Left ventricular noncompaction in children.

OBJECTIVE

Left ventricular noncompaction (LVNC) is an uncommon type of cardiomyopathy in children. We sought to determine the clinical presentations and outcomes of children diagnosed to have LVNC.

DESIGN

The case records of children diagnosed to have LVNC between 1999 and 2007 were reviewed. The diagnosis was based on echocardiographic finding of a thick noncompacted myocardium layer characterized by a trabecular meshwork with deep endomyocardial spaces.

RESULTS

Ten patients (seven males) were diagnosed to have LVNC at a median age of 2 years (range, 7 days to 12 years). Seven patients had isolated LVNC while three had associated structural congenital heart diseases. The right ventricle was also involved in two patients. Clinical presentations included congestive heart failure in eight patients, asymptomatic heart murmur in one, and syncope with exercise intolerance in one. At presentation, cardiomegaly was found in nine patients, electrocardiographic abnormalities in nine, and impaired LV contraction in six. Eight patients received anti-heart failure medications. Three patients died at a median of 1.5 years after diagnosis, two died suddenly of unknown causes, and one of heart failure. The seven surviving patients were followed up for a median of 2 years (range, 2 months to 3 years). Three patients developed cardiac arrhythmias. The LV function improved in three patients and worsened in one on follow-up. None of the patients developed thromboembolic complications.

CONCLUSIONS

Left ventricular noncompaction in children is a heterogeneous condition. Long-term follow-up for development of progressive LV dysfunction and cardiac arrhythmias is indicated.