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July 05, 2011; 77 (1) Articles

Cardiac abnormalities in a follow-up study on carriers of Duchenne and Becker muscular dystrophy

S.M. Schade van Westrum, E.M. Hoogerwaard, L. Dekker, T.S. Standaar, E. Bakker, P.F. Ippel, J.C. Oosterwijk, D.F. Majoor-Krakauer, A.J. van Essen, N.J. Leschot, A.A.M. Wilde, R.J. de Haan, M. de Visser, A.J. van der Kooi
First published June 22, 2011, DOI: https://doi.org/10.1212/WNL.0b013e318221ad14
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Abstract

Objectives: Cardiac involvement has been reported in carriers of dystrophin mutations giving rise to Duchenne muscular dystrophy (DMD) and Becker muscular dystrophy (BMD). The progress of these abnormalities during long-term follow-up is unknown. We describe the long-term follow-up of dilated cardiomyopathy (DCM) in DMD/BMD carriers.

Methods: A long-term follow-up study was performed among Dutch DMD/BMD carriers first analyzed in 1995. A cardiac history was taken, and all carriers were assigned a functional score to assess skeletal muscle involvement. Electrocardiography and M-mode and 2-D echocardiography were performed. DCM was defined as an enlarged left ventricle with a global left ventricle dysfunction or fractional shortening less than 28%. Slow vital capacity of the lung was measured by a hand-held spirometer.

Results: Ninety-nine carriers were monitored with a median follow-up of 9 years (range 7.0–10.6 years). Eleven carriers with DCM (10 DMD, 1 BMD) were identified. Nine of them developed DCM in the follow-up period. One of the patients with DCM reported in the 1995 study died of cardiac failure at age 57 years. DCM was more frequently found in carriers who were functionally symptomatic.

Conclusion: Cardiac abnormalities in DMD/BMD carriers are progressive, as in patients with DMD/BMD.

Footnotes

  • BMD=
    Becker muscular dystrophy;
    BSA=
    body surface area;
    DCM=
    dilated cardiomyopathy;
    DMD=
    Duchenne muscular dystrophy;
    ENMC=
    European Neuromuscular Centre;
    FSI=
    fractional shortening index;
    LAD=
    left atrial diameter;
    LVEDD=
    left ventricle end diastolic diameter;
    LVESD=
    left ventricle end systolic diameter;
    LVF=
    left ventricular function;
    sVC=
    slow vital capacity.

  • Received December 22, 2010.
  • Accepted February 25, 2011.
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