Sudden Cardiac Death in Congenital Heart Disease

Khairy P, Silka MJ, Moore JP, et al.
Sudden Cardiac Death in Congenital Heart Disease. Eur Heart J 2022;43:2103-2115.

The following are key points to remember from this state-of-the-art review related to sudden cardiac death (SCD) in congenital heart disease (CHD):

  1. SCD is a relatively common occurrence in patients with congenital heart disease (CHD), accounting for up to 25% of all deaths.
  2. The annual rate of SCD in patients with CHD varies based on the congenital heart lesion, and ranges from 0.28–2.7%. The rate for SCD in adults with tetralogy of Fallot (TOF) ranges from 0.9–1.5%.
  3. High risk substrates for SCD include TOF, transposition of the great arteries (TGA) after atrial switch surgeries, cyanotic heart disease, Ebstein anomaly, and Fontan circulation.
  4. Stratification schemes to predict SCD should include not only the risk of SCD but also competing cause of mortality, SCD related to nonshockable rhythms, and be balanced against the higher risk of device-related complications in adults with CHD.
  5. Two generic risk stratification schemes have recently been proposed to identify and validate factors with SCD in patients with CHD. The Prevention ACHD was derived from the Dutch CONCOR registry and the Toronto Adult Congenital Heart Program. The Spanish registry is derived from the Spanish adult CHD network. High-risk features common to both scores include systolic dysfunction of the systemic or subpulmonary ventricle, a wide QRS complex, and ischemic heart disease.
  6. There is consistent consensus that an implantable cardioverter-defibrillator (ICD) is indicated (Class I) in the following situations:
    • CHD patients who are survivors of cardiac arrest due to ventricular arrhythmias following evaluation of the cause of the event and after exclusion of reversible triggers.
    • Patients with symptomatic sustained ventricular tachycardia after hemodynamic and electrophysiologic evaluation.
  7. There is consensus that an ICD should be considered (Class IIa) in:
    • ┬áThe CHD patient with unexplained (presumably arrhythmic) syncope in the setting of impaired left ventricular function or inducible ventricular tachycardia or fibrillation at electrophysiologic study.
    • The TOF patient with multiple risk factors for SCD.
  8. There is consensus that an ICD is not indicated (Class III) for:
    • Patients with a life expectancy <1 year.
    • Incessant episodes of ventricular tachycardia or fibrillation.
    • Significant psychiatric illness, which might be aggravated by an ICD.
    • New York Heart Association class IV functional symptoms when a patient is not a candidate for cardiac resynchronization therapy.
  9. Complication rates remain high for CHD patients with ICDs. Device-related complications have been reported in 26% of patients and in appropriate shocks in 25% over a mean follow-up of 3.7–3.8 years.
  10. Subcutaneous ICD (S-ICD) has emerged as a viable option for some patients with CHD at risk for SCD. The main limitation is the absence of antibradycardia and antitachycardia pacing. As many as 40% of patients may be noneligible for S-SCD predominately due to a wide bundle branch block.

Clinical Topics: Arrhythmias and Clinical EP, Cardiac Surgery, Congenital Heart Disease and Pediatric Cardiology, Heart Failure and Cardiomyopathies, Invasive Cardiovascular Angiography and Intervention, Implantable Devices, EP Basic Science, SCD/Ventricular Arrhythmias, Atrial Fibrillation/Supraventricular Arrhythmias, Cardiac Surgery and Arrhythmias, Cardiac Surgery and CHD and Pediatrics, Cardiac Surgery and Heart Failure, Congenital Heart Disease, CHD and Pediatrics and Arrhythmias, CHD and Pediatrics and Interventions, CHD and Pediatrics and Prevention, CHD and Pediatrics and Quality Improvement, Acute Heart Failure, Interventions and Structural Heart Disease

Keywords: Arrhythmias, Cardiac, Bundle-Branch Block, Cardiac Resynchronization Therapy, Death, Sudden, Cardiac, Defibrillators, Implantable, Ebstein Anomaly, Electrophysiology, Fontan Procedure, Heart Arrest, Heart Defects, Congenital, Heart Failure, Myocardial Ischemia, Risk Assessment, Risk Factors, Syncope, Tachycardia, Ventricular, Tetralogy of Fallot, Transposition of Great Vessels, Ventricular Fibrillation

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