title: Left ventricular noncompaction.
abstract: Left Ventricular Noncompaction (LVNC) is a genetic cardiac disease of emerging importance with a distinct clinical and pathophysiological presentation. The most common clinical manifestations include heart failure, arrhythmias and embolic events, and in children it may be associated with facial dysmorphisms and Wolff-Parkinson-White syndrome. The diagnosis of LVNC, however, is often missed, most often as a consequence of ignorance of the condition. Echocardiography is considered the reference standard for the diagnosis of LVNC. Prognosis remains poor for patients with impaired systolic left ventricular function, as treatment options are very limited. Because of the familial association of LVNC, first-degree relatives should be screened by Echocardiography.
authors.affiliation: Department of Cardiology, Shahid Gangalal National Heart Center, Bansbari, Kathmandu, Nepal.
authors.forename: R
authors.initials: R
authors.lastname: Malla
authors.forename: R
authors.initials: R
authors.lastname: Sharma
authors.forename: B
authors.initials: B
authors.lastname: Rauniyar
authors.forename: M B
authors.initials: MB
authors.lastname: Kc
authors.forename: A
authors.initials: A
authors.lastname: Maskey
authors.forename: D
authors.initials: D
authors.lastname: Joshi
authors.forename: S
authors.initials: S
authors.lastname: Hamal
mesh_terms: D003937:Diagnosis, Differential; D003935:Diagnostic Techniques, Cardiovascular; D020022:Genetic Predisposition to Disease; D006352:Heart Ventricles / Q000002:abnormalities / Q000000981:diagnostic imaging / Q000503:physiopathology; D006801:Humans; D056830:Isolated Noncompaction of the Ventricular Myocardium / Q000145:classification / Q000175:diagnosis / Q000235:genetics; D011379:Prognosis; D014463:Ultrasonography