[Change in heart function in Duchênne de Boulogne myopathies at the severe progressive stage].
Left ventricular function was studied in 15 patients with rapidly progressive Duchenne de Boulogne muscular dystrophy. Only one patient had a previous history of cardiac failure. All patients had typical electrocardiographical changes. Haemodynamic and angiographic investigation was performed in all patients. Three groups of patients were distinguished according to angiographic criteria : group I comprising 7 normal patients. group II comprising 4 "intermediary" patients, and group III of 4 patients with hypokinetic cardiomyopathy. Segmental ventricular dyskinesis was observed in 6 patients. Mitral incompetence was present in 2 cases and mitral valve prolpse in 1 case. All patients had a normal resting cardiac index. Patients in group II and III had end diastolic left ventricular pressures greater than 15 mmHg. An intravenous angiotensin test was performed in 4 patients from groups I and II. Impaired left ventricular function was demonstrated in patients thought to be normal in the basal state. Finally, myocardial involvement in this group of patients runs a parallel course to the duration and severity of the peripheral muscular disease.
D000293:Adolescent; D000328:Adult; D002302:Cardiac Output; D002648:Child; D004562:Electrocardiography; D006331:Heart Diseases / Q000145:classification / Q000503:physiopathology*; D006333:Heart Failure / Q000150:complications; D006352:Heart Ventricles / Q000503:physiopathology; D006439:Hemodynamics; D006801:Humans; D007700:Kinetics; D008297:Male; D009136:Muscular Dystrophies / Q000150:complications / Q000503:physiopathology*
Altération de la fonction cardiaque dans les myopathies de Duchênne de Boulogne au stade évolutif sévère.