title: Left ventricular dysfunction and cardiac arrhythmias are frequent in type 2 myotonic dystrophy: a case control study.
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.
authors.affiliation: Myology Institute, Pitié-Salpétrière Hospital, APHP, 47-83 boulevard de l'Hôpital, Paris Cedex 13, France. karim.wahbi@psl.aphp.fr
authors.forename: Karim
authors.initials: K
authors.lastname: Wahbi
authors.forename: Christophe
authors.initials: C
authors.lastname: Meune
authors.forename: Henri Marc
authors.initials: HM
authors.lastname: Bécane
authors.forename: Pascal
authors.initials: P
authors.lastname: Laforêt
authors.forename: Guillaume
authors.initials: G
authors.lastname: Bassez
authors.forename: Arnaud
authors.initials: A
authors.lastname: Lazarus
authors.forename: Hélène
authors.initials: H
authors.lastname: Radvanyi-Hoffman
authors.forename: Bruno
authors.initials: B
authors.lastname: Eymard
authors.forename: Denis
authors.initials: D
authors.lastname: Duboc
mesh_terms: 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*