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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  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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  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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  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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  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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  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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