BACKGROUND AND OBJECTIVES: Left ventricular hypertrabeculation/noncompaction (LVHT) is a cardiac abnormality frequently associated with neuromuscular disorders (NMD). The data about long-term prognosis of patients with LVHT are controversial. Aim of the study in a cohort of LVHT patients was to assess the long-term prognosis regarding mortality, cardiac and neuromuscular comorbidity. METHODS AND RESULTS: In 86 patients LVHT was diagnosed echocardiographically between June 1995 and December 2004 (21 female, mean age 52 +/- 14, range 14-94 years). All patients underwent a baseline cardiologic investigation and were invited for a neurologic investigation. A specific NMD was diagnosed in 21, a NMD of unknown etiology in 33, the neurologic investigation was normal in 13 and 19 patients refused. During a mean follow-up of 51 months (range 3-106 months) the mortality rate was 5.3%/year. Predictors for an increased mortality were increased age (p=0.0134), presence of NMD (p=0.0324), exertional dyspnoea (p=0.0329), edema (p=0.0049), heart failure (p=0.0048), left anterior hemiblock (p=0.0078) and a left ventricular fractional shortening <25% (p=0.0648). CONCLUSION: The mortality of LVHT in adult patients depends on cardiac and neurologic comorbidity. Predictors for mortality are increased age, neuromuscular disorder, heart failure, left ventricular dilatation and systolic dysfunction.
BACKGROUND AND OBJECTIVES:Left ventricular hypertrabeculation/noncompaction (LVHT) is a cardiac abnormality frequently associated with neuromuscular disorders (NMD). The data about long-term prognosis of patients with LVHT are controversial. Aim of the study in a cohort of LVHT patients was to assess the long-term prognosis regarding mortality, cardiac and neuromuscular comorbidity. METHODS AND RESULTS: In 86 patients LVHT was diagnosed echocardiographically between June 1995 and December 2004 (21 female, mean age 52 +/- 14, range 14-94 years). All patients underwent a baseline cardiologic investigation and were invited for a neurologic investigation. A specific NMD was diagnosed in 21, a NMD of unknown etiology in 33, the neurologic investigation was normal in 13 and 19 patients refused. During a mean follow-up of 51 months (range 3-106 months) the mortality rate was 5.3%/year. Predictors for an increased mortality were increased age (p=0.0134), presence of NMD (p=0.0324), exertional dyspnoea (p=0.0329), edema (p=0.0049), heart failure (p=0.0048), left anterior hemiblock (p=0.0078) and a left ventricular fractional shortening <25% (p=0.0648). CONCLUSION: The mortality of LVHT in adult patients depends on cardiac and neurologic comorbidity. Predictors for mortality are increased age, neuromuscular disorder, heart failure, left ventricular dilatation and systolic dysfunction.