Cardiopathy in a patient with Emery-Dreifuss muscular dystrophy

Journal of Clinical and Basic Cardiology 2000; 3 (2): 145-146

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Keywords: atrialer Stillstand,  Emery-Dreifuss-Muskeldystrophie,  Kardiomyopathie,  Leitungsstörungen,  atrial standstill,  cardiomyopathy,  conduction disturbances,  Emery-Dreifuss

We report on a 25 year old man with the rare lesion of X-chromosomal recessive Emery-Dreifuss muscular dystrophy. The clinical picture is characterized by muscle weakness, contractures and progressive cardiopathy. The cardiac disease presents itself as progressive heart failure with dilatation of both atria and ventricles. The patient has pathognomonic atrial standstill, junctional bradycardia of about 40 bpm and cardiac arrests up to 3700 msec. These abnormalities are caused by pathologic changes with replacement of muscular tissue with adipose and fibrous tissue in the atria and ventricles. The patient belongs to a German family with 17 affected males over 3 generations, 9 of whom died suddenly at ages between 37 and 59 years. Whether these sudden deaths occurred due to asystoly or tachyarrhythmia is unknown. Based on his family history and on the progressive cardiopathy our patient received an implantable cardioverter/defibrillator (ICD) with an integrated VVIR pacemaker. Furthermore, medication with ACE-inhibitors and warfarin has been started. J Clin Basic Cardiol 2000; 3: 145-6.