Heart Failure, Atrial Fibrillation and Neuromuscular Disorders Influence Mortality in Left Ventricular Hypertrabeculation/Noncompaction

OBJECTIVES: The prognosis of patients with left ventricular hypertrabeculation/noncompaction (LVHT) is controversial. The aim of the study was to assess parameters influencing mortality and to compare the mortality of LVHT patients with that of the general population of Austria. METHODS AND RESULTS: Included were patients with LVHT diagnosed echocardiographically between June 1995 and February 2011. They underwent a baseline cardiologic examination and were invited for neurological investigation. Between January and February 2011, their survival status was assessed. LVHT was diagnosed in 162 patients (46 females, aged 53 ± 16 years) with a prevalence of 0.31%/year. One hundred and sixteen patients (72%) underwent a neurological investigation which revealed specific neuromuscular disorders (NMD) in 25 and NMD of unknown etiology in 74 patients, and 17 subjects were assessed as normal. During the follow-up of 67 months, mortality was 4.8%/year and the standardized mortality ratio was 6.27, compared with the Austrian population. By multivariate analysis, predictors of mortality were heart failure (hazard ratio 3.91, 4.48 and 5.37 for the New York Heart Association class II, III and IV, respectively), atrial fibrillation (hazard ratio 3.26) and NMD (hazard ratio 1.86). CONCLUSION: Prognostic predictors in LVHT are heart failure, atrial fibrillation and NMD. Whether optimal therapy of heart failure and atrial fibrillation will improve the prognosis of LVHT patients has to be addressed by further studies.