Texas Children's Hospital, 6621 Fannin Street, Houston, TX 77030, USA.
Circulation. 2011 Jul 26;124(4):388-96. doi: 10.1161/CIRCULATIONAHA.110.990549. Epub 2011 Jul 5.
Arterial tortuosity is described as a common and distinctive feature of Loeys-Dietz syndrome (LDS), yet reports on arterial tortuosity are based on qualitative observations and none have investigated an association between tortuosity and cardiovascular outcomes in LDS or other connective tissue disorders.
We performed a retrospective analysis of 90 patients ≤50 years of age with Marfan syndrome, LDS, Ehlers-Danlos syndrome, or nonspecific connective tissue disorder who underwent thoracic contrast-enhanced magnetic resonance angiography. Controls (n=30) underwent magnetic resonance imaging to exclude arrhythmogenic right ventricular dysplasia. Using a volume-rendered angiogram, vertebral arteries were measured along the curvature of the vessel (actual length) and linearly (straight length), and distance factor was calculated: [(actual/straight length-1)×100]. Each subject's maximum distance factor was designated the Vertebral Tortuosity Index (VTI). The VTI was compared among diagnostic groups and among patients with cardiac surgery, dissection, and death. Median age at magnetic resonance imaging was 19.6 years (range 0.2 to 50.1). VTI interrater reliability was excellent (intraclass correlation coefficient =0.987). The VTI was higher in Marfan syndrome (n=57, median 26; interquartile range 10 to 49) and LDS (n=13, median 58; interquartile range 18 to 92) compared with controls (median 4.5; interquartile range 3 to 6; P<0.001 for both). Higher VTI was associated with younger age at surgery even when controlling for root size (adjusted P=0.002). Vertebral tortuosity index ≥50 was associated with earlier age at dissection and death compared with VTI <50 (P=0.001 versus P<0.001). We found no difference in age at surgery, dissection, or death in Marfan syndrome compared with LDS.
Arterial tortuosity measured by magnetic resonance angiography is a reproducible marker of adverse cardiovascular outcomes in connective tissue disorders.
动脉迂曲是洛伊茨-迪茨综合征（LDS）的一个常见且独特的特征，但关于动脉迂曲的报告都是基于定性观察，没有研究过迂曲与 LDS 或其他结缔组织疾病的心血管结局之间的关系。
我们对 90 名≤50 岁的马凡综合征、LDS、埃勒斯-当洛斯综合征或非特异性结缔组织疾病患者进行了回顾性分析，这些患者均接受了胸主动脉对比增强磁共振血管造影检查。对照组（n=30）接受磁共振成像检查以排除致心律失常性右心室发育不良。使用容积再现血管造影，沿血管的弯曲（实际长度）和直线（直线长度）测量椎动脉，并计算距离因子：[（实际/直线长度-1）×100]。每位患者的最大距离因子被指定为椎动脉迂曲指数（VTI）。比较诊断组之间以及有心脏手术、夹层和死亡的患者之间的 VTI。磁共振成像时的中位年龄为 19.6 岁（范围 0.2 至 50.1）。VTI 的观察者间可靠性极好（组内相关系数=0.987）。马凡综合征（n=57，中位数 26；四分位间距 10 至 49）和 LDS（n=13，中位数 58；四分位间距 18 至 92）患者的 VTI 高于对照组（中位数 4.5；四分位间距 3 至 6；两者均 P<0.001）。即使在控制了根部大小的情况下，较高的 VTI 与手术时年龄较小相关（校正后 P=0.002）。与 VTI<50 相比，VTI≥50 与夹层和死亡的更早年龄相关（P=0.001 与 P<0.001）。我们没有发现马凡综合征与 LDS 相比，在手术、夹层或死亡的年龄上有差异。
磁共振血管造影测量的动脉迂曲是结缔组织疾病不良心血管结局的一个可重复的标志物。
