Rhythmic Collapse and Significant Pulmonary Hypertension Indicative of Arrhythmogenic Dysplasia of Right Ventricle: A Case Report

Arrhythmogenic right ventricular dysplasia (ARVD) or Right ventricular arrhythmogenic cardiomyopathyis a genetically determined of cardiac muscle disease. It can lead to serious rhythmic and hemodynamic consequences in direct relation to the progressive adipose transformation of the most often right ventricular myocardium. We report a case of a 35-year-old woman hospitalized for rhythmic collapse. The electrocardiogram showed a rapid atrial fibrillation reduced by an external electric shock. The echocardiography showed an important dilatation of the right heart chambers and a significant pulmonary hypertension on tricuspid regurgitation. Thoracic angioscanner has eliminated a thromboembolic cause that could be the cause of pulmonary hypertension. Cardiac MRI diagnosed ADRV by confirming the dilatation of the right cavities and revealed thinning and fibro-fatty transformation of the RV walls. The evolution under treatment with bases of phosphodiesterase inhibitors, amiodarone was favorable with a follow-up of 12 months.