CARDIOMYOPATHIC LENTIGINOSIS LEOPARD-SYNDROME PRESENTING AS SUDDEN CARDIAC-ARREST

A 26-year-old apparently healthy man with numerous pigmented skin lesi ons collapsed during an evening party and was resuscitated from ventri cular fibrillation. Hypertrophic cardiomyopathy and subaortic tunnel w ere disclosed by angiocardiography, A diagnosis of cardiomyopathic len tiginosis/lentigines (multiple), electrocardiographic abnormalities, o cular hypertelorism, pulmonary stenosis, abnormalities of the genitali a, retardation of growth, and deafness (sensorineural) syndrome was ma de. The patient then underwent treatment with an implantable pacer-car dioverter-defibrillator device. Further evaluation revealed several we ll-established features of the disorder. This is the first reported ca se of survival from ventricular fibrillation associated with this rare and little known multifaceted syndrome. Disseminated lentiginosis mus t prompt clinicians to evaluate such cases further since underlying di sorders may be associated with considerable morbidity and, apparently, sudden death.