Mitral insufficiency in an incomplete form of Marfan syndrome corrected with valve replacement

A 14-year-old boy with an incomplete form of the Marfan syndrome developed an acute episode of severe congestive heart failure following exertion. The preoperative studies indicated a mitral insufficiency, and a floppy, thickened mitral valve was removed and replaced with a Starr-Edwards prosthesis. Microscopically, the valve showed increase in collagen with central myxomatous transformation. Seven months postoperatively the patient is doing well and has no symptoms of heart failure.