Clinical features and cardiopulmonary function of patients with atrophic heart in Duchenne muscular dystrophy.
The clinical features and cardiopulmonary function of 5 patients with atrophic heart in Duchenne muscular dystrophy (DMD) were studied by echocardiography and spirometry. Atrophic heart was defined as a state where the left ventricular end-diastolic volume decreased markedly during long-term follow-up (over 5 years). The patients with atrophic heart were more emaciated and showed more severe motor disability than the controls (18 DMD patients with a normal left ventricular cavity size). The pre-ejection period/ejection time ratio was significantly higher in patients with atrophic heart than in controls, and the maximal diastolic posterior wall velocity was reduced. The forced vital capacity was lower in patients with atrophic heart than in controls, but the one-second forced expiratory volume rate was similar in the 2 groups. Three patients with atrophic heart died. Examination at autopsy showed that their hearts were small and of low weight, and showed not only fibrosis, but also brown atrophy and waxy degeneration. These findings indicate that cardiopulmonary function is significantly reduced in DMD patients with atrophic heart.
Matsuoka
S
S
Department of Pediatrics, School of Medicine, Tokushima University, Japan.
Ii
K
K
Akita
H
H
Tomimatsu
H
H
Kurahashi
Y
Y
Nakatsu
T
T
Miyao
M
M
D000293:Adolescent; D000328:Adult; D001284:Atrophy / Q000503:physiopathology; D002302:Cardiac Output; D009202:Cardiomyopathies / Q000503:physiopathology*; D004452:Echocardiography; D006801:Humans; D008124:Locomotion; D008297:Male; D009136:Muscular Dystrophies / Q000503:physiopathology*; D009200:Myocardial Contraction; D009206:Myocardium / Q000473:pathology*; D013318:Stroke Volume; D014797:Vital Capacity