Abstract 14983: Arterial Tortuosity and Cardiovascular Outcomes in Marfan Syndrome and Loeys-Dietz Syndrome
Background: Single-center research suggests that increased vertebral artery tortuosity is a biomarker of adverse cardiovascular events in young patients with Marfan and Loeys-Dietz syndromes (MFS and LDS). The objective of our study was to validate this finding in a larger, multicenter population with focus on specific cardiovascular events.
Methods: Patients were included from the National Registry of Genetically Triggered Thoracic Aortic Aneurysms and Cardiovascular Conditions (GenTAC) or our institutional Cardiovascular Genetics Clinic, with MFS or LDS (caused by TGFBR1/2 mutations) who underwent magnetic resonance or computed tomography angiography (MRA/CTA) < age 50 years that included the vertebral arteries. The vertebral artery tortuosity index (VTI) was calculated from the study including the largest proportion of the vertebral arteries. Outcomes were compared using Chi square, Wilcoxon rank sum, Poisson, and Cox regression analyses.
Results: A total of 203 patients were included. A VTI ≥ 50 was associated with earlier aortic surgery, earlier Type A and B aortic dissections, and more frequent aortic surgery (Table). In all multivariable models containing VTI and diagnosis, VTI retained significant association with the outcome, while the diagnosis of LDS versus MFS did not. For prophylactic aortic surgery, VTI remained significant when controlling for both diagnosis and maximum aortic dimension. Aortic dimensions were only available for 5 patients prior to Type A aortic dissection; those with VTI ≥ 50 (n=3) had a median dimension of 4.29 cm (range 4.02, 5.17) at dissection versus 5.88 cm (range 4.88,6.88) in those with VTI <50 (p=0.400).
Conclusions: In this multicenter study, VTI ≥ 50 is a marker of earlier adverse events in patients <age 50 with MFS and LDS. Further study is needed to assess whether VTI should influence treatment decisions for this population.
Author Disclosures: S.A. Morris: None. N.A. Dodd: None. S. Oda: None. K. Kancherla: None. F.M. Asch: None. M. Challman: None. S.S. Andreas: None. W. Payne: None. L. Preiss: None. S. LeMaire: Other Research Support; Modest; Vascutek Terumo, Baxter Healthcare, Medtronic, Inc., W.L. Gore & Associates, Cytosorbants. R.B. Devereux: None. R.E. Pyeritz: None. K.W. Holmes: None. M.J. Roman: None. R.V. Lacro: None. R.V. Shohet: None. R. Krishnamurthy: None. C. Pedroza: None. K. Eagle: Research Grant; Significant; Gore, Medtronic, Terumo. D.M. Milewicz: Research Grant; Significant; NIH, Bugher Fund, John Ritter Foundation. Consultant/Advisory Board; Significant; Marfan Foundation, John Ritter Foundation, Genetic Aortic Disease Association.
- © 2016 by American Heart Association, Inc.