Neurological comorbidity affects prognosis in left ventricular hypertrabeculation/noncompaction

Left ventricular hypertrabeculation/noncompaction (LVHT) is a cardiac abnormality frequently associated with neuromuscular disorders (NMDs). Whether differences exist between LVHT patients with and without NMDs remains unknown. This study compared the baseline characteristics and prognoses of LVHT patients with and without NMDs.

This prospective, observational study included patients in whom LVHT was diagnosed at one echocardiographic laboratory between June 1995 and June 2011. Patients underwent a baseline cardiologic examination, and were invited to participate in a neurological investigation. In June 2011, patients were contacted by telephone.

One hundred and seventy-two patients received a diagnosis of LVHT (53 female; mean age, 53 standard deviation [SD] ± 16 years). One hundred and twenty-three patients (72%) were investigated neurologically. A specific NMD was diagnosed in 25, including metabolic myopathy (n = 16), Leber's hereditary optic neuropathy (n = 3), myotonic dystrophy (n = 3), Becker muscular dystrophy (n = 1), postpoliomyelitis syndrome (n = 1), and Duchenne muscular dystrophy (n = 1). An NMD of unknown etiology was diagnosed in 79 patients, and the neurological investigation produced normal results in 19 patients. During a follow-up of 64 months, the mortality amounted to 4.84%/year. Baseline data did not differ between patients with and without NMDs. No deaths occurred among patients without NMDs, even though their observation periods were much longer (8 vs. 4 years, P = .01) than those of patients who were not investigated neurologically.

The presence or absence of NMDs influences the prognosis of patients with LVHT. Patients should be investigated by a neurologist when LVHT is diagnosed. Patients with LVHT and an NMD should be educated about the symptoms of arrhythmias and heart failure. They should be closely followed, and the implantation of cardiac electronic devices should be considered.