Mitral valve operation in patients with the Marfan syndrome

Abstract

Optimal surgical treatment of mitral regurgitation in the Marfan syndrome (valve repair versus replacement) is controversial because the underlying connective tissue defect theoretically might compromise repair durability. To examine the results of mitral valve repair in these patients, 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 were repairs. Mitral valve replacement was necessary in seven patients because of extensive annular calcification and/or severe anterior leaflet abnormalities. The 18 men and 11 women undergoing mitral valve repair 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 dilation or valvular insufficiency. AU 29 repairs included annuloplasty, and 11 patients also required leaflet resection. There were no operative deaths. At mean follow-up of 26.6 ± 4.8 months, there have been three late deaths, two caused by arrhythmia and one by complications of type m aortic dissection. All survivors are in New York Heart Association class I or II. In three patients recurrent mitral regurgitation developed (grade m or IV); 5-year actuarial freedom from significant mitral regurgitation was 88.3 %. One patient required repeat mitral annuloplasty after endocarditis of the composite aortic graft spread to the mitral valve. No patient required late mitral valve replacement. These results demonstrate that (1) 22 % of patients with the Madan syndrome 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. (J

Results of Modern Mitral Valve Repair in Patients with Marfan Syndrome

“…24 Although freedom from reoperation after 15 years after MVR is around 95% for idiopathic MVP, some studies have documented the potential for recurrence of significant MV regurgitation of 1 to 2% of patients per year. 18,25 In our cohort, we had no early mortality that was also previously reported by Gillinov et al and Bhudia et al In these studies, long-term survival after MVR was 83.3% after 10 years in the cohort of Gillinov et al that is comparable with the reported 80% of Bhudia et al and with 78.9% of Fuzzelier et al 5,26,27 No patient of our study needed late MV replacement. Other series also reported good long-term performance of the reconstructed valves with a low risk of late MV replacement with freedom from MV reoperation of 87.1 to 96% at 10 years.…”

Institutional Marfan syndrome surgical volume influences mitral valve surgical strategy in patients with Marfan syndrome

“…Most existing surgical literature has focused on the management of aortic disease in this patient population. The few reports regarding MV surgery in the MfS population are from major MfS reference centers and have shown that patients with MfS have superior long‐term outcomes when undergoing mitral valve repair (MVr), compared to mitral valve replacement (MVR) . This is congruent with current American and European guidelines stressing MVr over MVR in the management of degenerative mitral valve disease (DMVD) .…”

“…These reports, however, are from MfS surgical reference centers and demonstrate a robust MVr rate when compared to the rates in this study. Gillinov et al were able to achieve 81% MVr rate in their MfS population with mitral pathology. Similarly, Helder et al report a 60% MVr rate in their cohort.…”

Repair of Type A Dissection with Mitral Regurgitation Using Total Arch Replacement with Mitral Valve Surgery

“…Sixty to eighty percent of patients with Marfan's syndrome had mitral valve dysfunction . Mitral valve morphology demonstrated annular dilation, annular calcification, fibromyxomatous changes of leaflets and chordae, chordal rupture and elongation, and leaflet fenestration . Moreover, chronic type A dissection is not uncommon in China due to delayed diagnosis and surgical treatment for patients with acute type A dissection .…”