Left ventricular hypertrabeculation/noncompaction with and without neuromuscular disorders.
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.
Stöllberger
Claudia
C
2nd Medical Department, Krankenanstalt Rudolfstiftung, Steingasse 31/18, A-1030 Wien, Osterreich, Austria. claudia.stoellberger@chello.at
Winkler-Dworak
Maria
M
Blazek
Gerhard
G
Finsterer
Josef
J
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