Document Detail

Long-term implications of emergency versus elective proximal aortic surgery in patients with Marfan syndrome in the Genetically Triggered Thoracic Aortic Aneurysms and Cardiovascular Conditions Consortium Registry.
MedLine Citation:
PMID:  22104675     Owner:  NLM     Status:  MEDLINE    
OBJECTIVE: Patients with Marfan syndrome with aortic root aneurysms undergo elective aortic root replacement to avoid the life-threatening outcomes of aortic dissection and emergency repair. The long-term implications of failed aortic surveillance leading to acute dissection and emergency repair are poorly defined. We compared the long-term clinical courses of patients with Marfan syndrome who survive emergency versus elective proximal aortic surgery.
METHODS: The Genetically Triggered Thoracic Aortic Aneurysms and Cardiovascular Conditions Registry is a National Institutes of Health-funded multicenter database and biorepository that enrolls patients with genetically triggered thoracic aortic aneurysms. Of the 635 patients with Marfan syndrome enrolled as of March 2011, 194 had undergone proximal aortic replacement. Patients were grouped according to emergency (n = 47) or elective (n = 147) status at the time of surgery.
RESULTS: Patients in the emergency group were more likely to have incomplete proximal aortic resection; 83% of emergency procedures included aortic root replacement, compared with 95% of elective procedures. At long-term follow-up (mean, >6 years), the emergency group had a higher incidence of chronic dissection of the distal aorta and significantly larger diameters in distal aortic segments than elective patients. Patients in the emergency group had undergone more operations (1.31 vs 1.11 procedures/patient; P = .01) and had lower activity scores on a health-related quality of life survey.
CONCLUSIONS: For patients with Marfan syndrome, failed aortic surveillance and consequent emergency dissection repair have important long-term implications with regard to the status of the distal aorta, need for multiple procedures, and quality of life. These findings emphasize the importance of aortic surveillance and timely elective aortic root aneurysm repair for patients with Marfan syndrome.
Howard K Song; Mark Kindem; Joseph E Bavaria; Harry C Dietz; Dianna M Milewicz; Richard B Devereux; Kim A Eagle; Cheryl L Maslen; Barbara L Kroner; Reed E Pyeritz; Kathryn W Holmes; Jonathan W Weinsaft; Victor Menashe; William Ravekes; Scott A LeMaire;
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Publication Detail:
Type:  Comparative Study; Journal Article; Multicenter Study; Research Support, N.I.H., Extramural     Date:  2011-11-20
Journal Detail:
Title:  The Journal of thoracic and cardiovascular surgery     Volume:  143     ISSN:  1097-685X     ISO Abbreviation:  J. Thorac. Cardiovasc. Surg.     Publication Date:  2012 Feb 
Date Detail:
Created Date:  2012-01-17     Completed Date:  2012-02-28     Revised Date:  2013-06-27    
Medline Journal Info:
Nlm Unique ID:  0376343     Medline TA:  J Thorac Cardiovasc Surg     Country:  United States    
Other Details:
Languages:  eng     Pagination:  282-6     Citation Subset:  AIM; IM    
Copyright Information:
Copyright © 2012 The American Association for Thoracic Surgery. All rights reserved.
Division of Cardiothoracic Surgery, Oregon Health & Science University, Portland, OR 97239, USA.
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MeSH Terms
Aneurysm, Dissecting / diagnosis,  genetics,  surgery*
Aortic Aneurysm, Thoracic / diagnosis,  genetics,  surgery*
Blood Vessel Prosthesis Implantation* / adverse effects
Chi-Square Distribution
Child, Preschool
Chronic Disease
Marfan Syndrome / complications*,  diagnosis,  genetics
Middle Aged
Quality of Life
Risk Assessment
Risk Factors
Surgical Procedures, Elective
Time Factors
Treatment Outcome
United States
Young Adult
Grant Support
HHSN268201000048C/HL/NHLBI NIH HHS; N01-HV-08238/HHSN268201000048C/HV/NHLBI NIH HHS

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