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

Abstract

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.