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

  • Pediatric Congenital Heart Disease (G Singh, Section Editor)
  • Published:
Current Treatment Options in Cardiovascular Medicine Aims and scope Submit manuscript

Opinion statement

Progressive advances in the field of congenital heart disease (CHD) have led to a dramatic rise in the number of affected patients surviving well into adulthood. Groundbreaking and innovative achievements in the medical, surgical, and interventional management of these patients have allowed them to lead full adult lives that have included raising families, completing degrees in higher education, and maintaining successful careers. As patients are living longer, arrhythmias have emerged as one of the most significant factors affecting their morbidity, mortality, and quality of life. This article aims to review the current understanding of arrhythmia within the adult congenital heart disease (ACHD) population, emphasizing recent advances and providing recommendations pertaining to the management of these patients.

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References and Recommended Reading

Papers of particular interest, published recently, have been highlighted as: • Of importance

  1. Bouchardy J, Therrien J, Pilote L, Ionescu-Ittu R, Martucci G, Bottega N, et al. Atrial arrhythmias in adults with congenital heart disease. Circulation. 2009;120:1679–86.

    Article  PubMed  Google Scholar 

  2. Maxwell BG, Steppan J, Cheng A. Complications of catheter-based electrophysiology procedures in adults with congenital heart disease: a national analysis. J Cardiothorac Vasc Anesth. 2015;29:258–64.

    Article  PubMed  Google Scholar 

  3. Verheugt CL, Uiterwaal CS, van der Velde ET, Meijboom FJ, Pieper PG, Sieswerda GT, et al. The emerging burden of hospital admissions of adults with congenital heart disease. Heart. 2010;96:872–8.

    Article  PubMed  Google Scholar 

  4. Khairy P, Van Hare GF, Balaji S, Berul CI, Cecchin F, Cohen MI, et al. PACES/HRS expert consensus statement on the recognition and management of arrhythmias in adult congenital heart disease: developed in partnership between the Pediatric and Congenital Electrophysiology Society (PACES) and the Heart Rhythm Society (HRS). Endorsed by the governing bodies of PACES, HRS, the American College of Cardiology (ACC), the American Heart Association (AHA), the European Heart Rhythm Association (EHRA), the Canadian Heart Rhythm Society (CHRS), and the International Society for Adult Congenital Heart Disease (ISACHD). Can J Cardiol. 2014;30:e1–e63. This is a set of comprehensive consensus and evidence-based guidelines for the management of rhythm disorders in the ACHD population.

    Article  PubMed  Google Scholar 

  5. Gelatt M, Hamilton RM, McCrindle BW, Gow RM, Williams WG, Trusler GA, et al. Risk factors for atrial tachyarrhythmias after the Fontan operation. J Am Coll Cardiol. 1994;24:1735–41.

    Article  CAS  PubMed  Google Scholar 

  6. Stephenson EA, Lu M, Berul CI, Etheridge SP, Idriss SF, Margossian R, et al. Arrhythmias in a contemporary Fontan cohort: prevalence and clinical associations in a multicenter cross-sectional study. J Am Coll Cardiol. 2010;56:890–6.

    Article  PubMed Central  PubMed  Google Scholar 

  7. Marcelletti C, Corno A, Giannico S, Marino B. Inferior vena cava-pulmonary artery extracardiac conduit. A new form of right heart bypass. J Thorac Cardiovasc Surg. 1990;100:228–32.

    CAS  PubMed  Google Scholar 

  8. Balaji S, Daga A, Bradley DJ, Etheridge SP, Law IH, Batra AS, et al. An international multicenter study comparing arrhythmia prevalence between the intracardiac lateral tunnel and the extracardiac conduit type of Fontan operations. J Thorac Cardiovasc Surg. 2014;148:576–81.

    Article  PubMed  Google Scholar 

  9. Bossers SS, Duppen N, Kapusta L, Maan AC, Duim AR, Bogers AJ, et al. Comprehensive rhythm evaluation in a large contemporary Fontan population. Eur J Cardiothorac Surg. 2015.

  10. Moore JP, Russell M, Mandapati R, Aboulhosn JA, Shannon KM. Catheter ablation of tachycardia arising from the pulmonary venous atrium after surgical repair of congenital heart disease. Heart Rhythm. 2015;12:297–304.

    Article  PubMed  Google Scholar 

  11. Correa R, Sherwin ED, Kovach J, Mah DY, Alexander ME, Cecchin F, et al. Mechanism and ablation of arrhythmia following total cavopulmonary connection. Circ Arrhythm Electrophysiol. 2015;8:318–25.

    Article  PubMed  Google Scholar 

  12. Collins KK, Love BA, Walsh EP, Saul JP, Epstein MR, Triedman JK. Location of acutely successful radiofrequency catheter ablation of intraatrial reentrant tachycardia in patients with congenital heart disease. Am J Cardiol. 2000;86:969–74.

    Article  CAS  PubMed  Google Scholar 

  13. Gelatt M, Hamilton RM, McCrindle BW, Connelly M, Davis A, Harris L, et al. Arrhythmia and mortality after the Mustard procedure: a 30-year single-center experience. J Am Coll Cardiol. 1997;29:194–201.

    Article  CAS  PubMed  Google Scholar 

  14. Chen SC, Nouri S, Pennington DG. Dysrhythmias after the modified Fontan procedure. Pediatr Cardiol. 1988;9:215–9.

    Article  CAS  PubMed  Google Scholar 

  15. de Groot NM, Zeppenfeld K, Wijffels MC, Chan WK, Blom NA, Van der Wall EE, et al. Ablation of focal atrial arrhythmia in patients with congenital heart defects after surgery: role of circumscribed areas with heterogeneous conduction. Heart Rhythm. 2006;3:526–35.

    Article  PubMed  Google Scholar 

  16. Kirsh JA, Walsh EP, Triedman JK (2002) Prevalence of and risk factors for atrial fibrillation and intra-atrial reentrant tachycardia among patients with congenital heart disease. American Journal of Cardiology 90: 338 − +

  17. Teuwen CP, Ramdjan TT, Gotte M, Brundel BJ, Evertz R, Vriend JW, Molhoek SG, Dorman HG, van Opstal JM, Konings TC, van der Voort P, Delacretaz E, Houck C, Yaksh A, Jansz LJ, Witsenburg M, Roos-Hesselink JW, Triedman JK, Bogers AJ, de Groot NM (2015) Time course of atrial fibrillation in patients with congenital heart defects. Circ Arrhythm Electrophysiol: [in press] This is a large longitudinal study examining 199 ACHD patients with atrial fibrillation. The paper provides new insights into the mechanisms and natural history of this rhythm abnormality.

  18. Moore JP. Arrhythmia management for the adult patient with congenital heart disease: an update and analytical review. Minerva Pediatr. 2014;66:415–39.

    CAS  PubMed  Google Scholar 

  19. Anguera I, Dallaglio P, Macias R, Jimenez-Candil J, Peinado R, Garcia-Seara J, et al. Long-term outcome after ablation of right atrial tachyarrhythmias after the surgical repair of congenital and acquired heart disease. Am J Cardiol. 2015;115:1705–13.

    Article  PubMed  Google Scholar 

  20. Mah DY, Alexander ME, Cecchin F, Walsh EP, Triedman JK. The electroanatomic mechanisms of atrial tachycardia in patients with tetralogy of Fallot and double outlet right ventricle. J Cardiovasc Electrophysiol. 2011;22:1013–7.

    Article  PubMed  Google Scholar 

  21. de Groot NM, Atary JZ, Blom NA, Schalij MJ. Long-term outcome after ablative therapy of postoperative atrial tachyarrhythmia in patients with congenital heart disease and characteristics of atrial tachyarrhythmia recurrences. Circ Arrhythm Electrophysiol. 2010;3:148–54.

    Article  PubMed  Google Scholar 

  22. Akar JG, Kok LC, Haines DE, DiMarco JP, Mounsey JP. Coexistence of type I atrial flutter and intra-atrial re-entrant tachycardia in patients with surgically corrected congenital heart disease. J Am Coll Cardiol. 2001;38:377–84.

    Article  CAS  PubMed  Google Scholar 

  23. Yap SC, Harris L, Downar E, Nanthakumar K, Silversides CK, Chauhan VS. Evolving electroanatomic substrate and intra-atrial reentrant tachycardia late after Fontan surgery. J Cardiovasc Electrophysiol. 2012;23:339–45.

    Article  PubMed  Google Scholar 

  24. Lai YQ, Li JH, Li JW, Xu SD, Luo Y, Zhang ZG. Concomitant irrigated monopolar radiofrequency ablation of atrial fibrillation in adults with congenital heart disease. Interact Cardiovasc Thorac Surg. 2008;7:80–2.

    Article  PubMed  Google Scholar 

  25. Philip F, Muhammad KI, Agarwal S, Natale A, Krasuski RA. Pulmonary vein isolation for the treatment of drug-refractory atrial fibrillation in adults with congenital heart disease. Congenit Heart Dis. 2012;7:392–9. This is the largest report to date on the efficacy of catheter-based intervention for the treatment of atrial fibrillation in the ACHD community.

    Article  PubMed  Google Scholar 

  26. American College of Physicians Task Force on Clinical Competence, Training, Heart Rhythm Society, Tracy CM, Akhtar M, DiMarco JP, Packer DL, Weitz HH, et al. American College of Cardiology/American Heart Association 2006 update of the clinical competence statement on invasive electrophysiology studies, catheter ablation, and cardioversion: a report of the American College of Cardiology/American Heart Association/American College of Physicians Task Force on Clinical Competence and Training developed in collaboration with the Heart Rhythm Society. J Am Coll Cardiol. 2006;48:1503–17.

    Article  Google Scholar 

  27. Pass RH, Nappo L, Eugenio P, Lopez L. A transbaffle approach to ablation in a child with an extracardiac Fontan. Pacing Clin Electrophysiol. 2010;33:368–71.

    Article  PubMed  Google Scholar 

  28. Khairy P, Fournier A, Ruest P, Vobecky SJ. Transcatheter ablation via a sternotomy approach as a hybrid procedure in a univentricular heart. Pace. 2008;31:639–40.

    Article  PubMed  Google Scholar 

  29. Arana-Rueda E, Pedrote A, Duran-Guerrero JM, Santos de Soto J. Simplified approach for ablation of nodal reentrant tachycardia in a patient with tricuspid atresia and extracardiac Fontan palliation. Rev Esp Cardiol (Engl Ed). 2013;66:314–6.

    Article  Google Scholar 

  30. Nehgme RA, Carboni MP, Care J, Murphy JD. Transthoracic percutaneous access for electroanatomic mapping and catheter ablation of atrial tachycardia in patients with a lateral tunnel Fontan. Heart Rhythm. 2006;3:37–43.

    Article  PubMed  Google Scholar 

  31. Dave AS, Aboulhosn J, Child JS, Shivkumar K. Transconduit puncture for catheter ablation of atrial tachycardia in a patient with extracardiac Fontan palliation. Heart Rhythm. 2010;7:413–6.

    Article  PubMed Central  PubMed  Google Scholar 

  32. Moore JP, Hendrickson B, Brunengraber DZ, Shannon KM. Transcaval puncture for access to the pulmonary venous atrium after the extracardiac total cavopulmonary connection operation. Circ Arrhythm Electrophysiol. 2015;8:824–8.

    Article  PubMed  Google Scholar 

  33. Mavroudis C, Deal BJ, Backer CL, Stewart RD, Franklin WH, Tsao S, Ward KM, DeFreitas RA (2007) J. Maxwell Chamberlain Memorial Paper for congenital heart surgery. 111 Fontan conversions with arrhythmia surgery: surgical lessons and outcomes. Ann Thorac Surg 84: 1457–1465; discussion 1465–1456. This is a long-term follow-up study of arrhythmia surgery for ACHD patients with Fontan palliation undergoing conversion to TCPC. This study demonstrated improved efficacy with time and provides multivariate predictors for adverse outcome.

  34. Mavroudis C, Backer CL, Deal BJ, Stewart RD, Franklin WH, Tsao S, et al. Evolving anatomic and electrophysiologic considerations associated with Fontan conversion. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 2007;10:136–45.

    Article  Google Scholar 

  35. Banchs JE, Baquero GA, Nickolaus MJ, Wolbrette DL, Kelleman JJ, Samii S, et al. Clinical efficacy of dofetilide for the treatment of atrial tachyarrhythmias in adults with congenital heart disease. Congenit Heart Dis. 2014;9:221–7.

    Article  PubMed  Google Scholar 

  36. Koyak Z, Kroon B, de Groot JR, Wagenaar LJ, van Dijk AP, Mulder BA, et al. Efficacy of antiarrhythmic drugs in adults with congenital heart disease and supraventricular tachycardias. Am J Cardiol. 2013;112:1461–7.

    Article  CAS  PubMed  Google Scholar 

  37. Ammash NM, Phillips SD, Hodge DO, Connolly HM, Grogan MA, Friedman PA, et al. Outcome of direct current cardioversion for atrial arrhythmias in adults with congenital heart disease. Int J Cardiol. 2012;154:270–4.

    Article  PubMed  Google Scholar 

  38. Stephenson EA, Casavant D, Tuzi J, Alexander ME, Law I, Serwer G, et al. Efficacy of atrial antitachycardia pacing using the Medtronic AT500 pacemaker in patients with congenital heart disease. Am J Cardiol. 2003;92:871–6.

    Article  PubMed  Google Scholar 

  39. Kamp AN, LaPage MJ, Serwer GA, Dick 2nd M, Bradley DJ. Antitachycardia pacemakers in congenital heart disease. Congenit Heart Dis. 2015;10:180–4.

    Article  PubMed  Google Scholar 

  40. Rhodes LA, Walsh EP, Gamble WJ, Triedman JK, Saul JP. Benefits and potential risks of atrial antitachycardia pacing after repair of congenital heart disease. Pacing Clin Electrophysiol. 1995;18:1005–16.

    Article  CAS  PubMed  Google Scholar 

  41. Silka MJ, Manwill JR, Kron J, Mcanulty JH. Bradycardia-mediated tachyarrhythmias in congenital heart-disease and responses to chronic pacing at physiologic rates. Am J Cardiol. 1990;65:488–93.

    Article  CAS  PubMed  Google Scholar 

  42. Padeletti L, Purerfellner H, Mont L, Tukkie R, Manolis AS, Ricci R, et al. New-generation atrial antitachycardia pacing (Reactive ATP) is associated with reduced risk of persistent or permanent atrial fibrillation in patients with bradycardia: results from the MINERVA randomized multicenter international trial. Heart Rhythm. 2015;12:1717–25.

    Article  PubMed  Google Scholar 

  43. Zeppenfeld K, Schalij MJ, Bartelings MM, Tedrow UB, Koplan BA, Soejima K, et al. Catheter ablation of ventricular tachycardia after repair of congenital heart disease: electroanatomic identification of the critical right ventricular isthmus. Circulation. 2007;116:2241–52.

    Article  CAS  PubMed  Google Scholar 

  44. Moore JP, Seki A, Shannon KM, Mandapati R, Tung R, Fishbein MC. Characterization of anatomic ventricular tachycardia isthmus pathology after surgical repair of tetralogy of Fallot. Circ Arrhythm Electrophysiol. 2013;6:905–11.

    Article  PubMed  Google Scholar 

  45. Kapel GF, Reichlin T, Wijnmaalen AP, Piers SR, Holman ER, Tedrow UB, et al. Re-entry using anatomically determined isthmuses: a curable ventricular tachycardia in repaired congenital heart disease. Circ Arrhythm Electrophysiol. 2015;8:102–9. This is a recent study of the mechanisms and catheter-based treatment of ventricular tachycardia in patients with tetralogy of Fallot and related variants. The authors describe very good efficacy and low risk of recurrence in those patients who underwent successful catheter ablation.

    Article  PubMed  Google Scholar 

  46. Therrien J, Siu SC, Harris L, Dore A, Niwa K, Janousek J, et al. Impact of pulmonary valve replacement on arrhythmia propensity late after repair of tetralogy of Fallot. Circulation. 2001;103:2489–94.

    Article  CAS  PubMed  Google Scholar 

  47. Sabate Rotes A, Connolly HM, Warnes CA, Ammash NM, Phillips SD, Dearani JA, et al. Ventricular arrhythmia risk stratification in patients with tetralogy of Fallot at the time of pulmonary valve replacement. Circ Arrhythm Electrophysiol. 2015;8:110–6.

    Article  PubMed  Google Scholar 

  48. Khairy P, Landzberg MJ, Gatzoulis MA, Lucron H, Lambert J, Marcon F, et al. Value of programmed ventricular stimulation after tetralogy of Fallot repair: a multicenter study. Circulation. 2004;109:1994–2000.

    Article  PubMed  Google Scholar 

  49. Hayward RM, Dewland TA, Moyers B, Vittinghoff E, Tanel RE, Marcus GM, et al. Device complications in adult congenital heart disease. Heart Rhythm. 2015;12:338–44.

    Article  PubMed  Google Scholar 

  50. Bedair R, Babu-Narayan SV, Dimopoulos K, Quyam S, Doyle AM, Swan L, et al. Acceptance and psychological impact of implantable defibrillators amongst adults with congenital heart disease. Int J Cardiol. 2015;181:218–24.

    Article  PubMed  Google Scholar 

  51. Davies M, Anderson R, Becker A. The conduction system of the heart. London: Butterworths; 1983.

    Google Scholar 

  52. Simmons MA, Rollinson N, Fishberger S, Qin L, Fahey J, Elder RW. Modern incidence of complete heart block in patients with L-looped ventricles: does univentricular status matter? Congenit Heart Dis. 2015.

  53. American College of Cardiology Foundation, American Heart Association Task Force on Practice Guidelines, Heart Rhythm Society, Epstein AE, DiMarco JP, Ellenbogen KA, Estes 3rd NA, Freedman RA, et al. 2012 ACCF/AHA/HRS focused update incorporated into the ACCF/AHA/HRS 2008 guidelines for device-based therapy of cardiac rhythm abnormalities: a report of the American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines and the Heart Rhythm Society. J Am Coll Cardiol. 2013;61:e6–75.

    Google Scholar 

  54. Cohen MI, Wernovsky G, Vetter VL, Wieand TS, Gaynor JW, Jacobs ML, Spray TL, Rhodes LA (1998) Sinus node function after a systematically staged Fontan procedure. Circulation 98: II352-358; discussion II358-359

  55. Kucharczuk JC, Cohen MI, Rhodes LA, Karl TR, Spray TL, Gaynor JW. Epicardial atrial pacemaker lead placement after multiple cardiac operations. Ann Thorac Surg. 2001;71:2057–8.

    Article  CAS  PubMed  Google Scholar 

  56. Ceulemans A, Scavee C, Marchandise S, de Waroux JBL. Use of electroanatomic voltage mapping to guide lead implantation in young adults with congenital heart disease. Acta Cardiol. 2012;67:487–9.

    PubMed  Google Scholar 

  57. Moore JP, Shannon KM (2014) Transpulmonary atrial pacing: an approach to transvenous pacemaker implantation after extracardiac conduit Fontan surgery. J Cardiovasc Electrophysiol 25: 1028–1031. This is the first description of a novel approach to transvenous atrial pacing in ACHD patients with extracardiac conduit total cavopulmonary connection repair. A detailed description of the technique is provided.

  58. Arif S, Clift PF, De Giovanni JV. Permanent trans-venous pacing in an extra-cardiac Fontan circulation. Europace. 2015.

  59. Garnreiter J, Whitaker P, Pilcher T, Etheridge S, Saarel E. Lumenless pacing leads: performance and extraction in pediatrics and congenital heart disease. Pacing Clin Electrophysiol. 2015;38:42–7.

    Article  PubMed  Google Scholar 

  60. Shepherd E, Stuart G, Martin R, Walsh MA. Extraction of SelectSecure leads compared to conventional pacing leads in patients with congenital heart disease and congenital atrioventricular block. Heart Rhythm. 2015;12:1227–32.

    Article  PubMed  Google Scholar 

  61. Reddy VY, Exner DV, Cantillon DJ, Doshi R, Bunch TJ, Tomassoni GF, et al. Percutaneous implantation of an entirely intracardiac leadless pacemaker. N Engl J Med. 2015.

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Authors and Affiliations

  1. Department of Pediatrics, Division of Cardiology, UCLA Medical Center, 200 Medical Plaza Dr. Suite 330, Los Angeles, CA, 90095, USA

    Ian Lindsay MD & Jeremy P. Moore MD, MS, FHRS

  2. Ahmanson-UCLA Adult Congenital Heart Disease Center, University of California, Los Angeles, CA, USA

    Jeremy P. Moore MD, MS, FHRS

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Correspondence to Jeremy P. Moore MD, MS, FHRS.

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This article is part of the Topical Collection on Pediatric Congenital Heart Disease

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Lindsay, I., Moore, J.P. Cardiac Arrhythmias in Adults With Congenital Heart Disease: Scope, Specific Problems, and Management. Curr Treat Options Cardio Med 17, 56 (2015). https://doi.org/10.1007/s11936-015-0418-6

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