Institutional members access full text with Ovid®

Share this article on:

Progressive Cardiac Dysfunction in Bethlem Myopathy During Pregnancy

Flöck, Anne MD; Kornblum, Cornelia MD; Hammerstingl, Christoph MD; Claeys, Kristl G. MD; Gembruch, Ulrich MD; Merz, Waltraut Maria MD

doi: 10.1097/AOG.0000000000000101
Case Report

BACKGROUND: Bethlem myopathy is a congenital myopathy presenting with muscle weakness and joint abnormalities. Although cardiac involvement is frequent in other inherited myopathies, it has not yet been described in Bethlem myopathy.

CASE: We report a case of progressive deterioration of left ventricular function during pregnancy in a patient with Bethlem myopathy. Worsening of clinical symptoms, particularly dyspnea, necessitated delivery at 36 2/7 weeks of gestation. Myocardial function recovered postpartum with improvement of clinical symptoms.

CONCLUSION: Bethlem myopathy may be associated with progressive left ventricular dysfunction during pregnancy and may require early delivery.

Bethlem myopathy may be associated with progressive left ventricular dysfunction during pregnancy and may require early delivery.

Departments 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.

Correspondence to: Dr. Waltraut Maria Merz, Department of Obstetrics and Prenatal Medicine, University Medical School Bonn, Sigmund-Freud-Str. 25, 53105 Bonn, Germany; e-mail: waltraut.merz@ukb.uni-bonn.de.

Financial Disclosure The authors did not report any potential conflicts of interest.

© 2014 by The American College of Obstetricians and Gynecologists.