Aortic Root Surgery in Marfan Syndrome: Medium-Term Outcome in a Single-Center Experience.

Published on Jan 1, 2017in Journal of Heart Valve Disease
Christine H. Attenhofer Jost11
Estimated H-index: 11
(Mayo Clinic),
Heidi M. Connolly75
Estimated H-index: 75
(Mayo Clinic)
+ 3 AuthorsHartzell V. Schaff131
Estimated H-index: 131
(Mayo Clinic)
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.
Cited By7
#1Wen-Po Fan (Taipei Veterans General Hospital)
Last. Pi-Chang LeeH-Index: 21
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BACKGROUND Marfan syndrome is an inherited connective tissue disease that causes aortic root dilatation and dissection and requires surgical intervention. Apart from emergent surgery for aortic dissection or aortic aneurysmal rupture, prophylactic surgical intervention can also be administered, depending on the severity of aortic root dilatation. The direct relationship between surgical intervention and aortic regurgitation was seldom mentioned in previous studies. METHODS A retrospective cohort...
#1Nikolaos Konstantinou (LMU: Ludwig Maximilian University of Munich)H-Index: 3
#2Sven Peterss (LMU: Ludwig Maximilian University of Munich)
Last. Nikolaos Tsilimparis (LMU: Ludwig Maximilian University of Munich)H-Index: 8
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PurposeTo present a novel technique to successfully cross a mechanical aortic valve prosthesis.TechniqueA 55-year-old female patient with genetically verified Marfan syndrome presented with a 5-cm ...
#1Malak Elbatarny (Queen's University)H-Index: 5
#2Derrick Y. Tam (TGH: Toronto General Hospital)H-Index: 1
Last. Maral Ouzounian (TGH: Toronto General Hospital)H-Index: 25
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#1Reed E. Pyeritz (UPenn: University of Pennsylvania)H-Index: 91
Life expectancy for a person with Marfan syndrome has essentially doubled over the past four decades. During this period, the clinical histories of the organs managed routinely have improved, and will continue to be. Prominent examples are the eyes, the heart and aorta, and some features of the skeletal system. Meanwhile, the natural histories of organ systems that have not been subjected to treatment need to be described. This is particularly important as due to the improved life span many symp...
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#1Vicente Orozco-Sevilla (The Texas Heart Institute)H-Index: 2
#2Richard S. Whitlock (The Texas Heart Institute)H-Index: 1
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Aortic root aneurysm is the most common cardiovascular manifestation requiring surgical intervention in patients with Marfan syndrome (MFS), a heritable thoracic aortic disease. Elective replacement of the aortic root is the treatment of choice for patients with aneurysmal complications of the aortic root and ascending aorta. There are two basic approaches to aortic root replacement: valve-sparing (VS) and valve-replacing (VR) techniques. After successful aortic root replacement surgery, several...
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Ascending aortic replacement is usually performed with a cross-clamp at the distal ascending aorta. This cross-clamping is one of the routine procedures in cardiac surgery, and the risk of the cross-clamping has been lowered to minimum in regular practice. Theoretically, the cross-clamp itself is a risk to apply and might lead to aortic dissection (1) or create a source of embolization in the aortic wall. However, when there is no atheroma, calcification, or other abnormal situations including a...