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Updated: May 28, 2025

A Methodological Approach to Non-invasive Assessments of Vascular Function and Morphology
Published on: February 7, 2015
Three-Dimensional Curvature of the Cervical Carotid Artery Predicts Long-Term Neurovascular Risk in Loeys-Dietz
Jin Vivian Lee1,2, Anna L Huguenard1, Alan C Braverman3
1Department of Neurological Surgery (J.V.L., A.L.H., R.G.D., J.W.O.), Washington University School of Medicine, St. Louis, MO.
Insights
Cervical carotid tortuosity increases neurovascular event risk in Loeys-Dietz syndrome patients, especially those under 60. High carotid total absolute curvature (TAC) is a key predictor, aiding early identification of vulnerability.
Area of Science:
- Vascular Neurology
- Genetics
- Medical Imaging
Background:
- Loeys-Dietz syndrome (LDS) is a genetic disorder affecting connective tissue.
- The link between cervical carotid tortuosity and cardiovascular risk in LDS is known, but its impact on neurovascular events is unclear.
Purpose of the Study:
- To investigate whether cervical carotid tortuosity influences the risk of neurovascular events in patients with Loeys-Dietz syndrome.
- To identify specific tortuosity features and patient demographics associated with increased neurovascular risk.
Main Methods:
- Retrospective cohort study of 63 Loeys-Dietz syndrome patients with baseline cervical and cerebral artery imaging.
- Assessment of cervical carotid tortuosity and morphology.
- Analysis of composite neurovascular events at 5- and 10-year follow-ups using logistic regression and ROC analyses.
- Stratification by age (<60 and ≥60 years).
Main Results:
- 23% of patients experienced adverse neurovascular events during follow-up.
- Carotid total absolute curvature (TAC) and coiling morphology were independently associated with 5-year events.
- High TAC (≥16.5) was a significant predictor of 5- and 10-year neurovascular events in patients <60 years old.
Conclusions:
- Cervical carotid tortuosity is linked to long-term neurovascular event risk in Loeys-Dietz syndrome.
- Increased TAC and coiling morphology are high-risk angiographic features.
- Noninvasive imaging can identify neurovascular vulnerability early in at-risk LDS patients.
Background:
Although the relationship between cervical carotid tortuosity and cardiovascular risk in patients with Loeys-Dietz syndrome has been studied, it is unclear whether cervical carotid tortuosity influences the risk of neurovascular events.
Methods:
This is a single-institution retrospective cohort study. Cervical carotid tortuosity and morphology were assessed in patients with Loeys-Dietz syndrome who underwent baseline computed tomography/magnetic resonance imaging of the cervical and cerebral arteries from 2010 to 2022. The primary end point was a composite of adverse neurovascular events (multiple vessel cervical artery dissection, ischemic stroke, intracerebral hemorrhage, and any neurovascular intervention) at 5- and 10-year follow-ups. Independent risk factors were identified using univariate and multivariate logistic regression analyses. Single-variable predictors of 5- and 10-year outcomes were analyzed via receiver operating curve analyses. Cutoff values were determined per the Youden J index. Stratification analyses were performed for ages <60 and ≥60 years.
Results:
Of 105 eligible participants, 63 were included (mean age, 40±17 years; 52% female). During a mean follow-up of 8.7±4.1 years, 23 (37%) developed an adverse neurovascular event. Five-year follow-up was achieved in 86% and 10-year follow-up in 48%. Carotid total absolute curvature (TAC; P=0.008), coiling morphology (P=0.012), and TGFBR1/2 genetic variant (P=0.037) were independently associated with 5-year events. Stratification analyses revealed that the age group <60 years was more vulnerable to high TAC (unadjusted odds ratio, 7.2 [95% CI, 2.0-25.4]; P=0.002). Baseline TAC was the only independent predictor of adverse events at 5 years (area under the curve, 0.84; P<0.001) and 10 years (area under the curve, 0.75; P=0.007) in this age group. An optimal threshold for predicting neurovascular events was TAC ≥16.5. None were predictive in the age group ≥60 years.
Conclusions:
Cervical carotid tortuosity is associated with a long-term increased risk of neurovascular events in Loeys-Dietz syndrome. Angiographic findings of high-risk features such as increased TAC and coiling morphology may help to identify neurovascular vulnerability noninvasively at an early stage.
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