肺动脉闭锁合并室间隔缺损行肺动脉下拉重建右心室流出道

目的明确肺动脉闭锁合并室间隔缺损行肺动脉下拉重建右心室流出道后肺动脉发育、瓣膜反流及右心功能情况。方法回顾性分析2002年11月至2012年9月广东省人民医院31例肺动脉闭锁合并室间隔缺损患者的临床资料，其中男20例，女11例；患儿手术时14d～14．50岁（47．90±53．84）个月，均采用肺动脉下拉重建右心室流出道。随访评估其吻合口及肺动脉发育情况、肺动脉与三尖瓣反流及其与随访时间的相关关系；采用右心室应变等指标评估右心功能。结果术后早期死亡3例。随访27例（27／31，87．1％），随访时间4～129（35．97±28．24）个月。随访期间无死亡。根治术后元吻合口再狭窄，姑息术后吻合口相对狭窄4例。吻合口及左、右肺动脉的直径均明显大于术后早期；肺动脉反流及i尖瓣反流加重，但肺动脉反流量与随访时间无相关关系。远期右心功能整体良好。结论肺动脉闭锁合并室间隔缺损行肺动脉下拉重建右心室流出道效果良好，吻合口及肺动脉分支可生长发育，术后中远期右心功能良好。

Right Ventricle-pulmonary Anastomosis for Right Ventricle Outflow Reconstruction in Patients with Pulmonary Atresia and Ventricular Septal Defect

Objective To investigate pulmonary artery growth, valvular regurgitation and right heart function after right ventricle-pulmonary artery （RV-PA） anastomosis for right ventricle outflow （RVOT） reconstruction in patients with different types of pulmonary atresia and ventricle septal defect （PA/VSD）. Methods Clinical data of 31 PA/VSD patients who underwent right ventricle-pulmonary anastomosis for RVOT reconstruction from November 2002 to September 2012 in Guangdong General Hospital were retrospectively analyzed. There were 20 male and 11 female patients with their age ranging from 14 days to 14.50 years （47.90＋53.84 months）. Patients were followed up after discharge to evaluate the anastomasis, pulmonary artery growth, pulmonary artery and tricuspid regurgitation and their relationship with follow-up duration. Right ventrieular strain and other eehocardiography indexes were used to evaluate right heart function. Results Three patients died postoperatively. Twenty-seven patients （ 27/31, 87.1% ） were followed up for 4-129 （ 35.97 ± 28.24 ） months. There was no late death during follow-up. There was no anastomotic restenosis in patients who received radical surgery. Four patients who received palliative surgery had comparative anastomotic restenosis. The diameters of RV-PA anastomasis, left and right branches of the pulmonary artery during follow-up were significantly larger than early postoperative diameters （P 〈 0.05）. Echocardiography showed that pulmonary artery and tricuspid regurgitation were aggravated, which was not correlated to follow-up duration. Overall right heart function was good during follow-up. Conclusions Clinical outcomes of RV-PA anastomosis for RVOT reconstruction in patients with PA/VSD are satisfactory. RV-PA anastomasis, left and right branches of the pulmonary artery can keep their growth and development. Mid-term and long-term right heart function is good.