MITRAL-VALVE OPERATION IN PATIENTS WITH THE MARFAN-SYNDROME

Optimal surgical treatment of mitral regurgitation in the Marfan syndr ome (valve repair versus replacement) is controversial because the und erlying connective tissue defect theoretically might compromise repair durability. To examine the results of mitral valve repair in these pa tients, we did a retrospective review of 160 patients with the Marfan syndrome who had cardiac surgical procedures between January 1983 and January 1993. Thirty-six patients had mitral procedures, 29 of which w ere repairs. Mitral valve replacement was necessary in seven patients because of extensive annular calcification and/or severe anterior leaf let abnormalities. The 18 men and 11 women undergoing mitral valve rep air had a mean age of 26.5 +/- 2.6 years (range 9 months to 54 years); seven patients were less than 18 years of age. Twenty-four of the 29 patients had concomitant aortic root replacement because of aortic dil ation or valvular insufficiency. Ah 29 repairs included annuloplasty, and 11 patients also required leaflet resection. There were no operati ve deaths. At mean follow-up of 26.6 +/- 4.8 months, there have been t hree late deaths, two caused by arrhythmia and one by complications of type m aortic dissection. All survivors are in New York Heart Associa tion class I or II. In three patients recurrent mitral regurgitation d eveloped (grade III or IV); 5-year actuarial freedom from significant mitral regurgitation was 88.3%. One patient required repeat mitral ann uloplasty after endocarditis of the composite aortic graft spread to t he mitral valve. No patient required late mitral valve replacement, Th ese results demonstrate that (1) 22% of patients with the Marfan syndr ome who undergo cardiac operation require a mitral valve procedure, (2 ) most can be treated by mitral repair rather than replacement, and (3 ) at early follow-up, results of mitral repair in this population are satisfactory.