Poorly tolerated ventricular tachycardia revealing arrhythmogenic right ventricle dysplasia in a young black man.

Abstract

Arrhythmogenic right ventricle dysplasia (ARVD) is a hereditary cardiomyopathy characterized by progressive replacement of cardiomyocytes by fibro-adipose tissue, mainly in the right ventricle. Clinical manifestations are ventricular arrhythmia from the right ventricle revealing by palpitations which is the most common symptom, more rarely syncopia or sudden death. His diagnosis is often difficult, based on clinical, rhythmic, morphological and histological arguments. We report a case of poorly tolerated ventricular tachycardia revealing ARVD in a young black man. He was admitted in our department for paroxysmal palpitations associated with a fall without loss of consciousness and he was in cardiovascular collapse. The EKG showed a ventricular tachycardia. The hemodynamic state was restored after amiodarone and defibrillation. The transthoracic echocardiography found a moderate dilatation of the right ventricle associated with a hypokinesia of the inferior and apical wall. The systolic right ventricle function was moderately altered and pulmonary systolic pressures were normal.Cardiac MRI revealed severe fatty infiltration of the free wall of the right ventricle associated with fibrosis in late enhancement sequence. The patient received 200 mg of Amiodarone per day, five days out of seven. An implantable cardiac defibrillator (ICD) was proposed.