Yoshihara K, Ozawa T, Sakuragawa H, Fujii T, Shiono N, Watanabe Y, Koyama N, Matsuura H, Saji T, Takanashi Y
Department of Thoracic Cardio Vascular Surgery, Toho University School of Medicine, Tokyo, Japan.
Jpn J Thorac Cardiovasc Surg. 1998 Nov;46(11):1126-32. doi: 10.1007/BF03217887.
During November 1986 and May 1997, 19 patients with total anomalous pulmonary venous connection (TAPVC) underwent repair surgery. 20 operations including two reoperations were performed. 8 of 19 patients were classified as Darling type Ia, 5 as type IIa, 4 as type III and 2 patients were type IV. Two patients were operated under emergency circumstances within 24 hours after admission, 7 patients were after a short term stabilization of 4.4 days, and the other 11 patients received surgical treatment after a mean of 8.8 days as scheduled cases. For the anostomosis, the common pulmonary venous chamber or the vertical vein was connected with the left atrium in type Ia and III cases; in type IIa and IV cases the cut-back method was performed. Persistent pulmonary hypertension and post-operative pulmonary venous obstruction (PVO) affected the post-operative clinical course. Persistent pulmonary hypertension caused the death of one patient with type IIa and III each, just after operation. One type IV patient died 50 days after operation. The autopsy revealed post-operative obstructions of the remote parts of the pulmonary veins on the anostomosis site. Two patients (type IIa, III) successfully underwent reoperation due to PVO. Post-operative cardiac catheterization was performed after 12 month in 12 cases. Persistent pulmonary hypertension was found in 4 patients, and a type III patient was reoperated because of stenosis of the anostomosis site. The other three patients had persistent pulmonary hypertension without any demonstrable PVO. Persistent pulmonary hypertension and PVO are combined as TAPVC complex. The difficulty to reoperated patients with persistent pulmonary hypertension caused by PVO is one major problem. So preoperative prevention of PVO by normalization the morphologic changes of the pulmonary veins by using drugs could be a different view point in TAPVC therapy after the initial operation.
1986年11月至1997年5月期间，19例完全性肺静脉异位连接（TAPVC）患者接受了修复手术。共进行了20次手术，其中包括2次再次手术。19例患者中，8例被归类为达林（Darling）Ia型，5例为IIa型，4例为III型，2例为IV型。2例患者在入院后24小时内接受了急诊手术，7例患者在短期稳定4.4天后接受手术，另外11例患者作为择期病例在平均8.8天后接受了手术治疗。对于吻合术，Ia型和III型病例中，将共同肺静脉腔或垂直静脉与左心房相连；IIa型和IV型病例则采用折返法。持续性肺动脉高压和术后肺静脉梗阻（PVO）影响了术后临床病程。持续性肺动脉高压导致1例IIa型和1例III型患者术后即刻死亡。1例IV型患者术后50天死亡。尸检显示吻合部位肺静脉远端术后梗阻。2例患者（IIa型、III型）因PVO成功接受了再次手术。12例患者在术后12个月进行了心导管检查。4例患者发现持续性肺动脉高压，1例III型患者因吻合部位狭窄接受了再次手术。另外3例患者有持续性肺动脉高压，但未发现明显的PVO。持续性肺动脉高压和PVO合并为TAPVC综合征。因PVO导致持续性肺动脉高压的患者再次手术困难是一个主要问题。因此，术前通过药物使肺静脉形态改变正常化以预防PVO，可能是TAPVC初次手术后治疗的一个不同观点。
