Sudden cardiac death in patients with congenital heart disease

Adult patients with congenital heart disease are at risk of sudden cardiac death caused by malignant ventricular tachyarrhythmias. The reported prevalence of ventricular tachyarrhythmias is up to 30% and they are mainly reported in patients with tetralogy of Fallot (ToF) and transposition of the great arteries (TGA). These dysrhythmias may be preceded by non-sustained ventricular tachycardia. The aim of this chapter is to discuss the present knowledge on epidemiology and physiology of malignant ventricular tachyarrhythmias in patients with congenital heart disease and to demonstrate data concerning the time course of ventricular tachyarrhythmia in a large cohort of patients with a variety of congenital heart defects. In addition, the interrelationship between ventricular premature beats, (non)-sustained ventricular tachycardias and ventricular fibrillation are described. In our data, we show that ventricular tachyarrhythmias appear on average at the age of 40, but they rarely developed in patients with only non-sustained ventricular tachycardias.