Normal cardiac contractility in patients with congenital nemaline myopathy.
We made cardiac investigations in 12 patients aged 5-25 years with congenital nemaline myopathy by physical examination, electrocardiography, chest-X-ray and echocardiography to find out whether they showed signs of structural anomalies, specific heart muscle disease, conduction defects or cor pulmonale. One patient had a small ventricular septal defect and one had mitral valve prolapse, neither of which had caused haemodynamic alterations. In the other patients, cardiac findings were normal. Cardiac follow-up seems indicated, however, since cor pulmonale may develop in these patients because of their small vital capacity and restricted breathing movements.
Airenne
A L
AL
Jorvi Hospital, Espoo, Finland.
Wallgren-Pettersson
C
C
D000293:Adolescent; D000328:Adult; D009202:Cardiomyopathies / Q000151:congenital / Q000503:physiopathology*; D002648:Child; D002675:Child, Preschool; D004452:Echocardiography; D006321:Heart / Q000503:physiopathology*; D006330:Heart Defects, Congenital / Q000503:physiopathology*; D006801:Humans; D009200:Myocardial Contraction