Cardiomiopatia secundária á distrofia de Steinert

myotonic dystrophy type 1 (md1 or steinert's disease) is a genetic syndrome with multissistemic repercussions, and it is usual to the patient to seek for several specialists before clinical suspicion. it is related a case of a 28 years old male patient admitted with congestive heart failure. md1 has been diagnosed eight years before. at the time of the diagnosis, he presented syncope secondary to complete atrioventricular block. from that time on, he had a permanent pacemaker implanted. etiological, pathophysiological and clinical aspects of md1 with emphasis on cardiovascular manifestations are discussed.