Bethlem myopathy may be associated with progressive left ventricular dysfunction during pregnancy, which can recover postpartum following early delivery.
May warrant closer LV monitoring in pregnant patients with Bethlem myopathy; leaves open whether early delivery aids recovery in this rare setting.
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.
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Flöck et al. (2014) studied this question.
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