Arrhythmogenic right ventricular dysplasia: a sudanese experience

ABSTRACT

Arrhythmogenic right ventricular dysplasia cardiomyopathy [ARVDC] is a genetic cardiomyopathy characterized by ventricular arrhythmias and structural abnormalities of the right ventricle [RV].There were no reported studies on the presentation of Sudanese patients with ARVDC. This study aimed to describe patient characteristics and various manifestations of ARVDC among 4 patients seen at Shaab Teaching Hospital [STH] during the period from 2006-2010. This study was carried out in STH. STH is the central reference cardiology hospital in Sudan with the final diagnosis of ARVDC. Initially, none of those patients was diagnosed as having ARVDC. The first patient was treated as myocardial infarction and epilepsy. The second patient was diagnosed as Epilepsy. Both the third and fourth patients were treated for palpitations and the fourth patient was diagnosed as having heart failure as well. The first Patient died suddenly while the others stayed alive and have come for follow-up. They were prescribed anti-arrhythmic drugs, and showed remarkable improvement. The diagnosis of these cases based primarily on findings obtained from clinical assessment and simple laboratory tests, as neither MRI nor endomyocardial biopsy were available. Awareness of the protean presentations of ARVDC shouldn't be overlooked. Palpitation isn't always benign. Big heart shadows in X-rays may have many causes. T-wave changes not necessarily ischemia and Loss of consciousness isn't synonymous with epilepsy only. A national registry is a necessity