Myotonic dystrophy as a potential killer

Brenner, Roman; Joerg, Lucas; Rickli, Hans (2009). Myotonic dystrophy as a potential killer. Acta cardiologica, 64(4), pp. 567-9. Leuven: Peeters 10.2143/AC.64.4.2041628

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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.

Item Type:

Journal Article (Further Contribution)

Division/Institute:

04 Faculty of Medicine > Department of Cardiovascular Disorders (DHGE) > Clinic of Cardiology

UniBE Contributor:

Brenner, Roman

ISSN:

0001-5385

ISBN:

19725456

Publisher:

Peeters

Language:

English

Submitter:

Factscience Import

Date Deposited:

04 Oct 2013 15:09

Last Modified:

04 May 2014 23:22

Publisher DOI:

10.2143/AC.64.4.2041628

PubMed ID:

19725456

Web of Science ID:

000269196200022

URI:

https://boris.unibe.ch/id/eprint/30215 (FactScience: 191463)

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