Validating a Curvature-Based Marker of Cervical Carotid Tortuosity for Risk Assessment in Heritable Aortopathies

J Am Heart Assoc. 2024 Jul 2;13(13):e035171. doi: 10.1161/JAHA.124.035171. Epub 2024 Jun 21.

Abstract

Background: Cervical arterial tortuosity is associated with adverse outcomes in Loeys-Dietz syndrome and other heritable aortopathies.

Methods and results: A method to assess tortuosity based on curvature of the vessel centerline in 3-dimensional space was developed. We measured cervical carotid tortuosity in 65 patients with Loeys-Dietz syndrome from baseline computed tomography angiogram/magnetic resonance angiogram and all serial images during follow-up. Relations between baseline carotid tortuosity, age, aortic root diameter, and its change over time were compared. Patients with unoperated aortic roots were assessed for clinical end point (type A aortic dissection or aortic root surgery during 4 years of follow-up). Logistic regression was performed to assess the likelihood of clinical end point according to baseline carotid tortuosity. Total absolute curvature at baseline was 11.13±5.76 and was relatively unchanged at 8 to 10 years (fold change: 0.026±0.298, P=1.00), whereas tortuosity index at baseline was 0.262±0.131, with greater variability at 8 to 10 years (fold change: 0.302±0.656, P=0.818). Baseline total absolute curvature correlated with aortic root diameter (r=0.456, P=0.004) and was independently associated with aortic events during the 4-year follow-up (adjusted odds ratio [OR], 2.64 [95% CI, 1.02-6.85]). Baseline tortuosity index correlated with age (r=0.532, P<0.001) and was not associated with events (adjusted OR, 1.88 [95% CI, 0.79-4.51]). Finally, baseline total absolute curvature had good discrimination of 4-year outcomes (area under the curve=0.724, P=0.014), which may be prognostic or predictive.

Conclusions: Here we introduce cervical carotid tortuosity as a promising quantitative biomarker with validated, standardized characteristics. Specifically, we recommend the adoption of a curvature-based measure, total absolute curvature, for early detection or monitoring of disease progression in Loeys-Dietz syndrome.

Keywords: LDS; aortic dissection; arterial tortuosity; genetics; thoracic aortic aneurysm.

Publication types

  • Validation Study

MeSH terms

  • Adult
  • Aortic Dissection / diagnosis
  • Aortic Dissection / diagnostic imaging
  • Aortic Dissection / surgery
  • Carotid Arteries* / diagnostic imaging
  • Carotid Arteries* / pathology
  • Computed Tomography Angiography*
  • Female
  • Humans
  • Imaging, Three-Dimensional
  • Loeys-Dietz Syndrome* / complications
  • Loeys-Dietz Syndrome* / diagnosis
  • Loeys-Dietz Syndrome* / diagnostic imaging
  • Loeys-Dietz Syndrome* / genetics
  • Magnetic Resonance Angiography*
  • Male
  • Middle Aged
  • Predictive Value of Tests
  • Reproducibility of Results
  • Risk Assessment
  • Risk Factors
  • Skin Diseases, Genetic / diagnosis
  • Skin Diseases, Genetic / diagnostic imaging
  • Skin Diseases, Genetic / genetics
  • Vascular Malformations / diagnosis
  • Vascular Malformations / diagnostic imaging
  • Young Adult