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      Morphofunctional Abnormalities of Mitral Annulus and Arrhythmic Mitral Valve Prolapse

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          Abstract

          Supplemental Digital Content is available in the text.

          Abstract

          Background—

          Arrhythmic mitral valve prolapse (MVP) is characterized by myxomatous leaflets and left ventricular (LV) fibrosis of papillary muscles and inferobasal wall. We searched for morphofunctional abnormalities of the mitral valve that could explain a regional mechanical myocardial stretch.

          Methods and Results—

          Thirty-six (27 female patients; median age: 44 years) arrhythmic MVP patients with LV late gadolinium enhancement on cardiac magnetic resonance and no or trivial mitral regurgitation, and 16 (6 female patients; median age: 40 years) MVP patients without LV late gadolinium enhancement were investigated by morphofunctional cardiac magnetic resonance. Mitral annulus disjunction (median: 4.8 versus 1.8 mm; P<0.001), end-systolic mitral annular diameters (median: 41.2 versus 31.5; P=0.004) and end-diastolic mitral annular diameters (median: 35.5 versus 31.5; P=0.042), prevalence of posterior systolic curling (34 [94%] versus 3 [19%]; P<0.001), and basal to mid LV wall thickness ratio >1.5 (22 [61%] versus 4 [25%]; P=0.016) were higher in MVP patients with late gadolinium enhancement than in those without. A linear correlation was found between mitral annulus disjunction and curling ( R=0.85). A higher prevalence of auscultatory midsystolic click (26 [72%] versus 6 [38%]; P=0.018) was also noted. Histology of the mitral annulus showed a longer mitral annulus disjunction in 50 sudden death patients with MVP and LV fibrosis than in 20 patients without MVP (median: 3 versus 1.5 mm; P<0.001).

          Conclusions—

          Mitral annulus disjunction is a constant feature of arrhythmic MVP with LV fibrosis. The excessive mobility of the leaflets caused by posterior systolic curling accounts for a mechanical stretch of the inferobasal wall and papillary muscles, eventually leading to myocardial hypertrophy and scarring. These mitral annulus abnormalities, together with auscultatory midsystolic click, may identify MVP patients who would need arrhythmic risk stratification.

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          Most cited references41

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          A concordance correlation coefficient to evaluate reproducibility.

          L Lin (1989)
          A new reproducibility index is developed and studied. This index is the correlation between the two readings that fall on the 45 degree line through the origin. It is simple to use and possesses desirable properties. The statistical properties of this estimate can be satisfactorily evaluated using an inverse hyperbolic tangent transformation. A Monte Carlo experiment with 5,000 runs was performed to confirm the estimate's validity. An application using actual data is given.
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            Prevalence and clinical outcome of mitral-valve prolapse.

            Mitral-valve prolapse has been described as a common disease with frequent complications. To determine the prevalence of mitral-valve prolapse in the general population, as diagnosed with the use of current two-dimensional echocardiographic criteria, we examined the echocardiograms of 1845 women and 1646 men (mean [+/-SD] age, 54.7+/-10.0 years) who participated in the fifth examination of the offspring cohort of the Framingham Heart Study. Classic mitral-valve prolapse was defined as superior displacement of the mitral leaflets of more than 2 mm during systole and as a maximal leaflet thickness of at least 5 mm during diastasis, and nonclassic prolapse was defined as displacement of more than 2 mm, with a maximal thickness of less than 5 mm. A total of 84 subjects (2.4 percent) had mitral-valve prolapse: 47 (1.3 percent) had classic prolapse, and 37 (1.1 percent) had nonclassic prolapse. Their age and sex distributions were similar to those of the subjects without prolapse. None of the subjects with prolapse had a history of heart failure, one (1.2 percent) had atrial fibrillation, one (1.2 percent) had cerebrovascular disease, and three (3.6 percent) had syncope, as compared with unadjusted prevalences of these findings in the subjects without prolapse of 0.7, 1.7, 1.5, and 3.0 percent, respectively. The frequencies of chest pain, dyspnea, and electrocardiographic abnormalities were similar among subjects with prolapse and those without prolapse. The subjects with prolapse were leaner (P<0.001) and had a greater degree of mitral regurgitation than those without prolapse, but on average the regurgitation was classified as trace or mild. In a community based sample of the population, the prevalence of mitral-valve prolapse was lower than previously reported. The prevalence of adverse sequelae commonly associated with mitral-valve prolapse in studies of patients referred for that diagnosis was also low.
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              Arrhythmic Mitral Valve Prolapse and Sudden Cardiac Death.

              Mitral valve prolapse (MVP) may present with ventricular arrhythmias and sudden cardiac death (SCD) even in the absence of hemodynamic impairment. The structural basis of ventricular electric instability remains elusive.
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                Author and article information

                Journal
                Circ Cardiovasc Imaging
                Circ Cardiovasc Imaging
                HCI
                Circulation. Cardiovascular Imaging
                Lippincott Williams & Wilkins
                1941-9651
                1942-0080
                August 2016
                16 August 2016
                : 9
                : 8
                : e005030
                Affiliations
                From the Department of Cardiac, Thoracic, and Vascular Sciences (M.P.M., C.B., M.D.L., S.R., A.C., I.R., F.M., K.P., L.C., E.B., A.C.F., B.B., D.C., G.T., S.I.), Department of Medicine (B.G.), and Department of Radiology (C.L.), Azienda Ospedaliera-University of Padua Medical School, Italy.
                Author notes
                Correspondence to Martina Perazzolo Marra, MD, PhD, Department of Cardiac, Thoracic and Vascular Sciences, Via N. Giustiniani, 2 35100, Padova, Italy. E-mail martina.perazzolomarra@ 123456unipd.it
                Article
                00019
                10.1161/CIRCIMAGING.116.005030
                4991345
                27516479
                c2420a07-6566-4569-94cf-54001cbc619b
                © 2016 The Authors.

                Circ Cardiovasc Imaging is published on behalf of the American Heart Association, Inc., by Wolters Kluwer. This is an open access article under the terms of the Creative Commons Attribution Non-Commercial-NoDervis License, which permits use, distribution, and reproduction in any medium, provided that the original work is properly cited, the use is noncommercial, and no modifications or adaptations are made.

                History
                : 20 January 2016
                : 24 June 2016
                Categories
                10129
                Original Articles
                Valvular Heart Disease
                Custom metadata
                TRUE

                gadolinium,mitral valve,mitral valve annulus,mitral valve prolapse,papillary muscles

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