Aortic Root Surgery in Marfan Syndrome

Medium-Term Outcome in a Single-Center Experience

Christine H. Attenhofer Jost, Heidi M. Connolly, Christopher G. Scott, Naser M. Ammash, Juan M. Bowen, Hartzell V Schaff

Research output: Contribution to journalArticle

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Abstract

BACKGROUND: The study aim was to analyze the authors' experience with aortic root surgery in Marfan syndrome (MFS), and to expand the surgical outcome data of patients meeting the Ghent criteria (Marfan registry). METHODS: Analyses were performed of data acquired from MFS patients (who met the Ghent criteria), including an aortic root surgery and Kaplan-Meier survival. RESULTS: Between April 2004 and February 2012, a total of 59 MFS patients (mean age at surgery 36 ± 13 years) underwent 67 operations for aortic root aneurysm (n = 52), aortic valve (AV) regurgitation (n = 15), acute aortic dissection (n = 2), and/or mitral valve (MV) regurgitation resulting from MV prolapse (n = 7). Of 59 initial operations, 21 (36%) involved AV-replacing root surgery, 38 (64%) AV-sparing root surgery, seven (12%) aortic arch or hemi-arch repair, and five (8%) simultaneous MV surgery. There were no early mortalities. The mean follow up was 6.8 ± 1.2 years, with five deaths (8%) and a relatively low reoperation rate (10 reoperations in nine patients; 14%). Seven reoperations involved AV or aortic root surgery (including four for AV regurgitation following failed AV-sparing surgery), two MV repair/replacements, and one coronary artery bypass graft. Eight patients (21%) with AV-sparing surgery had moderate/severe AV regurgitation at the last follow up before re-intervention. The mean five-year freedom from postoperative death was 91.2 ± 8.8%, from cardiac reoperation 86.3 ± 4.5%, and more-than-moderate AV regurgitation 90.3 ± 4.8%. CONCLUSIONS: Prophylactic aortic surgery in MFS patients with AV-replacing root or AV-sparing root surgery carries a low risk of operative morbidity and death when performed at an experienced center. AV-sparing root surgery increases the risk of AV regurgitation and, possibly, of re-intervention. Regular clinical follow up is important after any aortic root surgery in MFS patients, with a delineation of risk factors for AV regurgitation after AV rootsparing surgery.

Original languageEnglish (US)
Pages (from-to)45-53
Number of pages9
JournalThe Journal of heart valve disease
Volume26
Issue number1
StatePublished - Jan 1 2017

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Marfan Syndrome
Aortic Valve
Aortic Valve Insufficiency
Reoperation
Mitral Valve
Mitral Valve Prolapse
Aortic Aneurysm
Mitral Valve Insufficiency
Thoracic Aorta
Coronary Artery Bypass
Registries

ASJC Scopus subject areas

  • Medicine(all)

Cite this

Attenhofer Jost, C. H., Connolly, H. M., Scott, C. G., Ammash, N. M., Bowen, J. M., & Schaff, H. V. (2017). Aortic Root Surgery in Marfan Syndrome: Medium-Term Outcome in a Single-Center Experience. The Journal of heart valve disease, 26(1), 45-53.

Aortic Root Surgery in Marfan Syndrome : Medium-Term Outcome in a Single-Center Experience. / Attenhofer Jost, Christine H.; Connolly, Heidi M.; Scott, Christopher G.; Ammash, Naser M.; Bowen, Juan M.; Schaff, Hartzell V.

In: The Journal of heart valve disease, Vol. 26, No. 1, 01.01.2017, p. 45-53.

Research output: Contribution to journalArticle

Attenhofer Jost, CH, Connolly, HM, Scott, CG, Ammash, NM, Bowen, JM & Schaff, HV 2017, 'Aortic Root Surgery in Marfan Syndrome: Medium-Term Outcome in a Single-Center Experience', The Journal of heart valve disease, vol. 26, no. 1, pp. 45-53.
Attenhofer Jost CH, Connolly HM, Scott CG, Ammash NM, Bowen JM, Schaff HV. Aortic Root Surgery in Marfan Syndrome: Medium-Term Outcome in a Single-Center Experience. The Journal of heart valve disease. 2017 Jan 1;26(1):45-53.
Attenhofer Jost, Christine H. ; Connolly, Heidi M. ; Scott, Christopher G. ; Ammash, Naser M. ; Bowen, Juan M. ; Schaff, Hartzell V. / Aortic Root Surgery in Marfan Syndrome : Medium-Term Outcome in a Single-Center Experience. In: The Journal of heart valve disease. 2017 ; Vol. 26, No. 1. pp. 45-53.
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abstract = "BACKGROUND: The study aim was to analyze the authors' experience with aortic root surgery in Marfan syndrome (MFS), and to expand the surgical outcome data of patients meeting the Ghent criteria (Marfan registry). METHODS: Analyses were performed of data acquired from MFS patients (who met the Ghent criteria), including an aortic root surgery and Kaplan-Meier survival. RESULTS: Between April 2004 and February 2012, a total of 59 MFS patients (mean age at surgery 36 ± 13 years) underwent 67 operations for aortic root aneurysm (n = 52), aortic valve (AV) regurgitation (n = 15), acute aortic dissection (n = 2), and/or mitral valve (MV) regurgitation resulting from MV prolapse (n = 7). Of 59 initial operations, 21 (36{\%}) involved AV-replacing root surgery, 38 (64{\%}) AV-sparing root surgery, seven (12{\%}) aortic arch or hemi-arch repair, and five (8{\%}) simultaneous MV surgery. There were no early mortalities. The mean follow up was 6.8 ± 1.2 years, with five deaths (8{\%}) and a relatively low reoperation rate (10 reoperations in nine patients; 14{\%}). Seven reoperations involved AV or aortic root surgery (including four for AV regurgitation following failed AV-sparing surgery), two MV repair/replacements, and one coronary artery bypass graft. Eight patients (21{\%}) with AV-sparing surgery had moderate/severe AV regurgitation at the last follow up before re-intervention. The mean five-year freedom from postoperative death was 91.2 ± 8.8{\%}, from cardiac reoperation 86.3 ± 4.5{\%}, and more-than-moderate AV regurgitation 90.3 ± 4.8{\%}. CONCLUSIONS: Prophylactic aortic surgery in MFS patients with AV-replacing root or AV-sparing root surgery carries a low risk of operative morbidity and death when performed at an experienced center. AV-sparing root surgery increases the risk of AV regurgitation and, possibly, of re-intervention. Regular clinical follow up is important after any aortic root surgery in MFS patients, with a delineation of risk factors for AV regurgitation after AV rootsparing surgery.",
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AU - Bowen, Juan M.

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N2 - BACKGROUND: The study aim was to analyze the authors' experience with aortic root surgery in Marfan syndrome (MFS), and to expand the surgical outcome data of patients meeting the Ghent criteria (Marfan registry). METHODS: Analyses were performed of data acquired from MFS patients (who met the Ghent criteria), including an aortic root surgery and Kaplan-Meier survival. RESULTS: Between April 2004 and February 2012, a total of 59 MFS patients (mean age at surgery 36 ± 13 years) underwent 67 operations for aortic root aneurysm (n = 52), aortic valve (AV) regurgitation (n = 15), acute aortic dissection (n = 2), and/or mitral valve (MV) regurgitation resulting from MV prolapse (n = 7). Of 59 initial operations, 21 (36%) involved AV-replacing root surgery, 38 (64%) AV-sparing root surgery, seven (12%) aortic arch or hemi-arch repair, and five (8%) simultaneous MV surgery. There were no early mortalities. The mean follow up was 6.8 ± 1.2 years, with five deaths (8%) and a relatively low reoperation rate (10 reoperations in nine patients; 14%). Seven reoperations involved AV or aortic root surgery (including four for AV regurgitation following failed AV-sparing surgery), two MV repair/replacements, and one coronary artery bypass graft. Eight patients (21%) with AV-sparing surgery had moderate/severe AV regurgitation at the last follow up before re-intervention. The mean five-year freedom from postoperative death was 91.2 ± 8.8%, from cardiac reoperation 86.3 ± 4.5%, and more-than-moderate AV regurgitation 90.3 ± 4.8%. CONCLUSIONS: Prophylactic aortic surgery in MFS patients with AV-replacing root or AV-sparing root surgery carries a low risk of operative morbidity and death when performed at an experienced center. AV-sparing root surgery increases the risk of AV regurgitation and, possibly, of re-intervention. Regular clinical follow up is important after any aortic root surgery in MFS patients, with a delineation of risk factors for AV regurgitation after AV rootsparing surgery.

AB - BACKGROUND: The study aim was to analyze the authors' experience with aortic root surgery in Marfan syndrome (MFS), and to expand the surgical outcome data of patients meeting the Ghent criteria (Marfan registry). METHODS: Analyses were performed of data acquired from MFS patients (who met the Ghent criteria), including an aortic root surgery and Kaplan-Meier survival. RESULTS: Between April 2004 and February 2012, a total of 59 MFS patients (mean age at surgery 36 ± 13 years) underwent 67 operations for aortic root aneurysm (n = 52), aortic valve (AV) regurgitation (n = 15), acute aortic dissection (n = 2), and/or mitral valve (MV) regurgitation resulting from MV prolapse (n = 7). Of 59 initial operations, 21 (36%) involved AV-replacing root surgery, 38 (64%) AV-sparing root surgery, seven (12%) aortic arch or hemi-arch repair, and five (8%) simultaneous MV surgery. There were no early mortalities. The mean follow up was 6.8 ± 1.2 years, with five deaths (8%) and a relatively low reoperation rate (10 reoperations in nine patients; 14%). Seven reoperations involved AV or aortic root surgery (including four for AV regurgitation following failed AV-sparing surgery), two MV repair/replacements, and one coronary artery bypass graft. Eight patients (21%) with AV-sparing surgery had moderate/severe AV regurgitation at the last follow up before re-intervention. The mean five-year freedom from postoperative death was 91.2 ± 8.8%, from cardiac reoperation 86.3 ± 4.5%, and more-than-moderate AV regurgitation 90.3 ± 4.8%. CONCLUSIONS: Prophylactic aortic surgery in MFS patients with AV-replacing root or AV-sparing root surgery carries a low risk of operative morbidity and death when performed at an experienced center. AV-sparing root surgery increases the risk of AV regurgitation and, possibly, of re-intervention. Regular clinical follow up is important after any aortic root surgery in MFS patients, with a delineation of risk factors for AV regurgitation after AV rootsparing surgery.

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