完全性肺静脉畸形连接于右上腔静脉的放射诊断

Radiological diagnosis of total anomalous pulmonary venous connection with right superior vena cava

目的 研究总结连接于右上腔静脉的完全性肺静脉畸形连接 (RSVC TAPVC)X线平片的征象及其价值 ,以及畸形连接肺静脉的变异及对手术的影响。方法 回顾性研究 1982～ 1998年收治并经手术证实的RSVC TAPVC 11例。逐个分析其X线平片征象并与心血管造影及手术结果对照 ,找出其征象形成的病理基础 ,以及肺静脉畸形连接的变异情况及其对手术的影响。结果 X线平片呈“右半头雪人征”者 6例 ,4例有右上纵隔旁增宽引流静脉影 ;引流静脉或肺静脉狭窄者 6例 ;右肺静脉汇合成 1支、2支或 3支引入引流静脉 (或称连接静脉 )者分别为 2、4及 3例。11例手术中死亡 3例 ,2例与右肺静脉连接变异有关。结论 X线平片对RSVC TAPVC的诊断有一定价值 ,大多数病例可作诊断。

Objective To evaluate radiographic diagnosis in total anomalous pulmonary venous connection with right superior vena cava (RSVC TAPVC) and analyze the variant of its pulmonary venous connection and the influence on surgical operation. Methods In 11 patients who were diagnosed by angiocardiography and confirmed by surgical operation, all chest radiographs were studied retrospectively and compared with their angiocardiography and surgical results. The variant of pulmonary venous connection was analyzed and the influence on surgical operation was also discussed. Results 6 patients showed “right hemi snowman sign” in posteroanterior view and 4 showed a pretracheal density on the lateral view or other sign caused by drainage vein. 6 of 9 angiocardiographs showed pulmonary or drainage venous stenosis. There were 2, 4 and 3 cases respectively whose right pulmonary veins connected with common pulmonary or drainage veins in one, two, and three branches. 2 of 3 died cases were caused by mistaken ligation of right superior pulmonary vein during the operation. Conclusion About 82% cases of the RSVC TAPVC can be diagnosed by chest radiograph because of the “right hemi snowmen sign” and other supplementary signs. In about 78% cases, the right pulmonary veins join right drainage (or connecting) vein in two or more branches at separate levels and this variant has surgical implications.