Symptomatic Valvular Myxomatous Transformation (The Floppy Valve Syndrome)

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Abstract
The clinical course of 9 patients aged 14 to 49 who demonstrated at surgery floppy aortic or mitral valves has been described. Microscopic examination revealed myxomatous transformation. Two of the 5 females with mitral regurgi-tation had had endocarditis. One of the 4 men with aortic disease had an aneurysm of the ascending aorta which, however, did not involve the valve ring. These patients were thought originally to have rheumatic heart disease but there was no fibrosis, calcification, or stenosis. One demonstrated arachnodactyly, the others had scattered stigmata of Marfan''s syndrome. Ocular signs were absent, Erdheim''s necrosis of the aortic or arterial media was found in 4 of 8. Prosthetic replacement performed in 8 patients resulted in dehiscence in 3. These studies indicate that idiopathic myxomatous valvular transformation can occur as an isolated cause of valvular insufficiency or as a forme fruste of Marfan''s syndrome.