Abdominal Aortic Aneurysm in Marfan Syndrome.
Ann Vasc Surg
; 40: 294.e1-294.e6, 2017 Apr.
Article
em En
| MEDLINE
| ID: mdl-27894713
ABSTRACT
BACKGROUND:
Marfan syndrome (MFS) leads to aortic root aneurysm, while descending thoracic aortic aneurysm (TAA) occurs less commonly. Abdominal aortic aneurysm (AAA) is rarely reported in MFS. Risk factors for AAA are poorly understood and there are no guidelines for AAA screening in MFS. We sought to characterize AAA among Marfan patients in our center.METHODS:
The records of 12 adults with MFS and AAA disease were reviewed. Clinical features, imaging, operative reports, and outcomes were analyzed.RESULTS:
Twelve adults with MFS and AAA were studied; age at AAA diagnosis was 44 ± 15 years (range 18-63). Nine patients smoked cigarettes. Eleven patients underwent prior aortic root replacement at age 31 ± 15 years. The size of AAA was 5.0 ± 1.3 cm (range 3.5-7.5) at the time of diagnosis. The AAA was suprarenal in 5, juxtarenal in 2, and infrarenal in 5 patients. Two patients had a descending TAA. Branch vessel aneurysms were present in 7 patients. Five patients underwent open surgical repair, 5 underwent endovascular repair, and 5 are being treated medically. One patient died suddenly with AAA size 5.7 cm, 2 months before death. Three patients subsequently developed type B aortic dissection, from 3 months to 9 years after AAA diagnosis.CONCLUSIONS:
Adults with MFS are at risk for developing AAA. Evaluation for AAA is recommended in adults with MFS and prior root replacement, especially if descending aortic or branch vessel aneurysm is present or the patient smokes cigarettes.
Texto completo:
1
Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Aneurisma da Aorta Abdominal
/
Síndrome de Marfan
Tipo de estudo:
Diagnostic_studies
/
Etiology_studies
/
Guideline
/
Observational_studies
/
Risk_factors_studies
Limite:
Adolescent
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Adult
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Female
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Humans
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Male
/
Middle aged
Idioma:
En
Revista:
Ann Vasc Surg
Assunto da revista:
ANGIOLOGIA
Ano de publicação:
2017
Tipo de documento:
Article
País de afiliação:
Macau