Skip to main content

Advertisement

Log in

Management of Late Arrhythmias in Adults With Repaired Congenital Heart Disease

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

Opinion statement

As survival of patients with congenital heart disease improves with surgery and the population of patients with repaired adult congenital heart disease (ACHD) age, understanding complications such as arrhythmias has become more important. Arrhythmias account for significant morbidity and mortality in the ACHD population. Atrial arrhythmias occur in 15 % of patients with ACHD and, in patients with severe congenital heart disease (CHD), more than 50 % develop atrial arrhythmias by the age of 65 years [1]. This article aims to give an overview of arrhythmias seen in the ACHD population and the treatment modalities available now.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Similar content being viewed by others

References and Recommended Reading

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

  1. Bouchardy J, Therrien J, Pilote L, et al. Atrial arrhythmias in adults with congenital heart disease. Circulation. 2009;120:1679–86.

    Article  PubMed  Google Scholar 

  2. Verheugt CL, Uiterwaal CSPM, van der Velde ET, et al. The emerging burden of hospital admissions of adults with congenital heart disease. Heart. 2010;96:872–8. Excellent, large national registry study from the Netherlands, including 28,990 patient years, identifying the admission rates and most common presentations for ACHD patients.

    Article  PubMed  Google Scholar 

  3. Rodriguez FH, Moodie DS, Neeland M, et al. Identifying arrhythmias in adults with congenital heart disease by 24-h ambulatory electrocardiography. Pediatr Cardiol. 2012;33:591–5. A single institution retrospective review of 140 adults with ACHD showing that 15 % of patients had arrhythmia on ECG, 31 % on Holter, and of those identified 76 % were asymptomatic.

    Article  PubMed  Google Scholar 

  4. Czosek RJ, Anderson J, Khoury PR, et al. Utility of ambulatory monitoring in patients with congenital heart disease. Am J Cardiol. 2013;111:723–30. Another single-center retrospective review of all adults with TOF, d-TGA status-post atrial switch, and Fontan palliation receiving Holter monitoring. Describes Holter monitor findings in this patient group and interestingly assigns a positive and negative predictive value for positive Holter monitoring results for future clinically significant arrhythmias and for significant changes in clinical management.

    Article  PubMed  Google Scholar 

  5. Daliento L, Corrado D, Buja GH, et al. Rhythm and conduction disturbances in isolated congenitally corrected transposition of the great arteries. Am J Cardiol. 1986;58:314–8.

    Article  PubMed  CAS  Google Scholar 

  6. Cordina RL, Celermajer DS, McGuire MA. Systemic venous anatomy in congenital heart disease: implications for electrophysiologic testing and catheter ablation. J Interv Card Electrophysiol. 2011, published online.

  7. Konings TC, Dekkers LR, Groenink M, et al. Transvenous pacing after the Mustard procedure: considering the complications. Neth Heart J. 2007;15(11):387–9.

    Article  PubMed  CAS  Google Scholar 

  8. Abraham WT, Fisher WG, Smith AL, et al. Cardiac resynchronization in chronic heart failure. NEJM. 2002;346(24):1845–53.

    Article  PubMed  Google Scholar 

  9. Khairy P, Landzberg MJ, Gatzoulis MA, et al. Tranvenous pacing leads and systemic thromboemboli in patients with intracardiac shunts. Circulation. 2006;113:2391–7.

    Article  PubMed  Google Scholar 

  10. Takahashi K, Cecchin F, Fortescue E, et al. Permanent atrial pacing lead implant route after Fontan operation. PACE. 2009;32:779–85.

    Article  PubMed  Google Scholar 

  11. Walsh EP, Cecchin F. Arrhythmias in adult patients with congenital heart disease. Circulation. 2007;115:534–45.

    Article  PubMed  Google Scholar 

  12. Li W, Somerville J. Atrial flutter in grown-up congenital heart patients: clinical characteristics of affected population. Int J Cardiol. 2000;75:129–37.

    Article  PubMed  CAS  Google Scholar 

  13. Khairy P, Aboulhosn J, Gurvitz MS, et al. Arrhythmia burden in adults with surgically-repaired tetralogy of Fallot: a multiinstitutional study. Circulation. 2010;122:868–75. This multicenter cross-sectional study assesses the prevalence and risk factors for arrhythmias in patients with TOF and interestingly concludes that left-heart disease is more predictive of arrhythmias then right-heart disease.

    Article  PubMed  Google Scholar 

  14. Roden DM. Ibutilide and the treatment of atrial arrhythmias. A new drug—almost unheralded—is now available to US physicians. Circulation. 1996;94:1499–502.

    Article  PubMed  CAS  Google Scholar 

  15. Hoyer A, Balaji S. The safety and efficacy of ibutilide in children and in patients with congenital heart disease. PACE. 2007;30:1003–8.

    Article  PubMed  Google Scholar 

  16. Patsilinakos S, Christou A, Kafkas N, et al. Effect of high doses of magnesium on converting ibutilide to a safe and more effective agent. Am J Cardiol. 2010;106(5):673–6.

    Article  PubMed  CAS  Google Scholar 

  17. Rao SO, Boramanand NK, Burton DA, et al. Atrial tachycardias in young adults and adolescents with congenital heart disease: cardioversion using single dose oral sotalol. Int J Cardiol. 2009;136:253–7.

    Article  PubMed  Google Scholar 

  18. Ammash NM, Phillips SD, Hodge DO, et al. Outcome of direct current cardioversion for atrial arrhythmias in adults with congenital heart disease. Int J Cardiol. 2012;154:270–4. Reports the success rates and complications of synchronized cardioversion in atrial arrhythmias ofACHD patients.

    Article  PubMed  Google Scholar 

  19. Klein AL, Grimm RA, Black IW, et al. Cardioversion guided by transesophageal echocardiography: the ACUTE pilot study. A randomized, controlled trial. Assessment of cardioversion using transesophageal echocardiography. Ann Int Med. 1997;126(3):200–9.

    Article  PubMed  CAS  Google Scholar 

  20. Hohnloser SH, Crijns HJ, van Eikels M, et al. Effect of dronedarone on cardiovascular events in atrial fibrillation. N Engl J Med. 2009;360:668–78.

    Article  PubMed  CAS  Google Scholar 

  21. Singh BN, Connolly SJ, Crijns HJ, et al. Dronedarone for maintenance of sinus rhythm in atrial fibrillation or flutter. N Engl J Med. 2007;357:987–99.

    Article  PubMed  CAS  Google Scholar 

  22. Piccini JP, Hasselblad V, Peterson ED, et al. Comparative efficacy of dronaderone and amiodarone for the maintenance of sinus rhythm in patients with atrial fibrillation. J Am Coll Cardiol. 2009;54:1089–95.

    Article  PubMed  CAS  Google Scholar 

  23. Peichl P, Kautzner J. Advances in irrigated tip catheter technology for treatment of cardiac arrhythmias. Recent Pat Cardiovasc Drug Discov. 2013, epub ahead of print.

  24. Kober L, Torp-Pedersen C, McMurray JJ, et al. Increased mortality after dronaderone therapy for severe heart failure. N Engl J Med. 2008;358:2678–87.

    Article  PubMed  Google Scholar 

  25. Bradfield J, Tung R, Mandapati R, et al. Catheter ablation utilizing remote magnetic navigation: a review of applications and outcomes. Pace. 2012;35:1021–34. A well-written review of remote magnetic navigation and various studies reporting applications and outcomes of this novel technique.

    Article  PubMed  Google Scholar 

  26. Wu J, Pflaumer A, Deisenhofer I, et al. Mapping of atrial tachycardia by remote magnetic navigation in postoperative patients with congenital heart disease. J Cardiovasc Electrophysiol. 2010;21:751–9.

    PubMed  Google Scholar 

  27. Ernst S, Babu-Narayan SV, Keegan J, et al. Remote-controlled magnetic navigation and ablation with 3D image integration as an alternate approach in patients with intra-atrial baffle anatomy. Circ Arrhythm Electrophsiol. 2012;5:131–9.

    Article  Google Scholar 

  28. Akca F, Onsesveren I, Jordaens L, et al. Safety and efficacy of the remote magnetic navigation for ablation of ventricular tachycardias: a systematic review. J Intervent Cardiac Electrophysiol. 2012;34:65–71.

    Article  Google Scholar 

  29. Akca F, Bauernfeind T, Witsenburg M, et al. Acute and long-term outcomes of catheter ablation using remote magnetic navigation in patients with congenital heart disease. Am J Cardiol. 2012;110:409–14.

    Article  PubMed  Google Scholar 

  30. Rhodes LA, Walsh EP, Gamble WJ, et al. Benefits and potential risks of atrial antitachycardia pacing after repair of congenital heart disease. Pacing Clin Electrophysiol. 1995;18:1005–16.

    Article  PubMed  CAS  Google Scholar 

  31. Stephenson EA, Casavant D, Tuzi J, 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 

  32. Stulak JM, Dearani JA, Puga FJ, et al. Right-sided Maze procedure for atrial tachyarrhythmias in congenital heart disease. Ann Thorac Surg. 2006;81:1780–5.

    Article  PubMed  Google Scholar 

  33. Stulak JM, Dearani JA, Burkhart HM, et al. The surgical treatment of concomitant atrial arrhythmias during redo cardiac operations. Ann Thorac Surg. 2012;94:1894–900. A nice retrospective review reporting high success and low complication rates of surgical ablation for arrhythmias performed in conjunction with redo cardiac operations.

    Article  PubMed  Google Scholar 

  34. Verheugt CL, Uiterwaal CSPM, van der Velde ET, et al. Mortality in adult congenital heart disease. Eur Hear J. 2010;31:1220–9. Another excellent, long-term national registry study from the Netherlands group looking at mortality and cause of death in ACHD patients.

    Article  Google Scholar 

  35. Silka MJ, Bar-Cohen Y. A contemporary assessment of the risk for sudden cardiac death in patients with congenital heart disease. Pediatr Cardiol. 2012;33:452–60. A review article discussing current knowledge on risk factors for SCD and the limitation of any single risk factor in predicting SCD.

    Article  PubMed  Google Scholar 

  36. Russo AM, Stainback RF, Bailey SR, et al. ACCF/HRS/AHA/ASE/HFSA/SCAI/SCCT/SCMR 2013 Appropriate Use Criteria for Implantable Cardioverter-Defibrillators and Cardiac Resynchronization Therapy: A Report of the American College of Cardiology Foundation Appropriate Use Criteria Task Force, Heart Rhythm Society, American Heart Association, American Society of Echocardiography, Heart Failure Society of America, Society for Cardiovascular Angiography and Interventions, Society of Cardiovascular Computed Tomography, and Society for Cardiovascular Magnetic Resonance. Heart Rhythm. 2013;e1547–5271.

  37. Khanna AD, Warnes CA, Phillips SD, et al. Single-center experience with implantable cardioverter-defibrillators in adults with complex congenital heart disease. Am J Cardiol. 2011;108:729–34. This is an excellent, large long-term study looking at the indications, success rates, and complications of ICDs placed in patients with ACHD.

    Article  PubMed  Google Scholar 

  38. Olde Nordkamp LRA, Abkenari LD, Boersma LVA, et al. The entirely subcutaneous implantable cardioverter-defrillator. J Am Coll Cardiol. 2012;60(19):1933–9. Exciting report describing the innovative use of a subcutaneous ICD that bypasses the need for intravenous access or sternotomy for epicardial lead placement.

    Article  PubMed  Google Scholar 

  39. Garson Jr A, Kanter RJ. Management of the child with Wolff-Parkinson-White syndrome and supraventricular tachycardia: model for cost-effectiveness. J Cardiovax Electrophysiol. 1997;8:1320–6.

    Article  Google Scholar 

  40. Gorenek B, Kudaiberdieva G. Cost analysis of radiofrequency catheter ablation for atrial fibrillation. Int J Cardiol. 2013. epub ahead of print.

  41. Hess EP, Russell JK, Liu PY, et al. A high peak current 150-J fixed-energy defibrillation protocol treats recurrent ventricular fibrillation (VF) as effectively as initial VF. Resuscitation. 2008;79(1):28–33.

    Article  PubMed  Google Scholar 

  42. Zhan C, Baine WB, Sedrakyan A, et al. Cardiac device implantation in the United States from 1997 to 2004: a population based analysis. J Gen Intern Med. 2007;23 suppl 1:13–9.

    Google Scholar 

  43. Mavroudis C, Deal BJ, Backer CL, et al. J. Maxwell Chamberlain memorial paper for congenital heart surgery. 111 Fontan conversions with arrhythmia surgery: surgical lessons and outcomes. Ann Thorac Surg. 2007;84(5):1457–65.

    Article  PubMed  Google Scholar 

  44. Quenneville SP, Xie X, Brophy JM. The cost-effectiveness of Maze procedures using ablation techniques at the time of mitral valve surgery. Int J Technol Assess Health Care. 2009;25(5):485–96.

    Article  PubMed  Google Scholar 

Download references

Compliance with Ethics Guidelines

Conflict of Interest

Dr. Jennifer H. Huang received a T32 grant from the National Institutes of Health.

Dr. Seshadri Balaji declares no potential conflicts of interest relevant to this article.

Human and Animal Rights and Informed Consent

This article does not contain any studies with human or animal subjects performed by any of the authors.

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Seshadri Balaji MBBS, MRCP, PhD.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Huang, J.H., Balaji, S. Management of Late Arrhythmias in Adults With Repaired Congenital Heart Disease. Curr Treat Options Cardio Med 15, 615–631 (2013). https://doi.org/10.1007/s11936-013-0254-5

Download citation

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s11936-013-0254-5

Keywords

Navigation