Literature DB >> 15700446

Surgical management of the aortic root in patients with Marfan syndrome.

Kenton J Zehr1, Alireza Matloobi, Heidi M Connolly, Thomas A Orszulak, Francisco J Puga, Hartzell V Schaff.   

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 (1.2%; 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 < or =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 < or =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.

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Year:  2005        PMID: 15700446

Source DB:  PubMed          Journal:  J Heart Valve Dis        ISSN: 0966-8519


  4 in total

1.  Cardiovascular management of marfan syndrome in the young.

Authors:  Angela M Sharkey
Journal:  Curr Treat Options Cardiovasc Med       Date:  2006-09

2.  Non-aortic cardiovascular disease in Marfan syndrome: a nationwide epidemiological study.

Authors:  Niels H Andersen; Kristian A Groth; Agnethe Berglund; Hanne Hove; Claus H Gravholt; Kirstine Stochholm
Journal:  Clin Res Cardiol       Date:  2021-04-22       Impact factor: 5.460

Review 3.  Systematic review and meta-analysis of surgical outcomes in Marfan patients undergoing aortic root surgery by composite-valve graft or valve sparing root replacement.

Authors:  Campbell D Flynn; David H Tian; Ashley Wilson-Smith; Tirone David; George Matalanis; Martin Misfeld; Stefano Mastrobuoni; Gebrine El Khoury; Tristan D Yan
Journal:  Ann Cardiothorac Surg       Date:  2017-11

4.  Mitral Valve Prolapse and Its Motley Crew-Syndromic Prevalence, Pathophysiology, and Progression of a Common Heart Condition.

Authors:  Jordan E Morningstar; Annah Nieman; Christina Wang; Tyler Beck; Andrew Harvey; Russell A Norris
Journal:  J Am Heart Assoc       Date:  2021-06-22       Impact factor: 5.501

  4 in total

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