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Increased aortic tortuosity indicates a more severe aortic phenotype in adults with Marfan syndrome
- Source :
- International Journal of Cardiology, 194, 7-12, International Journal of Cardiology, 194, pp. 7-12, International Journal of Cardiology, 194, 7-12. ELSEVIER IRELAND LTD, International journal of cardiology, 194, 7-12. Elsevier Ireland Ltd
- Publication Year :
- 2015
-
Abstract
- Background: Patients with Marfan syndrome (MFS) have a highly variable occurrence of aortic complications. Aortic tortuosity is often present in MFS and may help to identify patients at risk for aortic complications.Methods: 3D-visualization of the total aorta by MR imaging was performed in 211 adult MFS patients (28% with prior aortic root replacement) and 20 controls. A method to assess aortic tortuosity (aortic tortuosity index: ATI) was developed and reproducibility was tested. The relation between ATI and age, and body size and aortic dimensions at baseline was investigated. Relations between ATI at baseline and the occurrence of a clinical endpoint (aortic dissection, and/or aortic surgery) and aortic dilatation rate during 3 years of follow-upwere investigated.Results: ATI intra-and interobserver agreements were excellent (ICC: 0.968 and 0.955, respectively). Mean ATI was higher in 28 age-matched MFS patients than in the controls (1.92 +/- 0.2 vs. 1.82 +/- 0.1, p = 0.048). In the total MFS cohort, mean ATI was 1.87 +/- 0.20, and correlated with age (r=0.281, p 1.95 had a 12.8 times higher probability of meeting the combined endpoint (log rank-test, p Conclusions: Increased ATI is associated with a more severe aortic phenotype in MFS patients. (C) 2015 Elsevier Ireland Ltd. All rights reserved.
- Subjects :
- Marfan syndrome
Adult
Male
medicine.medical_specialty
Vascular damage Radboud Institute for Health Sciences [Radboudumc 16]
Aortic Diseases
Aortic dissection
Tortuosity
Fibrillins
Loeys–Dietz syndrome
Losartan
Aortic tortuosity
medicine.artery
Internal medicine
Ascending aorta
medicine
Clinical endpoint
Humans
ARTERIAL TORTUOSITY
Aorta
INDEX
business.industry
Microfilament Proteins
Other Research Radboud Institute for Health Sciences [Radboudumc 0]
Reproducibility of Results
TGF-BETA
Middle Aged
medicine.disease
Magnetic Resonance Imaging
Magnetic imaging
Surgery
LOEYS-DIETZ SYNDROME
Phenotype
PREGNANCY
Cohort
Mutation
Cardiology
cardiovascular system
Female
ASCENDING AORTA
Cardiology and Cardiovascular Medicine
business
Angiotensin II Type 1 Receptor Blockers
Dilatation, Pathologic
Follow-Up Studies
Subjects
Details
- Language :
- English
- ISSN :
- 01675273
- Volume :
- 194
- Database :
- OpenAIRE
- Journal :
- International Journal of Cardiology
- Accession number :
- edsair.doi.dedup.....6d3124f7f4a7b26d16041053dfcb6ef7