无脾综合征并发先天性心脏病心脏CT血管成像诊断  

Cardiac CT angiography diagnosis of asplenia syndrome combining congenital heart disease

作　　者：刘志[1] 高剑波[2] 张斯佳[1] 陈勇[1] LIU Zhi;GAO Jianbo;ZHANG Sijia;CHEN Yong(Department of Radiology,the People's Hospital of Zhengzhou,Zhengzhou 450003;Department of Radiology,the First Affiliated Hospital of Zhengzhou University,Zhengzhou 450052,China)

机构地区：[1]郑州人民医院医学影像科,河南郑州450003 [2]郑州大学第一附属医院放射科,河南郑州450052

出　　处：《实用放射学杂志》2021年第5期835-837,853,共4页Journal of Practical Radiology

摘　　要：目的分析无脾综合征并发先天性心脏病患者的心脏CT血管成像(CTA)表现.方法回顾性分析10例无脾综合征并发先天性心脏病患者的心脏CTA图像.结果10例患者均为复杂性先天性心脏病,CTA表现为同时并发单心房、单心室、完全型心内膜垫缺损、肺动脉狭窄,7例(70%)伴有右位主动脉弓,4例(40%)伴有大动脉转位,5例(50%)伴有右室双出口;5例(50%)伴有永存左上腔静脉,6例(60%)伴有肺静脉异位引流.结论无脾综合征并发先天性心脏病以同时并发单心房、单心室、心内膜垫缺损及肺动脉狭窄为主,同时伴有右位主动脉弓、大动脉转位、右心室双出口、永存左上腔静脉及肺静脉异位引流中1种或多种畸形,心脏CTA检查能提供比较全面、准确的诊断.Objective To study the cardiac CT angiography(CTA)findings of patients with asplenia syndrome combining congenital heart disease.Methods Cardiac CTA images of 10 patients with asplenia syndrome combining congenital heart disease were analyzed retrospectively.Results The 10 cases were complicated with complex congenital heart disease,all including single atrium,single ventricle,complete endocardial cushion defect and pulmonary stenosis.7 cases(70%)had right aortic arch,4 cases(40%)had transposition of the great arteries,5 cases(50%)had double outlet right ventricle,5 cases(50%)had permanent left superior vena cava,and 6 cases(60%)had anomalous pulmonary venous connection.Conclusion Asplenia syndrome combining with congenital heart disease is mainly complicated by single atrium,single ventricle,endocardial cushion defect and pulmonary artery stenosis,and combined with one or more malformations in right aortic arch,transposition of aorta,double outlet of right ventricle,permanent left superior vena cava and anomalous pulmonary venous connection.Cardiac CTA can provide a more comprehensive and accurate diagnosis.

关 键 词：无脾综合征 先天性心脏病 计算机体层成像 

分 类 号：R551.1[医药卫生—血液循环系统疾病] R541.1[医药卫生—内科学]