Congenital EP Featured Articles of June 2017

 

Congenital and Pediatric Cardiac EP Reviews of June 2017 Manuscripts

 

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

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

Circulation. 2017 Jun 20;135(25):2485-2493. doi: 10.1161/CIRCULATIONAHA.117.028087. Epub 2017 Apr 27.

PMID: 28450351

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Take Home Points:

  • Supraventricular tachycardia can be seen in up to 50% of children and adults after heart transplantation.
  • Adenosine, a endogenous nucleoside that results in sinus slowing and AV block, has been considered relatively contraindicated in heart transplant recipients due to post-transplant denervation
  • Adenosine use at low doses of 25 μg/kg or 1.5 mg were found to be effective in causing AV block in healthy heart transplant patients in sinus rhythm
  • AV block was seen in 96% of patients with escalating adenosine administration up to maximal dose of 200 μg/kg or 12 mg
  • There were no adverse events seen after adenosine administration including no clinically significant asystole (≥ 12 seconds) requiring rescue ventricular pacing
  • The use of adenosine during supraventricular tachycardia in post-heart transplants patients was not evaluated and therefore, the dosing, response and safety of adenosine in that scenario cannot be commented on based on this study.

 

A PatelComment from Dr. Akash Patel (San Francisco), section editor of Congenital Electrophysiology Journal Watch:  Previous limited data has suggested heart transplant patients are at adverse risk to adenosine in the setting of cardiac denervation after transplantation. In particular, adenosine exposure in this setting has been thought to lead to excessive AV block and bradycardia. Due to this concern, adenosine is relatively contraindicated in post-heart transplant patients and thus uncommonly used. However, up to 50% of children and adults with heart transplantation can be affected by supraventricular tachycardia with adenosine potentially providing a therapeutic and/or diagnostic option.  This single center prospective study from New York Presbyterian/Morgan Stanley Children’s Hospital, Columbia University Medical Center aimed to improve our understanding of the safety and efficacy of adenosine in pediatric and young adult patients who have undergone heart transplantation with hopes to reconsider its use in this population.

 

All healthy patients ≥ 2 weeks post-cardiac transplantation were included if they agreed to consent and were excluded if there was evidence of rejection (clinical/humeral/cellular), graft vasculopathy, concurrent use of intravenous inotropic medication, concurrent treatment for rejection, conduction disease (first-, second-, or third-degree AV block, preexisting sinus node dysfunction), or systolic ventricular dysfunction. At the time of routine post-cardiac transplant catheterization, eligible patients underwent adenosine testing.

The study group included 80 patients (55% male) with a median age of 13.6 years (Range: 1.1 – 24 year). The median weight was 50.4 kg (Range: 7.8 – 120 kg). The indications for transplantation included cardiomyopathy (60%), congenital heart disease (30%), retransplantation (7.5%), and other (2.5%). The most common type of transplantation was bicaval (71%) followed by biatrial (28%). Prior history of rejection was seen in 49%. There was use of dipyridamole in 53% which is a medication known to effect adenosine metabolism. This medication was stopped 3 days before catheterization in all patients. There was use of antiarrhythmic drugs – beta-blocker (24%) and digoxin (2.5%)- but no data on indications or prior arrhythmia status. The cardiac catheterization procedures were done under general anesthesia in 64%.

After catheterization and biopsy were complete but before the removal of central access, a 4F quadripolar pacing catheter was introduced into the right ventricle with pacing thresholds tested and outputs set appropriately. Adenosine was given via central access in serial escalating doses based on weight until AV block or clinically significant asystole (either a sinus pause or AV block ≥ 12 seconds which were the predetermined criterion to initiate ventricular pacing) were seen. If patients weighed <60 kg, patients were given 12.5, 25, 50, 100, and 200 μg/kg of adenosine. If patients weighed ≥ 60 kg, patients were given 0.8, 1.5, 3, 6, and 12 mg.  See below.  The median maximal dose of adenosine given was 3.6 mg (IQR: 2.0 – 6.0 mg).

June EP v1

The primary outcome of the study was to assess the safety of adenosine.  There were no patients (0%) who had clinically significant asystole requiring rescue ventricular pacing after adenosine administration during the escalating adenosine protocol. See below.

 June EP V2

 

The secondary objective was to assess the efficacy of adenosine in the post-heart transplant patient. There was AV block seen in 77/80 (96%) of the cohort (see above).  There was sinus pause > 2 seconds seen in 4 (5%). The effect of AV nodal blockade was seen at doses as low as 25 μg/kg (< 60 kg) or 1.5 mg (≥ 60kg) in 12%. As expected, incremental escalations in dosing increased the percent of patients demonstrating AV nodal block. At up to initial standard dosing of 100 μg/kg (< 60 kg) or 6 mg (≥ 60kg), there was AV nodal blockade seen in 72%.  See Below.

June EP V3The AV nodal effects of adenosine varied in duration. The median longest R-R interval during AV block was 1.9 seconds (IQR: 1.4 – 3.2 seconds) (Example See Figure Below (A)) The mean total adenosine effect was 4.3 seconds (SD: ±2.0 seconds)  (Example: See Figure Below (B)). The absolute longest R-R interval and total adenosine effect in any patient was 8.4 seconds. The median consecutive non-conducted P waves during the longest pause were 2 (IQR, 1–3). The authors stated no variable predicted adenosine effect including prior rejection, maximal dose, weight, or time from transplant.

June EP v6

The use of adenosine was well tolerated with typical symptoms reported in the non-anesthetized group including shortness of breath, discomfort, chest pain, headache, and flushing. There were no adverse symptoms that resulted in premature termination of the protocol.

This study demonstrates the safety of adenosine use in a large healthy pediatric and young adult post-cardiac transplant cohort with no adverse effects including no clinically significant asystole requiring rescue ventricular pacing.  The authors conclude that adenosine is effective in causing AV node blockade at much lower doses – 25 μg/kg (< 60kg) or 1.5mg (≥ 60 kg) –  than PALS or ACLS standard dosing. As the effect is dose-dependent, gradual dose escalation can be considered up to maximal doses of 200 μg/kg (< the 60kg) or 12 mg (≥60kg) until AV blockade is achieved.

This study however does not address if and why lower doses are required and if there is an exaggerated response with adenosine use in post-transplant patients. In particular, it is unclear if the same lower doses given centrally in healthy age-based controls would have resulted in the same effect. In addition, in 1990 Ellenbogen et al (Circulation 1990;81: 821-828  PMID: 2306833) demonstrated there was evidence of a supersensitivity effect in adult patients. Since the dose escalation was terminated once AV block was seen, it is unclear if maximal dosing in all 80 patients would have resulted in an exaggerated response. The study design did not allow for answering these important questions but did show safety with their adenosine dose escalation protocol.  The authors discuss the roles of cardiac denervation and reinnervation as it may play a factor in these issues, but interesting the study showed safe adenosine effect as early as 25 days post-transplant and overall no correlation between time of transplant and effective adenosine dose suggesting other factors may play a role.

Clearly there are limitations to this study with the most notable being that patients are known to be healthy, were not in supraventricular tachycardia at the time of adenosine administration, and the route of adenosine administration was via central access. From a practical standpoint, adenosine is often given peripherally and used in an acute setting. Therefore, the effect of adenosine in the “healthy patient” is a less likely scenario as their rejection status and function may not be known and they may be on antiarrhythmics or other medications that may affect the response to adenosine. Nevertheless, this study demonstrates that adenosine may be used safely in this population but a cautious approach using low doses should be undertaken.

 

Additional EP Articles Worth Reading.

 

Intraoperative arrhythmias in children with congenital heart disease: transient, innocent events? Houck CA, Ramdjan TTTK, Yaksh A, Teuwen CP, Lanters EAH, Bogers AJJC, de Groot NMS.Europace. 2017 Jun 28. doi: 10.1093/europace/eux072. [Epub ahead of print]

PMID: 28666343

 

Cardiac Fibroblast Transcriptome Analyses Support a Role for Interferogenic, Profibrotic and Inflammatory Genes in Anti-SSA/Ro-Associated Congenital Heart Block.

Clancy RM, Markham AJ, Jackson T, Rasmussen SE, Blumenberg M, Buyon JP.

Am J Physiol Heart Circ Physiol. 2017 Jun 16:ajpheart.00256.2017. doi: 10.1152/ajpheart.00256.2017. [Epub ahead of print]

PMID: 28626076

 

Cardiac Arrhythmias in Adults with Congenital Heart Disease.

Balaji S, Mandapati R, Shivkumar K.

Card Electrophysiol Clin. 2017 Jun;9(2):xv-xvi. doi: 10.1016/j.ccep.2017.04.001. No abstract available.

PMID: 28457248

 

Genotype-Phenotype Correlation of SCN5A Mutation for the Clinical and Electrocardiographic Characteristics of Probands With Brugada Syndrome: A Japanese Multicenter Registry.

Yamagata K, Horie M, Aiba T, Ogawa S, Aizawa Y, Ohe T, Yamagishi M, Makita N, Sakurada H, Tanaka T, Shimizu A, Hagiwara N, Kishi R, Nakano Y, Takagi M, Makiyama T, Ohno S, Fukuda K, Watanabe H, Morita H, Hayashi K, Kusano K, Kamakura S, Yasuda S, Ogawa H, Miyamoto Y, Kapplinger JD, Ackerman MJ, Shimizu W.

Circulation. 2017 Jun 6;135(23):2255-2270. doi: 10.1161/CIRCULATIONAHA.117.027983. Epub 2017 Mar 24.

PMID: 28341781

 

 

CHD EP June 2017

 

  1. Intraoperative arrhythmias in children with congenital heart disease: transient, innocent events?

Houck CA, Ramdjan TTTK, Yaksh A, Teuwen CP, Lanters EAH, Bogers AJJC, de Groot NMS.

Europace. 2017 Jun 28. doi: 10.1093/europace/eux072. [Epub ahead of print]

PMID: 28666343

 

Select item 28660303

 

  1. Cardiac Fibroblast Transcriptome Analyses Support a Role for Interferogenic, Profibrotic and Inflammatory Genes in Anti-SSA/Ro-Associated Congenital Heart Block.

Clancy RM, Markham AJ, Jackson T, Rasmussen SE, Blumenberg M, Buyon JP.

Am J Physiol Heart Circ Physiol. 2017 Jun 16:ajpheart.00256.2017. doi: 10.1152/ajpheart.00256.2017. [Epub ahead of print]

PMID: 28626076

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  1. Sudden cardiac death in the young: Epidemiology and overview.

Link MS.

Congenit Heart Dis. 2017 Jun 15. doi: 10.1111/chd.12494. [Epub ahead of print]

PMID: 28618149

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  1. Sudden death in athletes.

Corrado D, Zorzi A.

Int J Cardiol. 2017 Jun 15;237:67-70. doi: 10.1016/j.ijcard.2017.03.034. Epub 2017 Mar 10.

PMID: 28318658

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  1. <i>Nkx2.5</i> is Essential to Establish Normal Heart Rate Variability in the Zebrafish Embryo.

Harrington JK, Sorabella R, Tercek A, Isler JR, Targoff KL.

Am J Physiol Regul Integr Comp Physiol. 2017 Jun 14:ajpregu.00223.2016. doi: 10.1152/ajpregu.00223.2016. [Epub ahead of print]

PMID: 28615160

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  1. Calcium Signaling and Cardiac Arrhythmias.

Landstrom AP, Dobrev D, Wehrens XHT.

Circ Res. 2017 Jun 9;120(12):1969-1993. doi: 10.1161/CIRCRESAHA.117.310083. Review.

PMID: 28596175

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  1. Permanent chronotropic impairment after closure of atrial or ventricular septal defect.

Heiberg J, Nyboe C, Hjortdal VE.

Scand Cardiovasc J. 2017 Jun 8:1-6. doi: 10.1080/14017431.2017.1337216. [Epub ahead of print]

PMID: 28592193

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  1. Cardiac Arrhythmias in Adults with Congenital Heart Disease.

Balaji S, Mandapati R, Shivkumar K.

Card Electrophysiol Clin. 2017 Jun;9(2):xv-xvi. doi: 10.1016/j.ccep.2017.04.001. No abstract available.

PMID: 28457248

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  1. Arrhythmia Surgery for Adults with Congenital Heart Disease.

Deal BJ, Mavroudis C.

Card Electrophysiol Clin. 2017 Jun;9(2):329-340. doi: 10.1016/j.ccep.2017.02.014. Review.

PMID: 28457246

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  1. Cardiac Arrhythmias in Adults with Congenital Heart Disease: Pacemakers, Implantable Cardiac Defibrillators, and Cardiac Resynchronization Therapy Devices.

Cecchin F, Halpern DG.

Card Electrophysiol Clin. 2017 Jun;9(2):319-328. doi: 10.1016/j.ccep.2017.02.013. Epub 2017 Mar 31. Review.

PMID: 28457245

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  1. Catheter Ablation: General Principles and Advances.

Ernst S.

Card Electrophysiol Clin. 2017 Jun;9(2):311-317. doi: 10.1016/j.ccep.2017.02.012. Epub 2017 Mar 14. Review.

PMID: 28457244

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  1. Drug Therapy in Adult Congenital Heart Disease.

Contractor T, Levin V, Mandapati R.

Card Electrophysiol Clin. 2017 Jun;9(2):295-309. doi: 10.1016/j.ccep.2017.02.011. Review.

PMID: 28457243

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  1. Sudden Cardiac Death in Adult Congenital Heart Disease.

Ávila P, Chaix MA, Mondésert B, Khairy P.

Card Electrophysiol Clin. 2017 Jun;9(2):225-234. doi: 10.1016/j.ccep.2017.02.003. Epub 2017 Mar 18. Review.

PMID: 28457237

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  1. Ventricular Arrhythmias in Adult Congenital Heart Disease: Mechanisms, Diagnosis, and Clinical Aspects.

Sathananthan G, Harris L, Nair K.

Card Electrophysiol Clin. 2017 Jun;9(2):213-223. doi: 10.1016/j.ccep.2017.02.004. Epub 2017 Mar 18. Review.

PMID: 28457236

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  1. Supraventricular Tachycardia in Adult Congenital Heart Disease: Mechanisms, Diagnosis, and Clinical Aspects.

Janson CM, Shah MJ.

Card Electrophysiol Clin. 2017 Jun;9(2):189-211. doi: 10.1016/j.ccep.2017.02.005. Review.

PMID: 28457235

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  1. Bradyarrhythmias in Congenital Heart Disease.

Carlson SK, Patel AR, Chang PM.

Card Electrophysiol Clin. 2017 Jun;9(2):177-187. doi: 10.1016/j.ccep.2017.02.002. Epub 2017 Mar 22. Review.

PMID: 28457234

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  1. Introduction to the Congenital Heart Defects: Anatomy of the Conduction System.

Moore JP, Aboulhosn JA.

Card Electrophysiol Clin. 2017 Jun;9(2):167-175. doi: 10.1016/j.ccep.2017.02.001. Epub 2017 Mar 14. Review.

PMID: 28457233

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  1. Ventricular pacing in single ventricles-A bad combination.

Bulic A, Zimmerman FJ, Ceresnak SR, Shetty I, Motonaga KS, Freter A, Trela AV, Hanisch D, Russo L, Avasarala K, Dubin AM.

Heart Rhythm. 2017 Jun;14(6):853-857. doi: 10.1016/j.hrthm.2017.03.035.

PMID: 28528723

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

Wiles BM, Fitzsimmons SJ, Roberts PR.

Pacing Clin Electrophysiol. 2017 Jun;40(6):735-737. doi: 10.1111/pace.13089. Epub 2017 May 16. 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 Jun;38(5):1004-1009. doi: 10.1007/s00246-017-1608-7. Epub 2017 Apr 3.

PMID: 28374048

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  1. Measures of and changes in heart rate variability in pediatric heart transplant recipients.

Williams T, Tang X, Gilmore G, Gossett J, Knecht KR.

Pediatr Transplant. 2017 Jun;21(4). doi: 10.1111/petr.12894. Epub 2017 Feb 8.

PMID: 28181355

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  1. [News techniques of pacing in congenital heart diseases].

Le Bloa M, Thambo JB.

Presse Med. 2017 Jun;46(6 Pt 1):594-605. doi: 10.1016/j.lpm.2017.05.015. Epub 2017 Jun 5. French.

PMID: 28595994

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  1. Postnatal Outcomes of Fetal Supraventricular Tachycardia: a Multicenter Study.

Hinkle KA, Peyvandi S, Stiver C, Killen SAS, Weng HY, Etheridge SP, Puchalski MD.

Pediatr Cardiol. 2017 Jun 29. doi: 10.1007/s00246-017-1662-1. [Epub ahead of print]

PMID: 28664446

 

Select item 28664445

 

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

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

Circulation. 2017 Jun 20;135(25):2485-2493. doi: 10.1161/CIRCULATIONAHA.117.028087. Epub 2017 Apr 27.

PMID: 28450351

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  1. Right Atrial Diverticulosis and Early-onset Arrhythmia: Rare Cause of Incessant Neonatal Arrhythmia.

Aggarwal N, Joshi R, Joshi RK, Agarwal M.

Indian Pediatr. 2017 Jun 15;54(6):503-504.

PMID: 28667725

 

Select item 28620681

 

  1. Variation in Pediatric Post-Ablation Care: A Survey of the Pediatric and Congenital Electrophysiology Society (PACES).

Dechert BE, Dick M 2nd, Bradley DJ, LaPage MJ.

Pediatr Cardiol. 2017 Jun 15. doi: 10.1007/s00246-017-1654-1. [Epub ahead of print]

PMID: 28620754

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  1. Comparison of 6-mm Versus 8-mm-Tip Cryoablation Catheter for the Treatment of Atrioventricular Nodal Reentrant Tachycardia in Children: A Prospective Study.

Tuzcu V, Gul EE, Karacan M, Kamali H, Celik N, Akdeniz C.

Pediatr Cardiol. 2017 Jun 13. doi: 10.1007/s00246-017-1648-z. [Epub ahead of print]

PMID: 28612086

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  1. Genotype-Phenotype Correlation of SCN5A Mutation for the Clinical and Electrocardiographic Characteristics of Probands With Brugada Syndrome: A Japanese Multicenter Registry.

Yamagata K, Horie M, Aiba T, Ogawa S, Aizawa Y, Ohe T, Yamagishi M, Makita N, Sakurada H, Tanaka T, Shimizu A, Hagiwara N, Kishi R, Nakano Y, Takagi M, Makiyama T, Ohno S, Fukuda K, Watanabe H, Morita H, Hayashi K, Kusano K, Kamakura S, Yasuda S, Ogawa H, Miyamoto Y, Kapplinger JD, Ackerman MJ, Shimizu W.

Circulation. 2017 Jun 6;135(23):2255-2270. doi: 10.1161/CIRCULATIONAHA.117.027983. Epub 2017 Mar 24.

PMID: 28341781

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  1. “Chaotic Arrhythmia” During Successful Resuscitation After Ingestion of Yew (Taxus baccata) Needles.

Zutter A, Hauri K, Evers KS, Uhde S, Fassl J, Reuthebuch OT, Berset A, Kühne M, Donner BC.

Pediatr Emerg Care. 2017 Jun 6. doi: 10.1097/PEC.0000000000001196. [Epub ahead of print]

PMID: 28590987

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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 Jun;173(6):1495-1501. doi: 10.1002/ajmg.a.38191. Epub 2017 Apr 10.

PMID: 28394409

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  1. Cost Analysis of Patients Referred for Inherited Heart Rhythm Disorder Evaluation.

Janzen ML, Cheung C, Sanatani S, Cunningham T, Kerr C, Steinberg C, Sherwin E, Arbour L, Deyell MW, Andrade JG, Lehman AM, Gula LJ, Krahn AD.

Can J Cardiol. 2017 Jun;33(6):814-821. doi: 10.1016/j.cjca.2016.12.009. Epub 2016 Dec 20.

PMID: 28347582

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  1. Exome sequencing identifies primary carnitine deficiency in a family with cardiomyopathy and sudden death.

Lahrouchi N, Lodder EM, Mansouri M, Tadros R, Zniber L, Adadi N, Clur SB, van Spaendonck-Zwarts KY, Postma AV, Sefiani A, Ratbi I, Bezzina CR.

Eur J Hum Genet. 2017 Jun;25(6):783-787. doi: 10.1038/ejhg.2017.22. Epub 2017 Mar 15.

PMID: 28295041

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  1. Vectorcardiography identifies patients with electrocardiographically concealed long QT syndrome.

Cortez D, Bos JM, Ackerman MJ.

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PMID: 28279743

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  1. Single-incision percutaneous pericardial ICD lead placement in a piglet model.

Clark BC, Opfermann JD, Davis TD, Krieger A, Berul CI.

J Cardiovasc Electrophysiol. 2017 Jun 1. doi: 10.1111/jce.13263. [Epub ahead of print]

PMID: 28569424

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  1. KCNQ1 p.L353L affects splicing and modifies the phenotype in a founder population with long QT syndrome type 1.

Kapplinger JD, Erickson A, Asuri S, Tester DJ, McIntosh S, Kerr CR, Morrison J, Tang A, Sanatani S, Arbour L, Ackerman MJ.

J Med Genet. 2017 Jun;54(6):390-398. doi: 10.1136/jmedgenet-2016-104153. Epub 2017 Mar 6.

PMID: 28264985 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 Jun;40(6):745-747. doi: 10.1111/pace.13083. Epub 2017 May 16. No abstract available.

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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.

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

Pediatr Cardiol. 2017 Jun;38(5):909-914. doi: 10.1007/s00246-017-1596-7. Epub 2017 Mar 7.

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  1. Catecholaminergic Polymorphic Ventricular Tachycardia.

Wall JJ, Iyer RV.

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White MJ, Loccoh EC, Goble MM, Yu S, Odetola FO, Russell MW.

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CHD Intervention Featured Articles of June 2017

 

Interventional Cardiology Reviews of June 2017 Manuscripts

 

Safety, Feasibility, Results, and Economic Impact of Common Interventional Procedures in a Low-Volume Region of the United States.

Clem A, Awadallah S, Amin Z.

Pediatr Cardiol. 2017 Jun 21. doi: 10.1007/s00246-017-1664-z. [Epub ahead of print]

PMID: 28639149

 

Take Home Points:

  • Many patients that live in rural areas may incur an undue financial burden (personal and societal) related to travel and associated expenses to undergo relatively simple transcatheter interventions for common congenital heart defects
  • The authors present their experience with outreach interventional pediatric cardiology to reduce this burden on the patients and families and found a high success rate with minimal complications and high patient and family satisfaction
  • This study reports important findings that can improve the care and reduce the costs of treatment for patients that live in rural areas in the United States

Seckler, MikeCommentary from Dr. Michael Seckeler (Tucson), section editor of Interventional Cardiology Journal Watch:  Clem and colleagues present a retrospective review of their 10-year, single center experience utilizing outreach interventional pediatric cardiology to perform “simple” transcatheter interventions (mostly ASD and PDA closure). They utilized this management strategy to reduce the need to transport rural patients to larger centers for their procedures.

The analysis showed comparable success rates to other published reports of multi-center experience (IMPACT, MAGIC and C3PO registries). Importantly, they followed up with a subset of the families to assess patient satisfaction with receiving their care locally, with most respondents being quite satisfied. Finally, they looked at the potential cost savings for South Dakota, which was estimated at $3.4 million over the entire study period.

This study is important because it demonstrates the safety and success of performing simple transcatheter interventions at small centers without readily available surgical backup. Given the large number of patients that do not live near large pediatric medical centers, this is a viable option to provide their care without disrupting the family by out of state travel and the associated social and financial stressors. In addition, there is high potential cost savings for the local medical systems which can reduce overall healthcare expenditures without compromising patient care.

Twenty years of experience with intraoperative pulmonary artery stenting.

Zampi JD, Loccoh E, Armstrong AK, Yu S, Lowery R, Rocchini AP, Hirsch-Romano JC.

Catheter Cardiovasc Interv. 2017 May 4. doi: 10.1002/ccd.27094. [Epub ahead of print]

PMID: 28471080

 

Take Home Points:

  • Intraoperative pulmonary artery stent implantation is technically feasible with low procedural, stent-related complications.
  • Intraoperative stent implantation allows implantation of adult-size stents in small patients while avoiding injury to peripheral vessels, eliminates radiation exposure and positions stents to facilitate future percutaneous dilation.
  • Reintervention in these patients is common.

Averin , KonstantinCommentary from Dr. Konstantin Averin (Edmonton), section editor of Interventional Cardiology Journal Watch:   Branch pulmonary artery (PA) stenosis is a relatively common problem in congenital heart disease. Percutaneous techniques have provided good outcomes, but difficulties can arise when medium/large caliber stents are necessary in small patients. Intra-operative PA stent placement can be an alternative to exclusively trans-catheter or surgical therapy. The authors sought to describe their experience with intraoperative PA stent placement over a 20-year period and to examine long term outcomes, specifically focusing on the incidence of and risk factors for reintervention, due to recurrent PA stenosis.

Eighty-one PA stent were placed in 68 patients between 1994 and 2013 with 84% of patients receiving a stent implant as part of a procedure to palliate another cardiac defect. A left PA (59%) and proximal stenosis (85%) were most commonly treated. Stent implantation was acutely successful in all patients but one, with Palmaz XL Transhepatic Biliary Stent (58%) and ev3 Intrastent Mega LD Biliary stent (29%) being most commonly utilized.

During a median follow up of 6 years, 30 patients underwent reintervention on the intraoperatively placed stents (9 intraoperative dilation, 21 percutaneous) with a median time to first reintervention of 2.6 years. The majority of patients required reintervention for either in-stent stenosis (53%) or for somatic growth (30%). The overall freedom from reintervention was 83%, 74%, 51%, and 30% at 1, 2, 5 and 10 years respectively. A univariate cox regression analysis showed that age < 18 months (HR 2.97), BSA < 0.47 m2 (HR 3.20), and a cardiac diagnosis of TOF with MAPCAs (HR 4.61) had an increased risk of reintervention. There were no deaths or long-term complications related to intraoperative stent placement.

The authors conclude that intraoperative pulmonary artery stent implantation is technically feasible with low procedural, stent-related complications.  Intraoperative stent implantation allows implantation of adult-size stents in small patients while avoiding injury to peripheral vessels, eliminates radiation exposure and positions stents to facilitate future percutaneous dilation. Reintervention in these patients is common.

 

 

Outcomes After Decompression of the Right Ventricle in Infants with Pulmonary Atresia with Intact Ventricular Septum are Associated with Degree of Tricuspid Regurgitation: Results from the Congenital Catheterization Research Collaborative.

Petit CJ, Glatz AC, Qureshi AM, Sachdeva R, Maskatia SA, Justino H, Goldberg DJ, Mozumdar N, Whiteside W, Rogers LS, Nicholson GT, McCracken C, Kelleman M, Goldstein BH.

Circ Cardiovasc Interv. 2017 May;10(5). pii: e004428. doi: 10.1161/CIRCINTERVENTIONS.116.004428.

PMID: 28500137

 

Take Home Points:

  • In neonates with pulmonary atresia-intact ventricular septum, severity of tricuspid regurgitation pre-intervention is strongly associated with reintervention after RV decompression and ultimate circulatory status (biventricular v not).
  • Tricuspid regurgitation severity was associated with tricuspid valve annulus dimension and tricuspid inflow duration, suggesting that the physiology of moderate to severe TR may be favorable for RV development and growth.
  • Most patients who undergo RV decompression achieve biventricular circulation.

Averin , KonstantinCommentary from Dr. Konstantin Averin (Edmonton), section editor of Interventional Cardiology Journal Watch:  Pulmonary atresia with intact ventricular septum (PA-IVS) is a rare form of congenital heart disease marked by hypoplasia of the right ventricle (RV). Due to the sub-optimal outcomes associated with single ventricle physiology, decompression of the RV is pursued whenever possible in the hopes of achieving a 2 ventricle (2 V) circulation. Risk factors for not achieving 2 V circulation have only been studied in small cohort sizes and single-center experiences. The aim of this study was to determine broadly applicable risk factors for reintervention and for failure to achieve 2 V circulation in a large, multicenter cohort of patients.

From 2005-2015, 231 neonates were diagnosed with PA-IVS, 128 underwent BTS placement or primary heart transplantation, with the remaining 103 undergoing planned RV decompression at a median age of 3 days. Two patients died during the procedure and 2 had Ebstein’s anomaly (excluded), so the final cohort consisted of 99 patients (28 having ‘virtual atresia’ – pinhole discovered during the procedure that was not seen on pre-procedure echo). Freedom from reintervention was 51%, 27% and 23% at 1 month, 1 year, and 3 years post-RV decompression respectively, with 72% of patients undergoing at least 1 reintervention after decompression (to increase pulmonary blood flow or modify the RV outflow tract).

Having less than mild TR a significant predictive factor and was associated with needing reintervention (HR 3.58), number of reinterventions (HR 1.87), needing an additional source of pulmonary blood flow (HR 3.50), and not achieving 2 V circulation (OR 18.6). Other significant factors associated with needing reinterventions were virtual atresia (HR 0.51) and smaller RV length (HR 0.94); with needing an additional source of pulmonary blood flow was having virtual atresia (HR 0.36 – protective) and with not achieving a 2 V circulation was having a lower RV area (OR 0.81). The median duration of follow-up post-RV decompression was 3.0 years and at latest follow up 85% of patients had 2 V circulation.

The authors conclude that in neonates with PA-IVS who undergo RV decompression have a high burden of reintervention although most achieve 2 V circulation. Neonates with ≤mild TR before decompression seem to be at higher risk of reintervention and of not achieving 2V circulation in the medium term. TR may play an important role physiologically in the development of the RV in PA-IVS.

The association between TR to RV development has been reported previously in both fetal studies and post-natal studies but this is the first paper to pool data from multiple centers (as part of the Congenital Catheterization Research Collaborative) to develop a large data set of a rare cardiac lesion. The authors are to be commended for their contribution to the literature and their collaboration should serve as an example on how to successfully study rare defects in pediatric cardiology.

 

 

 

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

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

Pediatr Cardiol. 2017 Jun;38(5):1016-1023. doi: 10.1007/s00246-017-1611-z. Epub 2017 Apr 10.

PMID: 28396934

Similar articles

Select item 28396933

 

Take Home Points:

  • Transverse arch hypoplasia can occur in association with coarctation of the aorta.
  • Stent therapy for coarctation of the aorta generally does not address the hypoplastic transverse arch and, despite remodeling post-coarctation stent placement, the transverse arch may still prove to be problematic in follow-up.
  • At follow-up (median 37 months) after successful coarctation stent placement, there was no significant catch up growth of the transverse arch by echocardiography and elevated right arm blood pressure persisted.

 

Whiteside, WendyComment from Dr. Wendy Whiteside (Cincinnati), section editor of Congenital Heart Disease Interventions Journal Watch: Addressing coarctation of the aorta in the setting of transverse arch hypoplasia can be difficult.  Surgical repair under a month of age has been associated with improved arch growth, however it is unclear whether transverse arch growth occurs in the setting of coarctation stenting.  Generally, in these patients, this stent is placed more distal in the isthmus, leaving the transverse arch untreated as addressing the transverse arch percutaneously, would require stenting of the transverse arch directly, with stent material crossing other head and neck vessels. This then brings into question whether this area needs to be treated at the time of coarctation stenting—whether the transverse arch may grow now in the setting of an unobstructed distal vessel or whether this may play any role in development of systemic hypertension long-term. Lu et al provide the first systematic look at the clinical significance of transverse aortic arch hypoplasia in children following stent therapy for coarctation of the aorta.  They retrospectively reviewed 51 children who underwent stent placement for coarctation of the aorta, who were also found to have transverse arch hypoplasia (based on angiography).  Over their 20-year experience, this cohort represented about 1/3 of patients.

As expected, the average coarctation to descending aortic dimension increased significantly and the peak to peak systolic gradient decreased significantly following stent placement. More interesting, however, is that over the median follow-up of 37 months, there was not a significant increase in transverse arch z-score to suggest any catch up growth of the transverse arch. Additionally, while there was significant

 

 

CHD Interventions June 2017

 

  1. Aortic perfusion score for pulmonary atresia with intact ventricular septum: An antegrade coronary perfusion scoring system that is predictive of need for transplant and mortality.

Loomba RS, Pelech AN.

Congenit Heart Dis. 2017 Jun 27. doi: 10.1111/chd.12510. [Epub ahead of print]

PMID: 28653340

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Select item 28653463

 

  1. Real-time Three-dimensional Echocardiography: From Diagnosis to Intervention.

Orvalho JS.

Vet Clin North Am Small Anim Pract. 2017 Jun 29. pii: S0195-5616(17)30055-4. doi: 10.1016/j.cvsm.2017.05.003. [Epub ahead of print] Review.

PMID: 28669434

 

Select item 28662517

 

  1. The effect of balloon valvuloplasty for bioprosthetic valve stenosis at pulmonary positions.

Choi EY, Song J, Lee H, Lee CH, Huh J, Kang IS, Yang JH, Jun TG.

Congenit Heart Dis. 2017 Jun 23. doi: 10.1111/chd.12507. [Epub ahead of print]

PMID: 28643385

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Select item 28646501

 

  1. QRS Width as a Predictor of Right Ventricular Remodeling After Percutaneous Pulmonary Valve Implantation.

Paech C, Dähnert I, Riede FT, Wagner R, Kister T, Nieschke K, Wagner F, Gebauer RA.

Pediatr Cardiol. 2017 Jun 19. doi: 10.1007/s00246-017-1658-x. [Epub ahead of print]

PMID: 28631207

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Select item 28631156

 

  1. Transcatheter Treatment of Aortopulmonary Window with a Symmetrical Membranous Ventricular Septal Occluder.

Xu HX, Zheng DD, Pan M, Li XF.

Cardiology. 2017 Jun 14;138(2):76-79. doi: 10.1159/000475706. [Epub ahead of print]

PMID: 28609765

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Select item 28614966

 

  1. Catheter-based anatomic and functional assessment of coronary arteries in anomalous aortic origin of a coronary artery, myocardial bridges and Kawasaki disease.

Qureshi AM, Agrawal H.

Congenit Heart Dis. 2017 Jun 13. doi: 10.1111/chd.12500. [Epub ahead of print]

PMID: 28608519

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Select item 28608434

 

  1. Therapeutic catheterization in congenital heart disease: reflections on the value of risk scores.

Uebing A, Gatzoulis MA, Rigby ML.

Eur Heart J. 2017 Jun 13. doi: 10.1093/eurheartj/ehx299. [Epub ahead of print] No abstract available.

PMID: 28633370

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Select item 28633352

 

  1. Noninfective Transcatheter Pulmonary Valve Thrombosis: A Rare Cause of Post-Implantation Pulmonary Valve Obstruction.

Verhoeven PA, Learn CP, Brown NM, Goldstein BH.

JACC Cardiovasc Interv. 2017 Jun 7. pii: S1936-8798(17)30768-9. doi: 10.1016/j.jcin.2017.04.009. [Epub ahead of print] No abstract available.

PMID: 28624383

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Select item 28586404

 

  1. Interventional Treatment of Patients With Congenital Heart Disease: Nationwide Danish Experience Over 39 Years.

Larsen SH, Olsen M, Emmertsen K, Hjortdal VE.

J Am Coll Cardiol. 2017 Jun 6;69(22):2725-2732. doi: 10.1016/j.jacc.2017.03.587.

PMID: 28571637

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Select item 28588119

 

  1. Multicenter midterm follow-up results using the gore septal occluder for atrial septal defect closure in pediatric patients.

Grohmann J, Wildberg C, Zartner P, Abu-Tair T, Tarusinov G, Kitzmüller E, Schmoor C, Stiller B, Kampmann C.

Catheter Cardiovasc Interv. 2017 Jun 1;89(7):E226-E232. doi: 10.1002/ccd.26881. Epub 2016 Nov 30.

PMID: 27900839

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Select item 28473349

 

  1. Simultaneous transcatheter therapy for perimembranous ventricular septal defect combined with patent ductus arteriosus.

Wang Q, Zhu X, Duanzhen Z, Zhang P, Chen H, Han X, Sheng X, Meng L.

J Card Surg. 2017 Jun;32(6):370-375. doi: 10.1111/jocs.13138. Epub 2017 May 11.

PMID: 28497553

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Select item 28493392

 

  1. Percutaneous management of failed bioprosthetic pulmonary valves in patients with congenital heart defects.

Tarzia P, Conforti E, Giamberti A, Varrica A, Giugno L, Micheletti A, Negura D, Piazza L, Saracino A, Carminati M, Chessa M.

J Cardiovasc Med (Hagerstown). 2017 Jun;18(6):430-435. doi: 10.2459/JCM.0000000000000486.

PMID: 27828833

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Select item 23314595

 

  1. Short-term cardiopulmonary efficiency improvement after transcatheter baffle leak closure in a Mustard-operated patient.

Mezzani A, Butera G, Chessa M, Micheletti A, Negura D, Calaciura R, Carminati M.

J Cardiovasc Med (Hagerstown). 2017 Jun;18(6):447-449. doi: 10.2459/JCM.0b013e32835dbbde. No abstract available.

PMID: 23314595

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Select item 28436940

 

  1. Perceptions of transcatheter device closure of patent ductus arteriosus in veterinary cardiology and evaluation of a canine model to simulate device placement: a preliminary study.

Saunders AB, Keefe L, Birch SA, Wierzbicki MA, Maitland DJ.

J Vet Cardiol. 2017 Jun;19(3):268-275. doi: 10.1016/j.jvc.2017.04.002. Epub 2017 May 31.

PMID: 28576477

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Select item 28571753

 

  1. Real-time 3D transesophageal echocardiography-guided closure of a complicated patent ductus arteriosus in a dog.

Doocy KR, Nelson DA, Saunders AB.

J Vet Cardiol. 2017 Jun;19(3):287-292. doi: 10.1016/j.jvc.2017.04.001. Epub 2017 May 29.

PMID: 28571753

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Select item 28403448

 

  1. Utility and Safety of Combined Interventional Catheterization and Electrophysiology Procedures in a Children’s Hospital.

Asaki SY, Orcutt JW, Miyake CY, Justino H, de la Uz CM, Kim JJ, Valdes SO, Qureshi AM.

Pacing Clin Electrophysiol. 2017 Jun;40(6):661-666. doi: 10.1111/pace.13047. Epub 2017 May 3.

PMID: 28191656

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Select item 28599027

 

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

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

Pediatr Cardiol. 2017 Jun;38(5):1084-1086. doi: 10.1007/s00246-017-1613-x. Epub 2017 Apr 10.

PMID: 28396933

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Select item 28382463

 

  1. [Pulmonary revalvulation and rhythmologenic risks in patients with repaired tetralogy of Fallot].

Pilato R, Lacroix D, Domanski O, Godart F.

Presse Med. 2017 Jun;46(6 Pt 1):586-593. doi: 10.1016/j.lpm.2017.02.006. Epub 2017 Jun 2. French.

PMID: 28583744

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Select item 28576635

 

  1. RV to PA conduits: impact of transcatheter pulmonary valve replacement in adults – a national register study.

Skoglund K, Svensson G, Thilén U, Dellborg M, Eriksson P.

Scand Cardiovasc J. 2017 Jun;51(3):153-158. doi: 10.1080/14017431.2017.1291988. Epub 2017 Feb 15.

PMID: 28276717

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  1. Safety, Feasibility, Results, and Economic Impact of Common Interventional Procedures in a Low-Volume Region of the United States.

Clem A, Awadallah S, Amin Z.

Pediatr Cardiol. 2017 Jun 21. doi: 10.1007/s00246-017-1664-z. [Epub ahead of print]

PMID: 28639149

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  1. Mid-term results of percutaneous ventricular septal defect closure with Amplatzer Duct Occluder-II in children.

Pamukcu O, Narin N, Baykan A, Sunkak S, Tasci O, Uzum K.

Cardiol Young. 2017 Jun 20:1-6. doi: 10.1017/S104795111700107X. [Epub ahead of print]

PMID: 28631576

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

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

Pediatr Cardiol. 2017 Jun 7. doi: 10.1007/s00246-017-1643-4. [Epub ahead of print] No abstract available.

PMID: 28589406

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Select item 28589405

 

  1. Drug-Coated Balloon Angioplasty: A Novel Treatment for Pulmonary Artery In-Stent Stenosis in a Patient with Williams Syndrome.

Cohen JL, Glickstein JS, Crystal MA.

Pediatr Cardiol. 2017 Jun 7. doi: 10.1007/s00246-017-1646-1. [Epub ahead of print]

PMID: 28589405

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Select item 28592829

 

  1. Primary Coiling of the Left Ventricle in Hypoplastic Left Heart With Ventriculo-Coronary Connections.

Hummel J, Stiller B, Kroll J, Grohmann J.

Ann Thorac Surg. 2017 Jun;103(6):e559. doi: 10.1016/j.athoracsur.2017.01.057. No abstract available.

PMID: 28528069

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Select item 28262297

 

  1. Novel delivery technique for atrial septal defect closure in young children utilizing the GORE® CARDIOFORM® septal occluder.

Anderson JH, Fraint H, Moore P, Cabalka AK, Taggart NW.

Catheter Cardiovasc Interv. 2017 Jun 1;89(7):1232-1238. doi: 10.1002/ccd.26874. Epub 2016 Nov 29.

PMID: 27896910

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Select item 28398850

 

  1. Multicenter Experience Evaluating Transcatheter Pulmonary Valve Replacement in Bovine Jugular Vein (Contegra) Right Ventricle to Pulmonary Artery Conduits.

Morray BH, McElhinney DB, Boudjemline Y, Gewillig M, Kim DW, Grant EK, Bocks ML, Martin MH, Armstrong AK, Berman D, Danon S, Hoyer M, Delaney JW, Justino H, Qureshi AM, Meadows JJ, Jones TK.

Circ Cardiovasc Interv. 2017 Jun;10(6). pii: e004914. doi: 10.1161/CIRCINTERVENTIONS.116.004914.

PMID: 28600328

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Select item 28167306

 

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

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

Pediatr Cardiol. 2017 Jun;38(5):1016-1023. doi: 10.1007/s00246-017-1611-z. Epub 2017 Apr 10.

PMID: 28396934

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Select item 28396933

 

  1. [Closure of wide patent ductus arteriosus using a fenestrated muscular VSD occluder device in a pediatric patient with Down syndrome and pulmonary hypertension].

Güvenç O, Saygı M, Demir İH, Ödemiş E.

Turk Kardiyol Dern Ars. 2017 Jun;45(4):373-376. doi: 10.5543/tkda.2016.38283. Turkish.

PMID: 28595211 Free Article

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Select item 28614860

 

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CHD Surgery Featured Articles of June 2017

Congenital Heart Surgery Reviews of June 2017 Manuscripts

Ventricular assist devices for the failing univentricular circulation.

Buratto E, Shi WY, Ye XT, Konstantinov IE.

Expert Rev Med Devices. 2017 Jun;14(6):449-459. doi: 10.1080/17434440.2017.1332523. Epub 2017 May 26.

PMID: 28532240

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Select item 28266898

 

Take Home Points:

  • Failing single ventricle physiology presents unique challenges for mechanical circulatory support, especially when ventricular function is preserved but systemic venous pressures are elevated.
  • Applications and limitations of existing device technologies are reviewed and emerging technologies (e.g., cavopulmonary assistance) are discussed.

 

jeremy-herrmannCommentary from Dr. Jeremy Herrmann (Indianapolis), section editor of Congenital Heart Surgery Journal Watch:  As success of palliation for single ventricle palliation has improved over the past nearly 30 years, more patients are confronting the long-term challenges of this physiologic state including heart failure. Accordingly, options other than heart transplantation have historically been limited though there has been recent momentum in applying existing mechanical assistance technologies as well as developing novel technologies. An important consideration of failing single ventricle physiology is the distinction between those patients with ventricular dysfunction and those without. In the former, existing devices designed to augment ventricular function may be useful. However, these devices may have limited applicability for patients with preserved ventricular function but elevated systemic venous pressures.

This paper provides an excellent update of device therapies for patients with failing single ventricle physiology after any stage of palliation. The authors summarize key physiologic concepts and the experience to-date of mechanical circulatory support including pulsatile devices, continuous flow devices, and the total artificial heart. Limitations of existing device therapies for failing Fontan patients without ventricular function are discussed as related to their inability to mitigate elevated systemic venous pressures. Future directions for cavopulmonary assist devices (e.g., the Rodefeld pump) and other evolving technologies are also highlighted.

 

 

 

Long-Term Follow-Up After the Ross Procedure: A Single Center 22-Year Experience.

Schneider AW, Putter H, Klautz RJM, Bruggemans EF, Holman ER, Bökenkamp R, Hazekamp MG.

Ann Thorac Surg. 2017 Jun;103(6):1976-1983. doi: 10.1016/j.athoracsur.2016.11.064. Epub 2017 Feb 24.

PMID: 28242079

Similar articles

Select item 28223051

 

Take Home Points:

  • Excellent long-term outcomes can be achieved with the Ross aortic valve replacement procedure in pediatric and young adult patients and should be considered a viable option along with other bioprosthetic and mechanical valves.
  • The need for reoperation for pulmonary homografts is greater than for the autograft aortic root, but comparisons across eras with different surgical techniques and implants are difficult.
  • Prospective, multi-institutional studies comparing all options for aortic valve replacement in pediatric and young adult patients are still needed.

 

Commentary from Dr. Jeremy Herrmann (Indianapolis), section editor of Congenital Heart Surgery Journal Watch:  The choice of technique for replacing a stenotic and/or regurgitant native aortic valve in a pediatric or young adult patient can be challenging. Bioprosthetic valves do not require long-term anticoagulation but have very limited durability in younger patients. Mechanical valves are more durable but require anticoagulation. Both bioprosthetic and mechanical valves also may be limited by lack of growth potential. Advantages of the Ross procedure (pulmonary autograft replacement of the aortic valve) include growth potential, avoidance of long-term anticoagulation, and favorable hemodynamics compared to prosthetic valves. However, the Ross procedure involves replacement of two valves and a more technically demanding operation. As a result, the Ross procedure tends to be performed in a minority of congenital surgical programs that perform it with regularity and maintain clinical competence with it.

The group from Leiden University is the latest to offer a report of their long-term institutional experience with the Ross procedure. Over a 22-year period, they performed 105 Ross procedures and 49 Ross-Konno procedures. The median patient ages were 14 years (range, 8-25 years) for the Ross procedure and 1 year (range, 0.25-9 years) for the Ross-Konno procedure. Overall survival was good (and similar to other reports) with 20-year survival rates of 90.7% for the Ross group and 70% for the Ross-Konno group (see Figure). Of the 26 patients (18%) who required reoperation for the autograft, 20 were related to autograft dilatation. As with many other centers, their technique for autograft implantation has evolved to counter long-term aortic root dilation. However, surgical techniques were not compared, though the authors observed no difference between age and time and autograft reoperation. Thirty-one patients (21%) required pulmonary homograft reoperation. For the Ross group, the 5- and 20-year incidence of pulmonary homograft reoperation was 5.8% and 30.5%, respectively. The need for pulmonary homograft replacement was predictably higher in the younger Ross-Konno group with a 15-year incidence of RV-PA conduit replacement of 50.5%. Only four patients in the Ross group developed endocarditis, all with the pulmonary homograft.

The major limitations of this study are its single institutional experience, retrospective nature, and potentially strong selection bias given their preference for the Ross procedure in this patient population. In addition, the lack of comparison with other similarly aged prosthetic aortic valve replacement patients makes it difficult to judge their long-term results. However, their results are consistent with other reports and indicate the Ross procedure is a viable option for younger patients who cannot take or wish to avoid long-term anticoagulation. Future prospective, contemporary comparisons (as with the German Ross Registry) may help to clarify these decisions and facilitate patient counseling.

June surgery v1

 

 

 

 

 

CHD surgery June 2017

 

  1. The role of EEG recordings in children undergoing cardiac surgery for congenital heart disease.

Meyer S, Poryo M, Shatat M, Gortner L, Abdul-Khaliq H.

Wien Med Wochenschr. 2017 Jun 28. doi: 10.1007/s10354-017-0576-0. [Epub ahead of print]

PMID: 28660303

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Select item 28653809

 

  1. Does hypothermia impair cerebrovascular autoregulation in neonates during cardiopulmonary bypass?

Smith B, Vu E, Kibler K, Rusin C, Easley RB, Andropoulos D, Heinle J, Czosnyka M, Licht D, Lynch J, Brady K.

Paediatr Anaesth. 2017 Jun 27. doi: 10.1111/pan.13194. [Epub ahead of print]

PMID: 28653463

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Select item 28658196

 

  1. Late-Term Gestation Is Associated With Improved Survival in Neonates With Congenital Heart Disease Following Postoperative Extracorporeal Life Support.

McKenzie JM, Scodellaro T, d’Udekem Y, Chiletti R, Butt W, Namachivayam SP.

Pediatr Crit Care Med. 2017 Jun 27. doi: 10.1097/PCC.0000000000001249. [Epub ahead of print]

PMID: 28658196

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Select item 28653300

 

  1. Safety and effectiveness of tolvaptan for fluid management after pediatric cardiovascular surgery.

Katayama Y, Ozawa T, Shiono N, Masuhara H, Fujii T, Watanabe Y.

Gen Thorac Cardiovasc Surg. 2017 Jun 24. doi: 10.1007/s11748-017-0798-5. [Epub ahead of print]

PMID: 28647800

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Select item 28654552

 

  1. Severe Upper Airway Obstruction After Intraoperative Transesophageal Echocardiography in Pediatric Cardiac Surgery-A Retrospective Analysis.

Michel J, Hofbeck M, Schineis C, Kumpf M, Heimberg E, Magunia H, Schmid E, Schlensak C, Blumenstock G, Neunhoeffer F.

Pediatr Crit Care Med. 2017 Jun 24. doi: 10.1097/PCC.0000000000001252. [Epub ahead of print]

PMID: 28654552

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Select item 28643420

 

  1. Progressive aortic root dilatation in pediatric heart transplant recipients.

Sachdeva S, Zhang L, Simpson P, Frommelt PC.

Echocardiography. 2017 Jun 23. doi: 10.1111/echo.13570. [Epub ahead of print]

PMID: 28646501

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Select item 28641300

 

  1. Long-term outcome after right ventricle to pulmonary artery conduit surgery and reintervention.

Skoglund K, Svensson G, Thilén U, Dellborg M, Eriksson P.

Scand Cardiovasc J. 2017 Jun 23:1-8. doi: 10.1080/14017431.2017.1343492. [Epub ahead of print]

PMID: 28645241

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Select item 28648597

 

  1. Outcome of cardiac surgery in patients with congenital heart disease in England between 1997 and 2015.

Kempny A, Dimopoulos K, Uebing A, Diller GP, Rosendahl U, Belitsis G, Gatzoulis MA, Wort SJ.

PLoS One. 2017 Jun 19;12(6):e0178963. doi: 10.1371/journal.pone.0178963. eCollection 2017.

PMID: 28628610 Free Article

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Select item 28627279

 

  1. Effect of Obesity and Underweight Status on Perioperative Outcomes of Congenital Heart Operations in Children, Adolescents, and Young Adults: An Analysis of Data from the Society of Thoracic Surgeons Database.

O’Byrne ML, Kim S, Hornik CP, Yerokun BA, Matsouaka RA, Jacobs JP, Jacobs ML, Jonas RA.

Circulation. 2017 Jun 16. pii: CIRCULATIONAHA.116.026778. doi: 10.1161/CIRCULATIONAHA.116.026778. [Epub ahead of print]

PMID: 28626087

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Select item 28621042

 

  1. Paucicellular Fibrointimal Proliferation Characterizes Pediatric Pulmonary Vein Stenosis: Clinicopathologic Analysis of 213 Samples From 97 Patients.

Kovach AE, Magcalas PM, Ireland C, McEnany K, Oliveira AM, Kieran MW, Baird CW, Jenkins K, Vargas SO.

Am J Surg Pathol. 2017 Jun 15. doi: 10.1097/PAS.0000000000000892. [Epub ahead of print]

PMID: 28622179

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Select item 28618202

 

  1. Aortopulmonary window: results of repair beyond infancy†.

Talwar S, Siddharth B, Gupta SK, Choudhary SK, Kothari SS, Juneja R, Saxena A, Airan B.

Interact Cardiovasc Thorac Surg. 2017 Jun 13. doi: 10.1093/icvts/ivx158. [Epub ahead of print]

PMID: 28633352

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Select item 28614113

 

  1. Role of Octreotide in Pediatric Gastrointestinal Bleeding Secondary to Angiodysplasia in Children with Right Heart Failure.

Puri K, Caldwell R, Molleston JP.

J Pediatr Gastroenterol Nutr. 2017 Jun 13. doi: 10.1097/MPG.0000000000001659. [Epub ahead of print]

PMID: 28614113

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Select item 28607205

 

  1. Nutritional outcomes in infants with food allergy after cardiac surgery.

Luo WY, Xu ZM, Hong L, Wu QY, Zhang YY.

Congenit Heart Dis. 2017 Jun 9. doi: 10.1111/chd.12489. [Epub ahead of print]

PMID: 28598588

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Select item 28598551

 

  1. Coronary artery bypass grafting in infants, children, and young adults for acquired and congenital lesions.

Mavroudis C.

Congenit Heart Dis. 2017 Jun 9. doi: 10.1111/chd.12491. [Epub ahead of print]

PMID: 28598551

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Select item 28598033

 

  1. Aortopexy offers surgical options for a variety of pathological tracheal conditions in paediatric patients.

Gruszka A, Sachweh JS, Schnoering H, Tenbrock K, Muehler EG, Laschat M, Vazquez-Jimenez JF.

Interact Cardiovasc Thorac Surg. 2017 Jun 9. doi: 10.1093/icvts/ivx163. [Epub ahead of print]

PMID: 28605441

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Select item 28598000

 

  1. Use of Extracorporeal Membrane Oxygenation and Mortality in Pediatric Cardiac Surgery Patients With Genetic Conditions: A Multicenter Analysis.

Furlong-Dillard JM, Amula V, Bailly DK, Bleyl SB, Wilkes J, Bratton SL.

Pediatr Crit Care Med. 2017 Jun 9. doi: 10.1097/PCC.0000000000001225. [Epub ahead of print]

PMID: 28604574

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Select item 28498066

 

  1. Long-Term Outcomes of Pediatric Cardiac Patients Supported by Extracorporeal Membrane Oxygenation.

Elias MD, Achuff BJ, Ittenbach RF, Ravishankar C, Spray TL, Fuller S, Montenegro LM, Gaynor JW, O’Connor MJ.

Pediatr Crit Care Med. 2017 Jun 8. doi: 10.1097/PCC.0000000000001227. [Epub ahead of print]

PMID: 28598945

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Select item 28592193

 

  1. Long-term outcomes after aortic coarctation repair in Maltese patients: A population-based study.

Caruana M, Grech V.

Congenit Heart Dis. 2017 Jun 5. doi: 10.1111/chd.12488. [Epub ahead of print]

PMID: 28580660

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Select item 28580658

 

  1. Improvement in perioperative care in pediatric cardiac surgery by shifting the primary focus of treatment from cardiac output to perfusion pressure: Are beta stimulants still needed?

Hosseinpour AR, van Steenberghe M, Bernath MA, Di Bernardo S, Pérez MH, Longchamp D, Dolci M, Boegli Y, Sekarski N, Orrit J, Hurni M, Prêtre R, Cotting J.

Congenit Heart Dis. 2017 Jun 5. doi: 10.1111/chd.12485. [Epub ahead of print]

PMID: 28580658

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Select item 28580631

 

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

Gerrah R, Haller SJ, George I.

Ann Thorac Surg. 2017 Jun;103(6):2005-2014. doi: 10.1016/j.athoracsur.2017.01.068. Epub 2017 Apr 28. Review.

PMID: 28457478

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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 Jun;103(6):1941-1949. doi: 10.1016/j.athoracsur.2017.02.068. Epub 2017 Apr 26.

PMID: 28456396

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Select item 28223051

 

  1. Functional Status and Quality of Life in Survivors of Extracorporeal Membrane Oxygenation After the Norwood Operation.

Friedland-Little JM, Uzark K, Yu S, Lowery R, Aiyagari R, Hirsch-Romano JC.

Ann Thorac Surg. 2017 Jun;103(6):1950-1955. doi: 10.1016/j.athoracsur.2016.11.015. Epub 2017 Feb 21.

PMID: 28223051

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Select item 28504507

 

  1. Question 2 Is levosimendan better than milrinone in preventing post operative low cardiac output syndrome and improving cardiac function in children with congenital heart disease?

Udayasankar S.

Arch Dis Child. 2017 Jun;102(6):590-592. doi: 10.1136/archdischild-2017-312660. No abstract available.

PMID: 28536187

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Select item 27986699

 

  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 Jun;8(2):107-119. doi: 10.1007/s13239-017-0302-5. Epub 2017 Apr 5.

PMID: 28382440 Free PMC Article

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Select item 28612412

 

  1. Challenge of Timing Redo Aortic Valve Replacement: Is There a Potential Role for Left Ventricular Global Longitudinal Strain?

Baumgartner H.

Circ Cardiovasc Imaging. 2017 Jun;10(6). pii: e006556. doi: 10.1161/CIRCIMAGING.117.006556. No abstract available.

PMID: 28559421

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Select item 28392328

 

  1. Anesthesia in adults with congenital heart disease.

Baehner T, Ellerkmann RK.

Curr Opin Anaesthesiol. 2017 Jun;30(3):418-425. doi: 10.1097/ACO.0000000000000468.

PMID: 28306681

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Select item 28497583

 

  1. Ventricular assist devices for the failing univentricular circulation.

Buratto E, Shi WY, Ye XT, Konstantinov IE.

Expert Rev Med Devices. 2017 Jun;14(6):449-459. doi: 10.1080/17434440.2017.1332523. Epub 2017 May 26.

PMID: 28532240

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Select item 28266898

 

  1. Heart transplantation using a donor with partial anomalous pulmonary venous connection and atrial septal defect.

de Beco G, Duisit J, Poncelet AJ.

Interact Cardiovasc Thorac Surg. 2017 Jun 1;24(6):978-979. doi: 10.1093/icvts/ivw440.

PMID: 28379508

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Select item 28369475

 

  1. National trends and variability in blood utilization in paediatric cardiac surgery.

Karimi M, Sullivan JM, Lerer T, Hronek C.

Interact Cardiovasc Thorac Surg. 2017 Jun 1;24(6):938-943. doi: 10.1093/icvts/ivw439.

PMID: 28369475

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  1. Interventricular septal hematoma following repair of a ventricular septal defect.

Yamazawa H, Takeda A, Nakajima H, Tachibana T, Aoki M.

J Card Surg. 2017 Jun;32(6):390-393. doi: 10.1111/jocs.13145. Epub 2017 May 11.

PMID: 28493392

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Select item 27828833

 

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

Siacunco EA, Pacheco GS, Woolridge DP.

J Emerg Med. 2017 Jun;52(6):e239-e243. doi: 10.1016/j.jemermed.2017.01.051. Epub 2017 Mar 10.

PMID: 28285866

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Select item 28553761

 

  1. The use of neonatal extracorporeal life support in pediatric cardiac intensive care unit.

Öztürk E, Yıldız O, Çine N, Tüzün B, Onan S, Ergül Y, Güzeltaş A, Haydin S, Yeniterzi M, Bakır İ.

J Matern Fetal Neonatal Med. 2017 Jun;30(12):1397-1401. doi: 10.1080/14767058.2016.1214706. Epub 2016 Aug 10.

PMID: 27432486

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Select item 28585599

 

  1. Total cavopulmonary connection with a new bioabsorbable vascular graft: First clinical experience.

Bockeria LA, Svanidze O, Kim A, Shatalov K, Makarenko V, Cox M, Carrel T.

J Thorac Cardiovasc Surg. 2017 Jun;153(6):1542-1550. doi: 10.1016/j.jtcvs.2016.11.071. Epub 2017 Feb 7.

PMID: 28314534

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  1. Hands-on surgical training of congenital heart surgery using 3-dimensional print models.

Yoo SJ, Spray T, Austin EH 3rd, Yun TJ, van Arsdell GS.

J Thorac Cardiovasc Surg. 2017 Jun;153(6):1530-1540. doi: 10.1016/j.jtcvs.2016.12.054. Epub 2017 Feb 9.

PMID: 28268011

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Select item 28259455

 

  1. Variation in extubation failure rates after neonatal congenital heart surgery across Pediatric Cardiac Critical Care Consortium hospitals.

Benneyworth BD, Mastropietro CW, Graham EM, Klugman D, Costello JM, Zhang W, Gaies M.

J Thorac Cardiovasc Surg. 2017 Jun;153(6):1519-1526. doi: 10.1016/j.jtcvs.2016.12.042. Epub 2017 Feb 4.

PMID: 28259455

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  1. Fontan Revision with Y-Graft in a Patient with Unilateral Pulmonary Arteriovenous Malformation.

Lee JW, Park JJ, Goo HW, Ko JK.

Korean J Thorac Cardiovasc Surg. 2017 Jun;50(3):207-210. doi: 10.5090/kjtcs.2017.50.3.207. Epub 2017 Jun 5.

PMID: 28593158 Free PMC Article

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  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 Jun;38(5):1065-1070. doi: 10.1007/s00246-017-1620-y. Epub 2017 Apr 29.

PMID: 28456828

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  1. Perioperative and Anesthetic Considerations in Atrioventricular Septal Defect.

Ross FJ, Nasr VG, Joffe D, Latham GJ.

Semin Cardiothorac Vasc Anesth. 2017 Jun 1:1089253217706166. doi: 10.1177/1089253217706166. [Epub ahead of print]

PMID: 28592182

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Select item 28506196

 

  1. Sir William Harvey, Congenital Heart Disease and Anesthesia.

Twite MD.

Semin Cardiothorac Vasc Anesth. 2017 Jun;21(2):121-122. doi: 10.1177/1089253217704509. No abstract available.

PMID: 28506196

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Select item 28107801

 

  1. Surgical Considerations in Total Anomalous Pulmonary Venous Connection.

Shaw FR, Chen JM.

Semin Cardiothorac Vasc Anesth. 2017 Jun;21(2):132-137. doi: 10.1177/1089253216688535. Epub 2017 Jan 20.

PMID: 28107801

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Select item 27831520

 

  1. Total Anomalous Pulmonary Venous Connection: Preoperative Anatomy, Physiology, Imaging, and Interventional Management of Postoperative Pulmonary Venous Obstruction.

Files MD, Morray B.

Semin Cardiothorac Vasc Anesth. 2017 Jun;21(2):123-131. doi: 10.1177/1089253216672442. Epub 2016 Nov 29.

PMID: 27831520

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Select item 27694572

 

  1. Perioperative and Anesthetic Considerations in Total Anomalous Pulmonary Venous Connection.

Ross FJ, Joffe D, Latham GJ.

Semin Cardiothorac Vasc Anesth. 2017 Jun;21(2):138-144. doi: 10.1177/1089253216672012. Epub 2016 Sep 29.

PMID: 27694572

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Select item 27401860

 

  1. Effect of Induction Therapy on Graft Survival in Primary Pediatric Heart Transplantation: A Propensity Score Analysis of the UNOS Database.

Butts R, Davis M, Savage A, Burnette A, Kavarana M, Bradley S, Atz A, Nietert PJ.

Transplantation. 2017 Jun;101(6):1228-1233. doi: 10.1097/TP.0000000000001285.

PMID: 27362312

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Select item 28597724

 

  1. Influence of weight at the time of first palliation on survival in patients with a single ventricle.

Surendran S, Kumar TKS, Tansey B, Allen J, Zurakowski D, Boston U, Shah SH, Knott-Craig CJ.

Cardiol Young. 2017 Jun 27:1-8. doi: 10.1017/S1047951117001299. [Epub ahead of print]

PMID: 28651677

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  1. Echocardiographic Diagnosis, Surgical Treatment, and Outcomes of Anomalous Left Coronary Artery from the Pulmonary Artery.

Patel SG, Frommelt MA, Frommelt PC, Kutty S, Cramer JW.

J Am Soc Echocardiogr. 2017 Jun 23. pii: S0894-7317(17)30383-8. doi: 10.1016/j.echo.2017.05.005. [Epub ahead of print]

PMID: 28651802

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Select item 28651801

 

  1. Brain monitoring in adult and pediatric ECMO patients: the importance of early and late assessments.

Lorusso R, Taccone FS, Belliato M, Delnoij T, Zanatta P, Cvetkovic M, Davidson M, Belohlavek J, Matta N, Davis C, Ijsselstijn H, Mueller T, Muellenbach R, Donker D, David P, DI Nardo M, Vlasselaers D, Dos Reis Miranda D, Hoskote A; Euro-ELSO Working Group on Neurologic Monitoring and Outcome.

Minerva Anestesiol. 2017 Jun 22. doi: 10.23736/S0375-9393.17.11911-5. [Epub ahead of print]

PMID: 28643997

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Select item 28642988

 

  1. Advancing the field of anti-HLA sensitization management prior to pediatric heart transplantation.

Zuckerman WA.

Pediatr Transplant. 2017 Jun 22. doi: 10.1111/petr.13012. [Epub ahead of print] No abstract available.

PMID: 28639339

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Select item 28639488

 

  1. Control of a Pediatric Pulsatile Ventricular Assist Device: A Hybrid Cardiovascular Model Study.

Ferrari G, Di Molfetta A, Zieliński K, Fresiello L, Górczyńska K, Pałko KJ, Darowski M, Amodeo A, Kozarski M.

Artif Organs. 2017 Jun 16. doi: 10.1111/aor.12929. [Epub ahead of print]

PMID: 28621816

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Select item 28619123

 

  1. Outcomes of palliative right ventricle to pulmonary artery connection for pulmonary atresia with ventricular septal defect†.

Lenoir M, Pontailler M, Gaudin R, Gerelli S, Tamisier D, Bonnet D, Murtuza B, Vouhé PR, Raisky O.

Eur J Cardiothorac Surg. 2017 Jun 15. doi: 10.1093/ejcts/ezx194. [Epub ahead of print]

PMID: 28633393

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Select item 28617415

 

  1. Earlier Pulmonary Valve Replacement in Down Syndrome Patients Following Tetralogy of Fallot Repair.

Sullivan RT, Frommelt PC, Hill GD.

Pediatr Cardiol. 2017 Jun 14. doi: 10.1007/s00246-017-1653-2. [Epub ahead of print]

PMID: 28616649

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Select item 28606209

 

  1. Sedation Management in Children Supported on Extracorporeal Membrane Oxygenation for Acute Respiratory Failure.

Schneider JB, Sweberg T, Asaro LA, Kirby A, Wypij D, Thiagarajan RR, Curley MAQ; Randomized Evaluation of Sedation Titration for Respiratory Failure (RESTORE) Study Investigators.

Crit Care Med. 2017 Jun 13. doi: 10.1097/CCM.0000000000002540. [Epub ahead of print]

PMID: 28614197

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Select item 28614113

 

  1. Successful Treatment of Pediatric Ventricular Assist Device Thrombosis.

Chetan D, Buchholz H, Bauman M, Anand V, Holinski P, Conway J.

ASAIO J. 2017 Jun 7. doi: 10.1097/MAT.0000000000000606. [Epub ahead of print]

PMID: 28604570

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Select item 28592524

 

  1. Critically Underdeveloped Left Heart Morphology Associated with Prematurity and Low Birth Weight: Conditional Staged Rehabilitation Towards Biventricular Repair and Time-Related Growth of Left Heart Structures.

Ahmad F, Mangano R, Shore S, Polimenakos A.

Pediatr Cardiol. 2017 Jun 7. doi: 10.1007/s00246-017-1644-3. [Epub ahead of print]

PMID: 28589408

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Select item 28589407

 

  1. Factors associated with early extubation after superior cavopulmonary connection: analysis from single ventricle reconstruction trial.

Zakaria D, Rettiganti M, Gossett JM, Gupta P.

Acta Anaesthesiol Scand. 2017 Jun 1. doi: 10.1111/aas.12915. [Epub ahead of print]

PMID: 28568112

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Select item 28114148

 

  1. The Effects of Center Volume on Mortality in Pediatric Heart Transplantation-The Rest of the Story.

Canter CE.

Am J Transplant. 2017 Jun;17(6):1437-1438. doi: 10.1111/ajt.14277. Epub 2017 Apr 25. No abstract available.

PMID: 28332766

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Select item 28251816

 

  1. Inferior Outcomes on the Waiting List in Low-Volume Pediatric Heart Transplant Centers.

Rana A, Fraser CD, Scully BB, Heinle JS, McKenzie ED, Dreyer WJ, Kueht M, Liu H, Brewer ED, Rosengart TK, O’Mahony CA, Goss JA.

Am J Transplant. 2017 Jun;17(6):1515-1524. doi: 10.1111/ajt.14252. Epub 2017 Mar 30.

PMID: 28251816

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Select item 27931092

 

  1. The Influence of Race and Common Genetic Variations on Outcomes After Pediatric Heart Transplantation.

Green DJ, Brooks MM, Burckart GJ, Chinnock RE, Canter C, Addonizio LJ, Bernstein D, Kirklin JK, Naftel DC, Girnita DM, Zeevi A, Webber SA.

Am J Transplant. 2017 Jun;17(6):1525-1539. doi: 10.1111/ajt.14153. Epub 2017 Jan 23.

PMID: 27931092

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Select item 28509801

 

  1. Femoral Vein Homograft as Right Ventricle to Pulmonary Artery Conduit in Stage 1 Norwood Operation.

Kumar TKS, Briceno-Medina M, Sathanandam S, Joshi VM, Knott-Craig CJ.

Ann Thorac Surg. 2017 Jun;103(6):1969-1974. doi: 10.1016/j.athoracsur.2016.11.078. Epub 2017 Mar 3.

PMID: 28262297

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Select item 28242080

 

  1. Can Fontan Conversion for Patients Without Late Fontan Complications be Justified?

Higashida A, Hoashi T, Kagisaki K, Shimada M, Ohuchi H, Shiraishi I, Ichikawa H.

Ann Thorac Surg. 2017 Jun;103(6):1963-1968. doi: 10.1016/j.athoracsur.2016.11.062. Epub 2017 Feb 24.

PMID: 28242080

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Select item 28242079

 

  1. Long-Term Follow-Up After the Ross Procedure: A Single Center 22-Year Experience.

Schneider AW, Putter H, Klautz RJM, Bruggemans EF, Holman ER, Bökenkamp R, Hazekamp MG.

Ann Thorac Surg. 2017 Jun;103(6):1976-1983. doi: 10.1016/j.athoracsur.2016.11.064. Epub 2017 Feb 24.

PMID: 28242079

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Select item 28223051

 

  1. Functional Status and Quality of Life in Survivors of Extracorporeal Membrane Oxygenation After the Norwood Operation.

Friedland-Little JM, Uzark K, Yu S, Lowery R, Aiyagari R, Hirsch-Romano JC.

Ann Thorac Surg. 2017 Jun;103(6):1950-1955. doi: 10.1016/j.athoracsur.2016.11.015. Epub 2017 Feb 21.

PMID: 28223051

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Select item 28504505

 

  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 Jun 1;51(6):1044-1050. doi: 10.1093/ejcts/ezx038.

PMID: 28402400

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Select item 28422131

 

  1. Analgesia and Sedation Protocol for Mechanically Ventilated Postsurgical Children Reduces Benzodiazepines and Withdrawal Symptoms-But Not in All Patients.

Neunhoeffer F, Seitz G, Schmidt A, Renk H, Kumpf M, Fideler F, Esslinger M, Gerbig I, Fuchs J, Hofbeck M.

Eur J Pediatr Surg. 2017 Jun;27(3):255-262. doi: 10.1055/s-0036-1586202. Epub 2016 Jul 25.

PMID: 27454068

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Select item 27752831

 

  1. Complex Cardiac Surgery on Patients with a Body Weight of Less Than 5 kg without Donor Blood Transfusion.

Boettcher W, Dehmel F, Redlin M, Miera O, Musci M, Cho MY, Photiadis J.

J Extra Corpor Technol. 2017 Jun;49(2):93-97.

PMID: 28638157

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Select item 27485933

 

  1. The use of neonatal extracorporeal life support in pediatric cardiac intensive care unit.

Öztürk E, Yıldız O, Çine N, Tüzün B, Onan S, Ergül Y, Güzeltaş A, Haydin S, Yeniterzi M, Bakır İ.

J Matern Fetal Neonatal Med. 2017 Jun;30(12):1397-1401. doi: 10.1080/14767058.2016.1214706. Epub 2016 Aug 10.

PMID: 27432486

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Select item 28264985

 

  1. Comparison of right ventricle-pulmonary artery shunt position in the Single Ventricle Reconstruction trial.

Andersen ND, Meza JM, Byler MR, Lodge AJ, Hill KD, Hornik CP, Jaquiss RDB.

J Thorac Cardiovasc Surg. 2017 Jun;153(6):1490-1500.e1. doi: 10.1016/j.jtcvs.2016.10.104. Epub 2017 Feb 9.

PMID: 28274556

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Select item 28268011

 

  1. Arterial Switch Operation and Plasma Biomarkers: Analysis and Correlation with Early Postoperative Outcomes.

Giordano R, Cantinotti M, Arcieri L, Poli V, Pak V, Murzi B.

Pediatr Cardiol. 2017 Jun;38(5):1071-1076. doi: 10.1007/s00246-017-1621-x. Epub 2017 May 8.

PMID: 28480501

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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 Jun;38(5):1032-1041. doi: 10.1007/s00246-017-1615-8. Epub 2017 Apr 29.

PMID: 28456829

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Select item 28456828

 

  1. An Alternative Strategy for Bridge-to-Transplant/Recovery in Small Children with Dilated Cardiomyopathy.

Mets G, Panzer J, De Wolf D, Bové T.

Pediatr Cardiol. 2017 Jun;38(5):902-908. doi: 10.1007/s00246-017-1610-0. Epub 2017 Mar 29. Review.

PMID: 28357450

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Select item 28349207

 

  1. Surgical Correction of Aberrant Right Coronary Anomalies Stranding an Aortic Commissure with and Without Unroofing.

Abdelhady K, Durgam S, Elzein C, Ilbawi MN, Rhoiney D, Massad MG.

Pediatr Cardiol. 2017 Jun;38(5):1080-1083. doi: 10.1007/s00246-017-1581-1. Epub 2017 Feb 18.

PMID: 28214965

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Select item 28210768

 

  1. Complete Repair of Tetralogy of Fallot in the Neonatal Versus Non-neonatal Period: A Meta-analysis.

Loomba RS, Buelow MW, Woods RK.

Pediatr Cardiol. 2017 Jun;38(5):893-901. doi: 10.1007/s00246-017-1579-8. Epub 2017 Feb 11. Review.

PMID: 28190140

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Select item 28101662

 

  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 Jun;21(4). doi: 10.1111/petr.12936. Epub 2017 Apr 29.

PMID: 28455909

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Select item 28370952

 

  1. Feasibility and interpretation of global longitudinal strain imaging in pediatric heart transplant recipients.

Wisotzkey BL, Jorgensen NW, Albers EL, Kemna MS, Boucek RJ, Kronmal RA, Law YM, Bhat AH.

Pediatr Transplant. 2017 Jun;21(4). doi: 10.1111/petr.12909. Epub 2017 Mar 10.

PMID: 28295946

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Select item 28247591

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ACHD Featured Articles of June 2017

ACHD Manuscripts of June 2017 curated by members of the International Society of Adult Congenital Herat Disease

 

Platelet count and mean platelet volume predict outcome in adults with Eisenmenger syndrome.

Martin-Garcia AC, Arachchillage DR, Kempny A, Alonso-Gonzalez R, Martin-Garcia A, Uebing A, Swan L, Wort SJ, Price LC, McCabe C, Sanchez PL, Dimopoulos K, Gatzoulis MA.

Heart. 2017 Jun 29. pii: heartjnl-2016-311144. doi: 10.1136/heartjnl-2016-311144. [Epub ahead of print]

PMID: 28663364

 

Take Home Points:

  • Thrombocytopenia predicts mortality in Eisenmenger syndrome. Patients with a platelet count of <100×10⁹/L had double the mortality of those with higher platelet counts.
  • Raised mean platelet volume, very high hemoglobin (>220 g/L) or very low hemoglobin (<130 g/L), but not platelet count were associated with increased thrombotic events

 

jokhadar-maanCommentary from Dr. Maan Jokhadar (Atlanta), section editor of ACHD Journal Watch:   Eisenmenger syndrome (pulmonary hypertension with cyanosis (sat < 90%) and presence of a large intracardiac or extracardiac shunt) is associated high hemoglobin (erythrocytosis) and thrombocytopenia. Erythrocytosis as an expected compensatory response to cyanosis. Eisenmenger syndrome is also associated with thrombocytopenia that is caused by whole megakaryocytes bypassing the lungs via right to left shunting.  Megakaryocytes are normally fragmented into platelets in the pulmonary circulation.  Mean platelet volume (MPV) reflects platelet size and higher MPV is associated with higher platelet aggregation. Larger platelet may have more aggregation capacity and higher MPV is an independent risk factor for myocardial infarction and stroke in patients with coronary disease.

To further elucidate the role of erythrocytosis, thrombocytopenia, and MPV in Eisenmenger syndrome, Dr. Martin-Garcia and colleagues from the UK and Spain performed a retrospective analysis of 226 Eisenmenger syndrome patients who were seen at the Royal Brompton Hospital between 2005 in 2016 for median follow-up of about 8 years.  About a third of patients were male and the mean age was about 35 years.  Fifty percent of patients had complex anatomy, 43% had post tricuspid shunt, and about 7% of patients had a pre-tricuspid shunt.  About a quarter of patients were on pulmonary vasodilator therapy at baseline and about 58% of patients were started on pulmonary visit dilator therapy during follow-up.

About 44% of patients had Down syndrome, who tended to be younger (mean age 30), more complex anatomy (72%), similar oxygen saturations, similar aspirin use, and similar clopidogrel use.  However, Down syndrome patients were less likely to be on warfarin, novel anticoagulants, iron supplementation (despite more frequent iron deficiency), pulmonary vasodilator therapy, and also tended to have higher hemoglobin and lower platelet concentrations but similar MPV.

The mean hemoglobin concentration was 193.8 g/L; 90% of patients had an hemoglobin > 153.0 g/L, 75% > 172.3 g/L and 46.5% > 200 g/L. The mean platelet count was at low-normal levels (152.6×10⁹/L). About a quarter of patients had a platelet count of <100×10⁹/L, whereas 5.3% had platelet counts of <50×10⁹/L. The mean MPV was 9.6±1.2 fL. However, only about 5% of patients had MPV >11.5 fL.

As one would expect, lower oxygen saturations were associated with higher hemoglobin, lower platelet count was associated with higher hemoglobin, and lower platelet count was also associated with higher MPV.  MPV and hemoglobin concentrations had a positive correlation.  These patterns were present in both Down syndrome patients and non-Down syndrome patients.

Of interest, iron deficiency was present and about 45% of patients in this study, who had lower hemoglobin and higher platelet counts but similar MPV to those patients without iron deficiency.

Patients with a platelet count of <100×10⁹/L had double the mortality. MPV was not predictive of mortality. Patient with very high (>220 g/L) or very low (<130 g/L) Hb concentration were both associated with about a 4-fold increase in thrombotic events. (HR about 3.9 and 4.8, respectively). MPV >9.5 fL was associated with a 3-fold increase in thrombotic events (HR 3.5).

An interesting point made in the discussion of this paper was that iron deficiency, which is common in Eisenmenger syndrome patients, is associated with increased mortality in this population.  Iron replacement, though recommended, could worsen thrombocytopenia though this was not associated with increasing bleeding events.

Hemoglobin, platelet count, and MPV may be helpful in Eisenmenger syndrome risk stratification and could guide treatment. However, additional studies are needed.

 

 

 

How Pregnancy Impacts Adult Cyanotic Congenital Heart Disease: A Multicenter Observational Study.

Ladouceur M, Benoit L, Basquin A, Radojevic J, Hauet Q, Hascoet S, Moceri P, Le Gloan L, Amedro P, Lucron H, Richard A, Gouton M, Nizard J.

Circulation. 2017 Jun 13;135(24):2444-2447. doi: 10.1161/CIRCULATIONAHA.116.027152. No abstract available.

PMID: 28606952

 

Take Home Points:

  • Selected patients with cyanotic CHD can have successful pregnancies, especially when there’s adequate pre-pregnancy counseling and good follow-up with a multi-disciplinary team.
  • Although the risks to the mother are lower in this cohort than previously described, the potential for late chronic heart failure remains.
  • Further larger cohort studies are required to validate these findings.

 

Blanche CupidoCommentary by Dr. Blanche Cupido (Cape Town), section editor of ACHD Journal WatchMore women with unrepaired cyanotic CHD are surviving into adulthood. They are at high risk for both maternal and fetal complications during pregnancy. This study represents a retrospective review of 51 cyanotic patients presenting between 1997 and 2015 from 11 referral centers in the M3C French network (Center de reference des Malformations Cardiaques Congenitales Complexes). Patients with central cyanosis at rest or exercise and with an underlying ‘compatible’ lesion were included. Twenty patients were excluded because of pulmonary hypertension. The remaining 31 patients were divided into 4 categories according to their mechanism for cyanosis: 1. Ventricular right to left shunt and systemic LV, 2. Univentricular heart, 3. Ventricular right to left shunt and systemic RV, Atrial right to left shunt.

 

Seventy-one pregnancies occurred in 31 patients. 48% (n=15) had previous palliative shunts but had residual cyanosis. All but 2 patients had normal systemic ventricular function.  Cardiovascular complications (in 13 pregnancies) documented were: 3 with heart failure, 2 with de novo SVT, 7 with worsening hypoxemia. There was no distinct association between baseline maternal characteristics and outcomes.

No thromboembolic complications occurred during the pregnancies (13 patients received anticoagulation and 5 received antiplatelet agents) – these numbers are too small to draw any definitive conclusions. One patient developed infective endocarditis.

There were no deaths during the follow-up period (median 3.8 years).  Thirteen percent (n=4) developed late heart failure with a deterioration in systemic ventricular function. The study did not comment on fetal loss or fetal complications.

 

Longitudinal Outcomes of Patients with Single Ventricle after the Fontan Procedure.

Atz AM, Zak V, Mahony L, Uzark K, D’agincourt N, Goldberg DJ, Williams RV, Breitbart RE, Colan SD, Burns KM, Margossian R, Henderson HT, Korsin R, Marino BS, Daniels K, McCrindle BW; Pediatric Heart Network Investigators.

J Am Coll Cardiol. 2017 Jun 6;69(22):2735-2744. doi: 10.1016/j.jacc.2017.03.582.

PMID: 28571639

Similar articles

Select item 28571637

 

Take Home Points:

  • In the Fontan 3 study, a large, longitudinal cohort of Fontan patients (n=373 who consented to present study out of initial 546 enrolled in Fontan 1 study) followed by the Pediatric Heart Network (PHN) over 12 years, transplant-free survival was 90% at a mean age of 21.2 +5 years (mean 17.8 years after Fontan procedure).
  • Exercise capacity, on average, decreased from the initial Fontan 1 to Fontan 3 study, from a peak VO2 of 69% predicted to 61% predicted.
  • Risk of death or cardiac transplantation from Fontan 1 to Fontan 3 study was associated with poorer ventricular performance (either reduced EF or significant ventricular dilation), but was independent of ventricular morphology, age of the patient, or type of Fontan connection.
  • Worse measured exercise capacity correlated well with a lower Pediatric Quality of Life Inventory physical summary score.
  • Among the seven centers in the PHN study, there was significant variability regarding mortality outcome as well as percentage of patients receiving transplantation. A total of 21 patients in the study died without transplant, and 23 underwent transplantation.  Rate of transplantation ranged from 0% to 21.1%.  The center with zero transplants had the highest overall mortality rate (13.6%).  The center with the highest transplant rate (21.1%) had the second highest death rate (10.5%).  This level of discrepancy highlights need for more standardization in criteria for cardiac transplantation in the Fontan population.

 

Kay_William_MD.16.CVaCommentary from Dr. W. Aaron Kay (Indianapolis), section editor of ACHD Journal Watch:  Long term data in Fontan patients is lacking as most case series either have very small numbers of patients and/or have limited number of years of follow-up.  A few larger series encompass several surgical strategies and eras of surgery, and as a result using conclusions from these studies to care for younger patients in the modern surgical era may not be appropriate.

Over a decade ago, the Pediatric Heart Network (PHN) sought to start a longitudinal study of Fontan outcomes.  Out of 7 centers, the PHN initially enrolled 546 patients in the Fontan 1 study; this data was published long ago, and interval data of 416 patients was published in the Fontan 2 study more recently.  The present study, the Fontan 3 study, included 373 surviving patients who consented to remain enrolled in the study, or a total of 80% of patients still eligible to be in the study.  The present study population was enrolled at 21.2 + 3.5 years, at an average of 17.8 + 3.4 years from initial Fontan procedure.

Several outcomes were studied, including hard outcome of death or cardiac transplantation, quality of life (QOL) measures, exercise capacity variables, measurements of ventricular performance.  QOL was measured on a standardized test, the Pediatric Quality of Life Inventory, and/or the SF-36 for those > 19 years of age upon enrollment.  Other variables need for interval procedures, including need for medical or other treatment of arrhythmia, surgery, placement of cardiac rhythm device, or catheter intervention, as well as presence of significant noncardiac comorbidities including stroke, seizures, thrombus, protein-losing enteropathy, cirrhosis, or plastic bronchitis.  There was a significant increase from Fontan 1 to Fontan 3 of all comorbidties as well as need for catheterization and cardiac surgery.  Cirrhosis was found in only 8% of the cohort; however this paper does not mention how exactly cirrhosis was defined, nor did it mention any detail about liver biopsy, elastography, or imaging assessment of cirrhosis.

Most patients completed a QOL inventory (n=364), of which 74% had all laboratory testing performed, and 223 had maximal effort cardiopulmonary exercise testing.  The most common cardiac surgical procedure was placement or revision of a cardiac rhythm device, the majority of which were pacemakers (only 9/373 patients underwent interval placement of an ICD).

Overall systemic ventricle ejection fraction went down slightly during the study, from 58 + 11% to 55 + 10% (p < 0.001).  A lower QOL score correlated with poorer exercise performance.  Mean BNP levels were low in the population, although they were significantly higher at time of Fontan 3 compared with Fontan 1 cohort (18.0 pg/ml vs 13.0 pg/ml; p < 0.001).  Although overall levels were low, interestingly, mean BNP levels were statistically high in patients with atriopulmonary connection compared with intracardiac lateral tunnel, which in turn was higher than that seen in extracardiac conduit.  When the QOL scores were normalized on a 0-100 scale (with 100 representing best QOL), there was an association between higher logBNP and lower QOL score.  No association was found between QOL score and echocardiographic variables.

Hard outcomes of death or transplant represented approximately 10% of the initial Fontan study population, with a total of 54 patients having died (n=31) or undergoing transplant (n=23) by time of Fontan 3 study.  The authors found no association of high risk of death/transplant with patient sex, age, race, ethnicity, ventricular morphology, or type of Fontan procedure.  On univariate analysis, a poorer QOL physical summary score, higher BNP value, lack of completion of exercise test, lower resting oxygen saturation, lower predicted VO2 at anaerobic threshold, and poorer ventricular performance were associated with a higher risk of death/transplant.

Some information regarding socioeconomic status was included.  As a continuous variable, family income did not correlate with risk of death/transplantation.  However, when the population was dichotomized into low-income and high-income groups, a family income > $100,000 annually had better outcomes.  Overall, 96% of the study population maintained either private or public health insurance during the study.

Most of the study population is entering the age of “transition” from pediatrics to adulthood.  Patients at time of Fontan 3 were more likely to have seen an adult cardiologist in the past 2 years (35% versus 30%) as well as to have seen an adult congenital cardiologist (33% to 24%).  Female patients had a higher likelihood of having seen an OB-Gyn (41% versus 32%), but patients were found to receive less educational support compared to the prior study (decrease from 36% to 26%).

There was significant discrepancy from center to center regarding percentage of patients undergoing transplantation; this finding emphasizes the need for further research to better refine criteria for heart transplant evaluation in the Fontan population.

June ACHD 1

June ACHD 2

 

Mortality in pulmonary arterial hypertension due to congenital heart disease: serial changes improve prognostication.

Schuijt MTU1Blok IM2Zwinderman AH1van Riel ACMJ2Schuuring MJ2de Winter RJ1Duijnhouwer AL3van Dijk APJ3Mulder BJM2Bouma BJ4.

Int J Cardiol. 2017 Jun 1. pii: S0167-5273(17)30321-2. doi: 10.1016/j.ijcard.2017.05.101. [Epub ahead of print]

PMID: 28606655

 

Take Home Points:

  • Prognostic value of baseline parameters in patients with congenital heart disease and pulmonary hypertension weakens over time as the patient’s condition changes.
  • Serial changes in WHO functional class, 6-minute-walk distance, SaO2 at peak exercise, NT pro BNP and TAPSE were more potent than baseline parameters at predicting mortality.

 

Leong Ming ChernCommentary from Dr. MC Leong (Kuala Lumpur), section editor of ACHD Journal Watch: Baseline parameters of patients with congenital heart disease and pulmonary arterial hypertension (PAH-CHD) have been shown to be associated with mortality. However, the prognostic values of these baseline parameters weaken over time as the patient’s clinical condition changes and hence serial changes in clinical parameters should be a more apt way to prognosticate patients with PAH-CHD. This study aimed to investigate the prognostic value of serial changes in standard clinical parameters in PAD-CHD.

92 patients with PAH-CHD, either repaired or not, who were referred to 2 study centres in the Netherlands between January 2005 and May 2016 were included. Patients were given a standard monotherapy of either Bosentan (before 2014) or Macitentan (after 2014) after the first clinic visit and they were followed up 6 monthly with standard evaluation protocol which included SaO2 at peak exercise, six-minute-walk distance, biochemical (haemoglobin, urea, NT pro BNP and creatinine) and echocardiographic (TAPSE, RV S’ and echocardiogram-derived systolic pulmonary arterial pressure) parameters.

During a median follow up of 6.0 (IQR: 3.7-9.3) years, 35 (38%) patients died. Onset of WHO functional class IV (HR : 18.34), decrease of six-minute-walk distance (HR : 0.65 per 50m), SaO2 at peak exercise (HR : 0.74 per 5%), NT pro BNP (HR : 2.25 per 1000ng/l) and TAPSE (HR: 0.80 per 0.5cm) were significantly associated with higher mortality. Interestingly, a survival model, based on serial changes of these parameters in time-dependent univariate Cox regression, showed 25% mortality in 0.06 years with WHO functional class IV reached; 1.3 years with an increase of NT pro BNP of 1000ng/l; 3.7 years with TAPSE decreased 0.5cm; 4.4 years with six-minute-walk distance decreasing 50m; and 4.5 years with SaO2 at peak exercise decreasing 5%. (Figure 1) Impact of the serial changes was also shown in Table 3.

June ACHD 3 June ACHD 4 June ACHD 5

Undoubtedly, the use of serial changes in the parameters during follow up better reflects mortality in this group of patient and these parameters were shown to be useful to aid risk stratification of patients and subsequently guide medical therapy.

In this study, first year mortality was unusually high. The authors reasoned that this may due to the late initiation of PAH targeted therapy and older aged cohort. Meanwhile, the practice of starting all PAH-CHD on endothelin receptor antagonist at the first visit, which is practiced in the study centre is not conventional, especially in patients who are haemodynamically balanced. It would be interesting if the authors could analyse difference in serial changes in parameters in the patients with corrected PAH-CHD compared to those without, as patients with corrected PAH-CHD tended to fare worse than those without correction.

 

 

Effect of Fontan-Associated Morbidities on Survival with Intact Fontan Circulation.

Allen KY, Downing TE, Glatz AC, Rogers LS, Ravishankar C, Rychik J, Fuller S, Montenegro LM, Steven JM, Spray TL, Nicolson SC, Gaynor JW, Goldberg DJ.

Am J Cardiol. 2017 Jun 1;119(11):1866-1871. doi: 10.1016/j.amjcard.2017.03.004. Epub 2017 Mar 16.

PMID: 28385177

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Select item 28161345

 

Take Home Points:

  • Substantial mild plus pre-Fontan AV valve insufficiency is a significant risk factor for development of long-term comorbidities.
  • Ventricular morphology does not appear to differentially contribute to increased risks of cardiovascular comorbidities.
  • 20-year survival without comorbidities in TCPC is reported as 47% in this cohort.

 

 

Moe_Tabitha-WEBCommentary by Dr. Tabitha G. Moe (Phoenix), section editor of ACHD Journal WatchSurvival after the Fontan operation has improved over the course of time. Fontan operations can reasonably be categorized into separate and distinct eras. This paper from Children’s Hospital of Philadelphia [CHOP] outlines medical comorbidities in those patients in the second and third eras of Fontan or total cavopulmonary connections, specifically there are not any subjects who underwent atrio-pulmonary Fontan.  This review again emphasizes that ventricular morphology does not seem to be associated with long-term outcomes. They report a 20-year post-Fontan survival of 47% without PLE, PB, thrombosis, or tachyarrhythmia, consistent with previously reposted single-center studies at Mayo Clinic, and Australia-New Zealand. This study is valuable in that it defines the outcomes of 773 modern era Fontans.  Nearly 50% of the patients did not have any recent follow up at CHOP requiring the re-identification and contact of a significant number of patients, and in fact the records were collected from 70 centers worldwide. They report 79 deaths, 25 heart transplants, and 10 Fontan takedowns.  Of this cohort 163 experienced at least one component of the composite medical morbidity: PLE, PB, thrombosis, or tachyarrhythmia. Pre-operative AV valve insufficiency more than mild, post-op pleural drainage >14 days, and longer cross clamp times were associated with an increased risk of the composite risk factors. For those subjects with the ideal outcomes, and none of the composite risks experienced freedom from Fontan failure at 20 years was 98%.  The Fontan cohort is relatively young with average age at Fontan completion of 2.3 years, 50% of the cohort were HLHS. 90% of their cohort were initially fenestrated.

 June ACHD 6

 

 

 

The central figure for this article represents the remarkable outcomes of those without any reported comorbidities.  There is a marked decline in long-term outcomes with the addition of any one morbidity.  One of the unfortunate characteristics of this cohort was the reported 229 subjects who were lost to follow up.  This is a significant number of single number of single ventricle subjects, operated on at a prestigious institution with highly organized follow-up plans, who still managed to be lost to follow up. Further analysis of this subcohort of patients may reveal opportunities to maintain life-time congenital cardiac care at all centers and represents a valuable tool for identifying patients who may be at risk to being lost to follow up. Although thrombosis remains a risk factor long-term the highest risk time frame is perioperatively, and maintaining anti-platelet or anti-thrombotic management during this high-risk window continues to be of importance. Lateral tunnel, and extracardiac conduits seem to have similar risks for development of atrial arrhythmias, however, this may be further elucidated with a longer follow up period. AV-valve insufficiency is a more significant risk factor than ventricular morphology in the long-term outcomes, again these variables may diverge with longer term follow up.  Interestingly, the hemodynamic findings on pre-Fontan catheterization with pulmonary artery pressure variables did not contribute to long-term outcomes. This composite outcome allows for additional speculation in an otherwise very complex and heterogenous cohort to evaluate and the authors are to be commended for this unique approach.

 

ACHD June 2017

 

  1. Platelet count and mean platelet volume predict outcome in adults with Eisenmenger syndrome.

Martin-Garcia AC, Arachchillage DR, Kempny A, Alonso-Gonzalez R, Martin-Garcia A, Uebing A, Swan L, Wort SJ, Price LC, McCabe C, Sanchez PL, Dimopoulos K, Gatzoulis MA.

Heart. 2017 Jun 29. pii: heartjnl-2016-311144. doi: 10.1136/heartjnl-2016-311144. [Epub ahead of print]

PMID: 28663364

 

Select item 28669434

 

  1. Determinants of quality of life in adults with CHD: an Australian cohort.

Eaton SL, Wang Q, Menahem S.

Cardiol Young. 2017 Jun 27:1-6. doi: 10.1017/S1047951117000816. [Epub ahead of print]

PMID: 28651679

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  1. Illness perceptions in adult congenital heart disease: A multi-center international study.

Rassart J, Apers S, Kovacs AH, Moons P, Thomet C, Budts W, Enomoto J, Sluman MA, Wang JK, Jackson JL, Khairy P, Cook SC, Subramanyan R, Alday L, Eriksen K, Dellborg M, Berghammer M, Johansson B, Rempel GR, Menahem S, Caruana M, Veldtman G, Soufi A, Fernandes SM, White KS, Callus E, Kutty S, Luyckx K; APPROACH-IS consortium and the International Society for Adult Congenital Heart Disease (ISACHD).

Int J Cardiol. 2017 Jun 26. pii: S0167-5273(17)30122-5. doi: 10.1016/j.ijcard.2017.06.072. [Epub ahead of print]

PMID: 28669511

 

Select item 28652574

 

  1. Impact on Survival of Warfarin in Patients with Pulmonary Arterial Hypertension Receiving Subcutaneous Treprostinil.

Ascha M, Zhou X, Rao Y, Minai OA, Tonelli AR.

Cardiovasc Ther. 2017 Jun 23. doi: 10.1111/1755-5922.12281. [Epub ahead of print]

PMID: 28643420

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Select item 28643441

 

  1. Impact of growing cohorts of adults with con-genital heart disease on clinical workload: a 20-year experience at a tertiary care centre.

Padrutt M, Bracher I, Bonassin F, Santos Lopes B, Gruner C, Stämpfli SF, Wolber T, Kretschmar O, Oxenius A, De Pasquale G, Seeliger T, Lüscher TF, Attenhofer Jost C, Greutmann M.

Swiss Med Wkly. 2017 Jun 21;147:w14443. doi: smw.2017.14443. eCollection 2017 Jun 21.

PMID: 28634971 Free Article

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Select item 28645012

 

  1. Learning and Memory in Adolescents With Critical Biventricular Congenital Heart Disease.

Cassidy AR, Newburger JW, Bellinger DC.

J Int Neuropsychol Soc. 2017 Jun 20:1-13. doi: 10.1017/S1355617717000443. [Epub ahead of print]

PMID: 28629487

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Select item 28632506

 

  1. Impact of age and sex on survival and causes of death in adults with congenital heart disease.

Oliver JM, Gallego P, Gonzalez AE, Garcia-Hamilton D, Avila P, Alonso A, Ruiz-Cantador J, Peinado R, Yotti R, Fernandez-Aviles F.

Int J Cardiol. 2017 Jun 19. pii: S0167-5273(17)32510-X. doi: 10.1016/j.ijcard.2017.06.060. [Epub ahead of print]

PMID: 28648355

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Select item 28631106

 

  1. Transthoracic 3D echocardiographic left heart chamber quantification in patients with bicuspid aortic valve disease.

van den Hoven AT, Mc-Ghie JS, Chelu RG, Duijnhouwer AL, Baggen VJM, Coenen A, Vletter WB, Dijkshoorn ML, van den Bosch AE, Roos-Hesselink JW.

Int J Cardiovasc Imaging. 2017 Jun 19. doi: 10.1007/s10554-017-1192-1. [Epub ahead of print]

PMID: 28631106

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Select item 28625174

 

  1. The Role of Cardiopulmonary Exercise Testing for Decision Making in Patients with Repaired Tetralogy of Fallot.

Dallaire F, Wald RM, Marelli A.

Pediatr Cardiol. 2017 Jun 16. doi: 10.1007/s00246-017-1656-z. [Epub ahead of print] Review.

PMID: 28623384

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Select item 28622179

 

  1. Effects of persistent Fontan fenestration patency on cardiopulmonary exercise testing variables.

Heal ME, Jackson LB, Atz AM, Butts RJ.

Congenit Heart Dis. 2017 Jun 15. doi: 10.1111/chd.12451. [Epub ahead of print]

PMID: 28618202

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Select item 28618149

 

  1. Adult congenital heart disease: magnitude of the problem.

Thakkar AN, Chinnadurai P, Lin CH.

Curr Opin Cardiol. 2017 Jun 15. doi: 10.1097/HCO.0000000000000429. [Epub ahead of print]

PMID: 28617685

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Select item 28318658

 

  1. How Pregnancy Impacts Adult Cyanotic Congenital Heart Disease: A Multicenter Observational Study.

Ladouceur M, Benoit L, Basquin A, Radojevic J, Hauet Q, Hascoet S, Moceri P, Le Gloan L, Amedro P, Lucron H, Richard A, Gouton M, Nizard J.

Circulation. 2017 Jun 13;135(24):2444-2447. doi: 10.1161/CIRCULATIONAHA.116.027152. No abstract available.

PMID: 28606952

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Select item 28608969

 

  1. Effect of iloprost on biomarkers in patients with congenital heart disease-pulmonary arterial hypertension.

Li XY, Zheng Y, Long Y, Zhang X, Zhang L, Tian D, Zhou D, Lv QZ.

Clin Exp Pharmacol Physiol. 2017 Jun 13. doi: 10.1111/1440-1681.12796. [Epub ahead of print]

PMID: 28608969

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Select item 28608519

 

  1. Pregnancy, Marfan Syndrome and Type B Aortic Dissection.

Johnson MR, Roos Hesselink JW.

BJOG. 2017 Jun 10. doi: 10.1111/1471-0528.14781. [Epub ahead of print]

PMID: 28600893

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Select item 28601813

 

  1. Atrial fibrillation in pregnancy: a growing challenge.

Katsi V, Georgiopoulos G, Marketou M, Oikonomou D, Parthenakis F, Makris T, Nihoyannopoulos P, Vardas P, Tousoulis D.

Curr Med Res Opin. 2017 Jun 8:1-8. doi: 10.1080/03007995.2017.1330257. [Epub ahead of print]

PMID: 28498066

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Select item 28596305

 

  1. Genotype-phenotype correlations in Marfan syndrome.

Landis BJ, Veldtman GR, Ware SM.

Heart. 2017 Jun 8. pii: heartjnl-2017-311513. doi: 10.1136/heartjnl-2017-311513. [Epub ahead of print] No abstract available.

PMID: 28596305

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Select item 28532196

 

  1. Left ventricular thrombus or pseudothrombus? A rare cardiac CT artifact.

Rodrigues JCL, Yeong M, Curtis SL, Lyen SM.

Eur Heart J Cardiovasc Imaging. 2017 Jun 6. doi: 10.1093/ehjci/jex149. [Epub ahead of print] No abstract available.

PMID: 28586404

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Select item 28571640

 

  1. Cardiorespiratory Fitness, Not the Severity of the Condition, Dictates Late Outcomes After Fontan Procedures.

d’Udekem Y.

J Am Coll Cardiol. 2017 Jun 6;69(22):2745-2747. doi: 10.1016/j.jacc.2017.03.581. No abstract available.

PMID: 28571640

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Select item 28571639

 

  1. Longitudinal Outcomes of Patients With Single Ventricle After the Fontan Procedure.

Atz AM, Zak V, Mahony L, Uzark K, D’agincourt N, Goldberg DJ, Williams RV, Breitbart RE, Colan SD, Burns KM, Margossian R, Henderson HT, Korsin R, Marino BS, Daniels K, McCrindle BW; Pediatric Heart Network Investigators.

J Am Coll Cardiol. 2017 Jun 6;69(22):2735-2744. doi: 10.1016/j.jacc.2017.03.582.

PMID: 28571639

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Select item 28571637

 

  1. Mitral valve prolapse and Marfan syndrome.

Thacoor A.

Congenit Heart Dis. 2017 Jun 5. doi: 10.1111/chd.12467. [Epub ahead of print] Review.

PMID: 28580713

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Select item 28580712

 

  1. Clinical analyses of 383 cases with maternal cardiac diseases.

Aydin E, Ozyuncu O, Kasapoglu D, Orgul G, Ozer N, Yurdakok M, Beksac MS.

J Perinat Med. 2017 Jun 5. pii: /j/jpme.ahead-of-print/jpm-2017-0023/jpm-2017-0023.xml. doi: 10.1515/jpm-2017-0023. [Epub ahead of print]

PMID: 28622145

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Select item 28582432

 

  1. Feasibility of exercise stress echocardiography and myocardial response in patients with repaired congenital heart disease.

Hasan BS, Lunze FI, Alvi N, Shafer KM, Rhodes J.

Am Heart J. 2017 Jun;188:1-10. doi: 10.1016/j.ahj.2017.02.029. Epub 2017 Feb 23.

PMID: 28577664

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  1. Transition of Care in Congenital Heart Disease: Ensuring the Proper Handoff.

Lee A, Bailey B, Cullen-Dean G, Aiello S, Morin J, Oechslin E.

Curr Cardiol Rep. 2017 Jun;19(6):55. doi: 10.1007/s11886-017-0859-5. Review.

PMID: 28477319

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Select item 28432659

 

  1. Current Role of Blood and Urine Biomarkers in the Clinical Care of Adults with Congenital Heart Disease.

Rajpal S, Alshawabkeh L, Opotowsky AR.

Curr Cardiol Rep. 2017 Jun;19(6):50. doi: 10.1007/s11886-017-0860-z. Review.

PMID: 28432659

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Select item 28306681

 

  1. Evaluation of athletes with complex congenital heart disease.

Bates BA, Richards C, Hall M, Kerut EK, Campbell W, McMullan MR.

Echocardiography. 2017 Jun;34(6):934-936. doi: 10.1111/echo.13562. Epub 2017 May 12.

PMID: 28497583

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Select item 28386957

 

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

Bacich D, Braggion G, Faggian G.

Echocardiography. 2017 Jun;34(6):949-950. doi: 10.1111/echo.13537. Epub 2017 Apr 7.

PMID: 28386957

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Select item 28370416

 

  1. Right ventricular function and N-terminal pro-brain natriuretic peptide levels in adult patients with simple dextro-transposition of the great arteries.

Martínez-Quintana E, Marrero-Negrín N, Gopar-Gopar S, Rodríguez-González F.

Echocardiography. 2017 Jun;34(6):876-880. doi: 10.1111/echo.13526. Epub 2017 Mar 29.

PMID: 28370416

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Select item 28533195

 

  1. Valvular Heart Disease in Adults: Etiologies, Classification, and Diagnosis.

Hollenberg SM.

FP Essent. 2017 Jun;457:11-16.

PMID: 28671804

 

Select item 28620608

 

  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 Jun;65(6):309-320. doi: 10.1007/s11748-017-0777-x. Epub 2017 Apr 24. Review.

PMID: 28439697

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Select item 27831545

 

  1. Aortopathies in adult congenital heart disease and genetic aortopathy syndromes: management strategies and indications for surgery.

Kuijpers JM, Mulder BJ.

Heart. 2017 Jun;103(12):952-966. doi: 10.1136/heartjnl-2015-308626. Epub 2017 Mar 7. No abstract available.

PMID: 28270426

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Select item 28259844

 

  1. Cyanotic congenital heart disease and atherosclerosis.

Tarp JB, Jensen AS, Engstrøm T, Holstein-Rathlou NH, Søndergaard L.

Heart. 2017 Jun;103(12):897-900. doi: 10.1136/heartjnl-2016-311012. Epub 2017 Mar 4. Review.

PMID: 28259844

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Select item 27993911

 

  1. The management of the third stage of labour in women with heart disease.

Cauldwell M, Steer PJ, Swan L, Uebing A, Gatzoulis MA, Johnson MR.

Heart. 2017 Jun;103(12):945-951. doi: 10.1136/heartjnl-2016-310607. Epub 2016 Dec 19.

PMID: 27993911

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Select item 28528723

 

  1. Prognostic predictive value of gene mutations in Japanese patients with hypertrophic cardiomyopathy.

Chida A, Inai K, Sato H, Shimada E, Nishizawa T, Shimada M, Furutani M, Furutani Y, Kawamura Y, Sugimoto M, Ishihara J, Fujiwara M, Soga T, Kawana M, Fuji S, Tateno S, Kuraishi K, Kogaki S, Nishimura M, Ayusawa M, Ichida F, Yamazawa H, Matsuoka R, Nonoyama S, Nakanishi T.

Heart Vessels. 2017 Jun;32(6):700-707. doi: 10.1007/s00380-016-0920-0. Epub 2016 Nov 24.

PMID: 27885498

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Select item 28239760

 

  1. Mortality in pulmonary arterial hypertension due to congenital heart disease: Serial changes improve prognostication.

Schuijt MTU, Blok IM, Zwinderman AH, van Riel ACMJ, Schuuring MJ, de Winter RJ, Duijnhouwer AL, van Dijk APJ, Mulder BJM, Bouma BJ.

Int J Cardiol. 2017 Jun 1. pii: S0167-5273(17)30321-2. doi: 10.1016/j.ijcard.2017.05.101. [Epub ahead of print]

PMID: 28606655

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Select item 28132780

 

  1. Early mortality and concomitant procedures related to Fontan conversion: Quantitative analysis.

Brida M, Baumgartner H, Gatzoulis MA, Diller GP.

Int J Cardiol. 2017 Jun 1;236:132-137. doi: 10.1016/j.ijcard.2017.01.111. Epub 2017 Jan 25.

PMID: 28132780

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Select item 28635060

 

  1. Endothelin inhibitors lower pulmonary vascular resistance and improve functional capacity in patients with Fontan circulation.

Agnoletti G, Gala S, Ferroni F, Bordese R, Appendini L, Pace Napoleone C, Bergamasco L.

J Thorac Cardiovasc Surg. 2017 Jun;153(6):1468-1475. doi: 10.1016/j.jtcvs.2017.01.051. Epub 2017 Feb 10.

PMID: 28283234

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Select item 28268011

 

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

Davidson WR Jr.

JAMA Cardiol. 2017 Jun 1;2(6):671-672. doi: 10.1001/jamacardio.2017.0365. 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 Jun 1;2(6):664-671. doi: 10.1001/jamacardio.2017.0283.

PMID: 28403428

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Select item 28241248

 

  1. Value of Cardiovascular Magnetic Resonance Imaging in Noninvasive Risk Stratification in Tetralogy of Fallot.

Bokma JP, de Wilde KC, Vliegen HW, van Dijk AP, van Melle JP, Meijboom FJ, Zwinderman AH, Groenink M, Mulder BJM, Bouma BJ.

JAMA Cardiol. 2017 Jun 1;2(6):678-683. doi: 10.1001/jamacardio.2016.5818.

PMID: 28241248

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Select item 28384795

 

  1. Transition of Adolescents and Young Adults with Congenital Heart Disease: Challenges, Progress, and Future Improvements.

Nicolarsen J.

Pediatr Ann. 2017 Jun 1;46(6):e224-e228. doi: 10.3928/19382359-20170519-02.

PMID: 28599027

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Select item 28500413

 

  1. Impaired Pulmonary Function is an Additional Potential Mechanism for the Reduction of Functional Capacity in Clinically Stable Fontan Patients.

Turquetto ALR, Canêo LF, Agostinho DR, Oliveira PA, Lopes MICS, Trevizan PF, Fernandes FLA, Binotto MA, Liberato G, Tavares GMP, Neirotti RA, Jatene MB.

Pediatr Cardiol. 2017 Jun;38(5):981-990. doi: 10.1007/s00246-017-1606-9. Epub 2017 May 12.

PMID: 28500413

 

  1. Red Flags for Maltese Adults with Congenital Heart Disease: Poorer Dental Care and Less Sports Participation Compared to Other European Patients-An APPROACH-IS Substudy.

Caruana M, Apers S, Kovacs AH, Luyckx K, Thomet C, Budts W, Sluman M, Eriksen K, Dellborg M, Berghammer M, Johansson B, Soufi A, Callus E, Moons P, Grech V; APPROACH-IS consortium and the International Society for Adult Congenital Heart Disease (ISACHD).

Pediatr Cardiol. 2017 Jun;38(5):965-973. doi: 10.1007/s00246-017-1604-y. Epub 2017 Mar 24.

PMID: 28341902

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Select item 28210768

 

  1. The Lymphatic Circulation in Adaptations to the Fontan Circulation.

Menon S, Chennapragada M, Ugaki S, Sholler GF, Ayer J, Winlaw DS.

Pediatr Cardiol. 2017 Jun;38(5):886-892. doi: 10.1007/s00246-017-1576-y. Epub 2017 Feb 16. Review.

PMID: 28210768

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Select item 28199782

 

  1. Social independence of adult congenital heart disease patients in Japan.

Ochiai R, Ikeda Y, Kato H, Shiraishi I; Parents’ Association of Heart Disease Children.

Pediatr Int. 2017 Jun;59(6):675-681. doi: 10.1111/ped.13260. Epub 2017 Apr 17.

PMID: 28199782

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Select item 27816335

 

  1. A Rare Case of Anomalous Left Coronary Artery From the Pulmonary Artery (Bland-White-Garland Syndrome) in a 68-Year-Old Woman.

Roberts SM, Banbury T, Mehta A.

Semin Cardiothorac Vasc Anesth. 2017 Jun;21(2):186-190. doi: 10.1177/1089253216659146. Epub 2016 Jul 9.

PMID: 27401860

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Select item 27688032

 

  1. TGFβ1- and BMP2/PPARγ-regulated miRNAs in Pulmonary Arterial Hypertension.

Calvier L, Chouvarine P, Legchenko E, Hansmann G.

Am J Respir Crit Care Med. 2017 Jun 27. doi: 10.1164/rccm.201705-0923LE. [Epub ahead of print] No abstract available.

PMID: 28653861

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Select item 28651677

 

  1. Development of Pulmonary Hypertension During Treatment with Diazoxide: A Case Series and Literature Review.

Timlin MR, Black AB, Delaney HM, Matos RI, Percival CS.

Pediatr Cardiol. 2017 Jun 22. doi: 10.1007/s00246-017-1652-3. [Epub ahead of print]

PMID: 28642988

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Select item 28639339

 

  1. A case of intracardiac echinococcosis.

Kara SS, Gullu UU.

Cardiol Young. 2017 Jun 20:1-2. doi: 10.1017/S1047951117001196. [Epub ahead of print]

PMID: 28631585

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Select item 28631576

 

  1. Assessment of early diastolic intraventricular pressure gradient in the left ventricle among patients with repaired tetralogy of Fallot.

Kobayashi M, Takahashi K, Yamada M, Yazaki K, Matsui K, Tanaka N, Shigemitsu S, Akimoto K, Kishiro M, Nakanishi K, Kawasaki S, Nii M, Itatani K, Shimizu T.

Heart Vessels. 2017 Jun 20. doi: 10.1007/s00380-017-1011-6. [Epub ahead of print]

PMID: 28634695

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  1. Effective Cardiac Index and Systemic-Pulmonary Collaterals Evaluated by Cardiac Magnetic Resonance Late After Fontan Palliation.

Ait Ali L, Cadoni A, Rossi G, Keilberg P, Passino C, Festa P.

Am J Cardiol. 2017 Jun 15;119(12):2069-2072. doi: 10.1016/j.amjcard.2017.03.040. Epub 2017 Mar 29.

PMID: 28454633

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Select item 28430547

 

  1. Enhancing Insights into Pulmonary Vascular Disease through a Precision Medicine Approach. A Joint NHLBI-Cardiovascular Medical Research and Education Fund Workshop Report.

Newman JH, Rich S, Abman SH, Alexander JH, Barnard J, Beck GJ, Benza RL, Bull TM, Chan SY, Chun HJ, Doogan D, Dupuis J, Erzurum SC, Frantz RP, Geraci M, Gillies H, Gladwin M, Gray MP, Hemnes AR, Herbst RS, Hernandez AF, Hill NS, Horn EM, Hunter K, Jing ZC, Johns R, Kaul S, Kawut SM, Lahm T, Leopold JA, Lewis GD, Mathai SC, McLaughlin VV, Michelakis ED, Nathan SD, Nichols W, Page G, Rabinovitch M, Rich J, Rischard F, Rounds S, Shah SJ, Tapson VF, Lowy N, Stockbridge N, Weinmann G, Xiao L.

Am J Respir Crit Care Med. 2017 Jun 15;195(12):1661-1670. doi: 10.1164/rccm.201701-0150WS.

PMID: 28430547

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Select item 28618202

 

  1. Results of a phase I/II multi-center investigation of udenafil in adolescents after fontan palliation.

Goldberg DJ, Zak V, Goldstein BH, Chen S, Hamstra MS, Radojewski EA, Maunsell E, Mital S, Menon SC, Schumacher KR, Payne RM, Stylianou M, Kaltman JR, deVries TM, Yeager JL, Paridon SM; Pediatric Heart Network Investigators.

Am Heart J. 2017 Jun;188:42-52. doi: 10.1016/j.ahj.2017.02.030. Epub 2017 Mar 6.

PMID: 28577680

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Select item 28385177

 

  1. Effect of Fontan-Associated Morbidities on Survival With Intact Fontan Circulation.

Allen KY, Downing TE, Glatz AC, Rogers LS, Ravishankar C, Rychik J, Fuller S, Montenegro LM, Steven JM, Spray TL, Nicolson SC, Gaynor JW, Goldberg DJ.

Am J Cardiol. 2017 Jun 1;119(11):1866-1871. doi: 10.1016/j.amjcard.2017.03.004. Epub 2017 Mar 16.

PMID: 28385177

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Select item 28161345

 

  1. Associations of Age and Sex With Marfan Phenotype: The National Heart, Lung, and Blood Institute GenTAC (Genetically Triggered Thoracic Aortic Aneurysms and Cardiovascular Conditions) Registry.

Roman MJ, Devereux RB, Preiss LR, Asch FM, Eagle KA, Holmes KW, LeMaire SA, Maslen CL, Milewicz DM, Morris SA, Prakash SK, Pyeritz RE, Ravekes WJ, Shohet RV, Song HK, Weinsaft JW; GenTAC Investigators*.

Circ Cardiovasc Genet. 2017 Jun;10(3). pii: e001647. doi: 10.1161/CIRCGENETICS.116.001647.

PMID: 28600386

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Select item 28468790

 

  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 Jun;38(5):1087. doi: 10.1007/s00246-017-1617-6. No abstract available.

PMID: 28456830

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Select item 28456829

 

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

Friedland-Little JM, Gajarski RJ, Schumacher KR.

Pediatr Transplant. 2017 Jun;21(4). doi: 10.1111/petr.12925. Epub 2017 Mar 30.

PMID: 28370952

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Select item 28295946

 

  1. Mazindol: a risk factor for pulmonary arterial hypertension?

Konofal E, Benzouid C, Delclaux C, Lecendreux M, Hussey E.

Sleep Med. 2017 Jun;34:168-169. doi: 10.1016/j.sleep.2017.02.020. Epub 2017 Mar 18.

PMID: 28522087

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Select item 27860542

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Pediatric Cardiology Featured Articles of June 2017

 

 

Pediatric Cardiology Reviews of June 2017 Manuscripts

Improved long-term outcomes in double-inlet left ventricle and tricuspid atresia with transposed great arteries: systemic outflow tract obstruction present at birth defines long-term outcome.

Franken LC, Admiraal M, Verrall CE, Zannino D, Ayer JG, Iyengar AJ, Cole AD, Sholler GF, D’Udekem Y, Winlaw DS.

Eur J Cardiothorac Surg. 2017 Jun 1;51(6):1051-1057. doi: 10.1093/ejcts/ezx022.

PMID: 28329058

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Select item 28619364

 

Take home points:

  • The increased mortality in TA-TGA is primarily due to periprocedural events prior to Fontan completion, with systemic outflow tract obstruction as the major risk factor.
  • Similar survival was noted in both groups following the Fontan.

Hershenson, JaredCommentary from Dr. Jared Hershenson (Greater Washington DC), section editor of Pediatric Cardiology Journal Watch:  This Australian group studied outcomes in patients with Tricuspid atresia with transposed great arteries (TA-TGA) versus double inlet left ventricle with L-transposed great arteries (DILV), 2 subtypes of functional single left ventricle with similar features and longer-term issues. Historically, outcomes have been worse in the TA-TGA group. They studied 211 patients (59 TA-TGA and 152 DILV) over a median follow up of 17 years. Systemic outflow tract obstruction (SOTO) was present in 11% of the TA-TGA and 5% of the DILV at birth, and developed over time in 27% of the TA-TGA and 44 of the DILV. Fontan completion was achieved in 62% of the TA-TGA and 91% of the DILV groups. 5-year survival was 78% in the TA-TGA and 94% in the DILV groups. Multivariate Cox regression analysis showed SOTO at birth as the most important risk factor (HR 3.5). The study was unable to determine if initial surgical management affected the outcomes, but it seems clear that those with SOTO at birth, should undergo Norwood/DKS palliation and those that do not have significant SOTO at birth or arch hypoplasia requiring significant repair, undergo the DKS during Stage 2. Whether the initial Norwood/DKS would be best, as opposed to waiting due to the possible neurodevelopmental benefit of delayed major neonatal surgery, remains to be seen. This study does signify that discussion of mortality and long-term issues should not be generalized to single ventricles, but that small differences matter.

June Ped v1

June peds V2

 

Geometry and growth of the reconstructed aorta in patients with hypoplastic left heart syndrome and variants.

Haller C, Chetan D, Saedi A, Parker R, Van Arsdell GS, Honjo O.

J Thorac Cardiovasc Surg. 2017 Jun;153(6):1479-1487.e1. doi: 10.1016/j.jtcvs.2017.02.011. Epub 2017 Feb 14.

PMID: 28291606

 

Take home points:

  • The interdigitating technique for aortic arch reconstruction in the Norwood operation seems to have a much lower reintervention rate for recurrent coarctation than noted in the Single Ventricle Reconstruction trial. A possibly higher rate of reintervention for patching causing vascular compression or compression of adjacent structures was noted.
  • Growth of all segments of the reconstructed aorta is uniform with stable geometry noted after repair until the Fontan.
  • The balance towards preventing re-coarctation and needing intervention for excessive patching may not always be easy to achieve.

Commentary from Dr. Jared Hershenson (Greater Washington DC), section editor of Pediatric Cardiology Journal Watch: Reconstruction of the aortic arch is considered the most important part of the Norwood procedure in those with HLHS, as progressive arch obstruction is a major risk factor for post-operative mortality. The interdigitating technique is meant to decrease the risk of circumferential narrowing due to residual ductal tissue and achieve unobstructed blood flow via wide angles and larger dimensions of the reconstructed proximal arch via pulmonary homograft. The geometry of this neoaortic arch is quite abnormal, therefore long-term follow up has been undertaken to determine risk of future issues. Earlier studies from this group showed a lower incidence of arch obstruction before stage 2 palliation and this study continues to assess this group up to the Fontan. 73 patients with HLHS (or HLHS variants) underwent stage 1 Norwood, 52 patients had pre-stage 2 angiography and 21 patients had pre-Fontan angiography with measurements of the neo-aortic arch following the interdigitating technique. 4 measurements and z-scores were assessed, ascending aorta (AA) just distal to the DKS, transverse arch (TA) at take-off of first supraaortic vessel, interdigitating anastomosis (IA) narrowest diameter distal to the of last supraaortic vessel, and descending aorta (DA) at supradiaphragmatic level (see figure 1). Of the survivors past stage 1 Norwood and the interstage period, 4.1% of patients were treated for recoarctation and 5.5% of patients required reintervention for excessive arch dimensions. Larger AA and TA dimensions and z-scores were noted at both pre-stage 2 and pre-Fontan, and mildly lower dimension and z-scores of the IA and DA. There was no significant increase in the AA and TA by z-score, no significant change in the IA and a small increase in the DA by z-score. Intersegmental ratios remained unchanged except for a trend to smaller ratio between the TA-IA segment. This suggests that the growth of all segments are nearly uniform, the neo-aortic geometry remains the same, and there is no delayed growth restriction in the isthmus. The authors and an attached editorialist discuss that future 3D models may further improve outcomes and allow for individualized surgical treatment.

June peds v3 June peds v4 June peds v5 June peds v6 June peds v8

 

 


 

Cost of Congenital Heart Disease Hospitalizations in Canada: A Population-Based Study.

Mackie AS, Tran DT, Marelli AJ, Kaul P.

Can J Cardiol. 2017 Jun;33(6):792-798. doi: 10.1016/j.cjca.2017.01.024. Epub 2017 Feb 10.

PMID: 28457736

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Select item 28347581

 

Take Home Points:

  • This observational retrospective cohort study from Canada found the cost of inpatient hospitalizations in patients with congenital heart disease increased by almost 22% from 2004 to 2013.
  • The overall total cost of hospitalization was higher in children with CHD than adults.
  • However, comparatively the percentage of cost increase over time was higher in adults with CHD (4.5%) compared to the cost increase over time in children (0.7%).
  • This increase in cost of inpatient hospitalizations still remains less than the published cost increases over time among adults with CHD in the United States.

 

Abarbanell picture smallCommentary from Dr. Ginnie Abarbanell (Atlanta-St. Louis), section editor of Pediatric Cardiology Journal Watch:  This observational retrospective cohort study from Canada evaluated the cost of inpatient hospitalizations in children and adults with congenital heart disease using the Canadian Institute for Health Information (CIHI) Discharge Abstract Database (DAD).  All hospitalizations with a primary diagnosis of CHD or a comorbid condition related to CHD with CHD as the secondary diagnosis were included in the analysis.  Researchers found that the annual CHD inpatient hospitalization costs increased by 21.6% from $99.7 million in 2004 to $121.2 million in 2013 (all values in 2016 Canadian dollars). 64% of the hospitalizations were in children younger than 18 years with the majority of the hospitalizations occurring prior to 1 year of age.  During the study period the cost of hospitalizations was higher in children compared to adults with the majority of the inpatient expense occurring in infancy.  However, comparatively the percentage of cost increase over time was higher in the adults with CHD (4.5%) compared to the cost increase over time in children (0.7%). In 2004, inpatient hospitalizations for adults with CHD was 38.2% of the total costs compared to 45.8% in 2013.  The median length of hospital stay did not change significantly in both children and adults over the time period.  As would be expected, the annual hospitalization cost per patient increased with increasing CHD complexity among children.  The inverse relationship was found among adult patients with CHD in that the annual hospitalization cost per patient was highest among those with simple CHD.  The authors deduced this maybe secondary to an increased mean age and the number of comorbid conditions among adults with simple CHD.  Additionally, over 50% of the adults with a simple CHD were admitted with aortic valve disease or an atrial septal defect in which cases these diagnoses are often treated procedurally.  Also, among adults with CHD there were greater cost increases over time in men compared to women.  However, the cost of pregnancy among women with CHD was not included in the analysis.  As the authors discuss it is difficult to compare these results to published cost analyses from the United States given differences in methods of calculating hospital costs.  Additional, this study used longitudinal patient based cost compared to case based cost used in the US.  However, in the discussion these results were compared to 2 studies {Agarwal:2016gh, Opotowsky:2009df} from the US that looked at increasing costs among adult patients with CHD.  Both of the US studies found a higher increase in hospitalizations among adults with CHD compared to this study.  The study from Opotowsky et al{Opotowsky:2009df} found an increase of 127% in the mean per hospitalization charges from 1998-2005 with a projected increase in total national charges for hospitalizations of 357% from $691 million in 1998 to $3.16 billion in 2005 (2005 US dollars).  A similar study from Agarwal et al {Agarwal:2016gh} found an 81% increase in hospital admissions among adults with CHD from 2003 to 2013.   This current study from Canada as well as previously published studies highlights the increasing financial cost in caring for individuals with CHD with the greatest cost increases in adults with CHD.

 

June peds v9

June peds v10

 

 

 

 


 


 

Predictors of health-related quality of life in children with chronic heart disease.

Niemitz M, Gunst DCM, Hövels-Gürich HH, Hofbeck M, Kaulitz R, Galm C, Berger F, Nagdyman N, Stiller B, Borth-Bruhns T, Konzag I, Balmer C, Goldbeck L.

Cardiol Young. 2017 May 18:1-10. doi: 10.1017/S1047951117000440. [Epub ahead of print]

PMID: 28516823

Similar articles

Select item 28517030

 

Take Home Points:

  • This multicenter study from Germany and Switzerland found that many medical factors including NYHA class, use of current cardiac medication, uncertainty or limited medical prognosis, history of cardiac bypass and number of nights spent in the hospital were independently associated with a decreased health related quality of life.
  • Children and adolescents reported better health related quality of life than the parent proxy report.
  • From a psychosocial aspect school attendance was associated with a higher reported health related quality of life. This would suggest that better integration into the school system could possibly increase the health reported quality of life among children and adolescents with CHD.

 

Commentary from Dr. Ginnie Abarbanell (Atlanta/St. Louis), section editor of Pediatric Cardiology Journal Watch:  This multicenter study from Germany and Switzerland used the Pediatric Cardiac Quality of Life Inventory in 375 children with congenital heart disease and their parental caregivers.  The mean age of the children studied was nearly 13 years (8-18 years).  On patient self-report, NYHA class, school attendance and use of cardiac medication were independently associated with their health-related quality of life.  On the parental-report the following variables, in addition to those reported by the patient, including uncertainty or limited medical prognosis, history of cardiac bypass and number of nights spent in the hospital were also found to be independently associated with a decreased health related quality of life.   These findings indicated that patients with mild cardiac disease severity had higher quality of life inventory scores.  Children and adolescents reported better health related quality of life than their parents but researchers did find a significant strong correlation between the self and parent reported health related quality of life scores.  The only psychosocial variable found to be independently associated with the health-related quality of life was school attendance.   This study would suggest that better integration into the school system could possibly increase the health reported quality of life among children and adolescents with CHD.


 

 

Pediatric Cardiology June 2017

 

  1. Pediatric 320-row cardiac computed tomography using electrocardiogram-gated model-based full iterative reconstruction.

Shirota G, Maeda E, Namiki Y, Bari R, Ino K, Torigoe R, Abe O.

Pediatr Radiol. 2017 Jun 30. doi: 10.1007/s00247-017-3901-2. [Epub ahead of print]

PMID: 28667349

 

Select item 28669924

 

  1. Impact of standardized clinical assessment and management plans on resource utilization and costs in children after the arterial switch operation.

Rathod RH, Jurgen B, Hamershock RA, Friedman KG, Marshall AC, Samnaliev M, Graham DA, Jenkins K, Lock JE, Powell AJ.

Congenit Heart Dis. 2017 Jun 28. doi: 10.1111/chd.12508. [Epub ahead of print]

PMID: 28660735

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Select item 28666343

 

  1. Development of quality metrics for ambulatory pediatric cardiology: Chest pain.

Lu JC, Bansal M, Behera SK, Boris JR, Cardis B, Hokanson JS, Kakavand B, Jedeikin R.

Congenit Heart Dis. 2017 Jun 27. doi: 10.1111/chd.12509. [Epub ahead of print]

PMID: 28653469

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Select item 28653465

 

  1. Introduction to anomalous aortic origin of a coronary artery.

Brothers JA.

Congenit Heart Dis. 2017 Jun 27. doi: 10.1111/chd.12497. [Epub ahead of print]

PMID: 28653465

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Select item 28653340

 

  1. Down syndrome and Moyamoya disease: unusual cause of stroke.

Tavares Bello C, Barreiros C, Gil I, Vasconcelos C.

BMJ Case Rep. 2017 Jun 24;2017. pii: bcr-2017-219894. doi: 10.1136/bcr-2017-219894.

PMID: 28647711

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Select item 28647800

 

  1. Coronary artery anomalies: A multidisciplinary approach to shape the landscape of a challenging problem.

Molossi S, Agrawal H.

Congenit Heart Dis. 2017 Jun 23. doi: 10.1111/chd.12490. [Epub ahead of print]

PMID: 28643441

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Select item 28643402

 

  1. Clinical findings in right ventricular noncompaction in hypoplastic left heart syndrome.

Gardner MM, Cohen MS.

Congenit Heart Dis. 2017 Jun 23. doi: 10.1111/chd.12506. [Epub ahead of print]

PMID: 28643402

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Select item 28643385

 

  1. Pulmonary vascular volume ratio measured by cardiac computed tomography in children and young adults with congenital heart disease: comparison with lung perfusion scintigraphy.

Goo HW, Park SH.

Pediatr Radiol. 2017 Jun 23. doi: 10.1007/s00247-017-3912-z. [Epub ahead of print]

PMID: 28646273

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Select item 28645241

 

  1. Genotype-phenotype correlation for congenital heart disease in Down syndrome through analysis of partial trisomy 21 cases.

Pelleri MC, Gennari E, Locatelli C, Piovesan A, Caracausi M, Antonaros F, Rocca A, Donati CM, Conti L, Strippoli P, Seri M, Vitale L, Cocchi G.

Genomics. 2017 Jun 22. pii: S0888-7543(17)30045-9. doi: 10.1016/j.ygeno.2017.06.004. [Epub ahead of print]

PMID: 28648597

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Select item 28636109

 

  1. Xq26.1-26.3 duplication including MOSPD1 and GPC3 identified in boy with short stature and double outlet right ventricle.

Hirota Y, Minami T, Sato T, Yokomizo A, Matsumoto A, Goto M, Jinbo E, Yamamgata T.

Am J Med Genet A. 2017 Jun 21. doi: 10.1002/ajmg.a.38297. [Epub ahead of print]

PMID: 28636109

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Select item 28639359

 

  1. Interobserver variability in the classification of congenital coronary abnormalities: A substudy of the anomalous connections of the coronary arteries registry.

Koutsoukis A, Halna du Fretay X, Dupouy P, Ou P, Laissy JP, Juliard JM, Hyafil F, Aubry P; ANOCOR Investigators.

Congenit Heart Dis. 2017 Jun 21. doi: 10.1111/chd.12504. [Epub ahead of print]

PMID: 28639359

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Select item 28634971

 

  1. Protein-Losing Enteropathy in Patients With Congenital Heart Disease.

Itkin M, Piccoli DA, Nadolski G, Rychik J, DeWitt A, Pinto E, Rome J, Dori Y.

J Am Coll Cardiol. 2017 Jun 20;69(24):2929-2937. doi: 10.1016/j.jacc.2017.04.023.

PMID: 28619193

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Select item 28629487

 

  1. Adenotonsillectomy for the Management of Pulmonary Hypertension in a Patient With Complex Congenital Heart Disease: A Case Report.

Adler AC, Nicole Chao YJ.

A A Case Rep. 2017 Jun 19. doi: 10.1213/XAA.0000000000000593. [Epub ahead of print]

PMID: 28632506

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Select item 28627098

 

  1. Effect of concomitant birth defects and genetic anomalies on infant mortality in tetralogy of fallot.

Jernigan EG, Strassle PD, Stebbins RC, Meyer RE, Nelson JS.

Birth Defects Res. 2017 Jun 19. doi: 10.1002/bdr2.1057. [Epub ahead of print]

PMID: 28627098

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Select item 28628748

 

  1. Does young age really put the heart at risk?

Belanger MP, Tan LY, Wittnich C.

Can J Physiol Pharmacol. 2017 Jun 19. doi: 10.1139/cjpp-2017-0072. [Epub ahead of print]

PMID: 28628748

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Select item 28648355

 

  1. Diagnosis of secondary pulmonary lymphangiectasia in congenital heart disease: a novel role for chest ultrasound and prognostic implications.

Lam CZ, Bhamare TA, Gazzaz T, Manson D, Humpl T, Seed M.

Pediatr Radiol. 2017 Jun 19. doi: 10.1007/s00247-017-3892-z. [Epub ahead of print]

PMID: 28631156

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Select item 28628610

 

  1. Clinical evaluation of anomalous aortic origin of a coronary artery (AAOCA).

Molossi S, Agrawal H.

Congenit Heart Dis. 2017 Jun 16. doi: 10.1111/chd.12505. [Epub ahead of print]

PMID: 28621042

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Select item 28621552

 

  1. Complete isolation of right subclavian artery supplied by the thoracic aorta and bilateral patent ductus arteriosus.

Ghasemi A, Serati AR, Emami S, Movahed MR.

Future Cardiol. 2017 Jun 16. doi: 10.2217/fca-2016-0062. [Epub ahead of print]

PMID: 28621552

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Select item 28637627

 

  1. Pulmonary arterial resistance and compliance in preterm infants.

Okada S, Muneuchi J, Nagatomo Y, Watanabe M, Iida C, Shirouzu H, Matsuoka R, Joo K.

Int J Cardiol. 2017 Jun 16. pii: S0167-5273(17)31399-2. doi: 10.1016/j.ijcard.2017.06.056. [Epub ahead of print]

PMID: 28637627

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Select item 28629661

 

  1. Cornelia de Lange syndrome: Congenital heart disease in 149 patients.

Ayerza Casas A, Puisac Uriol B, Teresa Rodrigo ME, Hernández Marcos M, Ramos Fuentes FJ, Pie Juste J.

Med Clin (Barc). 2017 Jun 16. pii: S0025-7753(17)30344-5. doi: 10.1016/j.medcli.2017.03.051. [Epub ahead of print] English, Spanish.

PMID: 28629661

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Select item 28629660

 

  1. Characterization and risk factors for aortic dilatation in pediatric patients with bicuspid aortic valve.

Sabaté-Rotés A, Sabidó Sanchez L, Gran Ipiña F, Albert Brotons D, Abella RF, Rosés Noguer F.

Med Clin (Barc). 2017 Jun 16. pii: S0025-7753(17)30326-3. doi: 10.1016/j.medcli.2017.03.041. [Epub ahead of print] English, Spanish.

PMID: 28629660

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Select item 28617789

 

  1. Adequate adherence to benzathine penicillin secondary prophylaxis following the diagnosis of rheumatic heart disease by echocardiographic screening.

Culliford-Semmens N, Tilton E, Webb R, Lennon D, Paku B, Malcolm J, French S, Blair N, Wilson N.

N Z Med J. 2017 Jun 16;130(1457):50-57.

PMID: 28617789

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Select item 28623384

 

  1. Cardiac T1 mapping in congenital heart disease: bolus vs. infusion protocols for measurements of myocardial extracellular volume fraction.

Al-Wakeel-Marquard N, Rastin S, Muench F, O H-Ici D, Yilmaz S, Berger F, Kuehne T, Messroghli DR.

Int J Cardiovasc Imaging. 2017 Jun 15. doi: 10.1007/s10554-017-1191-2. [Epub ahead of print]

PMID: 28620681

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Select item 28614866

 

  1. Critical Congenital Heart Disease Screening in NICU: Need for Revision and Standardization.

Fernandes N, Short B, Manja V, Lakshminrusimha S.

Am J Perinatol. 2017 Jun 14. doi: 10.1055/s-0037-1603654. [Epub ahead of print] No abstract available.

PMID: 28614866

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Select item 28615160

 

  1. Use of 3D models of vascular rings and slings to improve resident education.

Jones TW, Seckeler MD.

Congenit Heart Dis. 2017 Jun 13. doi: 10.1111/chd.12486. [Epub ahead of print]

PMID: 28608434

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Select item 28633370

 

  1. Brain Injury and Neurodevelopmental Outcome in Congenital Heart Disease: A Systematic Review.

Mebius MJ, Kooi EMW, Bilardo CM, Bos AF.

Pediatrics. 2017 Jun 13. pii: e20164055. doi: 10.1542/peds.2016-4055. [Epub ahead of print] Review.

PMID: 28607205

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Select item 28663792

 

  1. Exercise Capacity and Self-Efficacy are Associated with Moderate-to-Vigorous Intensity Physical Activity in Children with Congenital Heart Disease.

Banks L, Rosenthal S, Manlhiot C, Fan CS, McKillop A, Longmuir PE, McCrindle BW.

Pediatr Cardiol. 2017 Jun 12. doi: 10.1007/s00246-017-1645-2. [Epub ahead of print]

PMID: 28608149

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Select item 28608148

 

  1. Hypoplastic Left Heart Syndrome Sequencing Reveals a Novel NOTCH1 Mutation in a Family with Single Ventricle Defects.

Durbin MD, Cadar AG, Williams CH, Guo Y, Bichell DP, Su YR, Hong CC.

Pediatr Cardiol. 2017 Jun 12. doi: 10.1007/s00246-017-1650-5. [Epub ahead of print]

PMID: 28608148

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Select item 28600893

 

  1. Clinical and molecular cytogenetic characterization of four unrelated patients carrying 2p14 microdeletions.

Mathieu ML, Demily C, Chantot-Bastaraud S, Afenjar A, Mignot C, Andrieux J, Gerard M, Catala-Mora J, Jouk PS, Labalme A, Edery P, Sanlaville D, Rossi M.

Am J Med Genet A. 2017 Jun 9. doi: 10.1002/ajmg.a.38307. [Epub ahead of print]

PMID: 28599093

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Select item 28596175

 

  1. Decision making in anomalous aortic origin of a coronary artery.

Mery CM.

Congenit Heart Dis. 2017 Jun 9. doi: 10.1111/chd.12493. [Epub ahead of print]

PMID: 28598033

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Select item 28649564

 

  1. Primary Ciliary Dyskinesia: An Update on Clinical Aspects, Genetics, Diagnosis, and Future Treatment Strategies.

Mirra V, Werner C, Santamaria F.

Front Pediatr. 2017 Jun 9;5:135. doi: 10.3389/fped.2017.00135. eCollection 2017. Review.

PMID: 28649564 Free PMC Article

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Select item 28605441

 

  1. High-Flow Nasal Cannula Utilization in Pediatric Critical Care.

Coletti KD, Bagdure DN, Walker LK, Remy KE, Custer JW.

Respir Care. 2017 Jun 6. pii: respcare.05153. doi: 10.4187/respcare.05153. [Epub ahead of print]

PMID: 28588119

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Select item 28580763

 

  1. Knowledge-based reconstruction for measurement of right ventricular volumes on cardiovascular magnetic resonance images in a mixed population.

Pieterman ED, Budde RPJ, Robbers-Visser D, van Domburg RT, Helbing WA.

Congenit Heart Dis. 2017 Jun 5. doi: 10.1111/chd.12484. [Epub ahead of print]

PMID: 28580763

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Select item 28580760

 

  1. Stress echocardiography: An overview for use in pediatric and congenital cardiology.

Ermis P.

Congenit Heart Dis. 2017 Jun 5. doi: 10.1111/chd.12495. [Epub ahead of print]

PMID: 28580760

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Select item 28580730

 

  1. Right aortic arch with situs solitus frequently heralds a vascular ring.

Evans WN, Acherman RJ, Ciccolo ML, Carrillo SA, Mayman GA, Luna CF, Rollins RC, Castillo WJ, Galindo A, Rothman A, Alexander JA, Kwan TW, Restrepo H.

Congenit Heart Dis. 2017 Jun 5. doi: 10.1111/chd.12487. [Epub ahead of print]

PMID: 28580730

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Select item 28580713

 

  1. Kawasaki disease: State of the art.

Newburger JW.

Congenit Heart Dis. 2017 Jun 5. doi: 10.1111/chd.12498. [Epub ahead of print]

PMID: 28580712

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Select item 28580660

 

  1. Kawasaki disease: Medical therapies.

Newburger JW.

Congenit Heart Dis. 2017 Jun 5. doi: 10.1111/chd.12502. [Epub ahead of print]

PMID: 28580631

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Select item 28622145

 

  1. In vivo efficacy of the AKT inhibitor ARQ 092 in Noonan Syndrome with multiple lentigines-associated hypertrophic cardiomyopathy.

Wang J, Chandrasekhar V, Abbadessa G, Yu Y, Schwartz B, Kontaridis MI.

PLoS One. 2017 Jun 5;12(6):e0178905. doi: 10.1371/journal.pone.0178905. eCollection 2017.

PMID: 28582432 Free PMC Article

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Select item 28662918

 

  1. Reproducibility of Cardiac Magnetic Resonance Imaging (CMRI)-Derived Right Ventricular Parameters in Repaired Tetralogy of Fallot (ToF).

Gnanappa GK, Rashid I, Celermajer D, Ayer J, Puranik R.

Heart Lung Circ. 2017 Jun 3. pii: S1443-9506(17)30491-2. doi: 10.1016/j.hlc.2017.04.017. [Epub ahead of print]

PMID: 28662918

 

Select item 28586709

 

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

Sharma A, Kumar S, Sharma S.

Acta Neurochir (Wien). 2017 Jun;159(6):1105-1106. doi: 10.1007/s00701-017-3175-3. Epub 2017 Apr 8.

PMID: 28391445

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Select item 28235129

 

  1. Pulse oximetry is a feasible method for detecting neonatal critical congenital heart disease.

Hu XJ, Huang GY.

Acta Paediatr. 2017 Jun;106(6):1008. doi: 10.1111/apa.13801. Epub 2017 Mar 21. No abstract available.

PMID: 28235129

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Select item 28075515

 

  1. The limitations of pulse oximetry for critical congenital heart disease screening in the neonatal intensive care units.

Fernandes N, Lakshminrusimha S.

Acta Paediatr. 2017 Jun;106(6):1007. doi: 10.1111/apa.13742. Epub 2017 Jan 30. No abstract available.

PMID: 28075515

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Select item 28577664

 

  1. Interruption of the Aortic Arch.

Combes N, Waldmann V, Heitz F, Bruguiere E, Quedreux JF, Vahdat O, Chambran P, Narayanan K, Marijon E.

Am J Med. 2017 Jun;130(6):e251-e252. doi: 10.1016/j.amjmed.2016.12.041. Epub 2017 Feb 2. No abstract available.

PMID: 28161345

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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 Jun;173(6):1575-1585. doi: 10.1002/ajmg.a.38237. Epub 2017 Apr 19.

PMID: 28425218

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Select item 28422456

 

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

Sun Z, Lee SY.

Anatol J Cardiol. 2017 Jun;17(6):423-435. doi: 10.14744/AnatolJCardiol.2017.7464. Epub 2017 Apr 10.

PMID: 28430115 Free PMC Article

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Select item 28457478

 

  1. [Mosaic trisomy 18. Series of cases].

Cammarata-Scalisi F, Lacruz-Rengel MA, Araque D, Da Silva G, Avendaño A, Callea M, Stock F, Guerrero Y, Aguilar E, Lacruz MJ, Sulbaran J.

Arch Argent Pediatr. 2017 Jun 1;115(3):e183-e186. doi: http://dx.doi.org/10.5546/aap.2017.e183. Spanish.

PMID: 28504507 Free Article

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Select item 28504505

 

  1. Retinovascular findings in newborns with critical congenital heart disease: A case series.

Fettah N, Kabatas EU, Doğan V, Zenciroğlu AL, Dilli D, Özyazıcı E, Koç M, Beken S, Dursun A, Kandemir S.

Arch Argent Pediatr. 2017 Jun 1;115(3):e175-e178. doi: http://dx.doi.org/10.5546/aap.2017.eng.e175. Spanish, English.

PMID: 28504505 Free Article

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Select item 28536187

 

  1. Ethnic and socioeconomic variation in incidence of congenital heart defects.

Knowles RL, Ridout D, Crowe S, Bull C, Wray J, Tregay J, Franklin RC, Barron DJ, Cunningham D, Parslow RC, Brown KL.

Arch Dis Child. 2017 Jun;102(6):496-502. doi: 10.1136/archdischild-2016-311143. Epub 2016 Dec 16.

PMID: 27986699 Free Article

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Select item 27530240

 

  1. Newborn pulse oximetry screening in practice.

Ismail AQT, Cawsey M, Ewer AK.

Arch Dis Child Educ Pract Ed. 2017 Jun;102(3):155-161. doi: 10.1136/archdischild-2016-311047. Epub 2016 Aug 16.

PMID: 27530240

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Select item 28544650

 

  1. Cost of Congenital Heart Disease Hospitalizations in Canada: A Population-Based Study.

Mackie AS, Tran DT, Marelli AJ, Kaul P.

Can J Cardiol. 2017 Jun;33(6):792-798. doi: 10.1016/j.cjca.2017.01.024. Epub 2017 Feb 10.

PMID: 28457736

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Select item 28347581

 

  1. Validity of Commercial Activity Trackers in Children With Congenital Heart Disease.

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

Can J Cardiol. 2017 Jun;33(6):799-805. doi: 10.1016/j.cjca.2016.11.024. Epub 2016 Dec 18.

PMID: 28347581

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Select item 28457248

 

  1. Ebstein Anomaly.

Sherwin ED, Abrams DJ.

Card Electrophysiol Clin. 2017 Jun;9(2):245-254. doi: 10.1016/j.ccep.2017.02.007. Review.

PMID: 28457239

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Select item 28457237

 

  1. Recent Advances in Cardiovascular Magnetic Resonance: Techniques and Applications.

Salerno M, Sharif B, Arheden H, Kumar A, Axel L, Li D, Neubauer S.

Circ Cardiovasc Imaging. 2017 Jun;10(6). pii: e003951. doi: 10.1161/CIRCIMAGING.116.003951. Review.

PMID: 28611116

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Select item 28559421

 

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

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

Clin Radiol. 2017 Jun;72(6):502-507. doi: 10.1016/j.crad.2017.01.017. Epub 2017 Mar 6.

PMID: 28267987

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Select item 28460704

 

  1. Continuous Capnography in Pediatric Intensive Care.

Riley CM.

Crit Care Nurs Clin North Am. 2017 Jun;29(2):251-258. doi: 10.1016/j.cnc.2017.01.010. Epub 2017 Mar 18. Review.

PMID: 28460704

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Select item 28477319

 

  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 Jun 1;18(6):621-628. doi: 10.1093/ehjci/jex063.

PMID: 28407053

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Select item 28329058

 

  1. Improved long-term outcomes in double-inlet left ventricle and tricuspid atresia with transposed great arteries: systemic outflow tract obstruction present at birth defines long-term outcome.

Franken LC, Admiraal M, Verrall CE, Zannino D, Ayer JG, Iyengar AJ, Cole AD, Sholler GF, D’Udekem Y, Winlaw DS.

Eur J Cardiothorac Surg. 2017 Jun 1;51(6):1051-1057. doi: 10.1093/ejcts/ezx022.

PMID: 28329058

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Select item 28619364

 

  1. Outcome of arterial ischemic stroke in children with heart disease.

Vázquez López M, de Castro de Castro P, Barredo Valderrama E, Miranda Herrero MC, Gil Villanueva N, Alcaraz Romero AJ, Pascual Pascual SI.

Eur J Paediatr Neurol. 2017 Jun 1. pii: S1090-3798(17)31684-7. doi: 10.1016/j.ejpn.2017.05.007. [Epub ahead of print]

PMID: 28619364

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Select item 27858403

 

  1. Cardiac rehabilitation in an adolescent with DiGeorge Syndrome.

Kim DJ, Lee KY, Choe Y, Han JY, Choi IS.

Eur J Phys Rehabil Med. 2017 Jun;53(3):462-465. doi: 10.23736/S1973-9087.16.04408-7. Epub 2016 Nov 18.

PMID: 27858403 Free Article

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Select item 27752831

 

  1. Effect of Fontan operation on liver stiffness in children with single ventricle physiology.

DiPaola FW, Schumacher KR, Goldberg CS, Friedland-Little J, Parameswaran A, Dillman JR.

Eur Radiol. 2017 Jun;27(6):2434-2442. doi: 10.1007/s00330-016-4614-x. Epub 2016 Oct 17.

PMID: 27752831

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Select item 28532240

 

  1. Hepatic Malignancy in an Infant with Wolf-Hirschhorn Syndrome.

Rutter S, Morotti RA, Peterec S, Gallagher PG.

Fetal Pediatr Pathol. 2017 Jun;36(3):256-262. doi: 10.1080/15513815.2017.1293201. Epub 2017 Mar 7.

PMID: 28266898

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Select item 28671804

 

  1. A Path to Implement Precision Child Health Cardiovascular Medicine.

Touma M, Reemtsen B, Halnon N, Alejos J, Finn JP, Nelson SF, Wang Y.

Front Cardiovasc Med. 2017 Jun 1;4:36. doi: 10.3389/fcvm.2017.00036. eCollection 2017. Review.

PMID: 28620608 Free PMC Article

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Select item 28439697

 

  1. Neuroimaging findings in Mowat-Wilson syndrome: a study of 54 patients.

Garavelli L, Ivanovski I, Caraffi SG, Santodirocco D, Pollazzon M, Cordelli DM, Abdalla E, Accorsi P, Adam MP, Baldo C, Bayat A, Belligni E, Bonvicini F, Breckpot J, Callewaert B, Cocchi G, Cuturilo G, Devriendt K, Dinulos MB, Djuric O, Epifanio R, Faravelli F, Formisano D, Giordano L, Grasso M, Grønborg S, Iodice A, Iughetti L, Lacombe D, Maggi M, Malbora B, Mammi I, Moutton S, Møller R, Muschke P, Napoli M, Pantaleoni C, Pascarella R, Pellicciari A, Poch-Olive ML, Raviglione F, Rivieri F, Russo C, Savasta S, Scarano G, Selicorni A, Silengo M, Sorge G, Tarani L, Tone LG, Toutain A, Trimouille A, Valera ET, Vergano SS, Zanotta N, Zollino M, Dobyns WB, Paciorkowski AR.

Genet Med. 2017 Jun;19(6):691-700. doi: 10.1038/gim.2016.176. Epub 2016 Nov 10.

PMID: 27831545 Free PMC Article

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Select item 28270426

 

  1. Incidence and Outcome of Acute Cardiorenal Syndrome in Hospitalized Children.

Athwani V, Bhargava M, Chanchlani R, Mehta AJ.

Indian J Pediatr. 2017 Jun;84(6):420-424. doi: 10.1007/s12098-017-2307-3. Epub 2017 Feb 27.

PMID: 28239760

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Select item 28606655

 

  1. Stress and coping in Japanese mothers whose infants required congenital heart disease surgery.

Nakazuru A, Sato N, Nakamura N.

Int J Nurs Pract. 2017 Jun;23 Suppl 1. doi: 10.1111/ijn.12550.

PMID: 28635060

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Select item 28379508

 

  1. Echocardiography-Derived Left Ventricular Outflow Tract Gradient and Left Ventricular Posterior Wall Thickening Are Associated with Outcomes for Anatomic Repair in Congenitally Corrected Transposition of the Great Arteries.

Moodley S, Balasubramanian S, Tacy TA, Chan F, Hanley FL, Punn R.

J Am Soc Echocardiogr. 2017 Jun 1. pii: S0894-7317(17)30219-5. doi: 10.1016/j.echo.2017.03.019. [Epub ahead of print]

PMID: 28579248

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Select item 28391001

 

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

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

J Am Soc Echocardiogr. 2017 Jun;30(6):561-571. doi: 10.1016/j.echo.2017.01.018. Epub 2017 Apr 6.

PMID: 28391001

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Select item 28497553

 

  1. Regression equations for calculation of z scores for echocardiographic measurements of right heart structures in healthy Han Chinese children.

Wang SS, Zhang YQ, Chen SB, Huang GY, Zhang HY, Zhang ZF, Wu LP, Hong WJ, Shen R, Liu YQ, Zhu JX.

J Clin Ultrasound. 2017 Jun;45(5):293-303. doi: 10.1002/jcu.22436. Epub 2017 Jan 25.

PMID: 28121016

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Select item 28230599

 

  1. Teaching congenital heart disease: A new era?

Beckerman Z, Mery CM.

J Thorac Cardiovasc Surg. 2017 Jun;153(6):1541. doi: 10.1016/j.jtcvs.2017.02.045. Epub 2017 Mar 9. No abstract available.

PMID: 28359576

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Select item 28314534

 

  1. Geometry and growth of the reconstructed aorta in patients with hypoplastic left heart syndrome and variants.

Haller C, Chetan D, Saedi A, Parker R, Van Arsdell GS, Honjo O.

J Thorac Cardiovasc Surg. 2017 Jun;153(6):1479-1487.e1. doi: 10.1016/j.jtcvs.2017.02.011. Epub 2017 Feb 14.

PMID: 28291606

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Select item 28283234

 

  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, Damtew SA, Ahmed MB, Akseer N, Al-Raddadi R, Alemayohu MA, Altirkawi K, Abajobir AA, Amare AT, Antonio CAT, Arnlov J, Artaman A, Asayesh H, Avokpaho EFGA, Awasthi A, Ayala Quintanilla BP, Bacha U, Betsu BD, 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, Ding EL, Dubey M, Endries AY, Erskine HE, Faraon EJA, 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 IAM, 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 El Razek H, 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 AMN, 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, Silpakit 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 CDA, Woodbrook R, Yano Y, Yonemoto N, Yoon SJ, Younis MZ, Yu C, Zaki MES, Zegeye EA, Zuhlke LJ, Murray CJL, Vos T.

JAMA Pediatr. 2017 Jun 1;171(6):573-592. doi: 10.1001/jamapediatrics.2017.0250.

PMID: 28384795

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Select item 28593158

 

  1. Critical Congenital Heart Disease Newborn Screening Implementation: Lessons Learned.

McClain MR, Hokanson JS, Grazel R, Van Naarden Braun K, Garg LF, Morris MR, Moline K, Urquhart K, Nance A, Randall H, Sontag MK.

Matern Child Health J. 2017 Jun;21(6):1240-1249. doi: 10.1007/s10995-017-2273-4.

PMID: 28092064

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Select item 28591039

 

  1. Genetic polymorphism of methylenetetrahydrofolate reductase as a potential risk factor for congenital heart disease: A meta-analysis in Chinese pediatric population.

Yuan Y, Yu X, Niu F, Lu N.

Medicine (Baltimore). 2017 Jun;96(23):e7057. doi: 10.1097/MD.0000000000007057.

PMID: 28591039 Free PMC Article

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Select item 28393221

 

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

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

Mol Med Rep. 2017 Jun;15(6):3658-3664. doi: 10.3892/mmr.2017.6425. Epub 2017 Apr 3.

PMID: 28393221 Free PMC Article

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Select item 28393185

 

  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 Jun;38(5):991-1003. doi: 10.1007/s00246-017-1607-8. Epub 2017 Apr 5.

PMID: 28382463

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Select item 28374048

 

  1. Trends in National Institutes of Health-Funded Congenital Heart Disease Research from 2005 to 2015.

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

Pediatr Cardiol. 2017 Jun;38(5):974-980. doi: 10.1007/s00246-017-1605-x. Epub 2017 Mar 27.

PMID: 28349207

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Select item 28341902

 

  1. [Infective endocarditis prophylaxis in congenital heart disease].

Di Filippo S.

Presse Med. 2017 Jun;46(6 Pt 1):606-611. doi: 10.1016/j.lpm.2017.05.016. Epub 2017 May 30. French.

PMID: 28576635

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Select item 28555455

 

  1. Motivations and Features of Co-Parenting an Infant With Complex Congenital Heart Disease.

Pridham K, Harrison TM, McKechnie AC, Brown R.

West J Nurs Res. 2017 Jun 1:193945917712693. doi: 10.1177/0193945917712693. [Epub ahead of print]

PMID: 28597724

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Select item 28606231

 

  1. Pediatric Cardiology Provider Attitudes About Palliative Care: A Multicenter Survey Study.

Balkin EM, Kirkpatrick JN, Kaufman B, Swetz KM, Sleeper LA, Wolfe J, Blume ED.

Pediatr Cardiol. 2017 Jun 29. doi: 10.1007/s00246-017-1663-0. [Epub ahead of print]

PMID: 28664445

 

Select item 28662346

 

  1. FOXC1 haploinsufficiency due to 6p25 deletion in a patient with rapidly progressing aortic valve disease.

Ovaert C, Busa T, Faure E, Missirian C, Philip N, Paoli F, Milh M, Macé L, Zaffran S.

Am J Med Genet A. 2017 Jun 28. doi: 10.1002/ajmg.a.38331. [Epub ahead of print]

PMID: 28657660

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Select item 28658286

 

  1. MYBPC3 mutations are associated with a reduced super-relaxed state in patients with hypertrophic cardiomyopathy.

McNamara JW, Li A, Lal S, Bos JM, Harris SP, van der Velden J, Ackerman MJ, Cooke R, Dos Remedios CG.

PLoS One. 2017 Jun 28;12(6):e0180064. doi: 10.1371/journal.pone.0180064. eCollection 2017.

PMID: 28658286 Free Article

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Select item 28660303

 

  1. Assessment of large copy number variants in patients with apparently isolated congenital left-sided cardiac lesions reveals clinically relevant genomic events.

Hanchard NA, Umana LA, D’Alessandro L, Azamian M, Poopola M, Morris SA, Fernbach S, Lalani SR, Towbin JA, Zender GA, Fitzgerald-Butt S, Garg V, Bowman J, Zapata G, Hernandez P, Arrington CB, Furthner D, Prakash SK, Bowles NE, McBride KL, Belmont JW.

Am J Med Genet A. 2017 Jun 27. doi: 10.1002/ajmg.a.38309. [Epub ahead of print]

PMID: 28653806

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Select item 28653861

 

  1. Anomalous origin of the left coronary artery from the right pulmonary artery in a univentricular heart.

Binsalamah ZM, Lara DA, McKenzie ED.

Cardiol Young. 2017 Jun 27:1-4. doi: 10.1017/S1047951117001317. [Epub ahead of print]

PMID: 28651676

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Select item 28651675

 

  1. Responses of mothers of children with CHD: quality of life, anxiety and depression, parental attitudes, family functionality.

Alkan F, Sertcelik T, Yalın Sapmaz S, Eser E, Coskun S.

Cardiol Young. 2017 Jun 27:1-7. doi: 10.1017/S1047951117001184. [Epub ahead of print]

PMID: 28651675

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Select item 28651669

 

  1. Gastrointestinal haemorrhage due to lymphangiectasia caused by protein-losing enteropathy in the Fontan circulation.

Gras P, Gottrand F, Godart F.

Cardiol Young. 2017 Jun 27:1-3. doi: 10.1017/S1047951117000853. [Epub ahead of print]

PMID: 28651669

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Select item 28653469

 

  1. Development of quality metrics for ambulatory pediatric cardiology: Chest pain.

Lu JC, Bansal M, Behera SK, Boris JR, Cardis B, Hokanson JS, Kakavand B, Jedeikin R.

Congenit Heart Dis. 2017 Jun 27. doi: 10.1111/chd.12509. [Epub ahead of print]

PMID: 28653469

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Select item 28656474

 

  1. Dissecting Kawasaki disease: a state-of-the-art review.

Dietz SM, van Stijn D, Burgner D, Levin M, Kuipers IM, Hutten BA, Kuijpers TW.

Eur J Pediatr. 2017 Jun 27. doi: 10.1007/s00431-017-2937-5. [Epub ahead of print] Review.

PMID: 28656474

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Select item 28653463

 

  1. Cardiac Events and the Maximum Diameter of Coronary Artery Aneurysms in Kawasaki Disease.

Tsuda E, Tsujii N, Hayama Y.

J Pediatr. 2017 Jun 26. pii: S0022-3476(17)30748-5. doi: 10.1016/j.jpeds.2017.05.055. [Epub ahead of print]

PMID: 28662948

 

  1. Right ventricular function during exercise in children after heart transplantation.

Cifra B, Morgan CT, Dragulescu A, Guerra VC, Slorach C, Friedberg MK, Manlhiot C, McCrindle BW, Dipchand AI, Mertens L.

Eur Heart J Cardiovasc Imaging. 2017 Jun 23. doi: 10.1093/ehjci/jex137. [Epub ahead of print]

PMID: 28655190

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Select item 28651802

 

  1. Physician Attitudes toward the First Pediatric Appropriate Use Criteria and Engagement With Educational Intervention to Improve the Appropriateness of Outpatient Echocardiography.

Sachdeva R, Kelleman MS, McCracken CE, Campbell RM, Lai WW, Lopez L, Stern KWD, Welch E, Douglas PS.

J Am Soc Echocardiogr. 2017 Jun 23. pii: S0894-7317(17)30385-1. doi: 10.1016/j.echo.2017.05.007. [Epub ahead of print]

PMID: 28651801

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Select item 28646244

 

  1. In-scan and scan-rescan assessment of LV in- and outflow volumes by 4D flow MRI versus 2D planimetry.

Kamphuis VP, van der Palen RLF, de Koning PJH, Elbaz MSM, van der Geest RJ, de Roos A, Roest AAW, Westenberg JJM.

J Magn Reson Imaging. 2017 Jun 22. doi: 10.1002/jmri.25792. [Epub ahead of print]

PMID: 28640394

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Select item 28643997

 

  1. How Should We Diagnose Persistence of the Artery of the Fifth Pharyngeal Arch?

Tretter JT, Crotty EJ, Anderson RH, Taylor MD.

Pediatr Cardiol. 2017 Jun 21. doi: 10.1007/s00246-017-1665-y. [Epub ahead of print] No abstract available.

PMID: 28639150

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Select item 28639149

 

  1. Gadolinium Brain Deposition after Macrocyclic Gadolinium Administration: A Pediatric Case-Control Study.

Tibussek D, Rademacher C, Caspers J, Turowski B, Schaper J, Antoch G, Klee D.

Radiology. 2017 Jun 21:161151. doi: 10.1148/radiol.2017161151. [Epub ahead of print]

PMID: 28640695

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Select item 28631585

 

  1. Echocardiographic Evaluation of Coronary Abnormalities and Cardiac Function in a Murine Model of Kawasaki Disease Using High-frequency Ultrasound.

Zhang XX, Du ZD, Wen SG, Sun XP.

Chin Med J (Engl). 2017 Jun 20;130(12):1467-1474. doi: 10.4103/0366-6999.207461.

PMID: 28584211 Free PMC Article

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Select item 28450351

 

  1. Evaluation and Management of Pulmonary Hypertension in Children with Bronchopulmonary Dysplasia.

Krishnan U, Feinstein JA, Adatia I, Austin ED, Mullen MP, Hopper RK, Hanna B, Romer L, Keller RL, Fineman J, Steinhorn R, Kinsella JP, Ivy DD, Rosenzweig EB, Raj U, Humpl T, Abman SH; Pediatric Pulmonary Hypertension Network (PPHNet).

J Pediatr. 2017 Jun 20. pii: S0022-3476(17)30650-9. doi: 10.1016/j.jpeds.2017.05.029. [Epub ahead of print] No abstract available.

PMID: 28645441

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Select item 28630369

 

  1. ALPK3 GENE MUTATION IN A PATIENT WITH CONGENITAL CARDIOMYOPATHY AND DYSMORPHIC FEATURES.

Cağlayan AO, Sezer RG, Kaymakcalan H, Ulgen E, Yavuz T, Baranoski JF, Bozaykut A, Serin Harmanci A, Yalcin Y, Youngblood MW, Yasuno K, Bilguvar K, Gunel M.

Cold Spring Harb Mol Case Stud. 2017 Jun 19. pii: mcs.a001859. doi: 10.1101/mcs.a001859. [Epub ahead of print]

PMID: 28630369 Free Article

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Select item 28627028

 

  1. Pulmonary Vein Doppler Patterns in Infants with Single Right Ventricle Anomalies After Initial Staged Palliations.

Kirkpatrick EC, Steltzer J, Simpson P, Pan A, Dragulescu A, Falkensammer CB, Gelehrter S, Lai WW, Levine J, Miller S, Miller TA, Pruetz J, Sachdeva R, Thacker D, Frommelt P.

Pediatr Cardiol. 2017 Jun 19. doi: 10.1007/s00246-017-1660-3. [Epub ahead of print]

PMID: 28631208

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Select item 28631207

 

  1. A Computable Phenotype Improves Cohort Ascertainment in a Pediatric Pulmonary Hypertension Registry.

Geva A, Gronsbell JL, Cai T, Cai T, Murphy SN, Lyons JC, Heinz MM, Natter MD, Patibandla N, Bickel J, Mullen MP, Mandl KD; Pediatric Pulmonary Hypertension Network and National Heart, Lung, and Blood Institute Pediatric Pulmonary Vascular Disease Outcomes Bioinformatics Clinical Coordinating Center Investigators.

J Pediatr. 2017 Jun 15. pii: S0022-3476(17)30658-3. doi: 10.1016/j.jpeds.2017.05.037. [Epub ahead of print]

PMID: 28625502

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Select item 28620754

 

  1. Massive aortic root aneurysm in an infant with the Loeys-Dietz syndrome.

Molina-Sánchez T, Calderón-Colmenero J, Sandoval JP.

Cardiol Young. 2017 Jun 13:1-2. doi: 10.1017/S1047951117001056. [Epub ahead of print]

PMID: 28606209

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Select item 28611076

 

  1. Learning a Comorbidity-Driven Taxonomy of Pediatric Pulmonary Hypertension.

Ong MS, Mullen MP, Austin ED, Szolovits P, Natter M, Geva A, Cai T, Kong SW, Mandl KD.

Circ Res. 2017 Jun 13. pii: CIRCRESAHA.117.310804. doi: 10.1161/CIRCRESAHA.117.310804. [Epub ahead of print]

PMID: 28611076

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Select item 28606952

 

  1. Exercise Capacity and Self-Efficacy are Associated with Moderate-to-Vigorous Intensity Physical Activity in Children with Congenital Heart Disease.

Banks L, Rosenthal S, Manlhiot C, Fan CS, McKillop A, Longmuir PE, McCrindle BW.

Pediatr Cardiol. 2017 Jun 12. doi: 10.1007/s00246-017-1645-2. [Epub ahead of print]

PMID: 28608149

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Select item 28608148

 

  1. Coarctation Index Predicts Recurrent Aortic Arch Obstruction Following Surgical Repair of Coarctation of the Aorta in Infants.

Adamson G, Karamlou T, Moore P, Natal-Hernandez L, Tabbutt S, Peyvandi S.

Pediatr Cardiol. 2017 Jun 12. doi: 10.1007/s00246-017-1651-4. [Epub ahead of print]

PMID: 28608147

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Select item 28603837

 

  1. Strain Rate in Children and Young Piglets Mirrors Changes in Contractility and Demonstrates a Force-Frequency Relationship.

Alvarez SV, Fortin-Pellerin E, Alhabdan M, Lomelin JS, Kantoch M, Atallah J, Hornberger LK, Coe JY, Cheung PY, Tham EB, Mah K, Lin L, Mills L, Khoo NS.

J Am Soc Echocardiogr. 2017 Jun 8. pii: S0894-7317(17)30273-0. doi: 10.1016/j.echo.2017.04.001. [Epub ahead of print]

PMID: 28602206

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Select item 28598945

 

  1. Changes of Cerebral Oxygenation in Sequential Glenn and Fontan Procedures in the Same Children.

Yagi Y, Yamamoto M, Saito H, Mori T, Morimoto Y, Oyasu T, Tachibana T, Ito YM.

Pediatr Cardiol. 2017 Jun 7. doi: 10.1007/s00246-017-1647-0. [Epub ahead of print]

PMID: 28589407

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Select item 28589406

 

  1. Comparison of two-dimensional strain analysis using vendor-independent and vendor-specific software in adult and pediatric patients.

Anwar S, Negishi K, Borowszki A, Gladding P, Popović ZB, Erenberg F, Thomas JD.

JRSM Cardiovasc Dis. 2017 Jun 6;6:2048004017712862. doi: 10.1177/2048004017712862. eCollection 2017 Jan-Dec.

PMID: 28660071 Free PMC Article

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Select item 28590987

 

  1. Stress echocardiography: An overview for use in pediatric and congenital cardiology.

Ermis P.

Congenit Heart Dis. 2017 Jun 5. doi: 10.1111/chd.12495. [Epub ahead of print]

PMID: 28580760

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Select item 28580730

 

  1. Pediatric pulmonary arterial hypertension: on the eve of growing up.

Douwes JM, Berger RMF.

Curr Opin Pulm Med. 2017 Jun 5. doi: 10.1097/MCP.0000000000000406. [Epub ahead of print]

PMID: 28590293

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Select item 28595766

 

  1. Improving Universal Pediatric Lipid Screening.

DeSantes K, Dodge A, Eickhoff J, Peterson AL.

J Pediatr. 2017 Jun 5. pii: S0022-3476(17)30651-0. doi: 10.1016/j.jpeds.2017.05.030. [Epub ahead of print]

PMID: 28595766

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Select item 28582794

 

  1. Non-receptor type, proline-rich protein tyrosine kinase 2 (Pyk2) is a possible therapeutic target for Kawasaki disease.

Suzuki C, Nakamura A, Miura N, Fukai K, Ohno N, Yahata T, Okamoto-Hamaoka A, Fujii M, Yoshioka A, Kuchitsu Y, Ikeda K, Hamaoka K.

Clin Immunol. 2017 Jun;179:17-24. doi: 10.1016/j.clim.2017.01.013. Epub 2017 Feb 3.

PMID: 28167306

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Select item 28089618

 

  1. Infective endocarditis in children: an update.

Dixon G, Christov G.

Curr Opin Infect Dis. 2017 Jun;30(3):257-267. doi: 10.1097/QCO.0000000000000370.

PMID: 28319472

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Select item 28480564

 

  1. Distribution of strain patterns in children with dilated cardiomyopathy.

den Boer SL, du Marchie Sarvaas GJ, Klitsie LM, van Iperen GG, Tanke RB, Helbing WA, Backx APCM, Rammeloo LAJ, Dalinghaus M, Ten Harkel ADJ.

Echocardiography. 2017 Jun;34(6):881-887. doi: 10.1111/echo.13548. Epub 2017 May 7.

PMID: 28480564

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Select item 28370259

 

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

Echocardiography. 2017 Jun;34(6):888-897. doi: 10.1111/echo.13529. Epub 2017 Apr 1.

PMID: 28370259

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Select item 28329048

 

  1. Isolated left subclavian artery arising from the main pulmonary artery.

Ciliberti P, Toscano A, Drago F, Secinaro A.

Eur Heart J Cardiovasc Imaging. 2017 Jun 1;18(6):716. doi: 10.1093/ehjci/jew337. No abstract available.

PMID: 28329048

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Select item 28402400

 

  1. Arthrogryposis as neonatal presentation of Loeys-Dietz syndrome due to a novel TGFBR2 mutation.

Valenzuela I, Fernández-Alvarez P, Munell F, Sanchez-Montanez A, Giralt G, Vendrell T, Tizzano EF.

Eur J Med Genet. 2017 Jun;60(6):303-307. doi: 10.1016/j.ejmg.2017.03.010. Epub 2017 Mar 24.

PMID: 28344185

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Select item 27454068

 

  1. Importance of multidisciplinary management for pulmonary atresia, ventricular septal defect, major aorto-pulmonary collateral arteries and completely absent central pulmonary arteries.

Hoashi T, Yazaki S, Kagisaki K, Kitano M, Shimada M, Shiraishi I, Ichikawa H.

Gen Thorac Cardiovasc Surg. 2017 Jun;65(6):337-342. doi: 10.1007/s11748-017-0765-1. Epub 2017 Mar 4.

PMID: 28260150

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Select item 28394184

 

  1. Pilot study analyzing automated ECG screening of hypertrophic cardiomyopathy.

Campbell MJ, Zhou X, Han C, Abrishami H, Webster G, Miyake CY, Sower CT, Anderson JB, Knilans TK, Czosek RJ.

Heart Rhythm. 2017 Jun;14(6):848-852. doi: 10.1016/j.hrthm.2017.02.011. Epub 2017 Feb 11.

PMID: 28193509

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Select item 27885498

 

  1. Mutation in mitochondrial complex IV subunit COX5A causes pulmonary arterial hypertension, lactic acidemia, and failure to thrive.

Baertling F, Al-Murshedi F, Sánchez-Caballero L, Al-Senaidi K, Joshi NP, Venselaar H, van den Brand MA, Nijtmans LG, Rodenburg RJ.

Hum Mutat. 2017 Jun;38(6):692-703. doi: 10.1002/humu.23210. Epub 2017 Mar 23.

PMID: 28247525

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Select item 28408091

 

  1. Retroesophageal course of left brachiocephalic vein: The garland vein.

Auti OB, Kaushik PK, Ramachandra P, Raj V.

J Card Surg. 2017 Jun;32(6):394-395. doi: 10.1111/jocs.13141. Epub 2017 May 10. No abstract available.

PMID: 28493417

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Select item 28493392

 

  1. Corrigendum to “Risk stratification and pattern of cardiotoxicity in pediatric Ewing sarcoma” [J. Egypt Natl. Cancer Instit. 29 (2017) 53-56].

Moussa E, Zamzam M, Kamel A, Salah Z, Attia I, Gaber L, Soliman R, Ezzat S.

J Egypt Natl Canc Inst. 2017 Jun;29(2):119. doi: 10.1016/j.jnci.2017.06.001. Epub 2017 Jun 7. No abstract available.

PMID: 28601426 Free Article

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Select item 28638157

 

  1. Prevalence and risk factors associated with the patency of ductus arteriosus in premature neonates: a prospective observational study from Iran.

Pourarian S, Farahbakhsh N, Sharma D, Cheriki S, Bijanzadeh F.

J Matern Fetal Neonatal Med. 2017 Jun;30(12):1460-1464. doi: 10.1080/14767058.2016.1219991. Epub 2016 Aug 25.

PMID: 27485933

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Select item 27432486

 

  1. Technologist corner: Reducing the small-heart effect in pediatric gated myocardial perfusion single-photon emission computed tomography.

Pagnanelli R, Camposano H, Borges-Neto S.

J Nucl Cardiol. 2017 Jun;24(3):944-945. doi: 10.1007/s12350-016-0573-5. Epub 2016 Jun 20. No abstract available.

PMID: 27324348

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Select item 28497001

 

  1. Klinefelter Syndrome in Association with Tetralogy of Fallot and Congenital Diaphragmatic Hernia.

Swaminathan S, Gorla SR, Barbouth DS.

J Pediatr Genet. 2017 Jun;6(2):115-117. doi: 10.1055/s-0036-1597932. Epub 2017 Jan 3.

PMID: 28497001

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Select item 28283234

 

  1. Obstructive Sleep Apnea and Pulmonary Hypertension in Children.

Ingram DG, Singh AV, Ehsan Z, Birnbaum BF.

Paediatr Respir Rev. 2017 Jun;23:33-39. doi: 10.1016/j.prrv.2017.01.001. Epub 2017 Jan 11. Review.

PMID: 28185814

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Select item 27964948

 

  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 Jun;38(5):1042-1048. doi: 10.1007/s00246-017-1616-7. Epub 2017 Apr 29.

PMID: 28456833

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Select item 28456832

 

  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 Jun;38(5):1057-1064. doi: 10.1007/s00246-017-1619-4. Epub 2017 Apr 29.

PMID: 28456832

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Select item 28456831

 

  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 Jun;38(5):915-921. doi: 10.1007/s00246-017-1597-6. Epub 2017 Apr 12.

PMID: 28401252

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Select item 28401251

 

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

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

Pediatr Cardiol. 2017 Jun;38(5):1024-1031. doi: 10.1007/s00246-017-1612-y. Epub 2017 Apr 11.

PMID: 28401251

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Select item 28396934

 

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

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

Pediatr Cardiol. 2017 Jun;38(5):1010-1015. doi: 10.1007/s00246-017-1609-6. Epub 2017 Mar 27.

PMID: 28345114

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Select item 28341902

 

  1. Hypoplastic Left Heart Syndrome is not Associated with Worse Clinical or Neurodevelopmental Outcomes Than Other Cardiac Pathologies After the Norwood-Sano Operation.

Martin BJ, De Villiers Jonker I, Joffe AR, Bond GY, Acton BV, Ross DB, Robertson CMT, Rebeyka IM, Atallah J.

Pediatr Cardiol. 2017 Jun;38(5):922-931. doi: 10.1007/s00246-017-1598-5. Epub 2017 Mar 24.

PMID: 28341901

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Select item 28337515

 

  1. Distribution of Kawasaki Disease Coronary Artery Aneurysms and the Relationship to Coronary Artery Diameter.

Tsuda E, Tsujii N, Kimura K, Suzuki A.

Pediatr Cardiol. 2017 Jun;38(5):932-940. doi: 10.1007/s00246-017-1599-4. Epub 2017 Mar 20.

PMID: 28321483

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Select item 28315943

 

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

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

Pediatr Cardiol. 2017 Jun;38(5):959-964. doi: 10.1007/s00246-017-1602-0. Epub 2017 Mar 18.

PMID: 28315943

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Select item 28271153

 

  1. The Lymphatic Circulation in Adaptations to the Fontan Circulation.

Menon S, Chennapragada M, Ugaki S, Sholler GF, Ayer J, Winlaw DS.

Pediatr Cardiol. 2017 Jun;38(5):886-892. doi: 10.1007/s00246-017-1576-y. Epub 2017 Feb 16. Review.

PMID: 28210768

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Select item 28190140

 

  1. Strategies to Prevent Cast Formation in Patients with Plastic Bronchitis Undergoing Heart Transplantation.

Parent JJ, Darragh RK, Gossett JG, Ryan TD, Villa CR, Lorts A, Jefferies JL, Towbin JA, Chin C.

Pediatr Cardiol. 2017 Jun;38(5):1077-1079. doi: 10.1007/s00246-017-1568-y. Epub 2017 Jan 19.

PMID: 28101662

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Select item 28574914

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

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