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Arrhythmias in Patients with Congenital Heart Disease

  • Published:
Cardiac Electrophysiology Review

Abstract

Improved surgical outcome for patients with congenital heart disease (CHD) has created a rapidly expanding population of adolescents and young adult survivors. Cardiac arrhythmias are a common late sequelae of this form of heart disease. Effective treatment requires clear understanding of the underlying anatomic defect as well as the specific surgical interventions. Intraatrial reentrant tachycardia (IART) is the most common and difficult arrhythmia encountered in these patients. Traditional IART treatment with medication has been largely unsuccessful, but radiofrequency ablation has emerged in recent years as a promising option for many patients. The availability of three-dimensional mapping systems and irrigated-tip ablation catheters has improved acute success rates for IART to better than 90%. Postablation recurrence of IART still remains problematic for patients who have undergone the Fontan operation, in which case atrial maze surgery may be considered. Ventricular tachycardia (VT) is seen in a smaller number of CHD patients, most notably those with tetralogy of Fallot or aortic stenosis. The adoption of implantable defibrillator (ICD) therapy for these patients has improved outcome. Owing to their complex anatomy, the CHD population presents unique challenges during both catheterization and device implant. Multicenter study of this unique patient group is needed in order to develop more objective treatment guidelines.

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References

  1. Smith WM, Gallagher JJ, Kerr CR, Sealy WC, Kasell JH, Benson DW, Reiter MJ, Sterba R, Grant AO. The electrophysiologic basis and management of symptomatic recurrent tachycardia in patients with Ebstein's anomaly of the tricuspid valve. Am J Cardiol 1982;49:1223–1234.

    Google Scholar 

  2. Levine J, Walsh EP, Saul JP. Catheter ablation of accessory pathways in patients with congenital heart disease including heterotaxy syndrome. Am J Cardiol 1993;72:689–694.

    Google Scholar 

  3. Flinn CJ, Wolff GS, Dick M, Campbell RM, Borkat G, Casta A, Hordof A, Hougen TJ, Kavey RE, Kugler J. Cardiac rhythm after the Mustard operation for complete transposition of the great arteries.N Engl JMed 1984;3310:1635–1638.

    Google Scholar 

  4. Fishberger SB, Wernovsky G, Gentles TL, Gauvreau K, Burnett J, Mayer JE, Walsh EP. Factors that influence the development of atrial flutter after the Fontan operation. J Thorac Cadiovas Surg 1997;113:80–86.

    Google Scholar 

  5. Walsh EP, Rockenmacher S, Keane JF, Hougen TJ, Lock JE, Castaneda AR. Late results in patients with tetralogy of Fallot repaired during infancy. Circulation1988;77:1062–1067.

    Google Scholar 

  6. Rhodes LA, Wernovsky G, Keane JF, Mayer JE, Shuren A, Dindy C, Colan SD, Walsh EP. Arrhythmias and intracardiac conduction after the arterial switch operation. J Thor Cardiovas Surg 1995;109:303–310.

    Google Scholar 

  7. Stamm C, Friehs I, Mayer JE, Zurakowski D, Triedman JK, Moran AM, Walsh EP, Lock JE, Jonas RA, DelNido PJ. Long-term results of the lateral tunnel Fontan operation. J Thorac Cardiovasc Surg 2001;121:28–41.

    Google Scholar 

  8. Triedman JK, Bergau DM, Saul JP, Epstein MR, Walsh EP. Efficacy of radiofrequency ablation for control of intraatrial reentrant tachycardia in patients with congenital heart disease. J Am Coll Cardiol 1997;30:1032–1038.

    Google Scholar 

  9. Garson A, Bink-Boelkens MTE, Hesslein PS, Hordof AJ, Keane JF, Neches WH, Porter CJ. Atrial flutter in the young: A collaborative study of 380 cases. J Am Coll Cardiol1985;6:871–878.

    Google Scholar 

  10. 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;18:1005–1016.

    Google Scholar 

  11. Triedman JK, Alexander ME, Berul CI, Bevilacqua LM, Walsh EP. Electroanatomic mapping of entrained and exit zones in patients with repaired congenital heart disease and intra-atrial reentrant tachycardia. Circulation2001;103:2060–2065.

    Google Scholar 

  12. Triedman JK, Alexander MA, Love BA, Collins KK, Berul CI, Bevilacqua LM, Walsh EP. Influence of patient factors and ablative technologies on outcomes of radiofrequency ablation of intra-atrial tachycardia in patients with congenital heart disease. J Am Coll Cardiol 2002;39:1827–1835.

    Google Scholar 

  13. 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.AmJ Cardiol2000;86:969–974.

    Google Scholar 

  14. Mavroudis C, Backer CL, Deal BJ, Johnsrude C, Strasburger J. Total cavopulmonary conversion and maze procedure for patients with failure of the Fontan operation. J Thorac Cardiovasc Surg 2001;122:863–871.

    Google Scholar 

  15. Kirsh JA, Walsh EP, Triedman JK. Prevalence of and risk factors for atrial fibrillation and intraatrial reentrant tachycardia among patients with congenital heart disease. Am J Cardiol 2002;90:338–340.

    Google Scholar 

  16. Roos-Hesselink J, Perlroth MG, McGhie J, Spitaels S. Atrial arrhythmias in adults after repair of tetralogy of Fallot: Correlations with clinical, exercise, and echocardiographic findings. Circulation 1995;91:2214–2219.

    Google Scholar 

  17. Chandar JS, Wolff GS, Garson A, Jr, Bell TJ, Beder SD, Bink-Boelkens M, Byrum CJ, Campbell RM, Deal BJ, Dick M, Flinn CJ, Guam WE. Gillette PC, Hordorf AJ, Kugler JD, Porter CJ, Walsh EP. Ventricular arrhythmia in postoperative tetralogy of Fallot. Am J Cardiol 1990;65:655–661.

    Google Scholar 

  18. Gatzoulis MA, Till JA, Redington AN. Depolarizationrepolarization inhomogeneity after repair of tetralogy of Fallot: The substrate for malignant ventricular tachycardia? Circulation 1997;95:401–404.

    Google Scholar 

  19. Alexander ME, Walsh EP,Saul JP, Epstein MR, Triedman JK. Value of programmed ventricular stimulation in patients with congenital heart disease. J Cardiovasc Electrophysiol 1999;10:1033–1044.

    Google Scholar 

  20. Bilbo LA, Carlson MD. Transcatheter ablation of ventricular tachycardia following surgical correction of tetralogy of Fallot. Pacing Clin Electrophysiol 1994;17:1556–1560.

    Google Scholar 

  21. Gonska BD, Cao K, Raab J, Eigster G, Kreuzer H. Radiofrequency catheter ablation of right ventricular tachycardia late after repair of congenital heart defects. Circulation 1996;94:1902–1908.

    Google Scholar 

  22. Berul CI, Barrett KS, Walsh EP. Implantable cardioverterdefibrillators in pediatric patients. In: Walsh EP, Saul JP, Triedman JK, eds. Cardiac Arrhythmias in Children and Young Adults with Congenital Heart Disease. Philadelphia: Lippincott Williams and Wilkins, 2001:93–111.

    Google Scholar 

  23. Alexander MA, Walsh EP, Triedman JK, Bevilacqua L, Epstein LM, Berul CI. Evolving experience with ICD technology in pediatrics and congenital heart disease (abstract). Pacing Clin Electrophysiol 2002;25: 625.

    Google Scholar 

  24. ACC/AHA Task Force on Practice Guidelines (Committee on Pacemaker Implantation). ACC/AHA guidelines for implantation of cardiac pacemakers and antiarrhythmia devices: Executive Summary. Circulation 1998;97:1325–1335.

    Google Scholar 

  25. Weindling SN, Gamble WJ, Mayer JE, Walsh EP. Duration of complete atrioventricular block after congenital heart disease surgery. Am J Cardiol 1998;82:525–527.

    Google Scholar 

  26. Hurwitz RA, Riemenschneider TA, Moss AJ. Chronic postoperative heart block in children. Am J Cardiol 1968;21:185–189.

    Google Scholar 

  27. Mullen MP, VanPraagh R, Walsh EP. Development and anatomy of the cardiac conduction system. In: Walsh EP, Saul JP, Triedman JK, eds. Cardiac Arrhythmias in Children and Young Adults with Congenital Heart Disease. Philadelphia: Lippincott Williams and Wilkins, 2001:93–111.

    Google Scholar 

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Walsh, E.P. Arrhythmias in Patients with Congenital Heart Disease. Card Electrophysiol Rev 6, 422–430 (2002). https://doi.org/10.1023/A:1021192526642

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