The prognosis of patients with left ventricular hypertrabeculation/noncompaction (LVHT) is controversial. We assessed cardiologic and neurologic predictors for mortality in LVHT patients and how many received implantable cardioverters/defibrillators(ICD) or cardiac resynchronization devices (CRT).Included were patients with LVHT diagnosed echocardiographically between June 1995 and May 2009. All patients underwent a baseline cardiologic examination, and were invited for a neurological investigation. During June 2009, the patients were contacted by telephone and their records were screened if they had received ICD or CRT.In 140 patients (29% females, mean age 53±16, range 14–94years) LVHT was diagnosed. The neurologic investigation, carried out in 76%, disclosed a neuromuscular disorder of definite (n=22) or unknown (n=68) etiology or was normal (n=16). During a follow-up of 4.5years the mortality was 5.7%/year. Causes of death were heart failure(n=11), pneumonia (n=6), sudden cardiac death (n=3), malignancy (n=3), pulmonary embolism(n=2), sepsis (n=2), stroke (n=2), hepatic failure (n=1) or unknown (n=6). Sixteen patients received devices (ICD n=4, CRT n=3, ICD plus CRT n=9). Predictors for mortality were increased age (p=0.0307), neuromuscular disorder of definite or unknown etiology (p=0.0063), exertional dyspnea (p=0.0018), edema (p=0.0000), heart failure (p=0.0002), ventricular ectopic beats (p=0.0119), atrial fibrillation (p=0.0000), low voltage (p=0.0139), presence of one or more ECG abnormalities (p=0.0420), left ventricular fractional shortening <25% (p=0.0046), extension of LVHT (p=0.0063) and LVHT affecting the lateral wall (p=0.0110).Mortality in LVHT is high and due to cardiac and neuromuscular comorbidity, why monitoring and therapy, including device therapy, should be improved.