Neuromuscular comorbidity, heart failure, and atrial fibrillation as prognostic factors in left ventricular hypertrabeculation/noncompaction.

BACKGROUND: There is some controversy concerning the prognosis of patients with left ventricular hypertrabeculation/noncompaction (LVHT). LVHT is frequently associated with neuromuscular disorders (NMDs). The aim of this study was to assess cardiac and neurological findings as predictors of mortality in patients with LVHT. PATIENTS AND METHODS: The study included patients with LVHT diagnosed between June 1995 and January 2014 in one echocardiographic laboratory. They underwent a baseline cardiologic examination and were invited for a neurological examination. Between January and February 2014, their survival status was assessed. RESULTS: LVHT was diagnosed in 220 patients (68 female, aged 52 ± 17 years) with a prevalence of 0.35 %/year. During a follow-up of 72 ± 61 months, 65 patients died. The mortality was 5 %/year. A neurological investigation was performed on 173 patients (79 %) and revealed specific NMDs in 31 (14 %), NMD of unknown etiology in 103 (47 %), and normal findings in 39 (18 %) patients. In multivariate analysis, the predictors of mortality were increased age (p = 0.0001), presence of a specific NMD (p = 0.0062) or NMD of unknown etiology (p = 0.0062), heart failure NYHA III (p = 0.0396), atrial fibrillation (p = 0.0022), and sinus tachycardia (p = 0.0395). CONCLUSIONS: LVHT patients should undergo systematic neurological examinations. Whether an optimal therapy of heart failure and atrial fibrillation will improve the prognosis of LVHT patients needs to be addressed in further studies.

Age-dependency of cardiac and neuromuscular findings in adults with left ventricular hypertrabeculation/noncompaction.

Left ventricular hypertrabeculation/noncompaction (LVHT) is diagnosed in all ages and is frequently associated with neuromuscular disorders (NMDs). The aim of the study was to compare patients with LVHT depending on age at diagnosis. Included were 232 patients with LVHT (72 women, mean age 52±17 years) diagnosed from 1995 to 2014 at 1 echocardiographic laboratory. In 2014, their survival was assessed. Seventy-six percent of the patients were neurologically investigated, revealing specific NMDs in 18%, unspecific NMDs in 60%, and normal findings in 22%. Forty-five patients (19%) received electronic devices: implantable cardioverter-defibrillators in 26 patients, combined with cardiac resynchronization systems (n=14) or an antibradycardic pacemaker (n=1); antibradycardic pacemakers (n=8); cardiac resynchronization systems (n=4); implantable loop recorders (n=4); life vests (n=2); and a left ventricular assist device as a bridge to transplantation (n=1). During 72-month follow-up, mortality was 4.9% per year. In younger age groups, more patients were referred for syncope or palpitations, whereas in older age groups, more patients were referred for heart failure. Classic cardiovascular risk factors such as hypertension and diabetes, as well as coronary artery stenosis, were rare in the young age groups but were more prevalent in older age groups. Differences between age groups were found regarding cardiac symptoms, NMDs, electrocardiographic findings, rate of device implantation, and mortality but not in location and extension of LVHT. None of the neurologically investigated patients≥70 years of age was neurologically normal. Prevalence of heart failure, electrocardiographic abnormalities, and mortality were highest in the oldest age group. In conclusion, LVHT must be considered as an echocardiographic diagnosis in all age groups. The morphologic pattern of LVHT is similar, whereas clinical manifestations and prognosis are variable among age groups.