Other genetic and acquired cardiomyopathies

Abstract

Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an inherited, progressive cardiomyopathy associated with high risk of ventricular tachycardia associated with right-sided structural disorders. More recent reports have shown that although the right ventricle (RV) is most often affected, the left ventricle (LV) is also commonly involved. This has led to the more recent term arrhythmogenic cardiomyopathy (AC). Men are more frequently affected than women and it is usually diagnosed between the second and fourth decade of life. The most common presentation is ventricular arrhythmia, specifically ventricular tachycardia originating from the RV with a characteristic left bundle-branch block (LBBB) morphology. ARVC is also an important cause of sudden death in individuals <30 years of age and has been found in up to 20% of sudden deaths in young individuals. Furthermore, ARVC is more common in athletes and disease expression is associated with high intensity exercise.