Surgical management of the aortic root in patients with Marfan syndrome

Kenton J. Zehr, Alireza Matloobi, Heidi M. Connolly, Thomas A. Orszulak, Francisco J. Puga, Hartzell V. Schaff

Research output: Contribution to journalArticle

Abstract

Background and aim of the study: Surgical aortic root reconstruction techniques are standard therapy to avoid catastrophic vascular events in patients with Marfan syndrome with a dilated and/or dissected aortic root. The study aim was to evaluate the long-term results of aortic root reconstruction. Methods: Eighty-three patients (54 males, 29 females; mean age 37 ± 17 years) fulfilling strict Ghent criteria for Marfan syndrome underwent aortic root surgery between 1971 and 2001. Of these patients, 65 (78%) underwent a composite valve conduit repair and 18 (22%) a valve-sparing aortic root reconstruction. Six patients (7%) suffered from an acute type A dissection, and 16 (19%) a chronic type A dissection. Results: In-hospital and 30-day mortality was 3.6% (n = 3). Morbidity included stroke (1.2%; n = 1), perioperative myocardial infarction (1.27c; n = 1) and reoperation for bleeding (10%; n = 8). Of 21 late deaths, the cause was cardiac in nine cases. Actuarial survival at 5, 10, 15 and 20 years was 84% (95% CI 76-93%), 73% (CI 61-86%), 59% (CI 45-77%) and 43% (CI 26-72%), respectively. Multivariate predictors for late death were postoperative dysrhythmias and need for inotropes (p ≤0.01). Freedom from reoperation at 5, 10,15 and 20 years was 86% (CI 78-95%), 69% (CI 56-85%), 53% (CI 38-74%) and 48% (CI 23-71%), respectively. Multivariate predictors for reoperation were preoperative mitral valve prolapse and an initial valve-sparing aortic procedure (p ≤0.05). In the composite valve conduit patients, freedom from thromboembolism was 88% (CI 76-100%), and from endocarditis was 99% (CI 93-100%) at 15 years. Conclusion: Composite valve conduit replacement of the aortic root in patients with Marfan syndrome offers a durable result, with low mortality and long-term complication rates. Reoperation was most commonly required for cardiac and vascular disease unrelated to the initial operation and in patients undergoing a valve-sparing aortic root procedure.

Original languageEnglish (US)
Pages (from-to)121-128
Number of pages8
JournalJournal of Heart Valve Disease
Volume14
Issue number1
StatePublished - Dec 1 2005

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine

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    Zehr, K. J., Matloobi, A., Connolly, H. M., Orszulak, T. A., Puga, F. J., & Schaff, H. V. (2005). Surgical management of the aortic root in patients with Marfan syndrome. Journal of Heart Valve Disease, 14(1), 121-128.