Mitral regurgitation in children is most commonly associated with chronic rheumatic heart disease, and a number of congenital cardiac lesions may produce a similar lesion. Not uncommonly, a diagnostic problem arises in the older child with classical findings of mitral regurgitation without a history of rheumatic fever. An unusual cause of acquired mitral insufficiency to be considered is myxomatous transformation of the mitral valve.
Myxomatous transformation is pathologically characterized by disruption and loss of normal valvular architecture accompanied by an increase in ground substance with no appreciable inflammatory reaction. The gross appearance of the affected valve at open heart surgery presents a characteristic appearance of lifeless leaflets which hang loosely in the ventricular cavity, hence, the name floppy valve, as coined by Read and Thal.1 Recent literature associates this lesion with Marfan's syndrome and its forme fruste, particularly where Erdheim's necrosis is found in the aorta or arterial media.
Sherman EB, Char F, Dungan WT, Campbell GS. Myxomatous Transformation of the Mitral Valve Producing Mitral Insufficiency: Floppy Valve Syndrome. Am J Dis Child. 1970;119(2):171–175. doi:10.1001/archpedi.1970.02100050173019
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