[Progressive cardiomyopathic lentiginosis]

We report on a 30-year-old man with progressive cardiomyopathic lentiginosis. This syndrome is first described by Polani and Moynahan 1972. They distinguish between the earlier described multiple lentigines syndrome, the LEOPARD syndrome (L-entiginosis, E-lectrocardiographic conduction defects, O-cular hypertelorism, P-ulmonary stenosis, A-bnormalities of genitalia, R-etardation of growth, D-eafness) and the progressive cardiomyopathic syndrome. The progressive cardiomyopathic syndrome is characterised by multiple symmetrical lentigines, hypertrophic obstructive cardiomyopathy and retardation of growth. All characteristics were present in our patient with only mild right ventricular hypertrophic cardiomyopathy.