/familial isolated arrhythmogenic right ventricular dysplasia

familial isolated arrhythmogenic right ventricular dysplasia

Rare Disease

MONDO:0016342

Also Known As

familial isolated ARVCfamilial isolated ARVDfamilial isolated arrhythmogenic right ventricular cardiomyopathyfamilial isolated arrhythmogenic right ventricular dysplasiafamilial isolated arrhythmogenic ventricular cardiomyopathyfamilial isolated arrhythmogenic ventricular dysplasia

Definition

Familial isolated arrhythmogenic right ventricular dysplasia (ARVC) is the familial autosomal dominant form of ARVC, a heart muscle disease characterized by life-threatening ventricular arrhythmias with left bundle branch block configuration that may manifest with palpitations, ventricular tachycardia, syncope and sudden fatal attacks, and that is due to dystrophy and fibro-fatty replacement of the right ventricular myocardium that may lead to right ventricular aneurysms.