右房异构单心室伴完全性肺静脉异位引流的外科治疗

Surgical treatment of single ventricle and total anomalous pulmonary venous connection with right atrial isomerism.

目的 介绍右房异构、单心室伴完全性肺静脉异位引流 (TAPVC)的外科治疗经验和TAPVC在右房异构纠治手术中意义。方法 1999年 6月至 2 0 0 0年 3月手术治疗右房异构、单心室伴TAPVC 5例。其中 4例为心上型 ,1例为混合型。所有病儿均施行一侧或双侧的双向腔肺血管吻合术(BCPC) ,4例同时进行TAPVC纠治术。结果 手术死亡 1例 ,原因为术前诊断不明确、体外循环时间过长、术后严重低心排和低氧血症。生存 4例术后均无残余解剖梗阻 ,术后血流动力学稳定 ,血氧饱和度明显增高 ,随访结果满意。结论 术前明确TAPVC的诊断对指导手术具有重要意义 ;右房异构、单心室伴TAPVC在进行分流手术时应当同时施行TAPVC纠治术。

Objective: To present the experience of surgical treatment of single ventricle and total anomalous pulmonary venous connection (TAPVC) with right atrial isomerism, and demonstrate the significance of TAPVC to the surgical treatment in the right atrial isomerism. Methods: Between June 1999 and March 2000, five patients with right atrial isomerism, single ventricle and TAPVC underwent surgical treatment.There were four patients with supracardiac type and one with mixed TAPVC. The forward flow from the ventricle to the pulmonary arteries was preserved in three cases. Results: Uncertain diagnosis before the operation, prolonged cardiopulmonary bypass,severe low cardiac output and hypoxmia led to one operature death. Hemodynamics of survivals were stable after the operation,and postoperative saturation of oxygen increased significantly. The results in the follow-up were satisfactory.Conclusions: The diagnosis of TAPVC before surgery is very important to the operation in the right atrial isomerism.Concomitant TAPVC repair should be performed in the palliative operation. The role of the presence of forward flow from the ventricle to the pulmonary need further investigation.