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[Facioscapulohumeral muscular dystrophy with sinus dysfunction].

Hiroshi Shigeto1, Takuhisa Tamura, Yasushi Oya

  • 1Department of Medicine, National Sanatorium Higashi-Saitama Hospital

Rinsho Shinkeigaku = Clinical Neurology
|April 25, 2003
PubMed
Summary

Facioscapulohumeral muscular dystrophy (FSHD) can cause distinct sinus node dysfunction, particularly when accompanied by respiratory failure. This case highlights the critical link between neuromuscular and cardiac health in FSHD patients.

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Area of Science:

  • Neurology
  • Cardiology
  • Pulmonology

Background:

  • Facioscapulohumeral muscular dystrophy (FSHD) is a progressive myopathy affecting skeletal muscles.
  • Cardiac involvement in FSHD is recognized, but sinus node dysfunction is less commonly emphasized.

Observation:

  • A 47-year-old male with FSHD presented with severe motor decline and typical FSHD features.
  • He exhibited significant sinus node dysfunction, including transient P wave inversion and prolonged P-P intervals, predominantly during sleep.
  • Respiratory failure was evident with decreased vital capacity, hypercapnia, and hypoxia, worsening during sleep.

Findings:

  • Cardiac evaluation revealed right atrial and ventricular overload.
  • Holter monitoring demonstrated significant sinus node dysfunction without other major arrhythmias.

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  • Severe respiratory compromise was a key feature, independent of sleep apnea.

    • Implications:

    • Sinus node dysfunction may be a significant, under-recognized complication of FSHD, especially in the context of respiratory insufficiency.
    • This case underscores the importance of comprehensive cardiac and respiratory monitoring in FSHD patients.
    • Early recognition and management of sinus node dysfunction and respiratory failure are crucial for improving outcomes in FSHD.