Arrhythmogenic right ventricular cardiomyopathy/dysplasia: risk stratification and therapy.

摘要

Arrhythmogenic right ventricular cardiomyo-pathy/dysplasia (ARVC/D) is an inherited heart muscle disease that occurs primarily in young and middle-aged individuals. It is characterized by ventricular arrhythmias (VA), sudden death, and heart failure occurring later in life. Ventricular electrical instability may occur at any time during the disease depending upon possible different pathophysiologic mechanisms including (a) inflammation and apoptosis leading to ventricular fibrillation or (b) fibrofatty tissue repair leading to scar-related ventricular tachycardia (VT). Heart failure may occur later in life secondary to slow, progressive loss of right and left ventricular myocardium.