[Arrhythmogenic right ventricular dysplasia disclosed by ventricular extrasystole]

Arrhythmogenic right ventricular dysplasia is one of the principal causes of sudden cardiac death in young subjects. In the absence of ventricular tachycardia, the disease can be revealed by simple ventricular extrasystole. In the light of such a case, the authors discuss the value of various complementary investigations for the diagnosis of these clinical forms with little or no symptoms. Detailed examination of the electrocardiogram followed by a search for late ventricular potentials are decisive steps before proceeding to invasive investigations which often remain essential to confirm the diagnosis. The prognosis remains uncertain, and is always dominated by the risk of sudden death, even in these apparently minor forms of the disease. This point further emphasizes the need for detection, which should be facilitated by the recent establishment of a list of diagnostic criteria.