Cardiologic and neurologic findings in left ventricular hypertrabeculation/non-compaction related to wall thickness, size and systolic function.

Left ventricular hypertrabeculation/noncompaction (LVHT) is a rare cardiac abnormality, diagnosed echocardiographically when >3 left ventricular trabeculations are visible in one image plane apically to the papillary muscles and intertrabecular spaces are perfused from the ventricular cavity. In the majority of the cases, LVHT is associated with neuromuscular disorders (NMD). LVHT occurs in dilated as well as in normally sized ventricles, with or without systolic dysfunction and wall thickening. Aim of the study was to assess whether cardiologic and neurologic findings differ between patients according to echocardiographically determined left ventricular size, systolic function and wall thickness. In 77 patients (19 f, mean age 52 years) LVHT was diagnosed. LVHT was assessed as 'dilative' (enddiastolic diameter >or=60 mm and fractional shortening <or=25%) in 43 cases, in 18 patients as 'hypertrophic' (interventricular septum and posterior wall >or=12 mm and fractional shortening >or=26%) and in the remaining 16 patients as 'normally-dimensioned'. Dilative LVHT patients were older than hypertrophic or normally-dimensioned LHVT patients. The prevalence of NMD was 63% in dilative LVHT, 67% in hypertrophic LVHT and 56% in normally-dimensioned LVHT. LHVT is more frequently found in dilated than hypertrophic ventricles. NMD are equally frequent in dilative, hypertrophic and normally-dimensioned LVHT. Cardiac abnormalities may progress with age.