完全性肺静脉异位引流的外科治疗

Surgical treatment of total anomalous pulmonary venous drainage

目的探讨完全性肺静脉异位引流(TAPVD)的外科治疗及围术期处理的经验。方法选择经手术治疗完全性肺静脉异位引流患者37例。男25例,女l2例;年龄1.5～32岁;心上型TAPVD23例、心内型l3例、心下型1例;超声心动图证实完全性肺静脉异位引流33例;行心导管检查l2例;多排螺旋CT(MDCT)检查2例。心上型采用心脏上翻法3例,双心房横切口12例,左房顶部入路3例。肺总静脉引流入上腔静脉5例,作上腔静脉至右心房纵切口。心内型经右房斜切口,用较大的自体心包片修补房间隔缺损,将冠状静脉窦、肺静脉口隔入左心房。1例心下型,用心脏上翻方法将共同肺静脉干与左心房吻合。术中根据情况放置临时心外膜起搏导线。结果全组无手术死亡,并发心律失常9例,低心排综合征3例,经治疗均痊愈。随访2～l6年,心功能NYHAⅠ级34例,Ⅱ级3例。无心律失常、无吻合口狭窄及肺静脉梗阻发生。结论术前明确诊断对设计手术方案尤为重要,超声心动图可以明确诊断,必要时行心血管照影及MDCT检查。本病确诊后应尽早手术,手术的关键是吻合口够大,血流通畅,防止肺静脉梗阻。良好的围手术期处理是手术成功的保证。

Objective To summarize the experiences of surgical treatment and perioperative management of total anomalous pulmonary venous drainage（TAPVD）. Methods Thirty-seven consecutive patients with TAPVD underwent corrective operations. There were 25 males and 12 females and their age ranged from 1.5 to 32 years old. Twenty-three of them were supracardiac type,13 were intracardiac type and 1 were infracardiac type. Thirty-three cases were confirmed by Echocardiography, 12 cases were diagnosed by Catheterization and 2 were diagnosed based on Multidectector comography （MDCT）. For the supracardiac type of TAPVD, 3 cases were corrected by flipping up the heart, 12 cases by making biatrial transverse incision, 3cases by top approach of the left atrium, 5 cases by draining the common pulmonary venous to the superior cava vena, vertical incision was made from superior vena cava to right atrium. For the intracardiac type of TAPVD, the anomaly was corrected via the right atrium oblique incision, the atrial septal defect was repaired with a larger self-pericardial patch, while the coronary sinus and the pulmonary vein inlet were boarded to the left atrium. One patient with infracardiac type underwent anastomoses between the left atrium and the common pulmonary vein with the heart on the turn. Temporary epicardium pacing line was placed accordingly. Results There was no operative death in all patients. Major post-operative complications of arrhythmia occurred in 9 cases. Three cases with low cardiac output syndrome were fully recovered after treatment. The postoperative followed-up period ranged from 2 to 16 years. Thirty-fourof these patients were in NYHA function class Ⅰ, three in class Ⅱ. Conclusion Accurate pre-operative diagnosis is very important for making appropriate surgery strategy. The diagnosis of TAPVD can be confirmed by Echocardiography, Angiocardiogra- phy and MDCT may be performed when needed. Surgical operation should be applied as soon as the disease is diagnosed. The key point of the surgery is to make sure that the stoma is large enough so that blood flows smoothly in order to avoid pulmonary venous obstruction. Perfect peri-operative management is the guarantee of successful surgery.