Ventricular Arrhythmias in Adults With Congenital Heart Disease, Part II: JACC State-of-the-Art Review

J Am Coll Cardiol. 2023 Sep 12;82(11):1121-1130. doi: 10.1016/j.jacc.2023.06.036.

Abstract

There are marked variations in the incidence of sudden cardiac death (SCD) and in the substrates for ventricular arrhythmias (VAs) across the gamut of congenital heart defects. In this 2-part review, patients with higher-risk forms of congenital heart disease (CHD) were conceptually categorized into those with discrete anatomic isthmuses for macro-reentrant ventricular tachycardia (VT) (Group A) and those with more diffuse or less well-defined substrates (Group B) that include patchy or extensive myocardial fibrosis. The latter category encompasses CHD lesions such as Ebstein anomaly, transposition of the great arteries with a systemic right ventricle (RV), and congenital aortic stenosis. For Group B patients, polymorphic VT and ventricular fibrillation account for a higher proportion of VA. The prognostic value of programmed ventricular stimulation is less well established, and catheter ablation plays a less prominent role. As cardiomyopathies evolve over time, pathophysiological mechanisms for VA among Groups A and B become increasingly blurred.

Keywords: adult congenital heart disease; implantable cardioverter-defibrillator; sudden cardiac death; ventricular arrhythmias; ventricular fibrillation; ventricular tachycardia.

Publication types

  • Review

MeSH terms

  • Adult
  • Arrhythmias, Cardiac / epidemiology
  • Arrhythmias, Cardiac / etiology
  • Arrhythmias, Cardiac / therapy
  • Ebstein Anomaly*
  • Heart Defects, Congenital* / complications
  • Humans
  • Tachycardia, Ventricular* / diagnosis
  • Tachycardia, Ventricular* / epidemiology
  • Tachycardia, Ventricular* / etiology
  • Transposition of Great Vessels*