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

Background: 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). Methods and results: 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 14-94 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). Conclusion: Mortality in LVHT is high and due to cardiac and neuromuscular comorbidity, why monitoring and therapy, including device therapy, should be improved. (C) 2010 Elsevier Ireland Ltd. All rights reserved.