Progressive cardiac dysfunction in Bethlem myopathy during pregnancy.

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.

• Authors: Anne Flöck, Cornelia Kornblum, Christoph Hammerstingl, Kristl G Claeys, Ulrich Gembruch, Waltraut Maria Merz
• Journal: Obstetrics and gynecology (Obstet Gynecol) Vol. 123 Issue 2 Pt 2 Suppl 2 Pg. 436-438 (Feb 2014) ISSN: 1873-233X [Electronic] United States
• PMID: 24413245 (Publication Type: Case Reports, Journal Article)

Topics

• Adult
• Contracture (complications)
• Female
• Heart Failure (etiology)
• Humans
• Muscular Dystrophies (complications, congenital)
• Pregnancy
• Pregnancy Complications, Cardiovascular (etiology)
• Ventricular Dysfunction, Left (etiology)