Abstract

Floppy mitral valve/mitral valve prolapse (FMV/MVP) is a heritable connective tissue disorder with a prevalence of 2–3% in the general population. FMV/MVP is often associated with structural abnormalities of other organs; although involvement of the aorta has been suggested, there is a lack of

Highlights

  • Floppy mitral valve/mitral valve prolapse (FMV/MVP) is considered to be a heritable disorder of connective tissue and has a prevalence of 2 to 3% in the general population.[1,2,3] FMV/MVP is most commonly transmitted by an autosomal dominant inheritance with a variable degree of penetration.[1,4,5,6] The term FMV comes from surgical and pathologic studies, and refers to the expansion of the area of the mitral valve leaflets with elongated chordae tendineae and frequently dilated mitral annulus

  • Abnormal aortic function, which deteriorates with age, may play an important role in the natural history of mitral regurgitation (MR) due to FMV/MVP

  • The present study demonstrated that aortic distensibility in the ascending aorta is decreased in patients with FMV/MVP and significant MR

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Summary

Introduction

Floppy mitral valve/mitral valve prolapse (FMV/MVP) is considered to be a heritable disorder of connective tissue and has a prevalence of 2 to 3% in the general population.[1,2,3] FMV/MVP is most commonly transmitted by an autosomal dominant inheritance with a variable degree of penetration.[1,4,5,6] The term FMV comes from surgical and pathologic studies, and refers to the expansion of the area of the mitral valve leaflets with elongated chordae tendineae and frequently dilated mitral annulus. Connective tissue disorders may affect aortic function, and a stiff aorta may increase the severity of MR. FMV/MVP patients had greater left ventricular (LV) endsystolic (88 Æ 72 mL versus 35 Æ 15 mL, p 1⁄4 0.002) and end-diastolic (165 Æ 89 mL versus 100 Æ 41 mL, p 1⁄4 0.005) volumes, and lower LV ejection fraction, compared with control (50 Æ 12% versus 57 Æ 6%, p 1⁄4 0.034). Conclusion Aortic distensibility is decreased (consistent with a stiff aorta) in patients with FMV/MVP and MR. Abnormal aortic function, which deteriorates with age, may play an important role in the natural history of MR due to FMV/MVP

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