Clinical Presentation of Left Ventricular Noncompaction in Children

Left ventricular noncompaction (LVNC) is a heterogeneous form of cardiomyopathy associated with abnormal myocardial maturation and characteristic prominent trabeculation of the left ventricular wall. The classification of the disease remains controversial, and some authors consider LVNC to represent a phenotypic manifestation of other cardiomyopathies. The clinical presentation, particularly in children, is highly variable, ranging from asymptomatic forms to heart failure, arrhythmias, and thromboembolic events. Echocardiography remains the cornerstone of diagnosis, complemented by cardiac magnetic resonance imaging; however, the lack of universally accepted diagnostic criteria contributes to the risk of overdiagnosis. Treatment is primarily symptomatic and depends on the disease phenotype and the degree of left ventricular dysfunction. Prognosis is determined mainly by cardiac function and the presence of arrhythmias, whereas isolated LVNC with preserved systolic function is generally associated with a favorable clinical course.