Pacemaker malfunctions in Danon's disease

We describe a case of a 30-year-old man with Danon’s disease, an X-linked genetic disorder due todeficiency of lysosomal-associated membrane protein 2 with secondary intracytoplasmatic glycogen andautophagic material storage. This disease is characterized by skeletal muscle involvement, mental retar-dation, ophthalmic abnormalities, and cardiac disease. In this patient, cardiac involvement was charac-terized by hypertrophic cardiomyopathy in young age, preexcitation, and parossistic atrioventriular block.The patient underwent to an implantable cardioverter defibrillator implantation for conduction disordersand for primary prevention of sudden death, a frequent event in Danon’s disease. This case report de-scribes cardiac involvement with conduction disorders and multiple pacemaker malfunctions in Danon’sdisease.