Abstract 490: Vertebral Artery Tortuosity Index is a Novel Biomarker of Surgery and Aortic Dissection or Rupture in Children and Young Adults: Findings From the National Registry of Genetically Triggered Thoracic Aortic Aneurysms and Cardiovascular Conditions
Background: Little is known about reliable predictors of thoracic aortic dissection or rupture (TAD) in children and young adults. We sought to determine whether elevated vertebral artery tortuosity is a biomarker of TAD or aortic surgery at an early age.
Methods: We identified 208 patients ≤50 years old in the GenTAC Registry who had ≥2.5 cm of either vertebral artery visualized on a computed tomography angiogram (CTA). In a blinded fashion, using a volume-rendered projection, each patient’s vertebral artery tortuosity index (VTI) was calculated using the larger distance factor (% by which actual length exceeds straight-line length) of the two vertebral arteries. We then investigated associations between VTI and freedom from prophylactic or post-TAD surgery.
Results: Subjects included 73 with Marfan syndrome (MS), 34 with bicuspid aortic valve, 18 with Loeys-Dietz syndrome (LDS), 16 with familial thoracic aneurysms and dissections, 12 with Ehlers-Danlos syndrome type IV (EDS), 6 with congenital heart disease, and 49 with other aneurysms and dissections. Median age was 38.5 years [interquartile range (IQR) 29.6-44.6]; 23 were ≤18 years. Indication for first surgery was prophylactic in 80 patients and TAD in 49. VTI was highest in LDS (median 50, IQR 21-72), followed by MS (median VTI 27, IQR 16-52), and lowest in EDS (median 7, IQR 4-13). VTI was not associated with age at CTA. When controlling for diagnosis and race/ethnicity, higher VTI was associated with younger age at prophylactic surgery [HR 1.28, 95%CI 1.09-1.50 for every increase in VTI of 20 (VTI20)] and at surgery for TAD (HR 1.38, 95%CI 1.13-1.69 for VTI20). There was no difference in freedom from prophylactic or post-TAD surgery among LDS and MS subgroups (adjusted p=0.95 and p=0.17 respectively). In patients ≤18 years, the association between increased VTI and age at prophylactic surgery was strongest: HR 1.73 (95%CI 1.11-2.69 for VTI20). One patient ≤18 had TAD with VTI 105.
Conclusions: Increased vertebral artery tortuosity is associated with earlier thoracic aortic surgery and dissection in patients under 50 years, and may be most predictive in children. VTI can identify patients at high risk for TAD who may benefit from targeted therapies.
Author Disclosures: S.A. Morris: None. S. Oda: None. F.M. Asch: None. W. Payne: None. S.A. LeMaire: None. S. Prakash: None. R.B. Devereux: None. B.L. Kroner: None. D.S. Moodie: None. C.L. Maslen: None. R.E. Pyeritz: None. W. Ravekes: None. K.W. Holmes: None. M.J. Roman: None. N.B. McDonnell: None. R.V. Lacro: None. H.C. Dietz: None. K.A. Eagle: None. R. Krishnamurthy: None. D.M. Milewicz: None.
- © 2014 by American Heart Association, Inc.