasing age was also associated with increased risk of AF recurrence within 1 month of initial episode.  Interestingly, among patients 15-19 years of age, nearly 20% experienced AF recurrence.  The authors also found that 2% of patients experienced a stroke within 1 year of lone AF diagnosis.  Following exclusion of other concomitant diagnoses including sepsis, hypertension, hypertensive crisis, drug abuse, stimulant use, cancer, renal impairment, sleep apena, respiratory failure, and bone marrow transplant, a total of 1580 patient remained.  Among these patients, a male predominance was noted (61%), and most episodes of lone AF occurred in the 15-19 years age group (58%).  Those in the oldest age cohort had recurrence rates spanning 15-22% from 1 month up to 1 year after the initial event.  In this lone AF subset, 1% of patients had a stroke within 1 year of lone AF diagnosis.

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EP7

Study limitations include limited available variables in the database and inability to control for the quality and accuracy of the data provided.  For example, the authors noted that they were unable to review ECGs to confirm AF diagnosis or echocardiograms to evaluate chamber size and that some patients could have been misclassified as having AF in the first place.  Recurrence rates could have been overestimated if a single AF diagnosis reappeared on more than 1 occasion.  They also noted that the dataset itself has not been directly validated for pediatric studies, though adult studies have demonstrated its validity.  It is unclear as to why nearly 10% of the originally classified “lone AF” patients were excluded given other systemic diseases to come to the subgroup of 1580 patients that were further analyzed for lone AF incidence and recurrence.  By the authors’ definition, these patients would not have met a pure lone AF diagnosis given these concomitant conditions, many of which can certainly increase the risk of arrhythmias (AF being one of them).  If the subgroup patients were used to calculate incidence, the lone AF incidence would be even lower.  Finally, the authors did not have further details relating to concomitant SVT in the 10% of patients identified and there was no long-term data regarding treatment efficacy (antiarrhythmics or catheter ablation), prognosis, and outcomes.

 

Reviewer perspective and thoughts for pediatric/CHD EP:

This study is an excellent example of the use of large scale databases to evaluate otherwise rare conditions.  As such, it provides a faster, and perhaps more accurate, assessment of pediatric lone AF incidence, as well as general approaches to management, complications, and recurrence.  The study is obviously limited by the quality and accuracy of data entered into the database itself.

This study’s finding of the association between lone AF and obesity, male gender, and increasing age is helpful and consistent with previous publications noting these potential associations that had substantially smaller patient cohorts.  The association with obesity is likely multifactorial.  Obesity is frequently associated with other cardiovascular conditions including hypertension, diastolic dysfunction, and left atrial distension and pressure loading, though interestingly, the association between obesity and lone AF was apparently made in the study after excluding for these other systemic diseases.  It is possible that these other systemic diseases were under-reported in the dataset and echo data was not available to assess for evidence of diastolic dysfunction or left atrial abnormalities.  Obesity is also frequently associated with obstructive sleep apnea, which is increasingly recognized as having a strong association with arrhythmia risk including AF (though again, the study largely excluded patients with concomitant diagnoses of AF and sleep apnea).  The authors noted familial lone AF as a possible important contributor, and the genetics of AF remains an important area of ongoing research.

Several important findings from this study that are important for the pediatric EP community to be aware of is the low incidence of pediatric lone AF that is found and which is likely more reflective of its true incidence in the general pediatric population.  Furthermore, based on the study’s results, there appears to be a fairly high recurrence rate (up to 20% in the oldest age cohort) as well as a low but very concerning incidence of stroke.  Both of these appeared to be present in the setting of low utilization of antiarrhythmic agents and anticoagulation.  In fact, the stroke incidence that was noted in this study corresponded to an equivalent CHADS2VASc score that would qualify patients to receive anticoagulation with either warfarin or NOACs.  This study’s findings show that pediatric lone AF may not be as benign of a condition as some may think and that these patients require aggressive and close follow-up to address or prevent recurrences and to reduce AF-related complications.

CHD EP April 2017

 

  1. Atrial undersensing secondary to quiet timer blanking in pediatric and congenital heart disease patients.

von Alvensleben JC, Schaffer M, Brateng C, Collins KK.

Pacing Clin Electrophysiol. 2017 Apr 24. doi: 10.1111/pace.13101. [Epub ahead of print]

PMID: 28436549

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  1. Percutaneous Ventricular Assist Device for Circulatory Support During Ablation of Atrial Tachycardias in Patients With Fontan Circulation.

Hendriks A, De Vries L, Witsenburg M, Yap SC, Van Mieghem N, Szili-Torok T.

Rev Esp Cardiol (Engl Ed). 2017 Apr 18. pii: S1885-5857(17)30170-6. doi: 10.1016/j.rec.2017.03.010. [Epub ahead of print] English, Spanish. No abstract available.

PMID: 28431884

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  1. The electrical heart axis and ST events in fetal monitoring: A post-hoc analysis following a multicentre randomised controlled trial.

Vullings R, Verdurmen KMJ, Hulsenboom ADJ, Scheffer S, de Lau H, Kwee A, Wijn PFF, Amer-Wåhlin I, van Laar JOEH, Oei SG.

PLoS One. 2017 Apr 14;12(4):e0175823. doi: 10.1371/journal.pone.0175823. eCollection 2017.

PMID: 28410419 Free PMC Article

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  1. Sudden cardiac death in adults with congenital heart disease: does QRS-complex fragmentation discriminate in structurally abnormal hearts?

Vehmeijer JT, Koyak Z, Bokma JP, Budts W, Harris L, Mulder BJ, de Groot JR.

Europace. 2017 Apr 10. doi: 10.1093/europace/eux044. [Epub ahead of print]

PMID: 28402450

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  1. Post mortem therapy from a subcutaneous ICD: What is the mechanism?

Wiles BM, Fitzsimmons SJ, Roberts PR.

Pacing Clin Electrophysiol. 2017 Apr 4. doi: 10.1111/pace.13089. [Epub ahead of print] No abstract available.

PMID: 28374449

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  1. Lone Pediatric Atrial Fibrillation in the United States: Analysis of Over 1500 Cases.

El-Assaad I, Al-Kindi SG, Saarel EV, Aziz PF.

Pediatr Cardiol. 2017 Apr 3. doi: 10.1007/s00246-017-1608-7. [Epub ahead of print]

PMID: 28374048

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  1. [Clinical features and outcomes of radiofrequency catheter ablation of atrial flutter in children].

Jiang H, Li XM, Zhang Y, Liu HJ, Li MT, Ge HY.

Zhonghua Er Ke Za Zhi. 2017 Apr 2;55(4):267-271. doi: 10.3760/cma.j.issn.0578-1310.2017.04.007. Chinese.

PMID: 28441822

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  1. [Brief interpretation of “the Pediatric and Congenital Electrophysiology Society (PACES)and the Heart Rhythm Society (HRS) expert consensus statement on the use of catheter ablation in children and patients with congenital heart disease].

Wu JJ, Li F.

Zhonghua Er Ke Za Zhi. 2017 Apr 2;55(4):256-259. doi: 10.3760/cma.j.issn.0578-1310.2017.04.004. Chinese. No abstract available.

PMID: 28441820

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  1. Non-fluoroscopic cardiac ablation of neonates with CHD.

Bigelow AM, Arnold BS, Padrutt GC, Clark JM.

Cardiol Young. 2017 Apr;27(3):592-596. doi: 10.1017/S1047951116001554. Epub 2016 Oct 21.

PMID: 27766996

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  1. What Is the Best Age for Diagnostic Prediction of Pediatric Long-QT Syndrome With a Borderline QT Interval?

Miyazaki A, Doi H.

Circ Arrhythm Electrophysiol. 2017 Apr;10(4). pii: e005119. doi: 10.1161/CIRCEP.117.005119. No abstract available.

PMID: 28356308

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  1. Left ventricular dysfunction is associated with frequent premature ventricular complexes and asymptomatic ventricular tachycardia in children.

Bertels RA, Harteveld LM, Filippini LH, Clur SA, Blom NA.

Europace. 2017 Apr 1;19(4):617-621. doi: 10.1093/europace/euw075.

PMID: 28431063

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  1. Outcomes of lead extraction in young adults.

El-Chami MF, Sayegh MN, Patel A, El-Khalil J, Desai Y, Leon AR, Merchant FM.

Heart Rhythm. 2017 Apr;14(4):537-540. doi: 10.1016/j.hrthm.2017.01.030. Epub 2017 Feb 16.

PMID: 28189822

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  1. Avoiding sports-related sudden cardiac death in children with congenital channelopathy : Recommendations for sports activities.

Lang CN, Steinfurt J, Odening KE.

Herz. 2017 Apr;42(2):162-170. doi: 10.1007/s00059-017-4549-2.

PMID: 28233036

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  1. Corrigendum to ‘Ventricular tachyarrhythmia during pregnancy in women with heart disease: Data from the ROPAC, a registry from the European Society of Cardiology’ [Int. J. Cardiol. 220 (2016) 131-136].

Ertekin E, van Hagen IM, Salam AM, Ruys TP, Johnson MR, Popelova J, Parsonage WA, Ashour Z, Shotan A, Oliver JM, Veldtman GR, Hall R, Roos-Hesselink JW.

Int J Cardiol. 2017 Apr 1;232:348. doi: 10.1016/j.ijcard.2017.01.077. Epub 2017 Jan 27. No abstract available.

PMID: 28139301 Free Article

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  1. Utility and safety of the SafeSept™ transseptal guidewire for electrophysiology studies with catheter ablation in pediatric and congenital heart disease.

Knadler JJ, Anderson JB, Chaouki AS, Czosek RJ, Connor C, Knilans TK, Spar DS.

J Interv Card Electrophysiol. 2017 Apr;48(3):369-374. doi: 10.1007/s10840-017-0224-z. Epub 2017 Jan 14.

PMID: 28091832

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  1. Evaluation of Prolonged QT Interval: Structural Heart Disease Mimicking Long QT Syndrome.

Weissler-Snir A, Gollob MH, Chauhan V, Care M, Spears DA.

Pacing Clin Electrophysiol. 2017 Apr;40(4):417-424. doi: 10.1111/pace.13040. Epub 2017 Mar 16.

PMID: 28155223

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  1. Arrhythmias in Adults with Congenital Heart Disease: What Are Risk Factors for Specific Arrhythmias?

Loomba RS, Buelow MW, Aggarwal S, Arora RR, Kovach J, Ginde S.

Pacing Clin Electrophysiol. 2017 Apr;40(4):353-361. doi: 10.1111/pace.12983. Epub 2017 Feb 27.

PMID: 27987225

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  1. Clinical Application of the QRS-T Angle for the Prediction of Ventricular Arrhythmias in Patients with the Fontan Palliation.

Tran TV, Cortez D.

Pediatr Cardiol. 2017 Apr 29. doi: 10.1007/s00246-017-1618-5. [Epub ahead of print]

PMID: 28456831

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  1. Fetal cardiac time intervals in healthy pregnancies – an observational study by fetal ECG (Monica Healthcare System).

Wacker-Gussmann A, Plankl C, Sewald M, Schneider KM, Oberhoffer R, Lobmaier SM.

J Perinat Med. 2017 Apr 28. pii: /j/jpme.ahead-of-print/jpm-2017-0003/jpm-2017-0003.xml. doi: 10.1515/jpm-2017-0003. [Epub ahead of print]

PMID: 28453441

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  1. Electrophysiological effects of anthracyclines in adult survivors of pediatric malignancy.

Markman TM, Ruble K, Loeb D, Chen A, Zhang Y, Beasley GS, Thompson WR, Nazarian S.

Pediatr Blood Cancer. 2017 Apr 28. doi: 10.1002/pbc.26556. [Epub ahead of print]

PMID: 28453898

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  1. Prospective Study of Adenosine on Atrioventricular Nodal Conduction in Pediatric and Young Adult Patients After Heart Transplant.

Flyer JN, Zuckerman WA, Richmond ME, Anderson BR, Mendelsberg TG, McAllister JM, Liberman L, Addonizio LJ, Silver ES.

Circulation. 2017 Apr 27. pii: CIRCULATIONAHA.117.028087. doi: 10.1161/CIRCULATIONAHA.117.028087. [Epub ahead of print]

PMID: 28450351

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  1. Mid-Term Follow-up of School-Aged Children With Borderline Long QT Interval.

Miyazaki A, Sakaguchi H, Matsumura Y, Hayama Y, Noritake K, Negishi J, Tsuda E, Miyamoto Y, Aiba T, Shimizu W, Kusano K, Shiraishi I, Ohuchi H.

Circ J. 2017 Apr 25;81(5):726-732. doi: 10.1253/circj.CJ-16-0991. Epub 2017 Feb 18.

PMID: 28216547 Free Article

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  1. Transesophageal and invasive electrophysiologic evaluation in children with Wolff-Parkinson-White pattern.

Koca S, Pac FA, Kavurt AV, Cay S, Mihcioglu A, Aras D, Topaloglu S.

Pacing Clin Electrophysiol. 2017 Apr 24. doi: 10.1111/pace.13100. [Epub ahead of print]

PMID: 28436586

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  1. The role of echocardiography in fetal tachyarrhythmia diagnosis. A burden for the pediatric cardiologist and a review of the literature.

Gozar L, Marginean C, Toganel R, Muntean I.

Med Ultrason. 2017 Apr 22;19(2):232-235. doi: 10.11152/mu-892.

PMID: 28440361 Free Article

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  1. Automated T-wave analysis can differentiate acquired QT prolongation from congenital long QT syndrome.

Sugrue A, Noseworthy PA, Kremen V, Bos JM, Qiang B, Rohatgi RK, Sapir Y, Attia ZI, Brady P, Caraballo PJ, Asirvatham SJ, Friedman PA, Ackerman MJ.

Ann Noninvasive Electrocardiol. 2017 Apr 21. doi: 10.1111/anec.12455. [Epub ahead of print]

PMID: 28429460

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  1. Cardiac Transplantation in Children and Adolescents with Long QT Syndrome.

Kelle AM, Bos JM, Etheridge SP, Cannon BC, Bryant RM, Johnson JN, Ackerman MJ.

Heart Rhythm. 2017 Apr 14. pii: S1547-5271(17)30451-4. doi: 10.1016/j.hrthm.2017.04.023. [Epub ahead of print]

PMID: 28416468

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  1. Lidocaine Attenuation Testing: An In Vivo Investigation of Putative LQT3-Associated Variants in the SCN5A-Encoded Sodium Channel.

Heather N Anderson MD, Bos JM, Kapplinger JD, Meskill JM, Ye D, Ackerman MJ.

Heart Rhythm. 2017 Apr 12. pii: S1547-5271(17)30448-4. doi: 10.1016/j.hrthm.2017.04.020. [Epub ahead of print]

PMID: 28412158

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  1. The KCNH2-IVS9-28A/G mutation causes aberrant isoform expression and hERG trafficking defect in cardiomyocytes derived from patients affected by Long QT Syndrome type 2.

Mura M, Mehta A, Ramachandra CJ, Zappatore R, Pisano F, Ciuffreda MC, Barbaccia V, Crotti L, Schwartz PJ, Shim W, Gnecchi M.

Int J Cardiol. 2017 Apr 12. pii: S0167-5273(17)30298-X. doi: 10.1016/j.ijcard.2017.04.038. [Epub ahead of print]

PMID: 28433559

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  1. Evaluation of QTc in Rett syndrome: Correlation with age, severity, and genotype.

Crosson J, Srivastava S, Bibat GM, Gupta S, Kantipuly A, Smith-Hicks C, Myers SM, Sanyal A, Yenokyan G, Brenner J, Naidu SR.

Am J Med Genet A. 2017 Apr 10. doi: 10.1002/ajmg.a.38191. [Epub ahead of print]

PMID: 28394409

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  1. Noninvasive predictors of perioperative atrial arrhythmias in patients with tetralogy of Fallot undergoing pulmonary valve replacement.

Cortez D, Barham W, Ruckdeschel E, Sharma N, McCanta AC, von Alvensleben J, Sauer WH, Collins KK, Kay J, Patel S, Nguyen DT.

Clin Cardiol. 2017 Apr 10. doi: 10.1002/clc.22707. [Epub ahead of print]

PMID: 28394443 Free Article

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  1. Tachycardia cycle and atrioventricular nodal conduction properties in children with supraventricular tachycardia.

Mills M, Dubin AM, Motonaga KS, Ceresnak SR.

Pacing Clin Electrophysiol. 2017 Apr 6. doi: 10.1111/pace.13083. [Epub ahead of print] No abstract available.

PMID: 28383202

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  1. To the Editors-Risk factors for complications in the implantation of epicardial pacemakers in neonates and infants.

Kean AC, Rodefeld M.

Heart Rhythm. 2017 Apr 5. pii: S1547-5271(17)30314-4. doi: 10.1016/j.hrthm.2017.03.021. [Epub ahead of print] No abstract available.

PMID: 28389305

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  1. Interpreting Incidentally Identified Variants in Genes Associated With Catecholaminergic Polymorphic Ventricular Tachycardia in a Large Cohort of Clinical Whole-Exome Genetic Test Referrals.

Landstrom AP, Dailey-Schwartz AL, Rosenfeld JA, Yang Y, McLean MJ, Miyake CY, Valdes SO, Fan Y, Allen HD, Penny DJ, Kim JJ.

Circ Arrhythm Electrophysiol. 2017 Apr;10(4). pii: e004742. doi: 10.1161/CIRCEP.116.004742.

PMID: 28404607

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  1. Effect of Age and Sex on the QTc Interval in Children and Adolescents With Type 1 and 2 Long-QT Syndrome.

Vink AS, Clur SB, Geskus RB, Blank AC, De Kezel CC, Yoshinaga M, Hofman N, Wilde AA, Blom NA.

Circ Arrhythm Electrophysiol. 2017 Apr;10(4). pii: e004645. doi: 10.1161/CIRCEP.116.004645.

PMID: 28356306

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  1. J-Wave syndromes expert consensus conference report: Emerging concepts and gaps in knowledge.

Antzelevitch C, Yan GX, Ackerman MJ, Borggrefe M, Corrado D, Guo J, Gussak I, Hasdemir C, Horie M, Huikuri H, Ma C, Morita H, Nam GB, Sacher F, Shimizu W, Viskin S, Wilde AAM.

Europace. 2017 Apr 1;19(4):665-694. doi: 10.1093/europace/euw235. No abstract available.

PMID: 28431071

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  1. Corrigendum to ‘Ventricular tachyarrhythmia during pregnancy in women with heart disease: Data from the ROPAC, a registry from the European Society of Cardiology‘ [Int. J. Cardiol. 220 (2016) 131-136].

Ertekin E, van Hagen IM, Salam AM, Ruys TP, Johnson MR, Popelova J, Parsonage WA, Ashour Z, Shotan A, Oliver JM, Veldtman GR, Hall R, Roos-Hesselink JW.

Int J Cardiol. 2017 Apr 1;232:348. doi: 10.1016/j.ijcard.2017.01.077. Epub 2017 Jan 27. No abstract available.

PMID: 28139301 Free Article

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  1. Precision Cardiovascular Medicine: State of Genetic Testing.

Giudicessi JR, Kullo IJ, Ackerman MJ.

Mayo Clin Proc. 2017 Apr;92(4):642-662. doi: 10.1016/j.mayocp.2017.01.015. Review.

PMID: 28385198

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  1. Early Repolarization in Normal Adolescents is Common.

Ahmed H, Czosek RJ, Spar DS, Knilans TK, Anderson JB.

Pediatr Cardiol. 2017 Apr;38(4):864-872. doi: 10.1007/s00246-017-1594-9. Epub 2017 Apr 3.

PMID: 28367598

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  1. Arrhythmias After Fontan Operation with Intra-atrial Lateral Tunnel Versus Extra-cardiac Conduit: A Systematic Review and Meta-analysis.

Li D, Fan Q, Hirata Y, Ono M, An Q.

Pediatr Cardiol. 2017 Apr;38(4):873-880. doi: 10.1007/s00246-017-1595-8. Epub 2017 Mar 7.

PMID: 28271152

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  1. Lateral Atrial Tunnel Fontan Operation Predisposes to the Junctional Rhythm.

Januszewska K, Schuh A, Lehner A, Dalla-Pozza R, Malec E.

Pediatr Cardiol. 2017 Apr;38(4):712-718. doi: 10.1007/s00246-017-1571-3. Epub 2017 Feb 10.

PMID: 28184977

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  1. Effects of Triple Cryoenergy Application on Lesion Formation and Coronary Arteries in the Developing Myocardium.

Krause U, Abreu da Cunha FD, Backhoff D, Jacobshagen C, Klehs S, Schneider HE, Paul T.

Pediatr Cardiol. 2017 Apr;38(4):663-668. doi: 10.1007/s00246-016-1564-7. Epub 2017 Jan 11.

PMID: 28078383

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  1. ECGs in the ED.

Tanel RE.

Pediatr Emerg Care. 2017 Apr;33(4):309-310. doi: 10.1097/PEC.0000000000001149. No abstract available.

PMID: 28353534

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  1. [Our experience in the diagnosis and treatment of postural orthostatic tachycardia syndrome, vasovagal syncope, and inappropriate sinus tachycardia in children].

Ugan Atik S, Dedeoğlu R, Koka A, Öztunç F.

Turk Kardiyol Dern Ars. 2017 Apr;45(3):227-234. doi: 10.5543/tkda.2017.36517. Turkish.

PMID: 28429690 Free Article

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CHD Surgery Featured Articles of April 2017

Congenital Heart Surgery Reviews of April 2017 Manuscripts

 

The effects of postoperative hematocrit on shunt occlusion for neonates undergoing single ventricle palliation.

Anderson BR, Blancha VL, Duchon JM, Chai PJ, Kalfa D, Bacha EA, Krishnamurthy G, Ratner V.

J Thorac Cardiovasc Surg. 2017 Apr;153(4):947-955. doi: 10.1016/j.jtcvs.2016.09.085. Epub 2016 Nov 14.

PMID: 27938905

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Select item 28350727

 

 

Take Home Points:

  • In patients undergoing placement of a systemic-to-pulmonary artery shunt, increased hematocrit levels may be associated with early shunt occlusion but not mortality.
  • The optimal anticoagulation strategy following shunt procedures (e.g., aspirin and/or systemic heparin) remains undetermined.

 

jeremy-herrmannCommentary from Dr. Jeremy Herrmann (Indianapolis), section editor of Congenital Heart Surgery Journal Watch:  Defining an optimal range of hematocrit values following cardiac surgery in pediatric patients remains a challenge, particularly for cyanotic patients undergoing placement of a systemic-to-pulmonary artery shunt. The Columbia group reports results of a retrospective analysis of their experience with these shunts from 2010-2015. Eighty neonates were included: 28 with Norwood with modified BT shunt; 39 with modified BT shunt; and 12 with a central shunt. The first postoperative hematocrit values were analyzed in comparison with shunt occlusion and 30-day mortality. Their standard postoperative anticoagulation regimen included aspirin but no systemic heparin. There were 12 instances of shunt occlusion, 5 within the first 24 hours (“early” shunt occlusions). Notably, none of these patients who developed early shunt occlusion died during the study period. There were 6 deaths in the series, 2 of which occurred after late shunt occlusion on postoperative days 12 and 18. On univariable analysis, the two factors associated with increased risk of early shunt occlusion were the first postoperative hematocrit level and later operative era (after July 1, 2014). Interestingly, the first postoperative hematocrit levels were higher in the later operative era, and for each additional 5 additional percentage points of hematocrit value, the risk of early shunt occlusion more than doubled (odds ratio [OR], 2.70; p=0.009). In terms of mortality, increased inotrope score and first postoperative PaO2 were associated with increased mortality in univariable analysis. Increased postoperative hematocrit was also associated with increased inotrope use and higher postoperative PRBC transfusion volumes were associated with increased risk of mortality, but no association between the first postoperative hematocrit and mortality was seen. The authors conclude that lower postoperative hematocrit values may be better for patients undergoing systemic-to-pulmonary artery shunt procedures, though the ideal range remains undetermined. They describe their practice of not transfusing PRBC for levels above 55%. With these results and those from other previous studies that do not strongly support or refute the routine use of systemic heparinization following shunt procedures, the authors continue to routinely use only aspirin in the postoperative period. The results of the present study are compelling regarding the avoidance of high hematocrit levels in the early postoperative period after shunt procedures. However, the question of how best to manage anticoagulation in these patients remains unanswered and may be beyond what a single institution, retrospective study can determine.

 

 


 

  1. Peritoneal Dialysis vs Furosemide for Prevention of Fluid Overload in Infants After Cardiac Surgery: A Randomized Clinical Trial.

Kwiatkowski DM, Goldstein SL, Cooper DS, Nelson DP, Morales DL, Krawczeski CD.

JAMA Pediatr. 2017 Apr 1;171(4):357-364. doi: 10.1001/jamapediatrics.2016.4538.

PMID: 28241247

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Select item 28385198

 

 

Take Home Points:

  • Peritoneal dialysis for postoperative oliguria in infants undergoing cardiac surgery is safe and may offer improved fluid balances, inotropic requirements, electrolyte levels, and less prolonged duration of mechanical ventilation compared to intravenous furosemide therapy.

 

Commentary from Dr. Jeremy Herrmann (Indianapolis), section editor of Congenital Heart Surgery Journal Watch:  Fluid management of neonates following cardiac surgery can be challenging, and fluid overload can be difficult to avoid. In this study, the Cincinnati group conducted a prospective, randomized study comparing the use of intravenous furosemide or peritoneal dialysis (PD) for neonates with postoperative oliguria (urine output <1 ml/kg/hr over a 4-hour period in the first 24 hours). PD catheter placement was planned preoperatively for high-risk patients (age <3 mo undergoing cardiopulmonary bypass, age <4 mo undergoing tetralogy of Fallot or double outlet right ventricle repair, or age <6 mo undergoing heart transplantation) and selectively for other moderate-risk patients. The surgeon was blinded to initial group assignment. Patients were excluded if they had preexisting kidney disease, did not undergo cardiopulmonary bypass during surgery, did not have a PD catheter placed as planned, died during the study period, or required ECMO support or reoperation. Seventy-three patients were included, 32 patients in the furosemide group and 41 in the PD group. The authors observed no difference in the proportion of patients with a negative fluid balance on by the first postoperative day between groups, the but patients in furosemide group were more likely to develop a 10% fluid overload (odds ratio [OR] 3.0, 95% CI 1.3 to 6.9) and require prolonged intubation of more than 72 hours (OR 3.1, 95% CI 1.2 to 8.2). Patients in the PD group demonstrated better fluid balances on postoperative days 1-3, required less inotropic support, and exhibited fewer electrolyte abnormalities compared to the furosemide group. There were 2 adverse events related to PD catheters involving bloody drainage in one patient and a hydrocele in the other, neither of which required intervention. Pleuro-peritoneal communications were identified in 9 patients in the PD group based on drainage of dialysate from the chest tubes, and these patients did not undergo PD as assigned. No difference in mortality was observed.

 

The authors conclude that PD is safe and offers better clinical performance compared to furosemide therapy in oliguric, postoperative infants based on more favorable inotrope use, electrolyte levels, rate of prolonged intubation, and avoidance of fluid overload. However, they also conclude that their study was underpowered to evaluate other clinical outcomes parameters (e.g., length of ICU stay). Given the prospective, randomized nature of this study, these findings are worthy of consideration. Of further interest would be a cost comparison study of PD and furosemide treatment pathways and related clinical outcomes.

CHD Surgery April 2017

 

  1. Resource Utilization Associated with Extracardiac Co-morbid Conditions Following Congenital Heart Surgery in Infancy.

Tuomela KE, Gordon JB, Cassidy LD, Johaningsmeir S, Ghanayem NS.

Pediatr Cardiol. 2017 Apr 29. doi: 10.1007/s00246-017-1620-y. [Epub ahead of print]

PMID: 28456828

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  1. Lung Transplantation for FLNA-Associated Progressive Lung Disease.

Burrage LC, Guillerman RP, Das S, Singh S, Schady DA, Morris SA, Walkiewicz M, Schecter MG, Heinle JS, Lotze TE, Lalani SR, Mallory GB.

J Pediatr. 2017 Apr 28. pii: S0022-3476(17)30457-2. doi: 10.1016/j.jpeds.2017.03.045. [Epub ahead of print]

PMID: 28457522

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Select item 28457478

 

  1. Mechanical Concepts Applied in Congenital Heart Disease and Cardiac Surgery.

Gerrah R, Haller SJ, George I.

Ann Thorac Surg. 2017 Apr 27. pii: S0003-4975(17)30178-9. doi: 10.1016/j.athoracsur.2017.01.068. [Epub ahead of print] Review.

PMID: 28457478

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Select item 28450553

 

  1. Outcomes of Totally Endoscopic Atrial Septal Defect Closure Using a Glutaraldehyde-Treated Autologous Pericardial Patch.

Nishida H, Nakatsuka D, Kawano Y, Hiraiwa N, Takanashi S, Tabata M.

Circ J. 2017 Apr 25;81(5):689-693. doi: 10.1253/circj.CJ-16-0888. Epub 2017 Feb 8.

PMID: 28179595 Free Article

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Select item 28427582

 

  1. Hospital costs and cost implications of co-morbid conditions for patients with single ventricle in the period through to Fontan completion.

Huang L, Dalziel KM, Schilling C, Celermajer DS, McNeil JJ, Winlaw D, Gentles T, Radford DJ, Cheung M, Bullock A, Wheaton GR, Justo RN, Selbie LA, Forsdick V, Du Plessis K, d’Udekem Y.

Int J Cardiol. 2017 Apr 20. pii: S0167-5273(17)30509-0. doi: 10.1016/j.ijcard.2017.04.056. [Epub ahead of print]

PMID: 28456482

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Select item 28426680

 

  1. Corrigendum to: “Glial fibrillary acidic protein plasma levels are correlated with degree of hypothermia during cardiopulmonary bypass in congenital heart disease surgery” [Interact CardioVasc Thorac Surg 2017]†.

Vedovelli L, Padalino M, D’Aronco S, Stellin G, Ori C, Carnielli VP, Simonato M, Cogo P.

Interact Cardiovasc Thorac Surg. 2017 Apr 17. doi: 10.1093/icvts/ivx072. [Epub ahead of print] No abstract available.

PMID: 28431165

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Select item 28427867

 

  1. New Regenerative Vascular Grafts for Hemodialysis Access: Evaluation of a Preclinical Animal Model.

Valencia Rivero KT, Jaramillo Escobar J, Galvis Forero SD, Miranda Saldaña MC, López Panqueva RDP, Sandoval Reyes NF, Briceño Triana JC.

J Invest Surg. 2017 Apr 17:1-9. doi: 10.1080/08941939.2017.1303100. [Epub ahead of print]

PMID: 28414565

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Select item 28419616

 

  1. The provision of pediatric cardiac anesthesia services in Germany: current status of structural and personnel organization.

Baehner T, Dewald O, Heinze I, Mueller M, Schindler E, Schirmer U, Baumgarten G, Hoeft A, Ellerkmann RK.

Paediatr Anaesth. 2017 Apr 17. doi: 10.1111/pan.13153. [Epub ahead of print]

PMID: 28419616

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Select item 28419229

 

  1. Non-invasive assessment of cerebral oxygen metabolism following surgery of congenital heart disease.

Neunhoeffer F, Sandner K, Wiest M, Haller C, Renk H, Kumpf M, Schlensak C, Hofbeck M.

Interact Cardiovasc Thorac Surg. 2017 Apr 16. doi: 10.1093/icvts/ivx080. [Epub ahead of print]

PMID: 28419229

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Select item 28233535

 

  1. Meta-Analysis of the Effectiveness of Heart Transplantation in Patients With a Failing Fontan.

Tabarsi N, Guan M, Simmonds J, Toma M, Kiess M, Tsang V, Ruygrok P, Konstantinov I, Shi W, Grewal J.

Am J Cardiol. 2017 Apr 15;119(8):1269-1274. doi: 10.1016/j.amjcard.2017.01.001. Epub 2017 Jan 25.

PMID: 28233535

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Select item 28320148

 

  1. Impact of International Quality Improvement Collaborative on Congenital Heart Surgery in Pakistan.

Khan A, Abdullah A, Ahmad H, Rizvi A, Batool S, Jenkins KJ, Gauvreau K, Amanullah M, Haq A, Aslam N, Minai F, Hasan B.

Heart. 2017 Apr 13. pii: heartjnl-2016-310533. doi: 10.1136/heartjnl-2016-310533. [Epub ahead of print]

PMID: 28408415

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Select item 28412186

 

  1. Cardiac surgery in patients with congenital heart disease is associated with acute kidney injury and the risk of chronic kidney disease.

Madsen NL, Goldstein SL, Frøslev T, Christiansen CF, Olsen M.

Kidney Int. 2017 Apr 12. pii: S0085-2538(17)30155-2. doi: 10.1016/j.kint.2017.02.021. [Epub ahead of print]

PMID: 28412020

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Select item 28411047

 

  1. Immunodepression after CPB: Cytokine dynamics and clinics after pediatric cardiac surgery – A prospective trial.

Justus G, Walker C, Rosenthal LM, Berger F, Miera O, Schmitt KRL.

Cytokine. 2017 Apr 11. pii: S1043-4666(17)30085-6. doi: 10.1016/j.cyto.2017.03.017. [Epub ahead of print]

PMID: 28411047

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Select item 28407053

 

  1. Early postoperative physical therapy for improving short-term gross motor outcome in infants with cyanotic and acyanotic congenital heart disease.

Haseba S, Sakakima H, Nakao S, Ohira M, Yanagi S, Imoto Y, Yoshida A, Shimodozono M.

Disabil Rehabil. 2017 Apr 7:1-8. doi: 10.1080/09638288.2017.1309582. [Epub ahead of print]

PMID: 28385091

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Select item 28386957

 

  1. Tetralogy of Fallot Surgical Repair: Shunt Configurations, Ductus Arteriosus and the Circle of Willis.

Piskin S, Unal G, Arnaz A, Sarioglu T, Pekkan K.

Cardiovasc Eng Technol. 2017 Apr 5. doi: 10.1007/s13239-017-0302-5. [Epub ahead of print]

PMID: 28382440

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Select item 28382463

 

  1. Anesthetic Management of a Tracheoesophageal Fistula in a Patient With a Large Uncorrected Aortopulmonary Window: A Case Report.

Adler AC, Steven JM, Jolley MA.

A A Case Rep. 2017 Apr 1;8(7):172-174. doi: 10.1213/XAA.0000000000000460.

PMID: 27941485

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Select item 28029179

 

  1. Transient cortical blindness following intracardiac repair of congenital heart disease in an 11-year-old boy.

Bharati S, Sharma MK, Chattopadhay A, Das D.

Ann Card Anaesth. 2017 Apr-Jun;20(2):256-258. doi: 10.4103/aca.ACA_159_16.

PMID: 28393793 Free Article

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Select item 28393785

 

  1. Modifying risks to improve outcome in cardiac surgery: An anesthesiologist’s perspective.

Chakravarthy M.

Ann Card Anaesth. 2017 Apr-Jun;20(2):226-233. doi: 10.4103/aca.ACA_20_17. Review.

PMID: 28393785 Free Article

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Select item 28393783

 

  1. Coagulopathies in cyanotic cardiac patients: An analysis with three point – of – care testing devices (Thromboelastography, rotational thromboelastometry, and sonoclot analyzer).

Bhardwaj V, Malhotra P, Hasija S, Chowdury UK, Pangasa N.

Ann Card Anaesth. 2017 Apr-Jun;20(2):212-218. doi: 10.4103/aca.ACA_4_17.

PMID: 28393783 Free Article

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Select item 27865477

 

  1. Tracheostomy Among Infants With Hypoplastic Left Heart Syndrome Undergoing Cardiac Operations: A Multicenter Analysis.

Prodhan P, Agarwal A, ElHassan NO, Bolin EH, Beam B, Garcia X, Gaies M, Tang X.

Ann Thorac Surg. 2017 Apr;103(4):1308-1314. doi: 10.1016/j.athoracsur.2016.09.016. Epub 2016 Nov 16.

PMID: 27865477

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Select item 27863728

 

  1. Fontan Patient Survival After Pediatric Heart Transplantation Has Improved in the Current Era.

Simpson KE, Pruitt E, Kirklin JK, Naftel DC, Singh RK, Edens RE, Barnes AP, Canter CE.

Ann Thorac Surg. 2017 Apr;103(4):1315-1320. doi: 10.1016/j.athoracsur.2016.08.110. Epub 2016 Nov 15.

PMID: 27863728

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Select item 28065447

 

  1. Postoperative Infection in Developing World Congenital Heart Surgery Programs: Data From the International Quality Improvement Collaborative.

Sen AC, Morrow DF, Balachandran R, Du X, Gauvreau K, Jagannath BR, Kumar RK, Kupiec JK, Melgar ML, Chau NT, Potter-Bynoe G, Tamariz-Cruz O, Jenkins KJ.

Circ Cardiovasc Qual Outcomes. 2017 Apr;10(4). pii: e002935. doi: 10.1161/CIRCOUTCOMES.116.002935.

PMID: 28408715

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Select item 27356630

 

  1. Comparison of paracorporeal and continuous flow ventricular assist devices in children: preliminary results.

Nassar MS, Hasan A, Chila T, Schueler S, Pergolizzi C, Reinhardt Z, Lord S, De Rita F, Ferguson L, Smith J, Haynes S, O’Sullivan J, Murtuza B.

Eur J Cardiothorac Surg. 2017 Apr 1;51(4):709-714. doi: 10.1093/ejcts/ezx006.

PMID: 28329112

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Select item 28204298

 

  1. Five-year experience with immediate extubation after arterial switch operations for transposition of great arteries.

Varghese J, Kutty S, Bisselou Moukagna KS, Craft M, Abdullah I, Hammel JM.

Eur J Cardiothorac Surg. 2017 Apr 1;51(4):728-734. doi: 10.1093/ejcts/ezw424.

PMID: 28199509

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Select item 28040675

 

  1. The problematic of polytetrafluoroethylene membranes for valvular repair.

Tasoglu I, Avci T, Collaku A, Paç M.

Eur J Cardiothorac Surg. 2017 Apr 1;51(4):805. doi: 10.1093/ejcts/ezw396. No abstract available.

PMID: 28040675

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Select item 28013288

 

  1. Total endoscopic repair of unroofed coronary sinus syndrome via right mini-thoracotomy.

Zaikokuji K, Sawazaki M, Tomari S, Imaeda Y.

Gen Thorac Cardiovasc Surg. 2017 Apr;65(4):206-208. doi: 10.1007/s11748-015-0601-4. Epub 2015 Nov 16.

PMID: 26572766

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Select item 27876287

 

  1. Mid-term Outcomes of Common Congenital Heart Defects Corrected Through a Right Subaxillary Thoracotomy.

An G, Zhang H, Zheng S, Wang W, Ma L.

Heart Lung Circ. 2017 Apr;26(4):376-382. doi: 10.1016/j.hlc.2015.05.028. Epub 2016 Feb 17.

PMID: 27876287

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Select item 28189822

 

  1. Bidirectional cavopulmonary anastomosis with additional pulmonary blood flow: good or bad pre-Fontan strategy†.

Nichay NR, Gorbatykh YN, Kornilov IA, Soynov IA, Ivantsov SM, Gorbatykh AV, Ponomarev DN, Bogachev-Prokophiev AV, Karaskov AM.

Interact Cardiovasc Thorac Surg. 2017 Apr 1;24(4):582-589. doi: 10.1093/icvts/ivw429.

PMID: 28093463

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Select item 28077509

 

  1. The “basic” approach: a single-centre experience with a cost-reducing model for paediatric cardiac extracorporeal membrane oxygenation.

Padalino MA, Tessari C, Guariento A, Frigo AC, Vida VL, Marcolongo A, Zanella F, Harvey MJ, Thiagarajan RR, Stellin G.

Interact Cardiovasc Thorac Surg. 2017 Apr 1;24(4):590-597. doi: 10.1093/icvts/ivw381.

PMID: 28077509

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Select item 28040751

 

  1. Lung ultrasound profile after cardiopulmonary bypass in paediatric cardiac surgery: first experience in a simple cohort†.

Vitale V, Ricci Z, Gaddi S, Testa G, Toma P, Cogo P.

Interact Cardiovasc Thorac Surg. 2017 Apr 1;24(4):598-602. doi: 10.1093/icvts/ivw357.

PMID: 28040749

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Select item 28145049

 

  1. The impact of liver disorders on perioperative management of reoperative cardiac surgery: a retrospective study in adult congenital heart disease patients.

Adachi K, Toyama H, Kaiho Y, Adachi O, Hudeta H, Yamauchi M.

J Anesth. 2017 Apr;31(2):170-177. doi: 10.1007/s00540-017-2308-4. Epub 2017 Jan 16.

PMID: 28091794

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Select item 28092290

 

  1. Preclinical study of patient-specific cell-free nanofiber tissue-engineered vascular grafts using 3-dimensional printing in a sheep model.

Fukunishi T, Best CA, Sugiura T, Opfermann J, Ong CS, Shinoka T, Breuer CK, Krieger A, Johnson J, Hibino N.

J Thorac Cardiovasc Surg. 2017 Apr;153(4):924-932. doi: 10.1016/j.jtcvs.2016.10.066. Epub 2016 Nov 14.

PMID: 27938900

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Select item 27913078

 

  1. Pilot randomized controlled trial on early and late remote ischemic preconditioning prior to complex cardiac surgery in young infants.

Guerra GG, Joffe AR, Seal R, Phillipos E, Wong M, Moez EK, Dinu IA, Duff JP, Ross D, Rebeyka I, Robertson CM.

Paediatr Anaesth. 2017 Apr;27(4):433-441. doi: 10.1111/pan.13125.

PMID: 28300357

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Select item 28239753

 

  1. Increased Atrial β-Adrenergic Receptors and GRK-2 Gene Expression Can Play a Fundamental Role in Heart Failure After Repair of Congenital Heart Disease with Cardiopulmonary Bypass.

Oliveira MS, Carmona F, Vicente WVA, Manso PH, Mata KM, Celes MR, Campos EC, Ramos SG.

Pediatr Cardiol. 2017 Apr;38(4):734-745. doi: 10.1007/s00246-017-1573-1. Epub 2017 Feb 18.

PMID: 28214967

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Select item 28184978

 

  1. Effect of Fenestration on Early Postoperative Outcome in Extracardiac Fontan Patients with Different Risk Levels.

Fan F, Liu Z, Li S, Yi T, Yan J, Yan F, Wang X, Wang Q.

Pediatr Cardiol. 2017 Apr;38(4):643-649. doi: 10.1007/s00246-016-1561-x. Epub 2017 Jan 23.

PMID: 28116475

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  1. Pediatric airway surgery.

Maeda K.

Pediatr Surg Int. 2017 Apr;33(4):435-443. doi: 10.1007/s00383-016-4050-7. Epub 2017 Jan 28. Review.

PMID: 28132084

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Select item 28025781

 

  1. Cardiac Biomarkers of Low Cardiac Output Syndrome in the Postoperative Period After Congenital Heart Disease Surgery in Children.

Pérez-Navero JL, de la Torre-Aguilar MJ, Ibarra de la Rosa I, Gil-Campos M, Gómez-Guzmán E, Merino-Cejas C, Muñoz-Villanueva MC, Llorente-Cantarero FJ.

Rev Esp Cardiol (Engl Ed). 2017 Apr;70(4):267-274. doi: 10.1016/j.rec.2016.09.011. Epub 2016 Oct 27. English, Spanish.

PMID: 28137395

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Select item 27515125

 

  1. Equine Cardiovascular Therapeutics.

Sleeper MM.

Vet Clin North Am Equine Pract. 2017 Apr;33(1):163-179. doi: 10.1016/j.cveq.2016.11.005. Review.

PMID: 28325177

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Select item 28101777

 

  1. [Association between fluid overload and acute renal injury after congenital heart disease surgery in infants].

Luo DQ, Chen ZL, Dai W, Chen F.

Zhongguo Dang Dai Er Ke Za Zhi. 2017 Apr;19(4):376-380. Chinese.

PMID: 28407819 Free Article

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  1. Cardioprotective Effects of Wharton Jelly Derived Mesenchymal Stem Cell Transplantation in a Rodent Model of Myocardial Injury.

Gaafar T, Attia W, Mahmoud S, Sabry D, Aziz OA, Rasheed D, Hamza H.

Int J Stem Cells. 2017 Apr 30. doi: 10.15283/ijsc16063. [Epub ahead of print]

PMID: 28446005 Free Article

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Select item 28456833

 

  1. The Evolution of a Pediatric Ventricular Assist Device Program: The Boston Children’s Hospital Experience.

Hawkins B, Fynn-Thompson F, Daly KP, Corf M, Blume E, Connor J, Porter C, Almond C, VanderPluym C.

Pediatr Cardiol. 2017 Apr 29. doi: 10.1007/s00246-017-1615-8. [Epub ahead of print]

PMID: 28456829

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  1. Three-Dimensional Modeling in Congenital and Structural Heart Perioperative Care and Education: A Path in Evolution.

Sarris GE, Polimenakos AC.

Pediatr Cardiol. 2017 Apr 29. doi: 10.1007/s00246-017-1614-9. [Epub ahead of print] No abstract available.

PMID: 28456827

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Select item 28455909

 

  1. Emergency department utilization in pediatric heart transplant recipients.

Wittlieb-Weber CA, Rossano JW, Weber DR, Lin KY, Ravishankar C, Mascio CE, Shaddy RE, O’Connor MJ.

Pediatr Transplant. 2017 Apr 29. doi: 10.1111/petr.12936. [Epub ahead of print]

PMID: 28455909

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Select item 28455631

 

  1. Impact of Surgery in Ebstein’s Anomaly Using Current Surgical Criteria.

Ávila-Vanzzini N, Fritche-Salazar JF, Herrera-Bello H, Espinola-Zavaleta N, Kuri Alfaro J, Rodriguez-Zanella H, Álvarez Bran MV, Cossio Aranda J, Gaxiola Macias MBA, Curi-Curi P.

Circ J. 2017 Apr 26. doi: 10.1253/circj.CJ-17-0062. [Epub ahead of print]

PMID: 28450668 Free Article

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Select item 28216547

 

  1. Heart Transplantation in a Toddler with Cardiac Kawasaki Disease.

Tirilomis T, Steinmetz M, Grossmann M, Bräuer A, Paul T, Ruschewski W, Schöndube FA.

Front Surg. 2017 Apr 20;4:21. doi: 10.3389/fsurg.2017.00021. eCollection 2017.

PMID: 28473974

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Select item 28438441

 

  1. Immunodepression after CPB: Cytokine dynamics and clinics after pediatric cardiac surgery – A prospective trial.

Justus G, Walker C, Rosenthal LM, Berger F, Miera O, Schmitt KRL.

Cytokine. 2017 Apr 11. pii: S1043-4666(17)30085-6. doi: 10.1016/j.cyto.2017.03.017. [Epub ahead of print]

PMID: 28411047

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Select item 28402400

 

  1. Unplanned cardiac reoperations and interventions during long-term follow-up after the Norwood procedure†.

Sames-Dolzer E, Gierlinger G, Kreuzer M, Schrempf J, Gitter R, Prandstetter C, Tulzer G, Mair R.

Eur J Cardiothorac Surg. 2017 Apr 11. doi: 10.1093/ejcts/ezx038. [Epub ahead of print]

PMID: 28402400

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Select item 28457561

 

  1. Complicated Postoperative Course after Pulmonary Artery Sling Repair and Slide Tracheoplasty.

Weber A, Donner B, Perez MH, Di Bernardo S, Trachsel D, Sandu K, Sekarski N.

Front Pediatr. 2017 Apr 10;5:67. doi: 10.3389/fped.2017.00067. eCollection 2017.

PMID: 28443268 Free PMC Article

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Select item 28394184

 

  1. Neonatal repair of left atrial diverticulum with gigantic thrombus without cardiopulmonary bypass.

Higashida A, Hoashi T, Sakaguchi H, Ichikawa H.

Gen Thorac Cardiovasc Surg. 2017 Apr 8. doi: 10.1007/s11748-017-0775-z. [Epub ahead of print]

PMID: 28391520

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Select item 28389745

 

  1. Cancer recurrence and mortality after pediatric heart transplantation for anthracycline cardiomyopathy: A report from the Pediatric Heart Transplant Study (PHTS) group.

Bock MJ, Pahl E, Rusconi PG, Boyle GJ, Parent JJ, Twist CJ, Kirklin JK, Pruitt E, Bernstein D.

Pediatr Transplant. 2017 Apr 4. doi: 10.1111/petr.12923. [Epub ahead of print]

PMID: 28378408

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Select item 28367761

 

  1. Twin-to-Twin Heart Transplantation: A Unique Event With a 25-Year Follow-Up.

Blitzer D, Yedlicka G, Manghelli J, Dentel J, Caldwell R, Brown JW.

Ann Thorac Surg. 2017 Apr;103(4):e341-e342. doi: 10.1016/j.athoracsur.2016.09.060.

PMID: 28359493

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Select item 28274521

 

  1. Cavopulmonary Anastomosis During Same Hospitalization as Stage 1 Norwood/Hybrid Palliative Surgery.

Tadphale SD, Tang X, ElHassan NO, Beam B, Prodhan P.

Ann Thorac Surg. 2017 Apr;103(4):1285-1291. doi: 10.1016/j.athoracsur.2017.01.013. Epub 2017 Mar 6.

PMID: 28274521

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Select item 27865477

 

  1. Allograft Heart Valves: Current Aspects and Future Applications.

Lisy M, Kalender G, Schenke-Layland K, Brockbank KG, Biermann A, Stock UA.

Biopreserv Biobank. 2017 Apr;15(2):148-157. doi: 10.1089/bio.2016.0070. Epub 2017 Feb 2. Review.

PMID: 28151005

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Select item 28067869

 

  1. Dissecting aneurysm of the interventricular septum due to rupture of the sinus of Valsalva.

Atiq M, Ali F, Hasan SB.

Cardiol Young. 2017 Apr;27(3):609-612. doi: 10.1017/S1047951116001621. Epub 2016 Nov 22.

PMID: 27873570

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  1. Successful replacement of the systemic tricuspid valve with a mechanical valve in a 3-month-old boy with congenitally corrected transposition of the great arteries having a dysplastic tricuspid valve.

Asada D, Ikeda K, Yamagishi M.

Cardiol Young. 2017 Apr;27(3):597-599. doi: 10.1017/S1047951116001578. Epub 2016 Sep 29.

PMID: 27680744

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  1. Impact of age on incidence and prevalence of moderate-to-severe cellular rejection detected by routine surveillance biopsy in pediatric heart transplantation.

Zinn MD, Wallendorf MJ, Simpson KE, Osborne AD, Kirklin JK, Canter CE.

J Heart Lung Transplant. 2017 Apr;36(4):451-456. doi: 10.1016/j.healun.2016.09.017. Epub 2016 Oct 7.

PMID: 27865735

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  1. Vascular endothelial growth factor A is associated with the subsequent development of moderate or severe cardiac allograft vasculopathy in pediatric heart transplant recipients.

Daly KP, Stack M, Eisenga MF, Keane JF, Zurakowski D, Blume ED, Briscoe DM.

J Heart Lung Transplant. 2017 Apr;36(4):434-442. doi: 10.1016/j.healun.2016.09.013. Epub 2016 Oct 3.

PMID: 27865734

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  1. The effects of postoperative hematocrit on shunt occlusion for neonates undergoing single ventricle palliation.

Anderson BR, Blancha VL, Duchon JM, Chai PJ, Kalfa D, Bacha EA, Krishnamurthy G, Ratner V.

J Thorac Cardiovasc Surg. 2017 Apr;153(4):947-955. doi: 10.1016/j.jtcvs.2016.09.085. Epub 2016 Nov 14.

PMID: 27938905

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  1. Peritoneal Dialysis vs Furosemide for Prevention of Fluid Overload in Infants After Cardiac Surgery: A Randomized Clinical Trial.

Kwiatkowski DM, Goldstein SL, Cooper DS, Nelson DP, Morales DL, Krawczeski CD.

JAMA Pediatr. 2017 Apr 1;171(4):357-364. doi: 10.1001/jamapediatrics.2016.4538.

PMID: 28241247

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  1. Veno-Venous ECMO in Europe: are we all speaking the same language?

Pappalardo F, Montisci A, Scandroglio A, Belliato M, Malfertheiner M, Broman M, David P, Lorusso R, Taccone F, Broccati F, DI Nardo M.

Minerva Anestesiol. 2017 Apr;83(4):424-425. doi: 10.23736/S0375-9393.16.11766-3. Epub 2016 Nov 9. No abstract available.

PMID: 27827522

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  1. Routine Application of Bloodless Priming in Neonatal Cardiopulmonary Bypass: A 3-Year Experience.

Boettcher W, Sinzobahamvya N, Miera O, Redlin M, Dehmel F, Cho MY, Murin P, Berger F, Photiadis J.

Pediatr Cardiol. 2017 Apr;38(4):807-812. doi: 10.1007/s00246-017-1585-x. Epub 2017 Feb 14.

PMID: 28197644

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  1. Predicting Fluid Responsiveness in Children Undergoing Cardiac Surgery After Cardiopulmonary Bypass.

Favia I, Romagnoli S, Di Chiara L, Ricci Z.

Pediatr Cardiol. 2017 Apr;38(4):787-793. doi: 10.1007/s00246-017-1582-0. Epub 2017 Feb 11.

PMID: 28190141

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  1. Association Between Hematologic and Inflammatory Markers and 31 Thrombotic and Hemorrhagic Events in Berlin Heart Excor Patients.

Iyengar A, Hung ML, Asanad K, Kwon OJ, Jackson NJ, Reemtsen BL, Federman MD, Biniwale RM.

Pediatr Cardiol. 2017 Apr;38(4):770-777. doi: 10.1007/s00246-017-1578-9. Epub 2017 Feb 10.

PMID: 28184979

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  1. Alteration of Cardiac Deformation in Acute Rejection in Pediatric Heart Transplant Recipients.

Chanana N, Van Dorn CS, Everitt MD, Weng HY, Miller DV, Menon SC.

Pediatr Cardiol. 2017 Apr;38(4):691-699. doi: 10.1007/s00246-016-1567-4. Epub 2017 Feb 4.

PMID: 28161809

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ACHD Featured Articles of April 2017

Adult Congenital Heart Reviews of April 2017 Manuscripts, sponsored by the International Society for Adult Congenital Heart Disease

 

  1. Maternal and Fetal Outcomes of Admission for Delivery in Women With Congenital Heart Disease. Hayward RM, Foster E, Tseng ZH. JAMA Cardiol. 2017 Apr 12. doi: 10.1001/jamacardio.2017.0283. [Epub ahead of print] PMID: 28403428 Similar articles Select item 28412020

 

Take Home Points:

  • California delivery hospitals demonstrated an association with congenital heart disease, and congestive heart failure admissions, atrial arrhythmias, and fetal growth restriction.
  • Maternal complex congenital heart disease is associated with ventricular arrhythmias and maternal in hospital mortality, though rare.
  • Increased monitoring at the time of delivery may be recommended as a result.
  • Post delivery there is an increased likelihood of readmission that is markedly increased when compared to pregnant women without CHD.

 

Moe_Tabitha-WEBCommentary from Dr. Tabitha Moe (Phoenix), section editor of ACHD Journal Watch:

This investigation from January 1st 2005 to December 31st, 2011 reviewed more than 3 million deliveries in the state of California with its diverse population, and a variety of centers.  Of those 3 million deliveries, 3189 women had noncomplex congenital heart disease and 262 had complex congenital heart disease. Women with CHD were more likely to undergo Caesarian delivery (39%), compared with women without CHD (32%). Interestingly 54.9% of the women in this cohort who had  Tetralogy of Fallot underwent C-section. This marked increase above baseline C-section rates clashes with the maternal indications for C-section, suggesting that the number of C-sections performed on women with TOF is out of proportion with those that are considered necessary by WHO risk stratification criteria. Complex congenital heart disease was associated with greater adjusted odds of serious ventricular arrhythmias and maternal in hospital mortality. Both simple and complex congenital heart disease women were at higher risk for readmission, atrial arrhythmias, heart failure, and fetal growth restriction as compared with the women without congenital heart disease.

This study expertly molds a large publicly available dataset from the HCUP data at UCSF allowing the authors to investigate an important question relating to outcomes associated with pregnancy in CHD.  Though valuable overall, this large database is limited by the specificity of coding classifications, and the transition from ICD 9 to ICD 10 in recent years, which occurred following the data collected in this paper.  Complex CHD included endocardial cushion defects, hypoplastic left heart syndrome, tetralogy of Fallot, transposition of the great arteries, truncus arteriosus, and univentricular heart defects.  All other defects were classified as noncomplex.

ACHD1 v2

The figure above alludes to the increasing likelihood of readmissions for patients with congenital heart disease over the course of the 6 years following delivery. Though this data does not differentiate non pregnancy related complications from overall cardiac complications, it does suggest that pregnancy may present an important milestone determining later outcomes. However, no comparative non-pregnant data is presented, making proper interpretation of such data somewhat speculative

This is nevertheless an important study as it demonstrates an overall decrease in maternal comorbidities compared with the previously reported data by Siu et al. More study as well as collaboration is certainly needed.

 

 

NT-proBNP predicts mortality in adults with transposition of the great arteries late after Mustard or Senning correction.

Popelová JR, Tomková M, Tomek J.

Congenit Heart Dis. 2017 Apr 17. doi: 10.1111/chd.12466. [Epub ahead of print]

PMID: 28419713

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Take Home Points:

 

  • Patients with a Mustard or Senning operation are at risk of unexpected deaths due to heart failure and arrhythmias
  • NT-proBNP was the most useful independent predictor of mortality (HR 281.39), using univariate Cox Regression. A value of 1000 pg/mL had a 88% sensitivity and 95% specificity for the prediction of mortality.
  • Surprisingly, mitral E velocity of 0.68 m/s or greater was associated with improved survival i.e. in the subpulmonary ventricle.

 

Blanche CupidoCommentary from Dr Blanche Cupido (Cape Town, South Africa), section editor of ACHD Journal Watch: Mustard and Senning corrections for transposition of the great arteries have largely been replaced by the arterial switch operation. However a number of such operations continue to be performed in the developing world for simple transposition of the great arteries. Although most patients remain asymptomatic for many years, they may die unexpectedly, usually as a result of heart failure of arrhythmias.  As risk factors for mortality, especially sudden cardiac death remain elusive, the authors of this study sought to investigate the predictors for mortality in adult patients with Mustard or Senning repairs.

 

Eighty-seven patients (39 Senning and 48 Mustard) were followed in an outpatient setting over a 10 years (2005-2015).  They underwent serial clinical [all], echocardiographic [all] and NT-proBNP measurements. CMR data was available in 18% and VO2max measurements in 44%.

 

The majority of patients were male (n=64, 74%). Those with a Mustard correction were significantly older (age 30 vs 21, p<0.0001) and had higher NT-proBNP levels (287 vs 144, p=0.00058) when compared to Senning patients.

 

The overall mortality was 9% (n=8) with no significant differences between the two groups. One of the patients died of early rejection post transplantation, the rest were due to heart failure.

ACHD2

 

NT-proBNP and the mitral E velocity were the only two independent predictors for death that emerged on multivariable analysis (NT-proBNP HR 281.39 (CI 13.06-6063, p=0.00048; and mitral E velocity HR 0.95 (CI 0.90-0.99, p=0.01815).

ACHD3

 

An NT-proBNP level of <1000 pg/mL correlated with improved survival.

ACHD4

 

A baseline mitral E velocity of >0.68m/s was associated with improved survival. Further validation is required. NYHA class and doppler and tissue doppler parameters relating to the systemic RV, surprisingly, did not show significant differences.

The findings of this study need verification however in larger datasets. Because of the relatively small numbers in this particular study, and the small number of outcomes, multivariable time dependent analysis was not possible.

 

Epidemiological changes in Eisenmenger syndrome in the Nordic region in 1977-2012.

Heart. 2017 Apr 27. pii: heartjnl-2016-310979. doi: 10.1136/heartjnl-2016-310979. [Epub ahead of print]

Hjortshøj CS1Jensen AS1Sørensen K2Nagy E3Johansson B4Kronvall T5Dellborg M6Estensen ME7Holmstrøm H8Turanlahti M9Thilén U10Søndergaard L1.

PMID: 28450553

Take Home Points:
• During the last 4 decades, the incidence and prevalence of Eisenmenger syndrome in the Nordic region has decreased.
• During the last 4 decades, the age of death increased, which may not necessarily reflect improved survival, but is more likely the result of decreased incidence and shifting of age distribution.
• Median survival of Eisenmenger syndrome patients is significantly lower than the background population.
• Down syndrome patients with Eisenmenger syndrome appear to have worse survival.

jokhadar-maanCommentary from Dr. Maan Jokhadar (Atlanta), section editor of ACHD Journal Watch: Eisenmenger syndrome (ES) is a late complication of unrepaired large systemic to pulmonary shunt that is characterized by increased pulmonary vascular resistance, pulmonary hypertension, and cyanosis with its associated features of digital clubbing and exertional dyspnea. ES was more common before modern diagnostic methods and surgical correction became available.
Though several studies report ES survival rates, the results are often influenced by so-called “immortal time bias” also known as survivor bias. Because recruit starts at time of diagnosis of start of late follow-up, there is an inherent bias toward survivors as earlier events such as mortality are not normally capture in such analyses. ES late survival is therefore likely to be overestimated when age is used as the time scale variable for Kaplan-Meier survival analyses without accounting for all events that have happened from the time of birth, which for most studies is not feasible.
Therefore, the time from diagnosis to start of follow-up should be removed from the analysis, by accounting for the age at entry to the study.
With this in mind, Dr. Hjortshøj and colleagues from Denmark, Sweden, Norway, and Finland performed a retrospective population based analysis through an extensive search of national registries and found 714 patients with ES between 1977 and 2012. It should be noted that patients with surgically corrected shunts but with persistent pulmonary hypertension were not included in the study.
The results were as follows:
ES incidence decreased from 2.5 to 0.23/million inhabitants/year.
ES prevalence decreased from 24.6 to 12.0/million inhabitants.
The mean age of the study population increased from about 19.9 in 1977 to 40.4 in 2012. The rate of deaths per million inhabitants increased as well. The absolute number of children with ES decreased throughout the study period.
The annual mortality rate was about 2.3%
The median survival was about 38.4 years, which is lower than the median survival of 81.6 years for the matched background control population.
Patients with simple shunts appear to survive longer than patients with complex lesions.
Down syndrome patients appeared to have worse mortality, particularly after factoring in the high preoperative mortality following shunt corrective surgery.
The mean age of death was 27.7 during the first era of the study (1977 to 1992), which was 38.8 during the second era (1993 to 2006), and was 46.3 during the last era (2006 to 2012). Though the study showed an increase in the age of death throughout the study, this may not represent improved longevity, but rather the result of decreasing incidence on age distribution that leads to an older study population.
The results of this study suggest that a reduced incidence of ES is due to earlier detection and treatment of patients with congenital heart disease. However, there will likely remain a small subset of patients with complex lesions who are unsuitable for surgical correction and will go on to develop ES. These findings in this Nordic population are likely similar to other developed countries, but quite different from developing countries where early access to diagnosis and treatment is less common.
Long-term outcomes of pulmonary arterial hypertension under specific drug therapy in Eisenmenger syndrome.

Hascoët S1, Baruteau AE2, Humbert M3, Simonneau G3, Jais X3, Petit J2, Laux D2, Sitbon O3, Lambert V4, Capderou A5.

J Heart Lung Transplant. 2017 Apr;36(4):386-398. doi: 10.1016/j.healun.2016.10.006. Epub 2016 Oct 15.

 

 

Take Home Points:

 

  • There is little long-term data in usefulness of PAH-specific drug therapy (PAH-SDT) in Eisenmenger syndrome
  • In this study, 69 patients with Eisenmenger syndrome were followed over 20 years using a structured follow-up including thorough hemodynamics (with measured VO2) and clinical status
  • PAH-SDT resulted in improvement in measured hemodynamics in 68.0% of patients; however after a median of 4.9 years, changes in hemodynamic measures were no longer significant
  • Even with PAH-SDT, there was a high event rate (death, heart-lung transplantation, or listing for transplantation), with a 15-year cumulative event rate of 49.2%
  • Independent predictors of events were superior vena cava oxygen saturation and hemodynamic reponse to PAH-SDT

 

Kay_William_MD.16.CVaCommentary from Dr. W. Aaron Kay (Indianapolis), section editor of ACHD Journal Watch: This study from France quite thoroughly evaluated a cohort of 69 patients with Eisenmenger syndrome who were treated with PAH-specific drug therapy (PAH-SDT).  All patients underwent thorough invasive hemodynamics, including a measured, rather than calculated, VO2, as well as 6-minute walk distance and WHO functional status.    All patients had measurement of indexed pulmonary output (Qpi), pulmonary vascular index (PVRi), and superior vena cava oxygen saturation.  All patients had an initial PVRi > 8 Woods units * m2 at the start of the study, and all patients were on PAH-SDT (i.e. there was no control group).

Measured hemodynamics improved in the majority of patients, 68.0% of the sample. The hemodynamic response and clinical response was generally initially good.  Overall increase in Qpi was 0.4 LPM/m2.  Overall increase in 6MW distane was +49 m.   Changes in hemodynamics and 6MW distance were assessed after each change in treatment (i.e. intensification or addition of a 2nd or 3rd drug).  The treatment effect was found not to be sustained over long-term follow-up, with changes no longer being significant after a median of 4.9 years.

Events were common; 33.3% of patients met the composite endpoint of death, heart-lung transplant, or listing for transplantation after a median of 7.2 years.  The 15-year cumulative event rate was 49.2%.  Median time to event was 8.1 years.  Patients with events had worse hemodynamics at baseline.  On univariate analysis, predictors of events were a PVRi > 30 Woods Units * m2, right atrial pressure > 4 mmHg, and SVC saturation < 65%, and pulmonary artery saturation < 70%.  On multivariate analysis,  SVC saturation was found to correlate with event rate.  Each 1% decrease in SVC saturation was associated with a 9.8% increase in hazard ratio for events.  SVC saturation in Eisenmenger syndrome reflects both arterial saturation as well as systemic cardiac output.

This study showed some evidence that patients with pretricuspid shunts may have more benefit from PAH-SDT than those with posttricuspid shunts; however the authors caution this may be artifact from “immortal survival bias.”  Atrial septal defects were the most common underlying CHD in this cohort (n=43, 62.3%).

The authors conclude that PAH-SDT can be helpful in Eisenmenger syndrome, but the regimen may need to be individualized base on hemodynamic changes for each patient.

ACHD5

 

 

Figure 4 Kaplan-Meier cumulative event curves in the sub-group (E),aortic oxygen saturation, (F) SVC oxygen saturation, (G),and pattern of hemodynamic change under PAH-SDT(H).

 

 

 

Is the presence of end-diastolic forward flow specific for restrictive right ventricular physiology in repaired tetralogy of Fallot?

 

Mori Y, Murakami T, Inoue N, Kaneko S, Nakashima Y, Koide M.

Int J Cardiol. 2017 Apr 11. pii: S0167-5273(16)34242-5. doi: 10.1016/j.ijcard.2017.04.021. [Epub ahead of print]

PMID

 

Take Home Points:

 

  • From past publications, end diastolic forward flow (EDFF) in repaired tetralogy of Fallot [TOF] has been considered to be synonymous with restrictive right ventricular (RV) physiology.
  • In the present study patients with EDFF have different hemodynamics as compared to those without: higher RA pressure, higher RA to PA pressures gradient, and had higher ANP levels
  • Not all patients who demonstrate EDFF, have restrictive RV physiology when taking invasive hemodynamics into account
  • Patients with a small RV and EDFF demonstrate restrictive RV physiology, and demonstrate associated invasive hemodynamics
  • Patients with large RV and EDFF do not demonstrate restrictive RV physiology. Instead, they may have very low pulmonary diastolic pressure, which is lower than right atrial pressure, resulting in EDFF during RA contraction.

 

Leong Ming ChernCommentary from Dr. M.C. Leong (Kuala Lumpur), section editor of ACHD Journal Watch:  The presence of end diastolic forward flow (EDFF) in the pulmonary arteries on echocardiography or cardiac magnetic resonance has been regarded as the hallmark echocardiopgraphic feature of restrictive right ventricular (RV) physiology in repaired Tetralogy of Fallot. However not all such patients have good late outcomes. A recent study found that TOF patients with EDFF have good effort tolerance only if associated with small right ventricles. The authors of the current study therefore sought to demonstrate that not all patients with EDFF on echocardiography have higher right ventricular filling pressures via cardiac catheterizations.

 

62 patients with repaired TOF were retrospectively reviewed. They were aged 15.7 ± 11.6 years, and underwent cardiac catheterization. Patients were then divided into Group 1 (n=23) with and Group 2 (n=39) without EDFF; Group A (n=53) with small RVs (<150ml/m2) and Group B (n=9) with larger RV (≥150ml/m2). Group 1 had a higher right atrial pressure, pressure gradient between the right atrium and pulmonary diastolic pressure and atrial natriuretic peptide levels. Group A had better RV and left ventricular ejection fraction and smaller LV size.

 

However, on further subgroup analysis, patients with EDFF and smaller RV (Group 1A) had higher right atrial pressure and less pulmonary regurgitation than the rest of the groups. Patients with large RV and EDFF (Group 1B) had right atrial pressure and RV end diastolic pressure similar to those without EDFF. This group of patients had higher pressure gradient between the right atrium and pulmonary diastolic pressure compared to patients without EDFF.

ACHD6ACHD7-1

 

The authors hence postulate that patients in Group 1A corresponds to “true” restrictive RV physiology. However, patients in Group 1B may not have true restrictive RV physiology, despite EDFF. Instead, they may have severe pulmonary regurgitation which results in very low pulmonary diastolic pressure, which created a high gradient between the right atrium and pulmonary diastolic pressure and hence EDFF.

    

 

ACHD April 2017

 

  1. Epidemiological changes in Eisenmenger syndrome in the Nordic region in 1977-2012.

Hjortshøj CS, Jensen AS, Sørensen K, Nagy E, Johansson B, Kronvall T, Dellborg M, Estensen ME, Holmstrøm H, Turanlahti M, Thilén U, Søndergaard L.

Heart. 2017 Apr 27. pii: heartjnl-2016-310979. doi: 10.1136/heartjnl-2016-310979. [Epub ahead of print]

PMID: 28450553

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Select item 28449285

 

  1. Research Review: Childhood chronic physical illness and adult emotional health – a systematic review and meta-analysis.

Secinti E, Thompson EJ, Richards M, Gaysina D.

J Child Psychol Psychiatry. 2017 Apr 27. doi: 10.1111/jcpp.12727. [Epub ahead of print] Review.

PMID: 28449285

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Select item 28450386

 

  1. Heart transplantation for adults with congenital heart disease: current status and future prospects.

Matsuda H, Ichikawa H, Ueno T, Sawa Y.

Gen Thorac Cardiovasc Surg. 2017 Apr 24. doi: 10.1007/s11748-017-0777-x. [Epub ahead of print] Review.

PMID: 28439697

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  1. Critical involvement of ZEB2 in collagen fibrillogenesis: the molecular similarity between Mowat-Wilson syndrome and Ehlers-Danlos syndrome.

Teraishi M, Takaishi M, Nakajima K, Ikeda M, Higashi Y, Shimoda S, Asada Y, Hijikata A, Ohara O, Hiraki Y, Mizuno S, Fukada T, Furukawa T, Wakamatsu N, Sano S.

Sci Rep. 2017 Apr 19;7:46565. doi: 10.1038/srep46565.

PMID: 28422173 Free PMC Article

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  1. Attitudes and perceptions of pregnant women with CHD: results of a single-site survey.

Sabanayagam A, Briston D, Zaidi AN.

Cardiol Young. 2017 Apr 18:1-8. doi: 10.1017/S1047951116002857. [Epub ahead of print]

PMID: 28416028

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Select item 28430913

 

  1. Person-centred transition programme to empower adolescents with congenital heart disease in the transition to adulthood: a study protocol for a hybrid randomised controlled trial (STEPSTONES project).

Acuña Mora M, Sparud-Lundin C, Bratt EL, Moons P.

BMJ Open. 2017 Apr 17;7(4):e014593. doi: 10.1136/bmjopen-2016-014593.

PMID: 28420661 Free Article

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Select item 28412993

 

  1. Paediatric-onset coronary artery anomalies in pregnancy: a single-centre experience and systematic literature review.

Keir M, Bhagra C, Vatenmakher D, Arancibia-Galilea F, Jansen K, Toh N, Silversides CK, Colman J, Siu SC, Sermer M, Crean AM, Wald RM.

Cardiol Young. 2017 Apr 17:1-9. doi: 10.1017/S1047951117000658. [Epub ahead of print]

PMID: 28412993

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Select item 28419713

 

  1. NT-proBNP predicts mortality in adults with transposition of the great arteries late after Mustard or Senning correction.

Popelová JR, Tomková M, Tomek J.

Congenit Heart Dis. 2017 Apr 17. doi: 10.1111/chd.12466. [Epub ahead of print]

PMID: 28419713

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Select item 28417257

 

  1. Stroke in adults with Down syndrome.

Marto JP, Dias M, Alves JN, Montes V, Beato-Coelho J, Marques-Matos C, de Sousa A, Bernardo F, Calado S, Viana-Baptista M.

J Neurol Sci. 2017 Apr 15;375:279-280. doi: 10.1016/j.jns.2017.02.014. Epub 2017 Feb 8. No abstract available.

PMID: 28320148

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Select item 28408455

 

  1. Pregnancy in Adult Congenital Heart Disease: Special Delivery.

Davidson WR Jr.

JAMA Cardiol. 2017 Apr 12. doi: 10.1001/jamacardio.2017.0365. [Epub ahead of print] No abstract available.

PMID: 28403448

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Select item 28403428

 

  1. Maternal and Fetal Outcomes of Admission for Delivery in Women With Congenital Heart Disease.

Hayward RM, Foster E, Tseng ZH.

JAMA Cardiol. 2017 Apr 12. doi: 10.1001/jamacardio.2017.0283. [Epub ahead of print]

PMID: 28403428

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Select item 28412020

 

  1. Is Takotsubo Syndrome a benign condition?

Drakopoulou MI, Gatzoulis MA.

Hellenic J Cardiol. 2017 Apr 8. pii: S1109-9666(17)30141-0. doi: 10.1016/j.hjc.2017.03.009. [Epub ahead of print]

PMID: 28400292 Free Article

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Select item 28385091

 

  1. Hypoplasia of the posterior mitral leaflet: A rare cause of mitral regurgitation in adulthood.

Bacich D, Braggion G, Faggian G.

Echocardiography. 2017 Apr 7. doi: 10.1111/echo.13537. [Epub ahead of print]

PMID: 28386957

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Select item 28396253

 

  1. Plastic bronchitis: An unusual complication of acute chest syndrome in adult.

Feray S, Mora P, Decavele M, Pham T, Hafiani EM, Fartoukh M.

Respir Med Case Rep. 2017 Apr 7;21:93-95. doi: 10.1016/j.rmcr.2017.04.006. eCollection 2017.

PMID: 28443234 Free PMC Article

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Select item 28343629

 

  1. Cardiopulmonary Exercise Testing in Adult Congenital Heart Disease.

Mantegazza V, Apostolo A, Hager A.

Ann Am Thorac Soc. 2017 Apr 4. doi: 10.1513/AnnalsATS.201611-876FR. [Epub ahead of print]

PMID: 28375677

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Select item 28395882

 

  1. Incidence and predictors of obstetric and fetal complications in women with structural heart disease.

van Hagen IM, Roos-Hesselink JW, Donvito V, Liptai C, Morissens M, Murphy DJ, Galian L, Bazargani NM, Cornette J, Hall R, Johnson MR.

Heart. 2017 Apr 4. pii: heartjnl-2016-310644. doi: 10.1136/heartjnl-2016-310644. [Epub ahead of print]

PMID: 28377476

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Select item 28377475

 

  1. Diagnosis, imaging and clinical management of aortic coarctation.

Dijkema EJ, Leiner T, Grotenhuis HB.

Heart. 2017 Apr 4. pii: heartjnl-2017-311173. doi: 10.1136/heartjnl-2017-311173. [Epub ahead of print] Review.

PMID: 28377475

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Select item 28359509

 

  1. Clinical Characteristics of Adult Dogs More Than 5 Years of Age at Presentation for Patent Ductus Arteriosus.

Boutet BG, Saunders AB, Gordon SG.

J Vet Intern Med. 2017 Apr 3. doi: 10.1111/jvim.14689. [Epub ahead of print]

PMID: 28370380 Free Article

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  1. Pregnancy-related acute aortic dissection in Marfan syndrome: A review of the literature.

Smith K, Gros B.

Congenit Heart Dis. 2017 Apr 2. doi: 10.1111/chd.12465. [Epub ahead of print] Review.

PMID: 28371362

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Select item 28441822

 

  1. [Assessment of undiagnosed critical congenital heart disease before discharge from the maternity hospital].

Zhao QM, Liu F, Wu L, Ye M, Jia B, Ma XJ, Huang GY.

Zhonghua Er Ke Za Zhi. 2017 Apr 2;55(4):260-266. doi: 10.3760/cma.j.issn.0578-1310.2017.04.006. Chinese.

PMID: 28441821

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Select item 28441820

 

  1. National In-Hospital Outcomes of Pregnancy in Women With Single Ventricle Congenital Heart Disease.

Collins RT 2nd, Chang D, Sandlin A, Goudie A, Robbins JM.

Am J Cardiol. 2017 Apr 1;119(7):1106-1110. doi: 10.1016/j.amjcard.2016.12.015. Epub 2017 Feb 16.

PMID: 28242012

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Select item 28328118

 

  1. High prevalence of fatigue in adults with a 22q11.2 deletion syndrome.

Vergaelen E, Claes S, Kempke S, Swillen A.

Am J Med Genet A. 2017 Apr;173(4):858-867. doi: 10.1002/ajmg.a.38094. Epub 2017 Feb 12.

PMID: 28190295

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  1. Biomarkers of Calcific Aortic Valve Disease.

Small A, Kiss D, Giri J, Anwaruddin S, Siddiqi H, Guerraty M, Chirinos JA, Ferrari G, Rader DJ.

Arterioscler Thromb Vasc Biol. 2017 Apr;37(4):623-632. doi: 10.1161/ATVBAHA.116.308615. Epub 2017 Feb 2. Review.

PMID: 28153876

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Select item 28212920

 

  1. Bicuspid aortic valve outcomes.

Rodrigues I, Agapito AF, de Sousa L, Oliveira JA, Branco LM, Galrinho A, Abreu J, Timóteo AT, Rosa SA, Ferreira RC.

Cardiol Young. 2017 Apr;27(3):518-529. doi: 10.1017/S1047951116002560. Epub 2016 Dec 12.

PMID: 27938448

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Select item 27766996

 

  1. Comparison of participants and non-participants in patient-reported outcome surveys: the case of Assessment of Patterns of Patient-Reported Outcomes in Adults with Congenital Heart disease – International Study.

Berghammer MC, Mattsson E, Johansson B, Moons P, Dellborg M.

Cardiol Young. 2017 Apr;27(3):427-434. doi: 10.1017/S1047951116000676. Epub 2016 May 11.

PMID: 27167242

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Select item 27416545

 

  1. Tricuspid annular plane systolic excursion is preserved in young patients with pulmonary hypertension except when associated with repaired congenital heart disease.

Hauck A, Guo R, Ivy DD, Younoszai A.

Eur Heart J Cardiovasc Imaging. 2017 Apr 1;18(4):459-466. doi: 10.1093/ehjci/jew068.

PMID: 27099276

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Select item 28329112

 

  1. Does functional capacity depend on the size of the shunt? A prospective, cohort study of adults with small, unrepaired ventricular septal defects.

Maagaard M, Heiberg J, Asschenfeldt B, Ringgaard S, Hjortdal VE.

Eur J Cardiothorac Surg. 2017 Apr 1;51(4):722-727. doi: 10.1093/ejcts/ezw420.

PMID: 28204298

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Select item 28199509

 

  1. The effect of a resilience improvement program for adolescents with complex congenital heart disease.

Lee S, Lee J, Choi JY.

Eur J Cardiovasc Nurs. 2017 Apr;16(4):290-298. doi: 10.1177/1474515116659836. Epub 2016 Jul 10.

PMID: 27400701

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Select item 28431063

 

  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, Hung J, Bermejo J, Chambers JB, Edvardsen T, Goldstein S, Lancellotti P, LeFevre M, Miller F Jr, Otto CM.

J Am Soc Echocardiogr. 2017 Apr;30(4):372-392. doi: 10.1016/j.echo.2017.02.009.

PMID: 28385280

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Select item 28306488

 

  1. Clinical recommendations of cardiac magnetic resonance, Part II: inflammatory and congenital heart disease, cardiomyopathies and cardiac tumors: a position paper of the working group ‘Applicazioni della Risonanza Magnetica’ of the Italian Society of Cardiology.

Pontone G, Di Bella G, Silvia C, Maestrini V, Festa P, Ait-Ali L, Masci PG, Monti L, di Giovine G, De Lazzari M, Cipriani A, Guaricci AI, Dellegrottaglie S, Pepe A, Marra MP, Aquaro GD.

J Cardiovasc Med (Hagerstown). 2017 Apr;18(4):209-222. doi: 10.2459/JCM.0000000000000499.

PMID: 28092290

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Select item 27986330

 

  1. Long-term outcomes of pulmonary arterial hypertension under specific drug therapy in Eisenmenger syndrome.

Hascoët S, Baruteau AE, Humbert M, Simonneau G, Jais X, Petit J, Laux D, Sitbon O, Lambert V, Capderou A.

J Heart Lung Transplant. 2017 Apr;36(4):386-398. doi: 10.1016/j.healun.2016.10.006. Epub 2016 Oct 15.

PMID: 27866929

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Select item 28415932

 

  1. [The Relationships among Perceived Parental Bonding, Illness Perception, and Anxiety in Adult Patients with Congenital Heart Diseases].

Shin N, Jang Y, Kang Y.

J Korean Acad Nurs. 2017 Apr;47(2):178-187. doi: 10.4040/jkan.2017.47.2.178. Korean.

PMID: 28470155

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Select item 28302382

 

  1. Use of Cardiac Computed Tomography for Ventricular Volumetry in Late Postoperative Patients with Tetralogy of Fallot.

Kim HJ, Mun DN, Goo HW, Yun TJ.

Korean J Thorac Cardiovasc Surg. 2017 Apr;50(2):71-77. doi: 10.5090/kjtcs.2017.50.2.71. Epub 2017 Apr 5.

PMID: 28382264 Free PMC Article

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Select item 26891904

 

  1. Readiness for Transition to Adult Health Care for Young Adolescents with Congenital Heart Disease.

Stewart KT, Chahal N, Kovacs AH, Manlhiot C, Jelen A, Collins T, McCrindle BW.

Pediatr Cardiol. 2017 Apr;38(4):778-786. doi: 10.1007/s00246-017-1580-2. Epub 2017 Feb 10.

PMID: 28184978

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Select item 28184976

 

  1. 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, Ramgren Johansson J, Malm T, Arheden H, Liuba P, Carlsson M.

Pediatr Cardiol. 2017 Apr;38(4):669-680. doi: 10.1007/s00246-016-1565-6. Epub 2017 Feb 10. Erratum in: Pediatr Cardiol. 2017 Apr 29;:.

PMID: 28184976 Free PMC Article

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Select item 28184975

 

  1. Magnetic resonance imaging 4-D flow-based analysis of aortic hemodynamics in Turner syndrome.

Arnold R, Neu M, Hirtler D, Gimpel C, Markl M, Geiger J.

Pediatr Radiol. 2017 Apr;47(4):382-390. doi: 10.1007/s00247-016-3767-8. Epub 2017 Feb 9.

PMID: 28184962

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Select item 28132084

 

  1. Relationship Between Fragmented QRS Complexes and Cardiac Status in Duchenne Muscular Dystrophy: Multimodal Validation Using Echocardiography, Magnetic Resonance Imaging, and Holter Monitoring.

Cho MJ, Lee JW, Lee J, Shin YB, Lee HD.

Pediatr Cardiol. 2017 Apr 29. doi: 10.1007/s00246-017-1616-7. [Epub ahead of print]

PMID: 28456833

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Select item 28456832

 

  1. Validation of numerical simulation methods in aortic arch using 4D Flow MRI.

Miyazaki S, Itatani K, Furusawa T, Nishino T, Sugiyama M, Takehara Y, Yasukochi S.

Heart Vessels. 2017 Apr 25. doi: 10.1007/s00380-017-0979-2. [Epub ahead of print]

PMID: 28444501

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Select item 28377612

 

  1. Natural history of aortic root dilation through young adulthood in a hypermobile Ehlers-Danlos syndrome cohort.

Ritter A, Atzinger C, Hays B, James J, Shikany A, Neilson D, Martin L, Weaver KN.

Am J Med Genet A. 2017 Apr 24. doi: 10.1002/ajmg.a.38243. [Epub ahead of print]

PMID: 28436618

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Select item 28436679

 

  1. Topsy-turvy heart and associated imaging findings.

Bayramoglu Z, Yılmaz R, Demir AA, Yekeler E, Dursun M, Dindar A, Nisli K, Omeroglu R.

J Cardiovasc Comput Tomogr. 2017 Apr 20. pii: S1934-5925(17)30093-X. doi: 10.1016/j.jcct.2017.04.008. [Epub ahead of print] No abstract available.

PMID: 28438441

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Select item 28366040

 

  1. Is the presence of end-diastolic forward flow specific for restrictive right ventricular physiology in repaired tetralogy of Fallot?

Mori Y, Murakami T, Inoue N, Kaneko S, Nakashima Y, Koide M.

Int J Cardiol. 2017 Apr 11. pii: S0167-5273(16)34242-5. doi: 10.1016/j.ijcard.2017.04.021. [Epub ahead of print]

PMID: 28457561

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Select item 28410946

 

  1. Aortopathy in an Adult With Tricuspid Atresia and Left Ventricular Non-Compaction After Fontan Procedure.

Murakami T, Mori Y, Inoue N, Kaneko S, Nakashima Y.

Circ J. 2017 Apr 5. doi: 10.1253/circj.CJ-17-0016. [Epub ahead of print] No abstract available.

PMID: 28381694 Free Article

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Select item 28389305

 

  1. Presentation of missed childhood Kawasaki disease in adults: Experience from a tertiary care center in north India.

Vijayvergiya R, Bhattad S, Varma S, Singhal M, Gordon J, Singh S.

Int J Rheum Dis. 2017 Apr 5. doi: 10.1111/1756-185X.13073. [Epub ahead of print]

PMID: 28378434

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Select item 28381550

 

  1. Revisiting the utility of technical performance scores following tetralogy of Fallot repair.

Lodin D, Mavrothalassitis O, Haberer K, Sunderji S, Quek RGW, Peyvandi S, Moon-Grady A, Karamlou T.

J Thorac Cardiovasc Surg. 2017 Apr 5. pii: S0022-5223(17)30640-2. doi: 10.1016/j.jtcvs.2017.02.066. [Epub ahead of print]

PMID: 28461051

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Select item 28382463

 

  1. Fontan survival: As good as it gets?

Thankavel P, Jaquiss RDB.

J Thorac Cardiovasc Surg. 2017 Apr 4. pii: S0022-5223(17)30633-5. doi: 10.1016/j.jtcvs.2017.03.114. [Epub ahead of print] No abstract available.

PMID: 28434620

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Select item 28378408

 

  1. Decreased false-positive adolescent pre-athletic screening with Seattle Criteria-interpreted electrocardiograms.

Colombo JN, Samson RA, Valdes SO, Meziab O, Sisk D, Klewer SE.

Cardiol Young. 2017 Apr;27(3):512-517. doi: 10.1017/S104795111600086X. Epub 2016 Jun 20.

PMID: 27322729

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Select item 27161562

 

  1. Mechanism of Progressive Heart Failure and Significance of Pulmonary Hypertension in Obstructive Hypertrophic Cardiomyopathy.

Covella M, Rowin EJ, Hill NS, Preston IR, Milan A, Opotowsky AR, Maron BJ, Maron MS, Maron BA.

Circ Heart Fail. 2017 Apr;10(4):e003689. doi: 10.1161/CIRCHEARTFAILURE.116.003689.

PMID: 28396501

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Select item 27905124

 

  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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Select item 27400701

 

  1. Loss-of-function mutations in the X-linked biglycan gene cause a severe syndromic form of thoracic aortic aneurysms and dissections.

Meester JA, Vandeweyer G, Pintelon I, Lammens M, Van Hoorick L, De Belder S, Waitzman K, Young L, Markham LW, Vogt J, Richer J, Beauchesne LM, Unger S, Superti-Furga A, Prsa M, Dhillon R, Reyniers E, Dietz HC, Wuyts W, Mortier G, Verstraeten A, Van Laer L, Loeys BL.

Genet Med. 2017 Apr;19(4):386-395. doi: 10.1038/gim.2016.126. Epub 2016 Sep 15.

PMID: 27632686 Free PMC Article

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Select item 28279417

 

  1. Does Body Mass Index Predict Premature Cardiomyopathy Onset for Duchenne Muscular Dystrophy?

McKane M, Soslow JH, Xu M, Saville BR, Slaughter JC, Burnette WB, Markham LW.

J Child Neurol. 2017 Apr;32(5):499-504. doi: 10.1177/0883073816687422. Epub 2017 Jan 13.

PMID: 28084148

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Select item 28324046

 

  1. Clinical and magnetic resonance evolution of “infarct-like” myocarditis.

Faletti R, Gatti M, Baralis I, Bergamasco L, Bonamini R, Ferroni F, Imazio M, Stola S, Gaita F, Fonio P.

Radiol Med. 2017 Apr;122(4):273-279. doi: 10.1007/s11547-016-0723-5. Epub 2017 Jan 9.

PMID: 28070840

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Select item 28096476

 

  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 Apr;36(4):239-246. doi: 10.1016/j.repc.2016.09.014. Epub 2017 Mar 18. English, Portuguese.

PMID: 28318851

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Select item 28142228

 

 

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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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Select item 28394409

 

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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Select item 28434117

 

 

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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Select item 28446798

 

  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

Similar articles

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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Select item 28179595

 

  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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Select item 28441153

 

  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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Select item 28436429

 

  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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Select item 28456482

 

  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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Select item 28425218

 

  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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Select item 28431165

 

  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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Select item 28414565

 

  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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Select item 28391445

 

  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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Select item 28191731

 

  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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Select item 27099276

 

  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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Select item 28007875

 

  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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Select item 27400701

 

  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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Select item 28223475

 

  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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Select item 27866929

 

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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Select item 28091832

 

  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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Select item 27986271

 

  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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Select item 27938900

 

  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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Select item 28403078

 

  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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Select item 28288113

 

  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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Select item 28155223

 

  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 28214968

 

  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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Select item 28214967

 

  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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Select item 28154912

 

  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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Select item 28150025

 

  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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Select item 28116475

 

  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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Select item 27543379

 

  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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Select item 28184962

 

  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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Select item 28325177

 

  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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Select item 27878778

 

  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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Select item 28407839

 

  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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Select item 28456831

 

  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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Select item 28456829

 

  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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Select item 28453898

 

  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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Select item 28459760

 

  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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Select item 28434117

 

  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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Select item 28473974

 

  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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Select item 28416468

 

  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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Select item 28399861

 

  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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Select item 28399889

 

  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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  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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  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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  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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  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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  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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  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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  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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  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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  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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  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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  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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  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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  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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  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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  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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  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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  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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  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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  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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  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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  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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  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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  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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  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. 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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  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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  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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  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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  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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  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. 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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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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  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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  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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  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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  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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