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Surgical treatment of patients enrolled in the national registry of genetically triggered thoracic aortic conditions.
- Source :
-
The Annals of thoracic surgery [Ann Thorac Surg] 2009 Sep; Vol. 88 (3), pp. 781-7; discussion 787-8. - Publication Year :
- 2009
-
Abstract
- Background: Genetic disorders are an important cause of thoracic aortic aneurysms (TAAs) in young patients. Despite advances in the treatment of genetically triggered TAAs, the optimal syndrome-specific treatment approach remains undefined. We used data from the National Institutes of Health-funded, multicenter National Registry of Genetically Triggered Thoracic Aortic Aneurysms and Cardiovascular Conditions (GenTAC) to characterize the contemporary surgical treatment of patients with genetically triggered TAAs.<br />Methods: GenTAC's aim is to collect longitudinal clinical data and banked biospecimens from 2800 patients with genetically triggered TAAs. We analyzed data from the 606 patients (mean age, 37.5 years) enrolled in GenTAC to date whose clinical data were available.<br />Results: The patients' primary diagnoses included Marfan syndrome (35.8%), bicuspid aortic valve with aneurysm (29.2%), and familial TAAs and dissections (10.7%). Of these, 56.4% had undergone at least one operation; the most common indications were aneurysm (85.7%), valve dysfunction (65.8%), and dissection (25.4%). Surgical procedures included replacement of the aortic root (50.6%), ascending aorta (64.8%), aortic arch (27.9%), and descending or thoracoabdominal aorta (12.4%). Syndrome-specific differences in age, indications for operation, and procedure type were identified.<br />Conclusions: Patients with genetically transmitted TAAs evaluated in tertiary care centers frequently undergo surgical repair. Aneurysm repairs most commonly involve the aortic root and ascending aorta; distal repairs are less common. Like TAAs themselves, complications of TAAs, including dissection and aortic valve dysfunction, are important indications for intervention. Future studies will focus on syndrome- and gene-specific phenotypes, biomarkers, treatments, and outcomes to improve the treatment of patients with TAAs.
- Subjects :
- Adolescent
Adult
Aortic Dissection genetics
Aortic Dissection surgery
Aortic Aneurysm, Thoracic genetics
Aortic Aneurysm, Thoracic surgery
Aortic Valve abnormalities
Aortic Valve surgery
Blood Vessel Prosthesis Implantation
Female
Hospitals, University
Humans
Longitudinal Studies
Male
Marfan Syndrome genetics
Marfan Syndrome surgery
Middle Aged
Multicenter Studies as Topic
Postoperative Complications surgery
Reoperation
Syndrome
United States
Young Adult
Aorta, Thoracic surgery
Aortic Diseases genetics
Aortic Diseases surgery
Registries
Subjects
Details
- Language :
- English
- ISSN :
- 1552-6259
- Volume :
- 88
- Issue :
- 3
- Database :
- MEDLINE
- Journal :
- The Annals of thoracic surgery
- Publication Type :
- Academic Journal
- Accession number :
- 19699898
- Full Text :
- https://doi.org/10.1016/j.athoracsur.2009.04.034