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Circulation. 2013 Sep 10;128(11 Suppl 1):S243-7. doi: 10.1161/CIRCULATIONAHA.112.000113.

Mitral valve disease in patients with Marfan syndrome undergoing aortic root replacement.

Author information

  • 1Division of Cardiovascular Surgery (M.R.K., H.V.S., Z.L.) and Division of Cardiovascular Diseases (H.C.), Mayo Clinic, Rochester, MN; Division of Cardiothoracic Surgery, Michael E. DeBakey Department of Surgery, Baylor College of Medicine, Houston, TX (I.V., S.A.L., J.S.C.); Department of Cardiovascular Surgery, Texas Heart Institute, Houston, TX (I.V., S.A.L., J.S.C.); and McKusick-Nathans Institute of Genetic Medicine and Department of Pediatrics, Johns Hopkins University School of Medicine and Howard Hughes Medical Institute, Baltimore, MD (H.C.D.).

Erratum in

  • Circulation. 2014 Mar 11;129(10):e390.

Abstract

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.

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 >2, and 10% had MR grade 2. The severity of MVP and MR was evaluated by echocardiography preoperatively and ≤ 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 >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 >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 >2 in only 3 patients (2%). One early death and 2 late deaths occurred.

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 ≤ 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 ≤ 2 would benefit from prophylactic MV intervention.

KEYWORDS:

Marfan syndrome; mitral valve; thoracic aorta

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