title: Predictors of outcome of mitral valve prolapse in patients with the Marfan syndrome.
abstract: Mitral valve prolapse has a prevalence of 2% to 3% in the general population, with adverse outcomes such as mitral valve regurgitation (MVR), heart failure, and endocarditis. Predictors of outcomes are used in idiopathic mitral valve prolapse for the timing of surgery, but such predictors are unknown in Marfan syndrome. Therefore, a population-based cohort study of 112 patients (49 male, 63 female; mean age 34 ± 15 years) with classic Marfan syndrome and mitral valve prolapse with moderate or less MVR at baseline was conducted. During 4.6 ± 3.6 years of follow-up, progression of MVR was observed in 41 patients and valve-related events, which comprised mitral valve endocarditis (7 events), heart failure (5 events), and mitral valve surgery (25 events), were observed in 31 patients. Multivariate Cox proportional-hazards regression analysis identified a flail mitral leaflet (hazard ratio [HR] 3.262, 95% confidence interval [CI] 1.406 to 7.566, p = 0.006) and increased indexed end-systolic left ventricular diameters (HR 1.113, 95% CI 1.043 to 1.188, p = 0.001) as independent predictors of progression of MVR. Similarly, mitral valve-related events were independently predicted by a flail mitral leaflet (HR 5.343, 95% CI 2.229 to 12.808, p <0.001), and mild (HR 14.336, 95% CI 1.873 to 109.755, p = 0.01) or moderate (HR 16.849, 95% CI 2.205 to 128.76, p = 0.006) degree of MVR. Conversely, aortic dilatation, dural ectasia, and sporadic mode of inheritance were not associated with outcome. In conclusion, the same clinical determinants that predict outcomes in idiopathic mitral valve prolapse also predict outcomes in mitral valve prolapse associated with Marfan syndrome.
authors.affiliation: Centre of Cardiology and Cardiovascular Surgery, University Hospital Eppendorf, Hamburg, Germany.
authors.forename: Meike
authors.initials: M
authors.lastname: Rybczynski
authors.forename: Hendrik
authors.initials: H
authors.lastname: Treede
authors.forename: Sara
authors.initials: S
authors.lastname: Sheikhzadeh
authors.forename: Eike F
authors.initials: EF
authors.lastname: Groene
authors.forename: Alexander M J
authors.initials: AM
authors.lastname: Bernhardt
authors.forename: Mathias
authors.initials: M
authors.lastname: Hillebrand
authors.forename: Thomas S
authors.initials: TS
authors.lastname: Mir
authors.forename: Kristine
authors.initials: K
authors.lastname: Kühne
authors.forename: Dietmar
authors.initials: D
authors.lastname: Koschyk
authors.forename: Peter N
authors.initials: PN
authors.lastname: Robinson
authors.forename: Jürgen
authors.initials: J
authors.lastname: Berger
authors.forename: Hermann
authors.initials: H
authors.lastname: Reichenspurner
authors.forename: Thomas
authors.initials: T
authors.lastname: Meinertz
authors.forename: Yskert
authors.initials: Y
authors.lastname: von Kodolitsch
mesh_terms: D000328:Adult; D018450:Disease Progression; D004452:Echocardiography; D005260:Female; D005500:Follow-Up Studies; D006352:Heart Ventricles / Q000000981:diagnostic imaging / Q000503:physiopathology; D006801:Humans; D008297:Male; D008382:Marfan Syndrome / Q000150:complications*; D008945:Mitral Valve Prolapse / Q000175:diagnosis* / Q000503:physiopathology; D011379:Prognosis; D012189:Retrospective Studies; D013318:Stroke Volume; D013997:Time Factors; D016277:Ventricular Function, Left