Life-threatening congestive heart failure as the presentation of centronuclear myopathy.


Journal Article

Myocardiopathy is associated infrequently with centronuclear myopathy. We present biopsy studies of a 15 1/2-year-old black male who presented with profound acute congestive heart failure and diffuse muscular atrophy. Cardiac symptoms had been present for 6 months; limb weakness had been unassociated with either infantile hypotonia or developmental delay. Cardiac catheterization demonstrated a dilated myocardiopathy and poor left ventricular contractility. Biopsies of both ventricles revealed striking hydropic degeneration and fibrosis. Right triceps biopsy disclosed centronuclear myopathy. Because the spectrum of disease expression in centronuclear myopathy is extensive, an association with cardiac disease always should be considered in these patients. In addition, we recommend that patients who present with idiopathic myocardiopathy should be evaluated for this and other skeletal muscle diseases.

Full Text

Duke Authors

Cited Authors

  • Gospe, SM; Armstrong, DL; Gresik, MV; Hawkins, HK

Published Date

  • March 1, 1987

Published In

Volume / Issue

  • 3 / 2

Start / End Page

  • 117 - 120

PubMed ID

  • 3508053

Pubmed Central ID

  • 3508053

International Standard Serial Number (ISSN)

  • 0887-8994

Digital Object Identifier (DOI)

  • 10.1016/0887-8994(87)90040-3


  • eng

Conference Location

  • United States