Left ventricular noncompaction: A cardiomyopathy often mistaken

Left ventricular noncompaction (LVNC) is a rare genetic cardiomyopathy postulated to be a defect in endomyocardial morphogenesis due to the embryonic arrest of the compaction of myocardial fibers. It could be isolated, without other structural heart defects; or associated with congenital heart defects. It is characterized by prominent ventricular myocardial trabeculations and deep intertrabecular recesses. The clinical manifestations, i.e., heart failure, arrhythmias or thromboembolism, overlap with those of other cardiac disorders. It is often misdiagnosed as restrictive or dilated cardiomyopathy. The high mortality and morbidity associated with it and familial occurrence make diagnosis important. Only 3 pediatric cases have been reported from India. We present 2 cases, that of an 11-year-old girl (familial case) with embolism (documented but rare in children) and atrial flutter (not yet reported), with mother having asymptomatic LVNC; and that of a 4-month-old girl. Both presented with heart failure. The 11-year-old child had sudden death, known to occur in LVNC.

Keywords
Arrhythmias, cardiomyopathy, embolism, heart failure, myocardium, noncompaction