Familial isolated arrhythmogenic right ventricular dysplasia
Familial isolated arrhythmogenic right 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.
Also known as familial isolated arrhythmogenic right ventricular cardiomyopathy, familial isolated arrhythmogenic right ventricular dysplasia, familial isolated arrhythmogenic ventricular cardiomyopathy, familial isolated arrhythmogenic ventricular dysplasia, familial isolated ARVC, familial isolated ARVD — per MONDO