Floppy Mitral Valve in Children with Marfan Syndrome

Marfan syndrome and mitral valve prolapse.

Mitral valve prolapse (MVP), an abnormal displacement into the left atrium of a thickened and redundant mitral valve during systole, is a relatively frequent abnormality in humans and may be associated with serious complications. A recent study implicates fibrillin-1, a component of extracellular matrix microfibrils, in the pathogenesis of a murine model of MVP. This investigation represents an...

Floppy Mitral Valve, Mitral Valve Prolapse, and Mitral Valvular Regurgitation.

It is well recognized that the floppy mitral valve (FMV) complex is the central issue in the FMV, mitral valve prolapse (MVP), and mitral valvular regurgitation (MVR) story. MVP associated with the FMV results from the systolic movement of portions or segments of the FMV complex into the left atrium (LA). Prolapse of the FMV results in unique forms of mitral valvular dysfunction and MVR. When t...

Bacterial endocarditis of mitral valve in Marfan syndrome.

An unusual case of bacterial endocarditis in Marfan syndrome is reported. A review of the published reports revealed 21 previously reported cases. The aortic valve, though commonly abnormal in Marfan syndrome, was rarely involved by the endocarditis. In contrast, the mitral valve was the favoured site of infection in these patients. Our own patient had staphylococcal endocarditis of both the ao...

The floppy mitral valve. Study on pathogenesis.

The pathogenesis of the floppy valve syndrome is not fully solved. An almost invariable histological finding is the great accumulation of mucinous material in the valve leaflets and constitutes the basis of the valvular theory of the syndrome. The presence of a mucinous layer in normal valves-- the zona spongiosa--is not well recognised. To establish the normal range of the extent of this zone,...

[Mitral insufficiency in Marfan syndrome].