Age-dependency of cardiac and neuromuscular findings in left ventricular noncompaction

Left ventricular hypertrabeculation/noncompaction (LVHT) is a cardiac abnormality characterized by prominent trabeculations and intertrabecular recesses, and frequently associated with neuromuscular disorders (NMD). Initially described in children and young adults, LVHT has been found also in elderly. Aim of the study was to assess the age-dependency of clinical, electrocardiographic (ECG) and echocardiographic findings, and whether they differ according to the neurologic diagnosis.

In 86 patients LVHT was diagnosed echocardiographically between June 1995 and December 2004 (65 male, median age 52 years, range 14-94). All patients underwent a baseline cardiologic investigation and were invited for a neurologic investigation. A specific NMD was diagnosed in 21 (metabolic myopathy, n = 14; Leber's hereditary optic neuropathy, n = 3; myotonic dystrophy, n = 2; Becker muscular dystrophy, n = 1; Duchenne muscular dystrophy, n = 1), a NMD of unknown etiology in 32. The neurologic investigation was normal in 13 and was refused by 20 patients. Patients above median age had more often heart failure (43% versus 95%, p < 0.02) and valvular abnormalities (71% versus 36%, p < 0.02) than below median age. Patients >71 years had more often exertional dyspnoea (39% versus 81%, p < 0.02), left bundle branch block (9% versus 38%, p = 0.031), larger left-ventricular enddiastolic diameters (68 versus 60 mm, p < 0.02) and a lower left-ventricular fractional shortening than patients <34 years (18% versus 29%, p < 0.02). No age-dependency was detected regarding location and extension of LVHT and the neurologic diagnosis.

There is no age-dependent typical pattern of LVHT regarding clinical, echocardiographic and neurologic findings. Echocardiographers should be aware of this cardiac abnormality when investigating patients of any age.