完全性肺静脉异位引流的CT诊断及合并畸形特点

CT diagnosis of total anomalous pulmonary venous connection and characteristics of coexistent malformation

目的 :提高对完全性肺静脉异位引流(Total anomalous pulmonary venous connection,TAPVC)及其合并畸形的认识。方法:回顾性分析70例TAPVC患儿的CT血管造影表现,并总结其合并畸形。结果:70例TAPVC中,心上型42例(60%),心内型13例(18.6%),心下型8例(11.4%),混合型7例(10%)。TAPVC的合并畸形可为合并单一畸形,也可为合并多种畸形。70例TAPVC的合并畸形包括:房间隔缺损70例(100%),动脉导管未闭31例(44.3%),永存左上腔静脉5例(7.1%),主动脉缩窄及肺动脉狭窄各3例(4.3%),肺动脉闭锁、单心室、右位心、室间隔缺损、迷走右锁骨下动脉各2例(2.9%),完全性心内膜垫缺损、单心房、无顶冠状窦、主肺动脉窗及右心室双出口各1例(1.4%)。除外合并房间隔缺损及动脉导管未闭,仅11例(15.7%)患儿合并有其它心血管畸形,其中3例(4.3%)患儿合并有5种及以上复杂畸形。结论:TAPVC形态学多样,影像表现复杂,其最常见的合并畸形为房间隔缺损及动脉导管未闭,呈集中并发趋势,其它合并畸形少见,但涵盖种类多样。

Objective: To improve the understanding of total anomalous pulmonary venous connection(TAPVC) and its coexistent malformation. Methods: CT angiography data of 70 patients with TAPVC were retrospectively analyzed, and the coexistent malformation of TAPVC were summarized. Results: Of 70 patients with TAPVC, 42 cases were supracardiac type, 13 cases were cardiac type, 8 cases were infracardiac type, and 7 cases were mixed type. The coexistent malformation of TAPVC can be single or multiple malformation. The coexistent malformation of 70 patients with TAPVC included: atrial septal defect70 cases(100%), patent ductus arteriosus 31 cases(44.3%), persistent left superior vena cava 5 cases(7.1%), coarctation of aorta and pulmonary valve stenosis 3 cases(4.3%) respectively, pulmonary atresia, single ventricle, dextrocardia, ventricular septal defect and aberrant right subclavian artery 2 cases(2.9%) respectively, endocardial cushions defect, single atrium, unroofed coronary sinus, aortopulmonary window and double outlet right ventricle 1 case(1.4%) respectively. In addition to the patients combined with atrial septal defect and patent ductus arteriosus, only 11 cases(15.7%) combined with other cardiovascular coexistent malformations. Three cases(4.3%) combined with five or more complex coexistent malformations. Conclusion: The morphology and CT findings of TAPVC were complex. The most common coexistent malformations of TAPVC were atrial septal defect and patent ductus arteriosus, which have the tendency of centralized concurrency. Other coexistent malformations were rare, but the types were diversified.