Clinical features and cardiopulmonary function of patients with atrophic heart in duchenne muscular dystrophy.

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概要

• 論文の詳細を見る

• 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 onesecond 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.

• International Heart Journal刊行会の論文

著者

• MATSUOKA SUGURU

  Department of Pediatrics

• Ii Kunio

  First Department Of Pathology School Of Medicine The University Of Tokushima

• Tomimatsu Hirofumi

  Department Of Pediatric Cardiology Tokyo Women's Medical University

• Nakatsu Tadanori

  Department Of Pediatrics School Of Medicine Tokushima University

• Miyao Masuhide

  Department Of Pediatrics School Of Medicine Tokushima University

• Kurahashi Yoshihide

  Department Of Pediatrics School Of Medicine Tokushima University

• Akita Hiroshi

  Department Of Civil Engineeringr Tohoku Institute Of Technology

• NAKATSU Tadanori

  Department of Pediatrics School of Medicine, Tokushima University

• II Kunio

  First Department of Pathology School of Medicine, Tokushima University

• MATSUOKA Suguru

  Department of Pediatrics School of Medicine, Tokushima University

• TOMIMATSU Hirofumi

  Department of Pediatrics School of Medicine, Tokushima University

• MIYAO Masuhide

  Department of Pediatrics School of Medicine, Tokushima University

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