title: Evaluation of heart involvement in calpainopathy (LGMD2A) using cardiovascular magnetic resonance.
abstract: BACKGROUND
  Cardiac dysfunction occurs in several forms of limb girdle muscular dystrophy (LGMD). The aim of this study was to investigate cardiac involvement in calpainopathy (LGMD2A).
  
  
  METHODS
  Cardiovascular evaluation was performed in 10 patients with genetically verified LGMD2A by echocardiography, 3 Tesla - cardiovascular magnetic resonance, 24-h electrocardiography recordings with heart rate variability (HRV) analysis, and 24-h blood pressure recordings.
  
  
  RESULTS
  No patient with calpainopathy showed impairment of left or right ventricular function. One patient had a small amount (2% of left ventricle mass) of late gadolinium enhancement. HRV analysis revealed no significant difference compared with external reference data.
  
  
  CONCLUSIONS
  The main finding of this study is the lack of cardiac involvement in patients with calpainopathy. Cardiac involvement was not found, even in individuals with advanced age and greater disease severity. Furthermore, we did not observe an overall reduction of cardiac autonomic regulation in calpainopathy.
authors.affiliation: Technische Universität Dresden, University Hospital, Heart Center, Department of Internal Medicine and Cardiology, Dresden, Germany.
authors.forename: Silvio
authors.initials: S
authors.lastname: Quick
authors.affiliation: Technische Universität Dresden, University Hospital, Department of Neurology, Dresden, Germany.
authors.forename: Jochen
authors.initials: J
authors.lastname: Schaefer
authors.affiliation: Technische Universität Dresden, University Hospital, Heart Center, Department of Internal Medicine and Cardiology, Dresden, Germany.
authors.forename: Nadine
authors.initials: N
authors.lastname: Waessnig
authors.affiliation: Technische Universität Dresden, University Hospital, Heart Center, Department of Internal Medicine and Cardiology, Dresden, Germany.
authors.forename: Thorsten
authors.initials: T
authors.lastname: Schultheiss
authors.affiliation: Technische Universität Dresden, University Hospital, Department of Neurology, Dresden, Germany.
authors.forename: Ulrike
authors.initials: U
authors.lastname: Reuner
authors.affiliation: Technische Universität Dresden, University Hospital, Heart Center, Department of Internal Medicine and Cardiology, Dresden, Germany.
authors.forename: Steffen
authors.initials: S
authors.lastname: Schoen
authors.affiliation: Technische Universität Dresden, University Hospital, Department of Neurology, Dresden, Germany.
authors.forename: Heinz
authors.initials: H
authors.lastname: Reichmann
authors.affiliation: Technische Universität Dresden, University Hospital, Heart Center, Department of Internal Medicine and Cardiology, Dresden, Germany.
authors.forename: Ruth
authors.initials: R
authors.lastname: Strasser
authors.affiliation: Technische Universität Dresden, University Hospital, Heart Center, Department of Internal Medicine and Cardiology, Dresden, Germany.
authors.forename: Uwe
authors.initials: U
authors.lastname: Speiser
mesh_terms: D000328:Adult; D001794:Blood Pressure; D002319:Cardiovascular System / Q000503:physiopathology*; D004452:Echocardiography; D005260:Female; D006321:Heart / Q000503:physiopathology*; D006339:Heart Rate / Q000502:physiology; D006801:Humans; D021621:Imaging, Three-Dimensional; D009682:Magnetic Resonance Spectroscopy; D008297:Male; D008875:Middle Aged; D049288:Muscular Dystrophies, Limb-Girdle / Q000473:pathology* / Q000503:physiopathology*; D055815:Young Adult
keywords: LGMD2A; calpainopathy; cardiac magnetic resonance imaging; echocardiography; heart rate variability; muscular dystrophy