Publication

Myotonic dystrophy as a potential killer

Journal Paper/Review - Aug 1, 2009

Units
PubMed

Citation
Brenner R, Rickli H. Myotonic dystrophy as a potential killer. Acta Cardiol 2009; 64:567-9.
Type
Journal Paper/Review (English)
Journal
Acta Cardiol 2009; 64
Publication Date
Aug 1, 2009
Issn Print
0001-5385
Pages
567-9
Brief description/objective

A 19-year-old man suffered a cardiac arrest during a promenade with his friends. Cardiac resuscitation was started immediately. Anamnesis uncovered that the father as well as a cousin of the patient suffered from myotonic dystrophy (MD). Follow-up ECG monitoring showed intercurrent III degree AV-block as well as several asymptomatic episodes of ventricular tachycardias, atrial flutter with changing conduction and atrial fibrillation. Neuromuscular testing and genetic analyses confirmed the diagnosis of a myotonic dystrophy. Myotonic dystrophy (MD) is a chronic, slowly progressing, autosomal dominant inherited multisystemic disease.The clinical presentation is characterized by wasting of the muscles with delayed relaxation, cataracts and endocrine changes. MD is associated with both cardiac conduction disturbances and structural heart abnormalities. Electrocardiographic abnormalities include conduction disturbances or tachyarrhythmias. This case illustrates that potentially lethal arrhythmias inducing sudden cardiac death may occur in MD patients even in the absence of neurologic symptoms characterizing the systemic illness.