血管造影评估马方综合征患者主动脉根部直径和Haller指数关系的研究

Study of the relationship between the aortic root diameter and Haller index in Marfan syndrome using CT angiography

目的:通过CT血管造影术(CTA)评估马方综合征主动脉根部直径和漏斗胸的关系,寻找马方综合征患者血管及骨骼病变的关系,增加马方综合征影像诊断准确性。方法:回顾性分析我院2012年以来26例马方综合症患者的胸部CTA表现,采用双源螺旋CT进行胸腹主动脉心电门控FLASH模式描方案。应用对比剂跟踪技术启动扫描,ROI置于降主动脉近段,ROI内CT值到达100HU时延迟10s钟自动触发扫描。结果:所有病例均存在主动脉窦扩张,16例升主动脉近段扩张。发生主动脉夹层5例,其中Stanford A型夹层3例,Stanford B型夹层2例。腹主动脉瘤1例。马方组和对照组主动脉窦直径和升主动脉近段直径存在明显统计学差异(P<0.05)。马方组主动脉窦直径(r=0.925)、升主动脉近段直径(r=0.819)与Haller指数存在明显相关性。马方组主动脉窦直径(r=-0.849)、升主动脉近段直径(r=-0.796)与胸廓前后径存在明显负相关性。升主动脉中段直径与Haller指数(r=-0.063)、胸廓前后径(r=0.034)相关性不高。胸廓前后径与Haller指数存在明显负相关性(r=-0.902)。马方组患者Haller指数正常组和异常组主动脉窦直径和升主动脉近段直径存在明显统计学差异(P<0.05)。异常组Haller指数轻度异常和中重度异常患者主动脉窦直径存在明显统计学差异(P<0.05)。结论:CTA是诊断马方综合征主动脉改变重要的影像学手段,也是马方综合征术后评价及随访首选检查方法。结合Haller指数的测量,有助于马方综合征的临床评估,从而选择合适的手术适应症。

Objective： To explore the relationship between the aortic root diameter and pectus excava- turn in Marfan syndrome patients by using CTA examination. And improve the diagnostic accuracy of Marfan syndrome. Methods： We analyzed 26 CTA characteristics of Marfan syndrome patients retrospectively since 2008. CTA exams were performed with ECG-gated FLASH scan mode by Dual-source CT. The scan is triggered by bolus tracking technique. The ROI is placed at the proximal segment of the descending aorta and CT scan was triggered after 10s as CT value of ROI reached 100HU. Results： Among all the 26 Marfan syndrome pa- tients, aortic root dilated in all patients, and proximal ascending aorta dilated in 16 patients. Aortic dissection happened in 5 patients, including 3 Stanford A aortic dissection and 2 Stanford B aortic dissection, in addition, abdominal aortic aneurysm in 1 patient. There were significant difference of aortic sinus diameter, proximal as- cending aorta diameter between Marfan group and control group （ P 〈 0. 05 ）. Good correlation were generatedbetween aortic sinus diameter（ r = 0. 925 ）, proximal segment of the ascending aorta diameter （ r = 0. 819） and Hailer Index. Negative correlation were existed between aortic sinus diameter（ r= -0. 849）, proximal ascend- ing aorta diameter（r = -0. 796） and thoracic cage anterior-posterior diameter. No significant correlation were found between middle segment of the ascending aorta diameter and Hailer Index（ r = -0. 063 ）, thoracic cage anterior-posterior diameter（r =0. 034）. Negative correlation was found between Hailer Index and thoracic cage anterior-posterior diameter（ r = -0. 902）. There were significant differences in the diameter of the aortic sinus and the proximal ascending aorta between the normal group and the abnormal group of Hailer index （P 〈 0. 05 ）. There were significant differences in the diameter of aortic sinus between the slight abnormal group and the moderate to severe abnormal group of Haller index in Marian patients （ P 〈 0. 05 ）. Conclusion： CTA is an important image modality for Maffan syndrome diagnosis, post-operative evaluation and follow-up. It is valuable by using Hailer Index measurement in Marian syndrome with CTA to make accurate diagnosis and choose proper management.