title: Left ventricular hypertrabeculation/noncompaction with and without neuromuscular disorders.
abstract: BACKGROUND
  Left ventricular hypertrabeculation/noncompaction (LVHT) is frequently associated with neuromuscular disorders (NMD). It is unknown, whether LVHT patients differ according to the presence or absence of NMD. Aim of the study was to assess, if clinical, ECG or echocardiographic findings differ between LVHT patients with and without NMD.
  
  
  METHODS
  Included were all patients, in whom LVHT was diagnosed between June 1995 and February 2003 in one echocardiographic laboratory. All patients underwent a cardiologic examination and were invited for a neurologic investigation.
  
  
  RESULTS
  Of 77 patients with LVHT (19 female, mean age 52 years), 59 were investigated neurologically. Eleven were neurologically normal, 21 had a definite NMD (metabolic myopathy, n=15; Leber's hereditary optic neuropathy, n=3; myotonic dystrophy, n=2 and Becker muscular dystrophy, n=1). The remaining 27 had a NMD of unknown etiology. Neurologically normal patients had more often anginal chest pain than patients with definite NMD (64% vs. 14%, P=0.0042) or NMD of unknown etiology (64% vs. 26%, P=0.0157). Neurologically normal patients were more often in NYHA class 0 or I than patients with NMD of unknown etiology (64% vs. 26%, P=0.0289) and had a thinner interventricular septum than patients with NMD (10.6 mm vs. 12.8 mm, P=0.0253).
  
  
  CONCLUSIONS
  Cardiac abnormalities are hardly different between patients with and without NMD.
authors.affiliation: 2nd Medical Department, Krankenanstalt Rudolfstiftung, Steingasse 31/18, A-1030 Wien, Osterreich, Austria. claudia.stoellberger@chello.at
authors.forename: Claudia
authors.initials: C
authors.lastname: Stöllberger
authors.forename: Maria
authors.initials: M
authors.lastname: Winkler-Dworak
authors.forename: Gerhard
authors.initials: G
authors.lastname: Blazek
authors.forename: Josef
authors.initials: J
authors.lastname: Finsterer
mesh_terms: D004562:Electrocardiography; D005260:Female; D006330:Heart Defects, Congenital / Q000150:complications* / Q000000981:diagnostic imaging; D006352:Heart Ventricles / Q000002:abnormalities*; D006801:Humans; D008297:Male; D008875:Middle Aged; D009468:Neuromuscular Diseases / Q000150:complications*; D014463:Ultrasonography