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Progressive cardiac dysfunction in Bethlem myopathy during pregnancy

Anne Flöck1, Cornelia Kornblum, Christoph Hammerstingl

  • 1Departments of Obstetrics and Prenatal Medicine, Neurology, and Cardiology, University Medical School, Bonn, and the Department of Neurology and the Institute of Neuropathology, RWTH Aachen University, Aachen, Germany.

Obstetrics and Gynecology
|January 14, 2014
PubMed

Insights

Bethlem myopathy, a congenital muscle disorder, can cause progressive heart dysfunction during pregnancy. This case highlights the need for close cardiac monitoring and potential early delivery in affected individuals.

Area of Science:

  • Cardiology
  • Neuromuscular Disorders
  • Genetics

Background:

  • Bethlem myopathy is a congenital myopathy characterized by muscle weakness and joint abnormalities.
  • Cardiac involvement is common in inherited myopathies but has not been previously documented in Bethlem myopathy.

Observation:

  • A pregnant patient with Bethlem myopathy experienced progressive left ventricular dysfunction.
  • Worsening dyspnea necessitated delivery at 36 2/7 weeks of gestation.

Findings:

  • Myocardial function improved postpartum, coinciding with clinical symptom resolution.
  • This case suggests a potential association between Bethlem myopathy and pregnancy-related cardiac complications.

Implications:

  • Bethlem myopathy may be linked to progressive left ventricular dysfunction during pregnancy.
  • Early delivery may be required in pregnant patients with Bethlem myopathy experiencing cardiac decline.
  • Further research is warranted to understand the cardiac manifestations of Bethlem myopathy.
Abstract

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