title: 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.
authors.affiliation: Division of Pediatric Cardiology, Department of Pediatrics, University at Buffalo, Women and Children's Hospital of Buffalo, 239 Bryant Street, Buffalo, NY, 14203, USA. umanggupta@yahoo.com.
authors.forename: Umang
authors.identifier: http://orcid.org/0000-0002-1492-2853
authors.initials: U
authors.lastname: Gupta
authors.affiliation: Division of Pediatric Cardiology, Department of Pediatrics, University at Buffalo, Women and Children's Hospital of Buffalo, 239 Bryant Street, Buffalo, NY, 14203, USA.
authors.forename: Pooja
authors.initials: P
authors.lastname: Makhija
mesh_terms: D000293:Adolescent; D002675:Child, Preschool; D004452:Echocardiography; D005260:Female; D006330:Heart Defects, Congenital / Q000175:diagnosis* / Q000000981:diagnostic imaging / Q000628:therapy; D006352:Heart Ventricles / Q000000981:diagnostic imaging*; D006801:Humans; D007223:Infant; D008297:Male
keywords: Cardiomyopathy; Coronary artery fistula; Left ventricular noncompaction; Pediatric patients