Neuromuscular and cardiac comorbidity determines survival in 140 patients with left ventricular hypertrabeculation/noncompaction

BackgroundThe 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).Methods and resultsIncluded 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 14–94 years) 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 4.5 years 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).ConclusionMortality in LVHT is high and due to cardiac and neuromuscular comorbidity, why monitoring and therapy, including device therapy, should be improved.