Cardiologic and neuromuscular co-morbidity influences mortality of patients with left ventricular hypertrabeculation/noncompaction: Comparison with the Austrian general population

Abstract

Objective — Left ventricular hypertrabeculation/noncompaction (LVHT) is characterized by prominent trabeculations and intertrabecular recesses. LVHT patients suffer from heart failure, arrhythmias, chest pain and neuromuscular disorders (NMD). Data about long-term prognosis of LVHT are controversial.The aim of the study was to compare the mortality of LVHT patients with that of the Austrian general population and to assess which clinical and echocardiographic parameters influence mortality, and if mortality differs between patients with and without NMD.

Methods and results — In 86patients LVHT was diagnosed echocardiographically between June 1995 and December 2004 (21 women, mean age 52±14, range 14 - 94years).A specific NMD was diagnosed in 21, a NMD of unknown aetiology in 33, the neurologic investigation was normal in 13 and 19patients refused examination. During a mean follow-up of 51months (range 3 – 106months) the mortality rate was 5.3%/year. Compared with the lifetable from the Austrian general population and considering the sex, the standardized mortality ratio (SMR) of LVHT patients was 5.584 (95% CI 3.562–8.754, p=0.000). The SMR was high in LVHT patients with NMD of unknown aetiology, who refused the neurologic investigation, with heart failure, diabetes mellitus, syncope, ventricular ectopic beats, pathologic Q waves, left anterior hemiblock, atrial fibrillation and low-voltage ECG. Patients with more extensive LVHT had a high SMR.

Conclusions — Mortality in LVHT patients is higher than in the general population and cardiac and neurologic morbidity is the presumed cause for the increased mortality.

Key Words:

• left ventricular noncompaction
• cardiomyopathy
• prognosis
• mortality