Mitral Valve Disease in Patients with Marfan Syndrome Undergoing Aortic Root Replacement

作者：Kunkala Meghana R; Schaff Hartzell V; Li Zhuo; Volguina Irina; Dietz Harry C; LeMaire Scott A; Coselli Joseph S; Connolly Heidi^*

来源：Circulation, 2013, 128(11): S243-S247.

DOI：10.1161/CIRCULATIONAHA.112.000113

• Marfan syndrome
• mitral valve
• thoracic aorta

Background-Cardiac manifestations of Marfan syndrome include aortic root dilation and mitral valve prolapse (MVP). Only scant data exist describing MVP in patients with Marfan syndrome undergoing aortic root replacement. %26lt;br%26gt;Methods and Results-We retrospectively analyzed data from 166 MFS patients with MVP who were enrolled in a prospective multicenter registry of patients who underwent aortic root aneurysm repair. Of these 166 patients, 9% had mitral regurgitation (MR) grade %26gt;2, and 10% had MR grade 2. The severity of MVP and MR was evaluated by echocardiography preoperatively and %26lt;= 3 years postoperatively. Forty-one patients (25%) underwent composite graft aortic valve replacement, and 125 patients (75%) underwent aortic valve-sparing procedures; both groups had similar prevalences of MR grade %26gt;2 (P=0.7). Thirty-three patients (20%) underwent concomitant mitral valve (MV) intervention (repair, n=29; replacement, n=4), including all 15 patients with MR grade %26gt;2. Only 1 patient required MV reintervention during follow-up (mean clinical follow-up, 31 +/- 10 months). Echocardiography performed 21 +/- 13 months postoperatively revealed MR %26gt;2 in only 3 patients (2%). One early death and 2 late deaths occurred. %26lt;br%26gt;Conclusions-Although the majority of patients with Marfan syndrome who undergo elective aortic root replacement have MVP, only 20% have concomitant MV procedures. These concomitant procedures do not seem to increase operative risk. In patients with MR grade %26lt;= 2 who do not undergo a concomitant MV procedure, the short-term incidence of progressive MR is low; however, more follow-up is needed to determine whether patients with MVP and MR grade %26lt;= 2 would benefit from prophylactic MV intervention.

• 出版日期2013-9-10