title: Replacement of Regurgitant Bicuspid Aortic Valve in a Dilated, Non-Compacted Left Ventricle.
abstract: Bicuspid aortic valve (BAV) is the most common form of congenital heart disease, with 20% of asymptomatic adults with BAV presenting with significant valve insufficiency. Yet, limited data exist regarding surgical indications and outcomes when BAV is accompanied by left ventricular dilation, systolic dysfunction, or left ventricle non-compaction (LVNC) syndrome. We present a case of dilated cardiomyopathy due to severe BAV regurgitation and partial LVNC syndrome and the decision to undergo aortic valve replacement. Our patient represents the most extreme documented case of regurgitant BAV with dilated, dysfunctional, and partially non-compacted left ventricle. Yet, surgical intervention provided improvement in systolic performance and ventricular dimensions.
authors.affiliation: Internal Medicine Department, Medicine Institute at Cleveland Clinic, Cleveland, OH, USA schenoa@ccf.org.
authors.forename: Aldo L
authors.initials: AL
authors.lastname: Schenone
authors.affiliation: Internal Medicine Department, Medicine Institute at Cleveland Clinic, Cleveland, OH, USA.
authors.forename: Aaron
authors.initials: A
authors.lastname: Cohen
authors.affiliation: Cardiothoracic Surgery Department, Heart and Vascular Institute at Cleveland Clinic, Cleveland, OH, USA.
authors.forename: Gosta
authors.initials: G
authors.lastname: Pettersson
authors.affiliation: Cardiology Department, Heart and Vascular Institute at Cleveland Clinic, Cleveland, OH, USA.
authors.forename: David
authors.initials: D
authors.lastname: Majdalany
mesh_terms: D000328:Adult; D001021:Aortic Valve / Q000002:abnormalities* / Q000000981:diagnostic imaging / Q000503:physiopathology / Q000601:surgery; D001022:Aortic Valve Insufficiency / Q000000981:diagnostic imaging* / Q000503:physiopathology / Q000601:surgery; D000082882:Bicuspid Aortic Valve Disease; D006349:Heart Valve Diseases / Q000000981:diagnostic imaging* / Q000503:physiopathology / Q000601:surgery; D006350:Heart Valve Prosthesis; D019918:Heart Valve Prosthesis Implantation; D006352:Heart Ventricles / Q000000981:diagnostic imaging / Q000503:physiopathology / Q000601:surgery; D006801:Humans; D008297:Male; D013599:Systole; D016896:Treatment Outcome; D016277:Ventricular Function, Left
keywords: bicuspid aortic valve; non-compaction left ventricle; surgical outcome; systolic dysfunction