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

18 Citations (Scopus)

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 - 2005

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Marfan Syndrome
Reoperation
Aortic Valve
Dissection
Mitral Valve Prolapse
Mortality
Thromboembolism
Endocarditis
Vascular Diseases
Blood Vessels
Cause of Death
Heart Diseases
Stroke
Myocardial Infarction
Hemorrhage
Morbidity
Survival

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine

Cite this

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.

Surgical management of the aortic root in patients with Marfan syndrome. / Zehr, Kenton J.; Matloobi, Alireza; Connolly, Heidi M.; Orszulak, Thomas A.; Puga, Francisco J.; Schaff, Hartzell V.

In: Journal of Heart Valve Disease, Vol. 14, No. 1, 2005, p. 121-128.

Research output: Contribution to journalArticle

Zehr, KJ, Matloobi, A, Connolly, HM, Orszulak, TA, Puga, FJ & Schaff, HV 2005, 'Surgical management of the aortic root in patients with Marfan syndrome', Journal of Heart Valve Disease, vol. 14, no. 1, pp. 121-128.
Zehr KJ, Matloobi A, Connolly HM, Orszulak TA, Puga FJ, Schaff HV. Surgical management of the aortic root in patients with Marfan syndrome. Journal of Heart Valve Disease. 2005;14(1):121-128.
Zehr, Kenton J. ; Matloobi, Alireza ; Connolly, Heidi M. ; Orszulak, Thomas A. ; Puga, Francisco J. ; Schaff, Hartzell V. / Surgical management of the aortic root in patients with Marfan syndrome. In: Journal of Heart Valve Disease. 2005 ; Vol. 14, No. 1. pp. 121-128.
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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.",
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N2 - 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.

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