Left Ventricular Noncompaction Cardiomyopathy in Pediatric Patients: A Case Series of a Clinically Heterogeneous Disease

Abstract

Left ventricular noncompaction is a rare form of cardiomyopathy, which results from multiple trabeculations in the left ventricular myocardium. The clinical presentation is highly variable, and spectrum includes asymptomatic patients diagnosed during family screening on one end to patients with depressed systolic function, heart failure, thromboembolic complications, and cardiac arrhythmias on the other (Kim et al in J Am Coll Cardiol 53: 2009, 2009). Further, the progression of the condition is highly variable. Hence, these patients require close follow-up, and management for each patient needs to be individualized and periodically reevaluated. Here, we present a series of five cases that have been followed in our practice and present our experience. A literature review of this rare form of congenital cardiomyopathy is also presented.