Congenital EP Featured Articles of May 2017

Congenital and Pediatric Cardiac EP Reviews of May 2017 Manuscripts

 

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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Take Home Points:

 

  • For single ventricle patients with ≥50% ventricular pacing, there was a high rate of moderate-to-severe ventricular systolic dysfunction (68%), AV valve regurgitation (55%), and death/transplantation (23%) in this study.
  • Compared to controls that were matched for age, similar ventricular morphology, and follow up duration, all 3 of the above endpoints were much more frequently observed in those with chronic ventricular pacing vs non-paced controls

 

J MooreCommentary by Dr. Jeremy Moore (Los Angeles) Congenital and Pediatric Cardiac EP section editor:  This was a single center study from 2 institutions over a 25-year period (1990 – 2015) that included patients with single ventricle congenital heart disease and ≥ 50% ventricular pacing. Cases with ventricular pacing were matched to a non-paced single ventricle control group in a 1:2 fashion for age, gender, single ventricle morphology, and similar follow-up duration.

The authors noted similar baseline characteristics between cases and controls, including similar degrees of ventricular systolic function and AV valve regurgitation before pacing. Ventricular morphology was also similar and was most commonly related to HLHS (40%), followed by DILV (24%), TA (13%), and DORV (12%). The pacing indication was divided nearly equally between postoperative AV block and nonsurgical advanced second or third-degree AV block.

The main study findings were that patients exposed to ventricular pacing fared far worse than their non-paced controls. Patients in the paced group were more likely to develop moderate-to-severe ventricular systolic dysfunction (68% vs 15%), moderate-to-severe AV valve regurgitation (55% vs 8%), were more often treated with heart failure medication (65% vs 21%), and experienced transplant/death (23% vs 6%) vs controls over a mean of 6.6 years.

May ep 1

 

Red, severe; yellow, moderate; green, mild; blue, no ventricular systolic dysfunction

The authors concluded that single ventricle patients requiring ventricular pacing are at a much higher risk for developing moderate-to-severe ventricular systolic dysfunction and AV valve regurgitation compared to those who are not paced. There was a 5-fold increased risk for death or heart transplantation. These risks appeared to be independent of baseline characteristics and attributable solely to chronic ventricular pacing.

 

Arrhythmia burden and related outcomes in Eisenmenger syndrome.

Baskar S, Horne P, Fitzsimmons S, Khoury PR, Vettukattill J, Miwa K, Awake T, Spence M, Sakazaki H, Veldtman G.

Congenit Heart Dis. 2017 May 19. doi: 10.1111/chd.12481. [Epub ahead of print]

PMID: 28523857

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Take Home Points:

 

  • AV valve regurgitation and prolonged QRS duration appear to be two important factors associated with arrhythmia in adult patients with Eisenmenger syndrome.
  • Arrhythmias occurred in 18% of Eisenmenger patients in this study, with the most common mechanisms being supraventricular tachycardia (57%), followed by NSVT (21%) and VT (21%).
  • The most important factor associated with sudden death by multivariate analysis in this study was prior therapy with antiarrhythmic drugs.

 

Commentary by Dr. Jeremy Moore (Los Angeles) Congenital and Pediatric Cardiac EP section editor:  This was a multicenter retrospective study of arrhythmias in adult Eisenmenger patients (≥ 18 years of age) at 2 centers in the U.K. and one in Japan. The authors were able to identify a total of 167 patients with Eisenmenger syndrome who had complete follow-up data. Clinical tachycardias were classified as NSVT, VT, or SVT (the latter further characterized as AF, AF/AFL, and paroxysmal SVT); whereas atrial and ventricular ectopy were excluded from the analysis. The authors looked at univariate and multivariate predictors of 3 major predefined outcomes that included 1) arrhythmia, 2) overall mortality, and 3) sudden death.

 

Arrhythmia occurrence was relatively common, being observed in 30 (18%) patients and were most frequently classified as SVT in 57%, followed by NSVT/VT in 42%. Predictors of arrhythmia included AV valve regurgitation, prolonged QRS duration, and absence of Downs syndrome.

May ep 2

 

Of the total cohort, 21 patients died and 8 patients died suddenly (3 with documented VF at the time of the arrest). Predictors of overall mortality included any history of arrhythmia, antiarrhythmic drug therapy, and higher NYHA class. Predictors of sudden death included documented arrhythmia and need for antiarrhythmic drug therapy. In the multivariate analysis, only antiarrhythmic drug therapy predicted sudden death.

May EP 3

 

 DTT = disease targeting therapy (i.e. advanced pulmonary hypertension therapy)

 

The authors concluded that arrhythmias in this group of ACHD patients are common, affecting approximately one-fifth of patients and that AV valve regurgitation and prolonged QRS duration were associated. Likewise, arrhythmias themselves were associated with all-cause mortality and sudden death.

 

These study findings are not novel, but do strengthen prior observations regarding risk for sudden death in the adult Eisenmenger population. The largest report evaluating this risk previously was the case-control study from the Netherlands in 2012 which included 33 cases of sudden death.* This prior study by Koyak and colleagues only reported bivariate associations, but found that heart failure symptoms (OR 2.7), heart failure medication (OR 3.), antiarrhythmic drug therapy (OR 3.4), QRS duration (1.34 per 10-ms increase), impaired systemic ventricular function (OR 8.2), impaired subpulmonary ventricular function (OR 5.0) and increased creatinine (OR 1.12 per 10 µMol increase) were all associated with sudden death. Clearly, more data on the risk for sudden death in this population is needed, but a relationship with prior arrhythmic event and especially antiarrhythmic drug therapy seems particularly important based on data from this smaller, but updated report.

 

*Koyak et al. Circulation 2012; 126(16):1944-1954

 

CHD EP May 2017

 

  1. Congenital heart block and immune mediated sensorineural hearing loss: possible cross reactivity of immune response.

Bason C, Pagnini I, Brucato A, Maestroni S, Puccetti A, Lunardi C, Cimaz R.

Lupus. 2017 Jul;26(8):835-840. doi: 10.1177/0961203316682099. Epub 2016 Dec 5.

PMID: 27913750

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

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

Presse Med. 2017 Jun 2. pii: S0755-4982(17)30112-4. doi: 10.1016/j.lpm.2017.02.006. [Epub ahead of print] French.

PMID: 28583744

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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. 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. Predictors of acute inefficacy and the radiofrequency energy time required for cavotricuspid isthmus-dependent atrial flutter ablation.

Pérez-Rodon J, Rodriguez-García J, Sarrias-Merce A, Rivas-Gandara N, Roca-Luque I, Francisco-Pascual J, Santos-Ortega A, Martín-Sánchez G, Ferreira-González I, Rodríguez-Palomares J, Evangelista-Masip A, García-Dorado D, Moya-Mitjans À.

J Interv Card Electrophysiol. 2017 Jun;49(1):83-91. doi: 10.1007/s10840-017-0232-z. Epub 2017 Mar 6.

PMID: 28265782

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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. Permanent nonselective His bundle pacing in an adult with L-transposition of the great arteries and complete AV block.

Kean AC, Kay WA, Patel JK, Miller JM, Dandamudi G.

Pacing Clin Electrophysiol. 2017 May 24. doi: 10.1111/pace.13107. [Epub ahead of print]

PMID: 28543795

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  1. Analysis of adults with congenital heart disease presenting to pediatric emergency departments with arrhythmias.

Mohan S, Moffett BS, Lam W, de la Uz C, Miyake C, Valdes SO, Kim JJ.

Congenit Heart Dis. 2017 May 22. doi: 10.1111/chd.12478. [Epub ahead of print]

PMID: 28544787

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  1. Analysis of Screening Electrocardiogram for the Subcutaneous Defibrillator in Adults with Congenital Heart Disease.

Thomas VC, Peterson M, McDaniel M, Restrepo H, Rothman A, Jain A.

Pediatr Cardiol. 2017 May 22. doi: 10.1007/s00246-017-1635-4. [Epub ahead of print]

PMID: 28534239

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  1. Arrhythmia burden and related outcomes in Eisenmenger syndrome.

Baskar S, Horne P, Fitzsimmons S, Khoury PR, Vettukattill J, Niwa K, Agaki T, Spence M, Sakazaki H, Veldtman G.

Congenit Heart Dis. 2017 May 19. doi: 10.1111/chd.12481. [Epub ahead of print]

PMID: 28523857

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  1. Force-Sensing Catheters During Pediatric Radiofrequency Ablation: The FEDERATION Study.

Dalal AS, Nguyen HH, Bowman T, Van Hare GF, Avari Silva JN.

J Am Heart Assoc. 2017 May 17;6(5). pii: e005772. doi: 10.1161/JAHA.117.005772.

PMID: 28515113 Free Article

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  1. Hypoplastic Left Heart Syndrome with Congenital Complete Heart Block.

Al-Kubaisi M, Aly SA, Mohammad Nijres B, Awad S.

Pediatr Cardiol. 2017 May 17. doi: 10.1007/s00246-017-1631-8. [Epub ahead of print]

PMID: 28512719

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  1. Epicardial cardioverter-defibrillator implantation in a 4-month-old infant bridged to heart transplantation.

Carro C, Cereda AF, Annoni G, Marianeschi SM.

Interact Cardiovasc Thorac Surg. 2017 May 16. doi: 10.1093/icvts/ivx129. [Epub ahead of print]

PMID: 28510647

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  1. Conventional and right-sided screening for subcutaneous ICD in a population with congenital heart disease at high risk of sudden cardiac death.

Alonso P, Osca J, Rueda J, Cano O, Pimenta P, Andres A, Sancho MJ, Martinez L.

Ann Noninvasive Electrocardiol. 2017 May 15. doi: 10.1111/anec.12461. [Epub ahead of print]

PMID: 28508439

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  1. Complete heart block complicating the acute phase of respiratory syncytial virus bronchiolitis.

Karatza AA, Kiaffas M, Rammos S.

Pediatr Pulmonol. 2017 May 9. doi: 10.1002/ppul.23714. [Epub ahead of print]

PMID: 28486756

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  1. Utility of Post-Mortem Genetic Testing in Cases of Sudden Arrhythmic Death Syndrome.

Lahrouchi N, Raju H, Lodder EM, Papatheodorou E, Ware JS, Papadakis M, Tadros R, Cole D, Skinner JR, Crawford J, Love DR, Pua CJ, Soh BY, Bhalshankar JD, Govind R, Tfelt-Hansen J, Winkel BG, van der Werf C, Wijeyeratne YD, Mellor G, Till J, Cohen MC, Tome-Esteban M, Sharma S, Wilde AAM, Cook SA, Bezzina CR, Sheppard MN, Behr ER.

J Am Coll Cardiol. 2017 May 2;69(17):2134-2145. doi: 10.1016/j.jacc.2017.02.046.

PMID: 28449774 Free PMC Article

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  1. Outcomes of Direct Current Cardioversion in Adults With Congenital Heart Disease.

Egbe AC, Asirvatham SJ, Connolly HM, Kapa S, Desimone CV, Vaidya VR, Deshmukh AJ, Khan AR, McLeod CJ, Melduni RM, Ammash NM.

Am J Cardiol. 2017 May 1;119(9):1468-1472. doi: 10.1016/j.amjcard.2017.01.018. Epub 2017 Feb 9.

PMID: 28262200

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  1. Increased risk of thromboembolic events in adult congenital heart disease patients with atrial tachyarrhythmias.

Masuda K, Ishizu T, Niwa K, Takechi F, Tateno S, Horigome H, Aonuma K.

Int J Cardiol. 2017 May 1;234:69-75. doi: 10.1016/j.ijcard.2017.02.004. Epub 2017 Feb 5.

PMID: 28209388

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  1. Acute Cardiac MRI Assessment of Radiofrequency Ablation Lesions for Pediatric Ventricular Arrhythmia: Feasibility and Clinical Correlation.

Grant EK, Berul CI, Cross RR, Moak JP, Hamann KS, Sumihara K, Cronin I, O’Brien KJ, Ratnayaka K, Hansen MS, Kellman P, Olivieri LJ.

J Cardiovasc Electrophysiol. 2017 May;28(5):517-522. doi: 10.1111/jce.13197. Epub 2017 Mar 28.

PMID: 28245348

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  1. Repetitive ventricular tachycardia in a syncopal child: Cause or incidental finding ?

Massin M, Jacquemart C, Khaldi K.

Ann Noninvasive Electrocardiol. 2017 May 30. doi: 10.1111/anec.12472. [Epub ahead of print]

PMID: 28557290

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  1. Family Screening for Brugada Syndrome in Asymptomatic Young Patients. Is it Better not to Know?

Gonzalez Corcia MC, Brugada P.

Pediatr Cardiol. 2017 May 26. doi: 10.1007/s00246-017-1637-2. [Epub ahead of print] No abstract available.

PMID: 28550419

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Select item 28549058

 

  1. Permanent nonselective His bundle pacing in an adult with L-transposition of the great arteries and complete AV block.

Kean AC, Kay WA, Patel JK, Miller JM, Dandamudi G.

Pacing Clin Electrophysiol. 2017 May 24. doi: 10.1111/pace.13107. [Epub ahead of print]

PMID: 28543795

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Select item 28540398

 

  1. Analysis of adults with congenital heart disease presenting to pediatric emergency departments with arrhythmias.

Mohan S, Moffett BS, Lam W, de la Uz C, Miyake C, Valdes SO, Kim JJ.

Congenit Heart Dis. 2017 May 22. doi: 10.1111/chd.12478. [Epub ahead of print]

PMID: 28544787

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Select item 28531295

 

  1. The effects of gender on electrical therapies for the heart: physiology, epidemiology, and access to therapies: A report from the XII Congress of the Italian Association on Arrhythmology and Cardiostimulation (AIAC).

Boriani G, Lorenzetti S, Cerbai E, Oreto G, Bronzetti G, Malavasi VL, Biffi A, Padeletti L, Botto G, Diemberger I.

Europace. 2017 May 19. doi: 10.1093/europace/eux068. [Epub ahead of print]

PMID: 28525585

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  1. Cost of Unnecessary Testing in the Evaluation of Pediatric Syncope.

Redd C, Thomas C, Willis M, Amos M, Anderson J.

Pediatr Cardiol. 2017 May 18. doi: 10.1007/s00246-017-1625-6. [Epub ahead of print]

PMID: 28523341

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  1. Pacing Induced Ventricular Dysfunction in a Child: Improvement with Reduction in Paced Rate.

Garg S, Balaji S.

Pediatr Cardiol. 2017 May 17. doi: 10.1007/s00246-017-1629-2. [Epub ahead of print]

PMID: 28512720

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  1. Propranolol Versus Digoxin in the Neonate for Supraventricular Tachycardia (from the Pediatric Health Information System).

Bolin EH, Lang SM, Tang X, Collins RT.

Am J Cardiol. 2017 May 15;119(10):1605-1610. doi: 10.1016/j.amjcard.2017.02.017. Epub 2017 Mar 1.

PMID: 28363353

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  1. A Mutation in the G-Protein Gene GNB2 Causes Familial Sinus Node and Atrioventricular Conduction Dysfunction.

Stallmeyer B, Kuß J, Kotthoff S, Zumhagen S, Vowinkel K, Rinné S, Matschke LA, Friedrich C, Schulze-Bahr E, Rust S, Seebohm G, Decher N, Schulze-Bahr E.

Circ Res. 2017 May 12;120(10):e33-e44. doi: 10.1161/CIRCRESAHA.116.310112. Epub 2017 Feb 20.

PMID: 28219978

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  1. Efficacy of Flecainide in the Treatment of Catecholaminergic Polymorphic Ventricular Tachycardia: A Randomized Clinical Trial.

Kannankeril PJ, Moore JP, Cerrone M, Priori SG, Kertesz NJ, Ro PS, Batra AS, Kaufman ES, Fairbrother DL, Saarel EV, Etheridge SP, Kanter RJ, Carboni MP, Dzurik MV, Fountain D, Chen H, Ely EW, Roden DM, Knollmann BC.

JAMA Cardiol. 2017 May 10. doi: 10.1001/jamacardio.2017.1320. [Epub ahead of print]

PMID: 28492868

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  1. Successful catheter ablation of ventricular ectopy in a young patient with implanted Melody valve.

Jan M, Žižek D, Mazic U, Klemen L, Mijovski G, Peichl P.

Europace. 2017 May 4. doi: 10.1093/europace/eux033. [Epub ahead of print] No abstract available.

PMID: 28472281

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  1. Phase-contrast magnet resonance imaging reveals regional, transmural, and base-to-apex dispersion of mechanical dysfunction in patients with long QT syndrome.

Brado J, Dechant MJ, Menza M, Komancsek A, Lang CN, Bugger H, Foell D, Jung BA, Stiller B, Bode C, Odening KE.

Heart Rhythm. 2017 May 4. pii: S1547-5271(17)30578-7. doi: 10.1016/j.hrthm.2017.04.045. [Epub ahead of print]

PMID: 28479515

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  1. Efficacy of Intravenous Sotalol for Treatment of Incessant Tachyarrhythmias in Children.

Li X, Zhang Y, Liu H, Jiang H, Ge H, Zhang Y.

Am J Cardiol. 2017 May 1;119(9):1366-1370. doi: 10.1016/j.amjcard.2017.01.034. Epub 2017 Feb 10.

PMID: 28283175 Free Article

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  1. Intraoperative Three-Dimensional Mapping of Supraventricular Tachycardia in a Young Fontan Patient.

Perry JC, Williams MR, Shepard S, Bratincsak A, Murphy C, Murthy R, Ebrahim M, Lamberti JJ.

Ann Thorac Surg. 2017 May;103(5):e453-e456. doi: 10.1016/j.athoracsur.2016.10.069.

PMID: 28431725

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  1. Retrosternal Implantation of the Cardioverter-Defibrillator Lead in an Infant.

Hata H, Sumitomo N, Nakai T, Amano A.

Ann Thorac Surg. 2017 May;103(5):e449-e451. doi: 10.1016/j.athoracsur.2016.10.063.

PMID: 28431724

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Select item 28431723

 

  1. Wolff-Parkinson-White syndrome: a single exercise stress test might be misleading.

Salavitabar A, Silver ES, Liberman L.

Cardiol Young. 2017 May;27(4):804-807. doi: 10.1017/S1047951116002055. Epub 2016 Nov 21.

PMID: 27869041

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Select item 27839529

 

  1. A Toddler With Central Venous Catheter Develops Tachycardia.

Shapiro DR, Dudek CJ, Ho JG.

Clin Pediatr (Phila). 2017 May;56(5):496-499. doi: 10.1177/0009922816661331. Epub 2016 Jul 26. No abstract available.

PMID: 27466077

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Select item 28235145

 

  1. Junctional ectopic tachycardia in late period after early postoperative complete atrioventricular block: Messenger of return to normal sinus rhythm? : Explanation with four case series.

Ozyilmaz I, Ergul Y, Ozyilmaz S, Guzeltas A.

J Electrocardiol. 2017 May – Jun;50(3):378-382. doi: 10.1016/j.jelectrocard.2017.01.015. Epub 2017 Feb 1.

PMID: 28189280

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CHD Electrophysiology Articles of February 2016

1. PACES/HRS Expert Consensus Statement on the use of Catheter Ablation in Children and Patients with Congenital Heart Disease: Developed in partnership with the Pediatric and Congenital Electrophysiology Society (PACES) and the Heart Rhythm Society (HRS). Endorsed by the governing bodies of PACES, HRS, the American Academy of Pediatrics (AAP), and the American Heart Association (AHA).

Philip Saul J, Kanter RJ, Abrams D, Asirvatham S, Bar-Cohen Y, Blaufox AD, Cannon B, Clark J, Dick M, Freter A, Kertesz NJ, Kirsh JA, Kugler J, LaPage M, McGowan FX, Miyake CY, Nathan A, Papagiannis J, Paul T, Pflaumer A, Skanes AC, Stevenson WG, Von Bergen N, Zimmerman F; Task Force Co-Chairs. Heart Rhythm. 2016 Feb 17. PMID: 26899545

 

A PatelComment from Dr. Akash Patel (San Francisco, CA), section editor of Congenital Heart Electrophysiology Journal Watch:  This 2016 consensus statement provides the most up-to-date guidelines for the use of catheter ablation in pediatric patients (0-18 years of age) and all congenital heart disease patients.  The last consensus statement was made in 2002 and since then there have been many advances in technical innovations to improve the safety and efficacy of catheter ablation.  In addition, ablation therapy has become an important option in arrhythmia management based on our improved understanding of arrhythmia mechanisms in these populations.

Overall, this consensus statement provides a nice review of the evidence used to make recommendations for catheter-based ablation in this population.

Take Home Points:

  1. Catheter ablation for children and patients with congenital heart disease can be very effective based on arrhythmia type and location.
  2. Catheter ablation for children and patients with congenital heart disease has a low risk profile that has improved over time.

 

 

2. Implantable cardioverter-defibrillators in adults with congenital heart disease: a systematic review and meta-analysis.

Vehmeijer JT, Brouwer TF, Limpens J, Knops RE, Bouma BJ, Mulder BJ, de Groot JR. Eur Heart J. 2016 Feb 11. PMID:26873095

Comment from Dr. Akash Patel (San Francisco, CA), section editor of Congenital Heart Electrophysiology Journal Watch:  This systematic review and meta-analysis of published studies prior to November 2014 was used to investigate the indications, efficacy, and ICD-related harm for ICD implantation in adults with congenital heart disease.

The study utilized standard medical publication databases and included all patients ≥ 16 years who had congenital heart disease and underwent ICD implantation and met inclusion/exclusion criteria for the study.

Twenty-four studies were included which identified 2162 ACHD patients who underwent ICD implantation.  Transvenous ICD was employed in at least 96% of patients. The mean age was 36 years with the majority of patients having repaired tetralogy of Fallot or transposition of the great arteries (see figure below adapted from paper).

ep 2.1

Approximately half (53%) underwent implantation for primary prevention. Indications included predominately non-sustained ventricular tachycardia, impaired systemic ventricular function, inducible ventricular tachycardia, and syncope (see figure below adapted from paper).

ep 2.2

The remainder underwent implantation for secondary prevention: sustained VT (61%) and cardiac arrest (39%).

Secondary aim analysis was based on complete data that was only available for 20-25% on to the total cohort.

Regarding appropriate implantable cardioverter-defibrillator interventions, 24% of patients received one or more appropriate interventions with a mean follow-up of 3.7 years.  Twenty-two percent of patients with primary prevention and 35% of patients with secondary prevention received appropriate interventions.  There was no difference seen in the pooled data between those with or without tetralogy of Fallot.

Regarding inappropriate implantable cardioverter-defibrillator interventions, 25% of patients received one or more inappropriate interventions with a mean follow-up of 3.7 years.  The majority of inappropriate shocks occurred due to supraventricular tachycardia (68%).

Regarding implantable cardioverter-defibrillator complications,  26% of patients had an ICD-related complication with a mean follow-up of 3.8 years.  The majority of complications (76%) involved lead- or generator-related issues (see figure below adapted from paper).

ep 2.3

Regarding mortality, there was no ICD-related death reported.  Overall, 10% of patients died (based on 15 studies with n=440) with a mean follow-up of 3.7 years.  The majority of deaths were due to heart failure (41%) and other cardiac causes (11%).  Interestingly, 18% of patients had sudden cardiac death despite ICD implantation. Data regarding the specifics were not provided.

This study nicely summarizes and adds to our understanding of who is receiving ICD implantation in patients with ACHD. Almost half of all ACHD patients who received ICDs are for primary prevention with the main indications including non-sustained or inducible ventricular arrhythmias, impaired systemic ventricular function, and syncope.  The largest cohorts include tetralogy of Fallot or Transposition of the great arteries.

However, this study does not aid our understanding of who should receive ICD therapy based on specific congenital defects and what pre-implantation risk factors predicted appropriate therapy.   Though ICD therapy was effective in 24% of patients, there continues to be an increased rate of inappropriate therapy and ICD-related complications as compared to the non-ACHD population.   Unfortunately, this study design does not provide the specifics of each individual case to allow for a more robust analysis that would impact further recommendations regarding ICD therapy use in the ACHD population.

Take Home Points:

  1. Knowledge of the outcomes of ICD implantation in ACHD patients is limited as compared to adult patients with non-structural adult heart disease and follow-up times are shorter.
  2. Almost half of patients (53%) underwent primary prevention implantation with the majority of patients having tetralogy of Fallot or transposition of the great arteries.
  3. Appropriate ICD therapy was demonstrated in 24% of patients.
  4. Inappropriate shocks occurred in 25% of patients.
  5. ICD-related complications were seen in 26% of patients.
  6. The overall mortality occurred in 10% of ACHD patients with ICD implantation though the follow-up duration and data is limited and not generalizable.

 

3. The Impact of Clinical and Genetic Findings on The Management of Young Brugada Syndrome Patients. Andorin A, Behr ER, Denjoy I, Crotti L, Dagradi F, Jesel L, Sacher F, Petit B, Mabo P, Maltret A, Wong LC, Degand B, Bertaux G, Maury P, Dulac Y, Delasalle B, Gourraud JB, Babuty D, Blom NA, Schwartz PJ, Wilde AA, Probst V. Heart Rhythm. 2016 Feb 24. PMID:26921764

Comment from Dr. Akash Patel (San Francisco, CA), section editor of Congenital Heart Electrophysiology Journal Watch:  This multi-center study from 16 European tertiary care centers aimed to describe the clinical presentation of Brugada Syndrome below the age of 19, to identify prognostic factors useful to improve risk stratification, and to propose an approach to management of this population.  This is the largest study to date regarding Brugada syndrome in young patients.

The study included 106 patients with a type 1 Brugada pattern either spontaneously or drug-induced.   The median age at diagnosis was 11 years with a median follow-up of 4.5 years with no sex differences seen under the age of 15.  Family history of sudden cardiac death was present in 43%.

Only 20% of patients were symptomatic at diagnosis which included syncope (71%), aborted sudden cardiac death (20%) and ventricular tachycardia (9%).  Males were 2.5 times more likely to have symptoms. The majority of patients were asymptomatic at diagnosis likely due to familial screening (63%).  Typically, sudden death with this disease is mainly seen in the adult population but interestingly, 5 of the 6 patients with aborted sudden cardiac death and ventricular tachycardia were under the age of 5 years.

The advent of genetic testing and familial screening has improved detection of this disease as mentioned.  Only 75 patients in the cohort underwent genetic testing but 77% were genotype positive.  In addition, 43% of index probands demonstrated a positive test for SCN5A mutations.  Interestingly, 16% of genotype positive patients had a life threatening event during follow-up while no patients with negative testing did.  However, this study lacked the ability to determine whether the presence or absence of an SCNA mutation would be useful for risk stratification.

ECG findings at diagnosis revealed that 34% presented with spontaneous type 1 Brugada pattern while the remaining were elicited with provocative testing.  Males were more likely to have a spontaneous type 1 Brugada pattern while females were more likely to have a drug-induced type 1 Brugada pattern.  Interestingly, spontaneous Brugada pattern patients were more symptomatic at diagnosis (39%) compared to drug-induced Brugada pattern (10%).   In addition, 19% of patients with spontaneous type I pattern only manifested the findings during a febrile episode.

The role of electrophysiology study (EPS) was assessed but limited to only 22 patients (21% of the total cohort) who underwent testing.  Nine patients were symptomatic and had a positive test or inducible VF.  Only 3 total patients (2 with a  positive EPS and 1 with a negative EPS) had a life threatening event during follow-up.  Based on the limited data, this study was unable to address the role of EPS in the risk stratification of young patients with Brugada syndrome.

Treatment for Brugada syndrome included 21% of patients with ICDs with the majority for primary prevention (86%), pacemakers (4%) for symptomatic sinus node dysfunction,  and quinidine (10%) for patients with the  spontaneous type I pattern and arrhythmic event(s).

Follow-up as mentioned was for a median of 4.5 years with 10 patients (9%) having 15 life threatening arrhythmias defined as sudden death, documented VT or VF, or appropriate ICD shock.  Fever was associated with 4 events (27%).  There were 3 deaths (3%), all in patients with a spontaneous Type 1 Brugada pattern. Two died during a febrile illness and 1 died following admission for an aborted sudden cardiac death.  There were 3 episodes of VT during hospital admission for fever related episodes.  The majority was symptomatic at diagnosis (70%) and was genotype positive for a SCN5A mutation (90%).

Predictors of life threatening arrhythmias that were significant included symptoms at diagnosis (Hazard Ratio: 5.9) and spontaneous Type 1 pattern on electrocardiogram  (Hazard Ratio: 2.7).  In addition patients with spontaneous Type 1 pattern and symptoms at presentation had a shorter time to first event (Hazard ratio: 28.9) as compared to those with drug-induced Type 1 pattern and no symptoms at diagnosis.

Overall, this study adds to our understanding of Brugada syndrome in young patients.  Patients with symptoms at diagnosis and a spontaneous type I pattern have a higher incidence of life threatening arrhythmias and a shorter time to first event.  Patients with drug-induced type I Brugada pattern and no symptoms at diagnosis have a low arrhythmic risk and good prognosis during study follow-up. There continues to be lack of clarity regarding EP study findings and genetic testing results in terms of risk stratification. Therapy in symptomatic patients with a spontaneous type I pattern should include consideration for ICD implantation and/or quinidine therapy.  As Brugada syndrome is rare diagnosis in the pediatric population, there is an increasing rate of diagnosis due to genetic testing.  Therapy should thus be considered on a case-by-case basis.  The study authors proposed the following paradigm which is a reasonable approach (see figure below adapted from paper).

Take Home Points:

  1. One in 5 patients with Brugada syndrome presented with symptoms at diagnosis with a male predominance.
  2. Syncope was the most common symptom (71%).
  3. Genetic testing is useful if genotype positive relatives are available.
  4. One-third of patients had a spontaneous Type I Brugada Pattern with 19% only manifesting this finding during fever.
  5. Spontaneous Type I Brugada Pattern and symptoms at diagnosis are predictive of a life threatening arrhythmia (43%) and a shorter time to the first life threatening arrhythmia.
  6. Drug-induced spontaneous Type I Brugada Pattern and no symptoms at diagnosis are predictive of a low arrhythmic risk (2%) and good prognosis.
  7. ICD and/or quinidine therapy should be considered in symptomatic patients with spontaneous type I Brugada Pattern.
  8. Fever triggered events occurred and the authors advise for aggressive treatment even though events during fevers may be unpredictable.
  9. A case-by-case approach should be used for management decisions in young patients with Brugada syndrome.

 

CHD Electrophysiology Articles of February 2016

NEW THIS MONTH  – Coloring denotes additional articles of interest

  1. Bipolar Versus Unipolar Temporary Epicardial Ventricular Pacing Leads Use in Congenital Heart Disease: A Prospective Randomized Controlled Study.

Mohari N, Starr JP, Gates RN, Domico MB, Batra AS.

Pacing Clin Electrophysiol. 2016 Feb 27. doi: 10.1111/pace.12836. [Epub ahead of print]

PMID: 26920816

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  1. Regional Implementation of a Pediatric Cardiology Syncope Algorithm Using Standardized Clinical Assessment and Management Plans (SCAMPS) Methodology.

Paris Y, Toro-Salazar OH, Gauthier NS, Rotondo KM, Arnold L, Hamershock R, Saudek DE, Fulton DR, Renaud A, Alexander ME; New England Congenital Cardiology Association (NECCA).

J Am Heart Assoc. 2016 Feb 19;5(2). pii: e002931. doi: 10.1161/JAHA.115.002931.

PMID: 26896480 Free Article

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Select item 26891904

 

  1. Effect of Left Cardiac Sympathetic Denervation on the Electromechanical Window in Patients with either Type 1 or Type 2 Long QT Syndrome: A Pilot Study.

Schneider AE, Bos JM, Ackerman MJ.

Congenit Heart Dis. 2016 Feb 17. doi: 10.1111/chd.12332. [Epub ahead of print]

PMID: 26887900

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Select item 26887835

 

  1. Lack of Association of ST-T Wave Abnormalities to Congenital Heart Disease in Neonates.

Gorla SR, Hsu DT, Kulkarni A.

Congenit Heart Dis. 2016 Feb 17. doi: 10.1111/chd.12329. [Epub ahead of print]

PMID: 26887835

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Select item 26887667

  1. A novel conditional mouse model for Nkx2-5 reveals transcriptional regulation of cardiac ion channels.

Furtado MB, Wilmanns JC, Chandran A, Tonta M, Biben C, Eichenlaub M, Coleman HA, Berger S, Bouveret R, Singh R, Harvey RP, Ramialison M, Pearson JT, Parkington HC, Rosenthal NA, Costa MW.

Differentiation. 2016 Feb 17. pii: S0301-4681(16)00009-8. doi: 10.1016/j.diff.2015.12.003. [Epub ahead of print]

PMID: 26897459

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Select item 26886902

 

  1. PACES/HRS Expert Consensus Statement on the use of Catheter Ablation in Children and Patients with Congenital Heart Disease: Developed in partnership with the Pediatric and Congenital Electrophysiology Society (PACES) and the Heart Rhythm Society (HRS). Endorsed by the governing bodies of PACES, HRS, the American Academy of Pediatrics (AAP), and the American Heart Association (AHA).

Philip Saul J, Kanter RJ, Abrams D, Asirvatham S, Bar-Cohen Y, Blaufox AD, Cannon B, Clark J, Dick M, Freter A, Kertesz NJ, Kirsh JA, Kugler J, LaPage M, McGowan FX, Miyake CY, Nathan A, Papagiannis J, Paul T, Pflaumer A, Skanes AC, Stevenson WG, Von Bergen N, Zimmerman F; Task Force Co-Chairs.

Heart Rhythm. 2016 Feb 17. pii: S1547-5271(16)00172-7. doi: 10.1016/j.hrthm.2016.02.009. [Epub ahead of print] No abstract available.

PMID: 26899545

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Select item 26922708

 

  1. Brugada syndrome and Brugada phenocopy. The importance of a differential diagnosis.

Dendramis G.

Int J Cardiol. 2016 Feb 17;210:25-27. doi: 10.1016/j.ijcard.2016.02.097. [Epub ahead of print]

PMID: 26922708

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Select item 26890199

 

  1. LV mechanical dispersion as a predictor of ventricular arrhythmia in patients with advanced systolic heart failure : A pilot study.

Banasik G, Segiet O, Elwart M, Szulik M, Lenarczyk R, Kalarus Z, Kukulski T.

Herz. 2016 Feb 16. [Epub ahead of print]

PMID: 26883898

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Select item 26868695

 

  1. Syncope with Swallowing.

Manu S, Aziz PF.

J Pediatr. 2016 Feb 16. pii: S0022-3476(16)00130-X. doi: 10.1016/j.jpeds.2016.01.053. [Epub ahead of print]

PMID: 26891888

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Select item 26882209

 

  1. Quality of Life of Pediatric Patients With Long QT Syndrome.

Czosek RJ, Kaltman JR, Cassedy AE, Shah MJ, Vetter VL, Tanel RE, Wernovksy G, Wray J, Marino BS.

Am J Cardiol. 2016 Feb 15;117(4):605-10. doi: 10.1016/j.amjcard.2015.11.051. Epub 2015 Dec 7.

PMID: 26721659

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Select item 26898634

 

  1. Sudden cardiac death during exercise in patients with congenital heart disease: the exercise paradox and the challenge of appropriate counselling.

Diller GP, Baumgartner H.

Eur Heart J. 2016 Feb 14;37(7):627-9. doi: 10.1093/eurheartj/ehv523. Epub 2015 Oct 21. No abstract available.

PMID: 26491107

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Select item 26341891

 

  1. Sudden unexpected death in children with congenital heart defects.

Jortveit J, Eskedal L, Hirth A, Fomina T, Døhlen G, Hagemo P, Tell GS, Birkeland S, Øyen N, Holmstrøm H.

Eur Heart J. 2016 Feb 14;37(7):621-6. doi: 10.1093/eurheartj/ehv478. Epub 2015 Sep 4.

PMID: 26341891

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Select item 26872844

 

  1. Implantable cardioverter-defibrillators in adults with congenital heart disease: a systematic review and meta-analysis.

Vehmeijer JT, Brouwer TF, Limpens J, Knops RE, Bouma BJ, Mulder BJ, de Groot JR.

Eur Heart J. 2016 Feb 11. pii: ehv735. [Epub ahead of print] Review.

PMID: 26873095

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Select item 26875011

 

  1. Rounding Up the Usual Suspects: Nonsustained Ventricular Tachycardia and Sustained Left Ventricular Dysfunction as Risk Factors for Sudden Death in Isolated AV Block.

Knilans TK, Czosek RJ.

J Cardiovasc Electrophysiol. 2016 Feb 3. doi: 10.1111/jce.12943. [Epub ahead of print] No abstract available.

PMID: 26839982

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Select item 26843489

 

  1. Comparison of the Frequency of Sudden Cardiovascular Deaths in Young Competitive Athletes Versus Nonathletes: Should We Really Screen Only Athletes?

Maron BJ, Haas TS, Duncanson ER, Garberich RF, Baker AM, Mackey-Bojack S.

Am J Cardiol. 2016 Feb 1. pii: S0002-9149(16)30148-5. doi: 10.1016/j.amjcard.2016.01.026. [Epub ahead of print]

PMID: 26949036

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Select item 26725103

 

  1. Cardiac MRI in patients with complex CHD following primary or secondary implantation of MRI-conditional pacemaker system.

Al-Wakeel N, O H-Ici D, Schmitt KR, Messroghli DR, Riesenkampff E, Berger F, Kuehne T, Peters B.

Cardiol Young. 2016 Feb;26(2):306-14. doi: 10.1017/S1047951115000190. Epub 2015 Feb 23.

PMID: 25704274

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Select item 25704070

 

  1. Congenital heart block related to maternal autoantibodies: descriptive analysis of a series of 18 cases from a single center.

Doti PI, Escoda O, Cesar-Díaz S, Palasti S, Teixidó I, Sarquella-Brugada G, Gómez O, Martínez JM, Espinosa G.

Clin Rheumatol. 2016 Feb;35(2):351-6. doi: 10.1007/s10067-016-3174-4. Epub 2016 Jan 20.

PMID: 26791874

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Select item 26830180

 

  1. Evaluation and management of bradycardia in neonates and children.

Baruteau AE, Perry JC, Sanatani S, Horie M, Dubin AM.

Eur J Pediatr. 2016 Feb;175(2):151-61. doi: 10.1007/s00431-015-2689-z. Epub 2016 Jan 16.

PMID: 26780751

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Select item 26362538

 

  1. Autoimmune congenital heart block: complex and unusual situations.

Brito-Zerón P, Izmirly PM, Ramos-Casals M, Buyon JP, Khamashta MA.

Lupus. 2016 Feb;25(2):116-28. doi: 10.1177/0961203315624024. Review.

PMID: 26762645

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Select item 26669606

 

  1. Evaluation of Permanent or Transient Complete Heart Block after Open Heart Surgery for Congenital Heart Disease.

Ayyildiz P, Kasar T, Ozturk E, Ozyilmaz I, Tanidir IC, Guzeltas A, Ergul Y.

Pacing Clin Electrophysiol. 2016 Feb;39(2):160-5. doi: 10.1111/pace.12778. Epub 2015 Dec 1.

PMID: 26517970

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  1. Changes in Myocardial Contractility and Electromechanical Interval During the First Month of Life in Healthy Neonates.

Kahr PC, Kahr MK, Dabral H, Agarwal R, Kothari SS, Saxena A, Ramakrishnan S.

Pediatr Cardiol. 2016 Feb;37(2):409-18. doi: 10.1007/s00246-015-1292-4. Epub 2015 Oct 24.

PMID: 26499358

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  1. Arrhythmias in Adult Congenital Patients With Bodily Isomerism.

Loomba RS, Aggarwal S, Gupta N, Buelow M, Alla V, Arora RR, Anderson RH.

Pediatr Cardiol. 2016 Feb;37(2):330-7. doi: 10.1007/s00246-015-1281-7. Epub 2015 Oct 19.

PMID: 26481118

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  1. Effects of chronic amiodarone treatment on rat testis.

Özkaya AK, Dilber E, Gürgen SG, Kutlu Ö, Cansu A, Gedik Y.

Acta Histochem. 2016 Feb 29. pii: S0065-1281(16)30027-7. doi: 10.1016/j.acthis.2016.02.003. [Epub ahead of print]

PMID: 26947592

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  1. Availability of Automated External Defibrillators in Public High Schools.

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

J Pediatr. 2016 Feb 27. pii: S0022-3476(16)00158-X. doi: 10.1016/j.jpeds.2016.02.010. [Epub ahead of print]

PMID: 26935784

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  1. QT Dynamics During Exercise in Asymptomatic Children with Long QT Syndrome Type 3.

Takahashi K, Nabeshima T, Nakayashiro M, Ganaha H.

Pediatr Cardiol. 2016 Feb 26. [Epub ahead of print]

PMID: 26921063

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  1. Clinical Features of Long QT Syndrome in Children.

Sumitomo N.

Circ J. 2016 Feb 25;80(3):598-600. doi: 10.1253/circj.CJ-16-0046. Epub 2016 Feb 1. No abstract available.

PMID: 26831253 Free Article

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Select item 26920722

 

  1. The Impact of Clinical and Genetic Findings on The Management of Young Brugada Syndrome Patients.

Andorin A, Behr ER, Denjoy I, Crotti L, Dagradi F, Jesel L, Sacher F, Petit B, Mabo P, Maltret A, Wong LC, Degand B, Bertaux G, Maury P, Dulac Y, Delasalle B, Gourraud JB, Babuty D, Blom NA, Schwartz PJ, Wilde AA, Probst V.

Heart Rhythm. 2016 Feb 24. pii: S1547-5271(16)00182-X. doi: 10.1016/j.hrthm.2016.02.013. [Epub ahead of print]

PMID: 26921764

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Select item 26911460

 

  1. Cardiac implantable electronic device lead-based masses and atrial fibrillation ablation: a case-based illustration of periprocedural anticoagulation management strategies.

Lenz CJ, DeSimone CV, Ponamgi SP, Sugrue A, Sinak LJ, Chandrasekaran K, Packer DL, Asirvatham SJ.

J Interv Card Electrophysiol. 2016 Feb 22. [Epub ahead of print]

PMID: 26898212

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Select item 26907646

 

  1. Accessory pathway ablation in childhood forming the substrate for ventricular tachycardia in adulthood.

Koch J, Lücke C, Gebauer R, Hindricks G, Arya A.

Eur Heart J. 2016 Feb 14;37(7):609. doi: 10.1093/eurheartj/ehv260. Epub 2015 Jun 20. No abstract available.

PMID: 26093639

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Select item 26878093

 

  1. Contribution of TGFB1 and TNF-α genes in one of twin pregnancies with congenital complete heart block phenotype.

Bornaun H, Oztarhan K, Ocak Z, Ekiz A, Ulucan K, Buyukkale G, Gedikbasi A.

Int J Cardiol. 2016 Feb 13;210:16-18. doi: 10.1016/j.ijcard.2016.01.214. [Epub ahead of print] No abstract available.

PMID: 26922706

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Select item 26896890

 

  1. Detecting Regional Myocardial Abnormalities in Patients With Wolff-Parkinson-White Syndrome With the Use of ECG-Gated Cardiac MDCT.

Lee HJ, Uhm JS, Joung B, Hong YJ, Hur J, Choi BW, Kim YJ.

AJR Am J Roentgenol. 2016 Feb 11:1-7. [Epub ahead of print]

PMID: 26866341

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Select item 26938796

 

  1. Combinatorial release of dexamethasone and amiodarone from a nano-structured parylene-C film to reduce perioperative inflammation and atrial fibrillation.

Robinson E, Kaushal S, Alaboson J, Sharma S, Belagodu A, Watkins C, Walker B, Webster G, McCarthy P, Ho D.

Nanoscale. 2016 Feb 11;8(7):4267-75. doi: 10.1039/c5nr07456h.

PMID: 26838117

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Select item 26883202

 

  1. Evaluating current implantable cardioverter defibrillator implantation procedures: can common complications be minimised?

Timmers L, Van Heuverswyn F, De Wilde H, Jordaens L.

Expert Rev Cardiovasc Ther. 2016 Feb 10:1-11. [Epub ahead of print]

PMID: 26790106

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  1. Recurrent Muscle Weakness with Rhabdomyolysis, Metabolic Crises, and Cardiac Arrhythmia Due to Bi-allelic TANGO2 Mutations.

Lalani SR, Liu P, Rosenfeld JA, Watkin LB, Chiang T, Leduc MS, Zhu W, Ding Y, Pan S, Vetrini F, Miyake CY, Shinawi M, Gambin T, Eldomery MK, Akdemir ZH, Emrick L, Wilnai Y, Schelley S, Koenig MK, Memon N, Farach LS, Coe BP, Azamian M, Hernandez P, Zapata G, Jhangiani SN, Muzny DM, Lotze T, Clark G, Wilfong A, Northrup H, Adesina A, Bacino CA, Scaglia F, Bonnen PE, Crosson J, Duis J, Maegawa GH, Coman D, Inwood A, McGill J, Boerwinkle E, Graham B, Beaudet A, Eng CM, Hanchard NA, Xia F, Orange JS, Gibbs RA, Lupski JR, Yang Y.

Am J Hum Genet. 2016 Feb 4;98(2):347-57. doi: 10.1016/j.ajhg.2015.12.008. Epub 2016 Jan 21.

PMID: 26805781

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  1. Is transesophageal electrophysiologic study valuable in children with successful radiofrequency ablation of supraventricular tachycardia on follow-up for recurrence?

Gülgün M, Özer S, Karagöz T, Akın A, Aykan HH, Özkutlu S, Alehan D, Çeliker A.

Anatol J Cardiol. 2016 Feb;16(2):126-30. doi: 10.5152/akd.2015.5895. Epub 2015 Mar 4.

PMID: 26467372 Free Article

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Select item 26777969

 

  1. Evaluation and management of bradycardia in neonates and children.

Baruteau AE, Perry JC, Sanatani S, Horie M, Dubin AM.

Eur J Pediatr. 2016 Feb;175(2):151-61. doi: 10.1007/s00431-015-2689-z. Epub 2016 Jan 16.

PMID: 26780751

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Select item 26350228

 

  1. A Pilot Study Assessing ECG versus ECHO Ventriculoventricular Optimization in Pediatric Resynchronization Patients.

Punn R, Hanisch D, Motonaga KS, Rosenthal DN, Ceresnak SR, Dubin AM.

J Cardiovasc Electrophysiol. 2016 Feb;27(2):210-6. doi: 10.1111/jce.12863. Epub 2015 Nov 23.

PMID: 26515428

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Select item 26702594

 

  1. Cardiac Autonomic Dysfunction in Patients With Infantile Spasm and the Effect of Adrenocorticotropic Hormone Treatment.

Gencpinar P, Kocabas A, Duman Ö, Dündar NO, Haspolat S, Kardelen F.

J Child Neurol. 2016 Feb;31(2):134-7. doi: 10.1177/0883073815583998. Epub 2015 May 7.

PMID: 25953826

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  1. Intrauterine Beta Blockade.

Abinader EG.

Pediatr Cardiol. 2016 Feb;37(2):428. doi: 10.1007/s00246-015-1337-8. Epub 2015 Dec 30. No abstract available.

PMID: 26717910

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Select item 26573816

 

  1. Characterization of Cardiac Troponin Elevation in the Setting of Pediatric Supraventricular Tachycardia.

Moore JP, Arcilla L, Wang S, Lee MS, Shannon KM.

Pediatr Cardiol. 2016 Feb;37(2):392-8. doi: 10.1007/s00246-015-1289-z. Epub 2015 Oct 19.

PMID: 26481117

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  1. Exploring the Discussion of Risk of Sudden Cardiac Death.

Wiley KA, Demo EM, Walker P, Osborne Shuler C.

Pediatr Cardiol. 2016 Feb;37(2):262-70. doi: 10.1007/s00246-015-1272-8. Epub 2015 Oct 3.

PMID: 26433939

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Select item 26433938

 

  1. ECGs in the ED.

Tanel RE.

Pediatr Emerg Care. 2016 Feb;32(2):136-7. doi: 10.1097/PEC.0000000000000740. No abstract available.

PMID: 26835575

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CHD Electrophysiology Articles of December 2015

 

  1. The genetics underlying acquired long QT syndrome: impact for genetic screening.

Itoh H, Crotti L, Aiba T, Spazzolini C, Denjoy I, Fressart V, Hayashi K, Nakajima T, Ohno S, Makiyama T, Wu J, Hasegawa K, Mastantuono E, Dagradi F, Pedrazzini M, Yamagishi M, Berthet M, Murakami Y, Shimizu W, Guicheney P, Schwartz PJ, Horie M.

Eur Heart J. 2015 Dec 28. pii: ehv695. [Epub ahead of print]

PMID: 26715165

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Select item 26708337

 

  1. Left and Right Parasternal Sensing for the S-Icd in Adult Congenital Heart Disease Patients and Normal Controls.

David G W, Mehmood Z, Gruschen V, Borislav D D, John M M.

Pacing Clin Electrophysiol. 2015 Dec 23. doi: 10.1111/pace.12802. [Epub ahead of print]

PMID: 26694691

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Select item 26701792

 

  1. Electro-mechanical dysfunction in long QT syndrome: Role for arrhythmogenic risk prediction and modulation by sex and sex hormones.

Lang CN, Menza M, Jochem S, Franke G, Feliz SP, Brunner M, Koren G, Zehender M, Bugger H, Jung BA, Foell D, Bode C, Odening KE.

Prog Biophys Mol Biol. 2015 Dec 21. pii: S0079-6107(15)00259-X. doi: 10.1016/j.pbiomolbio.2015.12.010. [Epub ahead of print]

PMID: 26718598

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Select item 26687150

 

  1. Familial Atrial Septal Defect and Sudden Cardiac Death: Identification of a Novel NKX2-5 Mutation and a Review of the Literature.

Ellesøe SG, Johansen MM, Bjerre JV, Hjortdal VE, Brunak S, Larsen LA.

Congenit Heart Dis. 2015 Dec 18. doi: 10.1111/chd.12317. [Epub ahead of print]

PMID: 26679770

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Select item 26685132

 

  1. QT Adaptation and Intrinsic QT Variability in Congenital Long QT Syndrome.

Seethala S, Singh P, Shusterman V, Ribe M, Haugaa KH, Němec J.

J Am Heart Assoc. 2015 Dec 16;4(12). pii: e002395. doi: 10.1161/JAHA.115.002395.

PMID: 26675252 Free Article

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Select item 26611123

 

  1. Minimally Invasive Percutaneous Pericardial ICD Placement in an Infant Piglet Model: Head-to-Head Comparison with an Open Surgical Thoracotomy Approach.

Clark BC, Davis TD, El-Sayed Ahmed MM, McCarter R, Ishibashi N, Jordan CP, Kane TD, Kim PC, Krieger A, Nath DS, Opfermann JD, Berul CI.

Heart Rhythm. 2015 Dec 9. pii: S1547-5271(15)01530-1. doi: 10.1016/j.hrthm.2015.12.015. [Epub ahead of print]

PMID: 26690060

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Select item 26646061

 

  1. Quality of Life of Pediatric Patients With Long QT Syndrome.

Czosek RJ, Kaltman JR, Cassedy AE, Shah MJ, Vetter VL, Tanel RE, Wernovksy G, Wray J, Marino BS.

Am J Cardiol. 2015 Dec 7. pii: S0002-9149(15)02335-8. doi: 10.1016/j.amjcard.2015.11.051. [Epub ahead of print]

PMID: 26721659

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  1. Arrhythmia diagnosis and management throughout life in congenital heart disease.

Clark BC, Berul CI.

Expert Rev Cardiovasc Ther. 2015 Dec 5. [Epub ahead of print]

PMID: 26642231

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  1. Cardiac Arrhythmias in Adults With Congenital Heart Disease: Scope, Specific Problems, and Management.

Lindsay I, Moore JP.

Curr Treat Options Cardiovasc Med. 2015 Dec;17(12):56. doi: 10.1007/s11936-015-0418-6.

PMID: 26467489

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  1. Comprehensive rhythm evaluation in a large contemporary Fontan population†.

Bossers SS, Duppen N, Kapusta L, Maan AC, Duim AR, Bogers AJ, Hazekamp MG, van Iperen G, Helbing WA, Blom NA.

Eur J Cardiothorac Surg. 2015 Dec;48(6):833-41. doi: 10.1093/ejcts/ezu548. Epub 2015 Jan 18.

PMID: 25602059

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  1. Reliability of Left Ventricular Hypertrophy by ECG Criteria in Children with Syncope: Do the Criteria Need to be Revised?

Banerjee MM, Ramesh Iyer V, Nandi D, Vetter VL, Banerjee A.

Pediatr Cardiol. 2015 Dec 31. [Epub ahead of print]

PMID: 26717911

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  1. Feasibility and safety of transseptal puncture procedures for radiofrequency catheter ablation in small children weighing below 30 kg: single-centre experience.

Yoshida S, Suzuki T, Yoshida Y, Watanabe S, Nakamura K, Sasaki T, Kawasaki Y, Ehara E, Murakami Y, Kato T, Nakamura Y.

Europace. 2015 Dec 24. pii: euv383. [Epub ahead of print]

PMID: 26705553

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  1. Arrhythmias Following Comprehensive Stage II Surgical Palliation in Single Ventricle Patients.

Wilhelm CM, Paulus D, Cua CL, Kertesz NJ, Cheatham JP, Galantowicz M, Fernandez RP.

Pediatr Cardiol. 2015 Dec 14. [Epub ahead of print]

PMID: 26667962

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  1. Hemodynamics in fetal arrhythmia.

Sonesson SE, Acharya G.

Acta Obstet Gynecol Scand. 2015 Dec 10. doi: 10.1111/aogs.12837. [Epub ahead of print]

PMID: 26660845

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  1. Implantable Cardioverter Defibrillator Explantation for Overdiagnosed or Overtreated Congenital Long QT Syndrome.

Gaba P, Bos JM, Cannon BC, Cha YM, Friedman P, Asirvatham S, Ackerman MJ.

Heart Rhythm. 2015 Dec 8. pii: S1547-5271(15)01521-0. doi: 10.1016/j.hrthm.2015.12.008. [Epub ahead of print]

PMID: 26681611

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  1. Protective effects of Hawthorn (Crataegus oxyacantha) extract against digoxin-induced arrhythmias in rats.

Alp H, Soner BC, Baysal T, Şahin AS.

Anatol J Cardiol. 2015 Dec;15(12):970-5. doi: 10.5152/akd.2014.5869. Epub 2014 Dec 25.

PMID: 25880053

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  1. Short QT Interval Prevalence and Clinical Outcomes in a Pediatric Population.

Guerrier K, Kwiatkowski D, Czosek RJ, Spar DS, Anderson JB, Knilans TK.

Circ Arrhythm Electrophysiol. 2015 Dec;8(6):1460-4. doi: 10.1161/CIRCEP.115.003256. Epub 2015 Sep 18.

PMID: 26386018

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  1. Implantable cardioverter-defibrillator and wait-list outcomes in pediatric patients awaiting heart transplantation.

El-Assaad I, Al-Kindi SG, Oliveira GH, Boyle GJ, Aziz PF.

Heart Rhythm. 2015 Dec;12(12):2443-8. doi: 10.1016/j.hrthm.2015.07.036. Epub 2015 Aug 3.

PMID: 26247317

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  1. Diagnosis and Acute Management of Tachyarrhythmias in Children.

Srinivasan C.

Indian J Pediatr. 2015 Dec;82(12):1157-63. doi: 10.1007/s12098-015-1881-5. Epub 2015 Sep 28.

PMID: 26411729

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  1. Validation of Standard and New Criteria for the Differential Diagnosis of Narrow QRS Tachycardia in Children and Adolescents.

Deutsch K, Stec S, Kukla P, Morka A, Jastrzebski M, Baszko A, Pitak M, Sledz J, Fijorek K, Mazij M, Ludwik B, Gubaro M, Szydlowski L.

Medicine (Baltimore). 2015 Dec;94(51):e2310. doi: 10.1097/MD.0000000000002310.

PMID: 26705217

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  1. Characterization of maturation of neuronal voltage-gated sodium channels SCN1A and SCN8A in rat myocardium.

Krause U, Alflen C, Steinmetz M, Müller MJ, Quentin T, Paul T.

Mol Cell Pediatr. 2015 Dec;2(1):5. doi: 10.1186/s40348-015-0015-5. Epub 2015 Mar 11.

PMID: 26542295 Free PMC Article

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  1. Changes in QTc associated with a rapid bolus dose of dexmedetomidine in patients receiving TIVA: a retrospective study.

Görges M, Whyte SD, Sanatani S, Dawes J, Montgomery CJ, Ansermino JM.

Paediatr Anaesth. 2015 Dec;25(12):1287-93. doi: 10.1111/pan.12780.

PMID: 26507917

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  1. ECGs in the ED.

Tanel RE.

Pediatr Emerg Care. 2015 Dec;31(12):891-2. doi: 10.1097/PEC.0000000000000686. No abstract available.

PMID: 26626902

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  1. QT dynamics evaluated on fully automated QT measurement in children.

Takahashi K, Nabeshima T, Sashinami A, Nakayashiro M, Ganaha H.

Pediatr Int. 2015 Dec;57(6):1067-71. doi: 10.1111/ped.12734. Epub 2015 Nov 5.

PMID: 26096703

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  1. Effects of atomoxetine on heart rhythm in children and adolescents.

Tanidir IC, Tanidir C, Ozturk E, Bahali K, Gunes H, Ergul Y, Uneri OS, Akdeniz C, Tuzcu V.

Pediatr Int. 2015 Dec;57(6):1078-85. doi: 10.1111/ped.12726. Epub 2015 Nov 5.

PMID: 26096186

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