In- and outpatients with noncompaction: differences in cardiac and neuromuscular co-morbidity

The prognosis of patients with left ventricular hypertrabeculation/noncompaction (LVHT) is controversial. LVHT is associated with neuromuscular disorders (NMD) and diagnosed echocardiographically in in- as well as outpatients. We compared cardiologic and neurologic findings and mortality in LVHT-patients according to their diagnosis established as in- or outpatients.

Among 113 patients (33 females, mean-age 53 years), 91 were investigated neurologically. Fifty-nine inpatients were older (55 versus 50 years, p<0.05), more frequently referred because of heart failure (73 versus 37%, p<0.001), had more often diabetes (24 versus 7%, p<0.05), heart failure (81 versus 57%, p<0.01), a lower left-ventricular fractional-shortening (21 versus 26%, p<0.05) and more extensive LVHT (1.7 versus 1.5 affected walls, p<0.05). Fifty-four outpatients were referred more often because of chest-pain (33 versus 12%, p<0.01), myopathy (13 versus 2%, p<0.05), were more often neurologically normal (20 versus 7%, p<0.05) or had a specific NMD (28 versus 12%, p<0.05). During a mean follow-up of 3.8 years, mortality was 5.8%/year. Inpatients had a higher mortality (12.1 versus 2.1%/year, p=0.0002) and a shorter time between LVHT-diagnosis and death (1.7 versus 4.6 years, p=0.0197) than outpatients.

Outpatients with LVHT have a better prognosis than inpatients. Inpatients with LVHT should be closely monitored.