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Neuromuscul Disord. 2009 Jul;19(7):468-72. doi: 10.1016/j.nmd.2009.04.012. Epub 2009 May 29.

Left ventricular dysfunction and cardiac arrhythmias are frequent in type 2 myotonic dystrophy: a case control study.

Author information

1
Myology Institute, Pitié-Salpétrière Hospital, APHP, 47-83 boulevard de l'Hôpital, Paris Cedex 13, France. karim.wahbi@psl.aphp.fr

Abstract

In contrast with Steinert's disease (DM1), type 2 muscular dystrophy (DM2) is not known to be associated with a high prevalence of cardiac involvement. Our objective was to compare the results of detailed cardiac investigations in populations of DM2 and DM1 patients, and in controls. Thirty-eight DM2 patients (17 males; age=57.1+/-15.2years) were investigated for possible heart involvement, and their results compared with 76 age-sex matched DM1 patients and 76 controls. Cardiac abnormalities were present in 15 DM2 patients, including conductive defects in 14, systolic dysfunction in 6, supraventricular arrhythmias in 6 and stroke in 5 patients and were significantly more frequent than in controls. When compared to DM1 patients, conductive defects were less frequent, supraventricular arrhythmias had similar prevalence and there was a trend towards more frequent left ventricular dysfunction in DM2 patients. Our study suggests that systematic cardiac investigations should be recommended in these patients.

PMID:
19481939
DOI:
10.1016/j.nmd.2009.04.012
[Indexed for MEDLINE]

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