2020 年第 7 期 第 15 卷

肺血管发育不良型法洛四联症分期矫治效果分析

Analysis of the effect of staging treatment of hypoplastic pulmonary artery in tetralogy of Fallot 

作者：刘健刘爱军李刚李斌苏俊武范祥明刘迎龙

英文作者：Liu Jian Liu Aijun Li Gang Li Bin Su Junwu Fan Xiangming Liu Yinglong

单位：首都医科大学附属北京安贞医院小儿心脏中心100029

英文单位：Pediatric Heart Center Beijing Anzhen Hospital Capital Medical University Beijing 100029 China 

关键词：先天性心脏病；法洛四联症；分期手术；体肺分流

英文关键词：Congenitalheartdisease；TetralogyofFallot；Stagedprocess；Aortopulmonaryshunt

• 摘要：

• 目的分析肺血管发育不良型法洛四联症（重症TOF）患者分期手术治疗效果。方法选取2010年6月至2018年6月首都医科大学附属北京安贞医院收治的行分期矫治14岁以下重症TOF患儿110例。以第1次分流手术至根治手术时间间隔将患儿分为分流效果好组（A组：间隔时间≤2年，84例）和分流效果差组（B组：间隔时间＞2年，26例）。回顾性分析患儿分流前及围术期基本资料。结果所有患儿第1次分流术后（1.6±1.1）年（0.42～7年）行根治术。99例患儿单纯行体肺分流手术，根治手术前McGoon比值、PAI指数较第1次分流术前均明显改善［（1.79±0.44）比（1.15±0.38）、（196±83）mm2/m2比（121±40）mm2/m2］（均P＜0.05）。11例患儿一次分流手术后行球囊扩张，根治手术前McGoon比值、PAI指数较球囊扩张前均明显改善［（1.84±0.39）比（1.20±0.07）、（194±93）mm2/m2比（108±21）mm2/m2］（均P＜0.05）。B组分流手术前PAI指数低于A组，根治围术期阻断时间、体外循环时间长于A组，使用球囊扩张比例高于A组（均P＜0.05），2组多次体肺分流比例、使用侧支封堵/结扎比例、呼吸机使用时间比较差异均无统计学意义（均P＞0.05）。全组死亡2例，病死率1.8%，1例死于术后早期低心排血量综合征，1例死于神经系统并发症。结论对于重症TOF患儿，单纯体肺分流术及一次分流手术后球囊扩张技术均可促进肺血管发育，可以作为肺血管反应较差患儿的有效治疗手段。分流时肺血管发育较差会影响体肺分流的效果，延长等待时间，但不影响根治手术早期效果。

• ObjectiveTo retrospectively analyze the staging surgical treatment of severe hypoplastic pulmonary artery in tetralogy of Fallot(severe TOF). Methods From June 2010 to June 2018, 110 children under 14 years old with severe TOF who were not suitable for one-stage radical treatment due to pulmonary vascular dysplasia admitted to Beijing Anzhen Hospital, Capital Medical University were enrolled. The children were divided into two groups according to the interval from the first shunt operation to the radical operation: group A (interval time≤2 years, 84 cases) with good shunt effect and group B (interval time＞2 years, 26 cases) with poor shunt effect. The basic data before and after shunt were analyzed retrospectively. Results  All the children underwent radical operation (1.6±1.1)years（0.42-7 years） after the first shunt. In 99 cases of patients who underwent only systemic pulmonary shunt, the McGoon ratio and PAI index were significantly improved before radical operation compared with those before the first shunt ［(1.79±0.44) vs (1.15±0.38), (196±83)mm2/m2 vs (121±40)mm2/m2］(all P＜0.05). Eleven children underwent balloon dilatation after one shunt operation. Before radical operation, the McGoon ratio and PAI index were significantly improved ［(1.84±0.39) vs (1.20±0.07), (194±93)mm2/m2 vs (108±21)mm2/m2］ (P＜0.05). The PAI index of group B was lower than that of group A. The perioperative block time and cardiopulmonary bypass time were longer than those of group A. The proportion of balloon dilation was higher than that in group A (both P＜0.05). There was no significant difference in the proportion of multiple shunts, occlusion / ligation ratio and ventilator use time between the two groups (all P＞0.05). In the whole group, 2 children died and the death rate was 1.8%； 1 child died of early postoperative low cardiac output syndrome； 1 child died of neurological complications. Conclusions  Aortopulmonary shunt and balloon dilation after a shunt may improve the growth of pulmonary artery effectively and can be an effective treatment for children with severe hypoplastic pulmonary artery. Severe hypoplastic pulmonary artery before shunt will affect the effect of aortopulmonary shunt and prolong the waiting time； it will not affect the early results   of radical surgery.