Abstract

Abstract Funding Acknowledgements PHRCI mitral 2012 Background Filamin-A mitral valve prolapse/dystrophy (FLNA-MVP) phenotype associates moderate MVP and a paradoxical restricted motion in diastole. Purpose We aimed to assess the association of MVP with restricted motion in diastole in MVP patients (restricted MVP). Methods We prospectively enrolled 433 MVP probands (57 ± 16 years). Patients underwent a clinical examination and a comprehensive echocardiographic analysis of mitral valve apparatus. Results Among the 433 probands, 27 (6.2%, 95% CI 3.9-8.5) had both a MVP and a doming aspect in diastole. Patients with restricted MVP exhibited shorter posterior chordae tendinaes (24.8 ± 6.3 vs 27.2 ± 5.9 mm, P = 0.037), and a shorter distance between papillary muscle (PM) tips and mitral annulus (anterior PM: P = 0.0001; posterior PM: P = 0.009). Anterior mitral valve leaflet was lengthened (15.5 ± 2.4 vs 14.3 ± 2.6 mm/m², P = 0.018), but leaflet thickness, leaflet prolapse, and mitral valve annulus did not differ between the 2 groups. Bicuspid aortic valve was more frequent in patients with restricted phenotype (14.8 vs 2.9%, P < 0.05). Familial recurrence of restricted MVP was identified even in the absence of Filamin-A mutation. Conclusion Restricted MVP is a quite frequent finding in MVP patients and is associated with PM tips location closer to mitral annulus. Restricted MVP can be regarded as a third type of MVP beside myxomatous Barlow disease and fibro-elastic deficiency MVP.

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