中国胸心血管外科临床杂志

中国胸心血管外科临床杂志

永存动脉干的外科治疗及早中期随访

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目的 本研究旨在回顾性分析永存动脉干患儿外科矫治的早中期结果。 方法 回顾性分析 2009 年 11 月至 2018 年 5 月期间,27 例在我科接受外科治疗的永存动脉干患儿的临床资料、外科手术方法、早期死亡率及中期随访结果。其中男 14 例,女 13 例。接受手术中位年龄 3 月(范围:13 d 至 11 岁,)大于 6 月龄者 10 例(37.0%)。中位体重 5.4 kg(范围:3.2~21.2 kg)。解剖分型包括 A1 型 13 例,A2 型 12 例,A3 和 A4 型各 1 例。共干瓣形态包括三叶瓣 21 例,四叶瓣 4 例,二叶瓣 2 例;合并共干瓣反流者 11 例(40.7%),轻度 5 例,中度及以上反流 6 例。RVOT 重建方式包括:无瓣自体心包管道 14 例,REV 术后 6 例,牛颈静脉带瓣管道 7 例。同期行共干瓣成形术 6 例,二尖瓣成形术 2 例,端侧吻合重建主动脉 2 例,冠状动脉瘘矫治 1 例。 结果 手术死亡 3 例(死亡率 11.1%),30 天存活率 88.9%。手术主要并发症包括:室间隔开窗 2 例,III°AVB 1 例。存活患儿平均随访时间 24.5±19.3 月(范围:1–76 月),随访期死亡 3 例,失访 2 例。随访发现:中度及以上肺动脉狭窄 7 例,包括肺动脉主干狭窄 3 例,左右肺动脉开口狭窄各 2 例;中度主动脉瓣反流 1 例。全组暂无再次干预病例。1 年和 5 年生存率均为 76.1%(95%CI:59.2%~92.9%)。 结论 永存动脉干的治疗仍面临挑战。早期一期根治手术风险较低,早中期效果满意;而患儿年龄偏大,合并重度肺动脉高压和/或共干瓣反流者,死亡风险较高。部分患儿仍存在较高的晚期右心室流出道或左/右肺动脉分支再梗阻风险,需要密切随访。

Objective To evaluate the early- and mid-term outcomes of surgical repair of persistent truncus arteriosus in children in a single institution of china. Methods The clinical data of 27consecutive patients with persistent truncus arteriosus undergoing surgical repair in Guangzhou Women and Children’s Medical Center from November 2009 to May 2018 were retrospectively reviewed. There were 14 males and 13 females. Median age was 3.0months (range: 13 days-11 years), of which 10 (37.0%) were older than 6 months. Anatomic type included: 13 cases of type A1, 12 cases of type A2, 1 case of type A3 and one type A4. Eleven patients (40.7%) have truncal valve insufficiency, of which 6 were more than moderate. Right ventricle-pulmonary artery continuity was established with a non-valved autologous pericardial conduit (n=14), BJVC (n=7), and direct anastomosis pulmonary artery to right ventriculotomy (n=6). Results There were three early deaths with a mortality of 11.1%. The main complications include VSD partial repair in 2 cases, complete atrioventricular block in one patient. The mean follow-up time was 24.5±19.3 months (range: 1–76 months). There were three late deaths, and two patients lost follow. Echocardiology showed seven cases of right heart outflow tract obstruction, including three in pulmonary artery trunk, and four cases of pulmonary artery branches. One patient showed moderate aortic valve regurgitation. None required re-intervention during the follow-up. Survival estimates for the entire cohort following surgery were both 76.1%(95%CI: 59.2%–92.9%)at 1 year and 5 years. Conclusions The surgical repair of PTA remains challenges. The Early- and mid-term outcomes of surgical repair of persistent truncus arteriosus were acceptable. For older children with severe pulmonary artery hypertension and/or trunk valve regurgitation, the risk of death is still higher. Some children have the higher risk of late right heart obstructive lesions.

关键词: 永存动脉干; 右室流出道重建; 共干瓣反流; 早中期结果

Key words: Persistent truncus arteriosus; trunk valve regurgitation; right ventricular outflow tract reconstruction; early- and mid-term results

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