Left ventricular dysfunction and cardiac arrhythmias are frequent in type 2 myotonic dystrophy: a case control study.
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.
Wahbi
Karim
K
Myology Institute, Pitié-Salpétrière Hospital, APHP, 47-83 boulevard de l'Hôpital, Paris Cedex 13, France. karim.wahbi@psl.aphp.fr
Meune
Christophe
C
Bécane
Henri Marc
HM
Laforêt
Pascal
P
Bassez
Guillaume
G
Lazarus
Arnaud
A
Radvanyi-Hoffman
Hélène
H
Eymard
Bruno
B
Duboc
Denis
D
D000328:Adult; D000368:Aged; D001145:Arrhythmias, Cardiac / Q000175:diagnosis / Q000453:epidemiology* / Q000503:physiopathology*; D016022:Case-Control Studies; D015897:Comorbidity; D004452:Echocardiography / Q000592:standards; D004562:Electrocardiography / Q000592:standards; D005260:Female; D020022:Genetic Predisposition to Disease / Q000235:genetics; D005820:Genetic Testing; D006323:Heart Arrest / Q000209:etiology / Q000378:metabolism / Q000503:physiopathology; D006352:Heart Ventricles / Q000378:metabolism / Q000473:pathology / Q000503:physiopathology; D006801:Humans; D008297:Male; D008403:Mass Screening; D008875:Middle Aged; D009206:Myocardium / Q000378:metabolism / Q000473:pathology; D020967:Myotonic Disorders / Q000453:epidemiology* / Q000235:genetics / Q000503:physiopathology*; D015995:Prevalence; D020521:Stroke / Q000209:etiology / Q000378:metabolism / Q000503:physiopathology; D018487:Ventricular Dysfunction, Left / Q000175:diagnosis / Q000453:epidemiology* / Q000503:physiopathology*