title: Echocardiographic versus histologic findings in Marfan syndrome.
abstract: This retrospective study attempted to establish the prevalence of multiple-valve involvement in Marfan syndrome and to compare echocardiographic with histopathologic findings in Marfan patients undergoing valvular or aortic surgery. We reviewed echocardiograms of 73 Marfan patients who underwent cardiovascular surgery from January 2004 through October 2009. Tissue histology was available for comparison in 29 patients. Among the 73 patients, 66 underwent aortic valve replacement or the Bentall procedure. Histologic findings were available in 29 patients, all of whom had myxomatous degeneration. Of 63 patients with moderate or severe aortic regurgitation as determined by echocardiography, 4 had thickened aortic valves. The echocardiographic findings in 18 patients with mitral involvement included mitral prolapse in 15. Of 11 patients with moderate or severe mitral regurgitation as determined by echocardiography, 4 underwent mitral valve repair and 7 mitral valve replacement. Histologic findings among mitral valve replacement patients showed thickened valve tissue and myxomatous degeneration. Tricuspid involvement was seen echocardiographically in 8 patients, all of whom had tricuspid prolapse. Two patients had severe tricuspid regurgitation, and both underwent repair. Both mitral and tricuspid involvement were seen echocardiographically in 7 patients. Among the 73 patients undergoing cardiac surgery for Marfan syndrome, 66 had moderate or severe aortic regurgitation, although their valves manifested few histologic changes. Eighteen patients had mitral involvement (moderate or severe mitral regurgitation, prolapse, or both), and 8 had tricuspid involvement. Mitral valves were most frequently found to have histologic changes, but the tricuspid valve was invariably involved.
authors: Xiaoyan
authors: X
authors: Gu
authors: Yihua
authors: Y
authors: He
authors: Zhian
authors: Z
authors: Li
authors: Jiancheng
authors: J
authors: Han
authors: Jian
authors: J
authors: Chen
authors: J V Ian
authors: JV
authors: Nixon
mesh_terms: D000293:Adolescent; D000328:Adult; D001021:Aortic Valve / Q000000981:diagnostic imaging / Q000473:pathology / Q000601:surgery; D001022:Aortic Valve Insufficiency / Q000175:diagnosis* / Q000000981:diagnostic imaging / Q000209:etiology / Q000473:pathology / Q000601:surgery; D005260:Female; D019918:Heart Valve Prosthesis Implantation; D006801:Humans; D008297:Male; D008382:Marfan Syndrome / Q000150:complications* / Q000175:diagnosis; D008875:Middle Aged; D008943:Mitral Valve / Q000000981:diagnostic imaging / Q000473:pathology / Q000601:surgery; D008944:Mitral Valve Insufficiency / Q000175:diagnosis* / Q000000981:diagnostic imaging / Q000209:etiology / Q000473:pathology / Q000601:surgery; D008945:Mitral Valve Prolapse / Q000175:diagnosis* / Q000000981:diagnostic imaging / Q000209:etiology / Q000473:pathology / Q000601:surgery; D012189:Retrospective Studies; D012720:Severity of Illness Index; D014261:Tricuspid Valve / Q000000981:diagnostic imaging / Q000473:pathology / Q000601:surgery; D014262:Tricuspid Valve Insufficiency / Q000175:diagnosis* / Q000000981:diagnostic imaging / Q000209:etiology / Q000473:pathology / Q000601:surgery; D014463:Ultrasonography; D055815:Young Adult
keywords: Aortic valve regurgitation; Marfan syndrome/complications/pathology/ultrasonography; cardiovascular abnormalities/epidemiology; echocardiography; mitral valve prolapse; pulmonary valve regurgitation; tricuspid valve regurgitation