Obstetrics & Gynecology

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Obstetrics & Gynecology:
doi: 10.1097/AOG.0000000000000101
Case Report

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

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Abstract

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.

© 2014 by The American College of Obstetricians and Gynecologists.

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