Ablation and Device Therapy in Adults with Repaired Congenital Heart Disease

Fri, 5/1/09 - 11:24am
0 Comments
4168 reads

Start Page:

End page:

Author(s):

Jason Bradfield, MD; Noel Boyle, MD, PhD;
David Cesario, MD, PhD*; Jamil Aboulhosn, MD;
Kalyanam Shivkumar, MD, PhD
UCLA Cardiac Arrhythmia Center, Department of Medicine,
David Geffen School of Medicine at UCLA;
*Keck School of Medicine at USC
Los Angeles, California

Some form of congenital heart disease is present in 75/1,000 live births. ¹ The majority of these patients live into adulthood, with approximately one half million adults living with congenital heart disease in the United States. Sudden cardiac death is now the leading cause of mortality in this patient population.^2,3

Advances in surgery for congenital heart disease have saved or prolonged the lives of thousands. As with any successful operation or procedure, there are trade-offs for success. Many years removed from their initial surgery, these patients are now at risk for morbidity and mortality from bradyarrhythmias and tachyarrhythmias.

Arrhythmias are common in this population and occur for a number of reasons. Some forms of congenital heart disease predispose to arrhythmia at baseline. After repair, the substrate created by scars, grafts, patches and suture lines further predispose to arrhythmia. These substrate abnormalities lead to slowed conduction and macroreentrant arrhythmias. ⁴Repaired congenital heart disease patients are further at risk for the development of arrhythmias due to abnormal hemodynamic changes within the affected cardiac chambers. ⁵ Once these patients develop arrhythmias, they can develop electrical remodeling as well. ^6,7

Developing an electrophysiology program for the treatment of arrhythmia in adult congenital heart disease requires a complete understanding of the anatomy and physiology underlying congenital heart disease. It also requires a detailed understanding of the individual patient’s surgical corrective and palliative procedures, which may vary significantly. Expertise in all areas of interventional electrophysiology is essential to successfully treat the congenital heart disease patient population. The frequency of underlying conduction system disease in this population, as well as the increased frequency of ventricular dysfunction that develops from the time of initial operation, make the options for pharmacologic antiarrhythmic therapy limited. Treatment of arrhythmia with ablation and implanted devices is often required.

The 2008 ACC/AHA guidelines for the treatment of adults with congenital heart disease give a class I recommendation for ablation and device implantation procedures to be undertaken at centers that “have significant experience with the complex anatomy and distinctive arrhythmia substrates encountered in congenital heart defects.” ⁸ The UCLA Congenital Heart Disease Center is responsible for the care of thousands of adult patients with congenital heart disease. These numbers only continue to grow.

References:

1. Hoffman JI, Kaplan S. The incidence of congenital heart disease. J Am Coll Cardiol 2002;39:1890-1900.

2. Oechslin EN, Harrison DA, Connelly MS, et al. Mode of death in adults with congenital heart disease. Am J Cardiol 2000;86:1111-1116.

3. Silka MJ, Hardy BG, Menashe VD, et al. A population-based prospective evaluation of risk of sudden cardiac death after operation for common congenital heart defects. J Am Coll Cardiol 1998;32:245-251.

4. Perloff JK, Middlekauf HR, Child JS, et al. Usefulness of post-ventriculotomy signal averaged electrocardiograms in congenital heart disease. Am J Cardiol 2006;98:1646-1651.

5. Perloff JK, Warnes CA. Challenges posed by adults with repaired congenital heart disease. Circulation 2001;103: 2637-2643.

6. Nattel S. Atrial electrophysiological remodeling caused by rapid atrial activation: Underlying mechanisms and clinical relevance to atrial fibrillation. Cardiovasc Res 1999;42:298-308.

7. Wijffels MC, Kirchhof CJ, Dorland R, Allessie MA. Atrial fibrillation begets atrial fibrillation. A study in awake chronically instrumented goats. Circulation 1995;92:1954-1968.

8. Warnes CA, Wiliams RG, Bashore TM, et al. ACC/AHA 2008 guidelines for adults with congenital heart disease: A report of the American College of Cardiology/American Heart Association task force on practice guidelines. J Am Coll Cardiol 2008;52:e1-121.

9. Triedman JK, DeLucca JM, Alexander ME, et al. Prospective trial of electroanatomically guided, irrigated catheter ablation of atrial tachycardia in patients with congenital heart disease. Heart Rhythm 2005;2:700-705.

10. Mustard WT. Successful two-stage correction of transposition of the great vessels. Surgery 1964;55:469-472.

11. Champsaur GL, Sokol DM, Trusler GA, Mustard WT. Repair of transposition of the great arteries in 123 pediatric patients: Early and long-term results. Circulation 1973;47:1032-1041.

12. Kalman JM, VanHare GF, Olgin JE, et al. Ablation of 'incisional' reentrant atrial tachycardia complicating surgery for congenital heart disease. Use of entrainment to define a critical isthmus of conduction. Circulation 1996;93:502-512.

13. Cecchin F, Johnsrude CL, Perry JC, Friedman RA. Effect of age and surgical technique on symptomatic arrhythmias after the Fontan procedure. Am J Cardiol 1995;76:386-391.

14. Balling G, Vogt M, Kaemmerer H, et al. Intracardiac thrombus after the fontan operation. J Thorac Cardiovasc Surg 2000;119:745-751.

15. Goldberger Z, Lampert R. Implantable cardioverter-defibrillators: Expanding indications and technologies. JAMA 2006;295:809-818.

16. Bar-Cohen Y, Berul CI, Alexander ME, et al. Age, size, and lead factors alone do not predict venous obstruction in children and young adults with transvenous lead systems. J Cardiovasc Electrophysiol 2006;17:754-759.

17. Karpawich PP. Technical aspects of pacing in adult and pediatric congenital heart disease. Pacing Clin Electrophysiol 2008;31:S28-31.

18. Sondheimer HM, Izukawa T, Olley PM, et al. Conduction disturbances after total correction of tetralogy of Fallot. Am Heart J 1976;92:278-282.

19. Godman MJ, Roberts NK, Izukawa T. Late postoperative conduction disturbances after repair of ventricular septal defect and tetralogy of Fallot. Analysis by his bundle recordings. Circulation 1974;49:214-221.

20. 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.

21. Waien SA, Liu PP, Ross BL, et al. Serial follow-up of adults with repaired tetralogy of Fallot. J Am Coll Cardiol 1992;20:295-300.

22. Chandar JS, Wolff GS, Garson A Jr, et al. Ventricular arrhythmias in postoperative tetralogy of Fallot. Am J Cardiol 1990;65:655-661.

23. Deanfield JE, Ho SY, Anderson RH, et al. Late sudden death after repair of tetralogy of Fallot: A clinicopathologic study. Circulation 1983;67:626-631.

24. Garson A Jr, Randall DC, Gillette PC, et al. Prevention of sudden death after repair of tetralogy of Fallot: Treatment of ventricular arrhythmias. J Am Coll Cardiol 1985;6:221-227.

25. Gatzoulis MA, Balaji S, Webber SA, et al. Risk factors for arrhythmia and sudden cardiac death late after repair of tetralogy of Fallot: A multicenter study. Lancet 2000;356:975-981.

26. Khairy P. EP challenges in congenital heart disease. Heart Rhythm 2008;5:1465-1472.

27. Khairy P, Harris L, Landzberg MJ, et al. Implantable cardioverter-defibrillators in tetralogy of Fallot. Circulation 2008;117:363-370.

28. Estner HL, Kolb C, Schmitt C, et al. Long-tern transvenous AV-sequential pacing in a failing atriopulmonary Fontan patient. Int J Cardiol 2008;127:e93-e95.

29. Fortescue EB, Berul CI, Cecchin F, et al. Patient, procedural, and hardware factors associated with pacemaker lead failures in pediatrics and congenital heart disease. Heart Rhythm 2004;1;150-159.

30. Cesario D, Kedia R, Desai N, et al. Device extraction in adults with congenital heart disease. Pacing Clin Electrophysiol 2009;32:340-345.

31. Cooper JM, Stephenson EA, Berul CI, et al. Implantable cardioverter defibrillator lead complications and laser extraction in children and young adults with congenital heart disease: Implications for implantation and management. J Cardiovasc Electrophysiol 2003;14:344-349.

32. Khairy P, Roux J, Dubuc M, et al. Laser lead extraction in adult congenital heart disease. J Cardiovasc Electrophysiol 2007;18:507-511.

33. Shivkumar K, Perloff JK. Electrophysiologic abnormalities: Unoperated and postoperative residua and sequelae. In: Perloff JK, Child JS, Aboulhosn J (eds). Congenital Heart Disease in Adults, 3rd edition. Saunders; 2008.