Résumé : We report the case of a young man without prior medical history who presented with a left ventricular heart failure because of a new onset atrial fibrillation. The evolution is characterized by a sinus rythm dysfunction, a complete atrioventricular block, a non-sustained ventricular tachycardia (which required a dual-chamber defibrillator implantation) and an ischemic testicular necrosis treated by orchidectomy. After ruling out differential diagnosis we evoked an hypertrophic cardiomyopathy. Medical family anamnesis revealed an hereditary component, and we concluded to a PRKAG2 cardiac syndrome presenting as a familial hypertrophic cardiomyopathy. Two years later, he presented with a type B aortic dissection. We review the literature and differentials diagnosis.