Abstract：
BACKGROUND: Cervical arterial tortuosity is associated with adverse outcomes in Loeys-Dietz syndrome and other heritable aortopathies.
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.
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.
摘要：
背景：颈动脉弯曲与Loeys-Dietz综合征和其他遗传性主动脉病变的不良结局相关。
结果：开发了一种基于三维空间中血管中心线曲率评估弯曲度的方法。我们从基线计算机断层扫描血管造影/磁共振血管造影和随访期间的所有系列图像中测量了65例Loeys-Dietz综合征患者的颈部颈动脉弯曲。基线颈动脉弯曲之间的关系，年龄,主动脉根部直径,并对其随时间的变化进行了比较。对未手术的主动脉根部患者进行临床终点评估（随访4年的A型主动脉夹层或主动脉根部手术）。根据基线颈动脉弯曲进行Logistic回归以评估临床终点的可能性。基线时的总绝对曲率为11.13±5.76，在8至10年时相对不变（倍数变化：0.026±0.298，P=1.00），而基线时弯曲指数为0.262±0.131,8~10年时变异性更大(倍数变化:0.302±0.656,P=0.818).基线总绝对曲率与主动脉根部直径相关（r=0.456，P=0.004），并且在4年随访期间与主动脉事件独立相关（调整后的比值比[OR]，2.64[95%CI，1.02-6.85]）。基线弯曲指数与年龄相关（r=0.532，P<0.001），与事件无关（校正OR，1.88[95%CI，0.79-4.51]）。最后,基线总绝对曲率对4年结局具有良好的区分度（曲线下面积=0.724，P=0.014），这可能是预后或预测。
结论：在这里，我们介绍颈部颈动脉弯曲作为一种有希望的定量生物标志物，标准化特征。具体来说,我们建议采用基于曲率的措施，总绝对曲率,用于早期发现或监测Loeys-Dietz综合征的疾病进展。
结果：开发了一种基于三维空间中血管中心线曲率评估弯曲度的方法。我们从基线计算机断层扫描血管造影/磁共振血管造影和随访期间的所有系列图像中测量了65例Loeys-Dietz综合征患者的颈部颈动脉弯曲。基线颈动脉弯曲之间的关系，年龄,主动脉根部直径,并对其随时间的变化进行了比较。对未手术的主动脉根部患者进行临床终点评估（随访4年的A型主动脉夹层或主动脉根部手术）。根据基线颈动脉弯曲进行Logistic回归以评估临床终点的可能性。基线时的总绝对曲率为11.13±5.76，在8至10年时相对不变（倍数变化：0.026±0.298，P=1.00），而基线时弯曲指数为0.262±0.131,8~10年时变异性更大(倍数变化:0.302±0.656,P=0.818).基线总绝对曲率与主动脉根部直径相关（r=0.456，P=0.004），并且在4年随访期间与主动脉事件独立相关（调整后的比值比[OR]，2.64[95%CI，1.02-6.85]）。基线弯曲指数与年龄相关（r=0.532，P<0.001），与事件无关（校正OR，1.88[95%CI，0.79-4.51]）。最后,基线总绝对曲率对4年结局具有良好的区分度（曲线下面积=0.724，P=0.014），这可能是预后或预测。
结论：在这里，我们介绍颈部颈动脉弯曲作为一种有希望的定量生物标志物，标准化特征。具体来说,我们建议采用基于曲率的措施，总绝对曲率,用于早期发现或监测Loeys-Dietz综合征的疾病进展。
