法洛四联症合并一侧肺动脉缺如的手术治疗

Surgical Treatment of Tetralogy of Fallot with Unilateral Absence of Pulmonary Artery

目的报告法洛四联症合并一侧肺动脉缺如的外科治疗。方法８例法洛四联症合并一侧肺动脉缺如患者，左侧肺动脉缺如７例，右侧肺动脉缺如１例；其中２例肺动脉闭锁。６例行一期根治术，２例先行体－肺动脉分流术，再分别于术后８个月和１１个月行二期根治术。６例应用带单瓣补片跨瓣加宽右心室流出道，２例采用右心室到肺动脉带瓣管道。结果全组术后出现呼吸衰竭２例，低心排血量综合征３例；其中１例死于心力衰竭，死亡率１２．５％。７例长期存活者术后随访０．５～５．０年，平均２．５６年，无晚期死亡，心功能Ⅰ级６例，心功能Ⅱ级１例。结论法洛四联症合并一侧肺动脉缺如根治术时，必须严格掌握手术适应证。

Objective To report the surgical treatment of tetralogy of Fallot with unilateral absence of a pulmonary artery. Methods There were 8 patients between the age of 1.5 to 13 years who suffered from tetralogy of Fallot with unilateral absence of a pulmonary artery. Among them, seven patients had absence of left pulmonary artery with pulmonary atresia in 2 and one absence of right pulmonary artery. The one stage corrective procedures were performed in 6 cases, Two cases had systemic pulmonary arterial shunting and then received second stage complete repair 8 and 11 months after first stage shunting respectively. The single valve patches were used to widen the right ventricular outflow tract in 6 patients requiring transannular enlargement. Right ventriculo pulmonary artery anastomosis with interposed valved conduits were used in 2 patients with pulmonary atresia. Results The postoperative complications included respiratory failure in 2 patients and low cardiac output syndrome in 3. There was one hospital death with mortality of 12.5%. Seven survivors were followed up from 0.5 to 5.0 years (mean 2.56 years). There was no late death. Cardiac function was class Ⅰ in 6 patients and class Ⅱ in 1. Conclusion The operative indications should be seriously elected when the corrective procedures of tetralogy of Fallot with unilateral absence of a pulmonary artery is performed. The patches with single valve should be used for patients requiring transannular enlargement.