Progressive cardiac dysfunction in Bethlem myopathy during pregnancy.

Flöck, Anne; Kornblum, Cornelia; Hammerstingl, Christoph; Claeys, Kristl G; Gembruch, Ulrich; Merz, Waltraut Maria · Obstet Gynecol · 2014

case_report · Level V

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Abstract

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

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