BACKGROUND: @entity1 with @entity1614 ( @entity1614 ) have a highly variable occurrence of aortic complications. Aortic tortuosity is often present in @entity1614 and may help to identify @entity1 at risk for aortic complications. METHODS: 3D-visualization of the total aorta by MR imaging was performed in 211 adult @entity1614 @entity1 (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 ( @entity1580 , and/or aortic surgery) and @entity384 rate during 3years of follow-up were investigated. RESULTS: ATI intra- and interobserver agreements were excellent (ICC: 0.968 and 0.955, respectively). Mean ATI was higher in 28 age-matched @entity1614 @entity1 than in the controls (1.92  0.2 vs. 1.82  0.1, p=0.048). In the total @entity1614 cohort, mean ATI was 1.87  0.20, and correlated with age (r=0.281, p<0.001), aortic root diameter (r=0.223, p=0.006), and aortic volume expansion rate (r=0.177, p=0.026). After 49.3  8.8months follow-up, 33 @entity1 met the combined clinical endpoint (7 dissections) with a significantly higher ATI at baseline than @entity1 without endpoint (1.98  0.2 vs. 1.86  0.2, p=0.002). @entity1 with an ATI>1.95 had a 12.8 times higher probability of meeting the combined endpoint (log rank-test, p<0.001) and a 12.1 times higher probability of developing an @entity1580 (log rank-test, p=0.003) compared to @entity1 with an ATI<1.95. CONCLUSIONS: Increased ATI is associated with a more severe aortic phenotype in @entity1614 @entity1 .

Increased aortic tortuosity indicates a more severe aortic phenotype in adults with XXXX .

@entity1 :: ('9606', 'Species') :: ['Patients', 'patients'] @entity384 :: ('MESH:D002311', 'Disease') :: ['aortic dilatation'] @entity1614 :: ('MESH:D008382', 'Disease') :: ['MFS', 'Marfan syndrome'] @entity1580 :: ('MESH:C562834', 'Disease') :: ['aortic dissection']
@entity1614 :: (MESH:D008382,Disease) :: ['Marfan syndrome']
