[Change in heart function in Duchênne de Boulogne myopathies at the severe progressive stage]

Arch Mal Coeur Vaiss. 1978 Dec;71(12):1417-23.
[Article in French]

Abstract

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.

Publication types

  • English Abstract

MeSH terms

  • Adolescent
  • Adult
  • Cardiac Output
  • Child
  • Electrocardiography
  • Heart Diseases / classification
  • Heart Diseases / physiopathology*
  • Heart Failure / complications
  • Heart Ventricles / physiopathology
  • Hemodynamics
  • Humans
  • Kinetics
  • Male
  • Muscular Dystrophies / complications
  • Muscular Dystrophies / physiopathology*