[The prevalence and clinical features of pathologically abnormal mitral valve leaflets (myxomatous mitral valve) in the mitral valve prolapse syndrome: an echocardiographic and pathological comparative study].

Abstract

We studied the prevalence and clinical features of pathologically abnormal mitral valve leaflets (myxomatous mitral valve: MMV) in consecutive 142 patients with the mitral valve prolapse syndrome (MVP). Our echocardiographic criteria for MMV were 1) thick leaflets 3 mm or greater, 2) redundant leaflet-motion, and 3) echo-density lower than that of the aortic walls. The echocardiographic measurements of left ventricular diastolic dimensions (LVDd), percent fractional shortening (%FS), mitral annular diameter (MAD), and LV mass were compared between MMV and non-MMV groups. Twelve patients (8%) were referred for surgery because of congestive heart failure, and two patients died during the observation periods. Gross morphology of the MMV was characterized by increased surface area, dome formation of the leaflet-body, and non-uniform leaflets in thickness, and histologic findings of the MMV were the infiltration of spongiosa layer into the fibrosa layer. The diagnostic accuracy of echocardiography for the MMV was examined in 14 patients underwent either surgery or autopsy, and it was high (78% in sensitivity and 80% in specificity). The progression of mitral regurgitation (MR) from mild to moderate grade or mild to severe grade was found in five of 26 patients during follow-up studies over 12 months (mean = 36 months). All of the five patients were aged 50 years and older. While, MR completely disappeared in a 17-year-old boy with marked physical development within three years of the observation period. Mitral annular diameter significantly increased in MMV with MR when compared to non-MMV with MR (4.1 +/- 0.7 vs 3.5 +/- 0.4). But no significant changes were noted in LVDd and LV mass between non-MMV with MR and MMV with MR. Of the 142 patients with MVP, 96 patients were non-MMV and 46 patients were MMV. Ruptured chordae tendineae were associated in 5/96 patients (5%) with non-MMV and 22/46 patients (48%) with MMV. Intracardiac vegetations were seen in four of the 96 patients (4%) with non-MMV. The prevalence of MMV in MVP was greater in older patients, and it reached nearly as high as 50% of MVP patients aged 60 years and older. In conclusion, the echocardiographic diagnostic criteria for MMV are reliable with high sensitivity and specificity, and are useful to predict the high risk patients in the MVP syndrome. MMV may be a potential etiology causing aggravation of mitral regurgitation and/or ruptured chordae tendineae.