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

C Conri, M Sicart, F Vallot

    Archives Des Maladies Du Coeur Et Des Vaisseaux
    |December 1, 1978
    PubMed

    Insights

    Duchenne muscular dystrophy patients show impaired left ventricular function, even those without overt cardiac issues. Myocardial involvement parallels the severity of muscle disease.

    Area of Science:

    • Cardiology
    • Neuromuscular Disorders
    • Cardiomyopathy

    Context:

    • Duchenne de Boulogne muscular dystrophy (DMD) is a progressive neuromuscular disorder.
    • Cardiac complications are common in DMD, but early functional changes are not fully understood.
    • This study investigates left ventricular function in DMD patients.

    Purpose:

    • To assess left ventricular (LV) function in patients with rapidly progressive Duchenne muscular dystrophy.
    • To correlate cardiac findings with the duration and severity of peripheral muscle disease.
    • To identify subclinical cardiac dysfunction in DMD.

    Summary:

    • Fifteen DMD patients underwent hemodynamic and angiographic assessment.
    • Three groups were identified: normal (I), intermediary (II), and hypokinetic cardiomyopathy (III).
    • Impaired LV function and segmental dyskinesis were observed, with elevated LV pressures in groups II and III. Even patients appearing normal at rest showed impaired function under stress (angiotensin test).

    Impact:

    • Reveals subclinical left ventricular dysfunction in DMD patients, even in the basal state.
    • Highlights the importance of cardiac monitoring in DMD.
    • Demonstrates a parallel progression between cardiac and peripheral muscle disease severity.

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