title: 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.
  
  
  METHODS
  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.
  
  
  CONCLUSIONS
  Bethlem myopathy may be associated with progressive left ventricular dysfunction during pregnancy and may require early delivery.
authors.affiliation: 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.
authors.forename: Anne
authors.initials: A
authors.lastname: Flöck
authors.forename: Cornelia
authors.initials: C
authors.lastname: Kornblum
authors.forename: Christoph
authors.initials: C
authors.lastname: Hammerstingl
authors.forename: Kristl G
authors.initials: KG
authors.lastname: Claeys
authors.forename: Ulrich
authors.initials: U
authors.lastname: Gembruch
authors.forename: Waltraut Maria
authors.initials: WM
authors.lastname: Merz
mesh_terms: D000328:Adult; D003286:Contracture / Q000150:complications*; D005260:Female; D006333:Heart Failure / Q000209:etiology; D006801:Humans; D009136:Muscular Dystrophies / Q000150:complications / Q000151:congenital*; D011247:Pregnancy; D011249:Pregnancy Complications, Cardiovascular / Q000209:etiology*; D018487:Ventricular Dysfunction, Left / Q000209:etiology*