2020 年第 8 期 第 15 卷

主动脉弓离断合并中重度肺动脉高压患儿一期外科手术治疗效果分析

Analysis of the effect of one-stage surgical treatment in children with aortic arch disconnection and moderate to severe pulmonary hypertension

作者：刘爱军李斌杨明程沛范祥明苏俊武

英文作者：Liu Aijun Li Bin Yang Ming Cheng Pei Fan Xiangming Su Junwu 

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：Department of Pediatric Heart Center Beijing Anzhen Hospital Capital Medical University Beijing 100029 China

关键词：主动脉弓离断；动脉导管未闭；肺动脉高压

英文关键词：Interruptedaorticarch；Patentductusarteriosus；Pulmonaryhypertension

• 摘要：

• 目的 分析主动脉弓离断（IAA）合并中重度肺动脉高压患儿一期外科手术治疗效果。方法 收集2010年1月至2017年9月在首都医科大学附属北京安贞医院小儿心脏中心行一期外科手术矫治的合并中重度肺动脉高压的IAA患儿33例病历资料。23例患儿合并重度肺动脉高压，口服肺动脉靶向药物治疗，下肢经皮血氧饱和度至少上升5%后手术。行端侧吻合21例，后壁端侧吻合+前壁心包片加宽12例。随访2～9年，分析治疗效果。结果 术后死亡2例（6.1%），其中术后17 d（出院后第7天）、术后4个月各死亡1例。出院时、术后6个月及术后2年主动脉吻合口压差分别为（17±11）、（15±8）、（14±7）mmHg(1 mmHg=0.133 kPa)，不同时点间差异无统计学意义（P=0.152）。术后6个月及术后2年患儿平均肺动脉压力均明显低于术前［（38±7）、（24±5）mmHg比（68±8）mmHg］，术后6个月患儿肺血管阻力明显低于术前［（493±248）dyn·s/cm5比（1 635±352）dyn·s/cm5］（均P＜0.05）。结论 一期外科手术治疗大龄IAA合并中重度肺动脉高压可以取得满意的手术疗效。围术期应用靶向药物降低肺动脉压力，选择恰当的手术方式是降低死亡率和防治术后吻合口狭窄的有效手段。

• Objective To analyze the effect of one-stage surgical treatment on children with aortic arch dissection (IAA) combined with moderate to severe pulmonary hypertension. Methods From January 2010 to September 2017, 33 children with IAA combined with moderate and severe pulmonary hypertension undergoing one-stage surgical treatment admitted to  Pediatric Heart Center, Beijing Anzhen Hospital, Capital Medical University were collected. Twenty-three children with severe pulmonary hypertension were treated by oral pulmonary artery targeting drugs after percutaneous oxygen saturation of the lower limbs increased 5% at least, the patient was considered operable. There were 21 cases of end-to-side anastomosis, and 12 cases of posterior end-to-side anastomosis+anterior wall pericardium widening. Followed up for 2-9 years, the treatment effect was analyzed. Results There were 2 cases (6.1%) died after operation, including 1 case died in 17 days (7 days after discharge) and 1 case died in 4 months after operation. At the time of discharge, 6 months after operation and 2 years after operation, the pressure difference of aortic anastomosis were (17±11), (15±8), (14±7)mmHg respectively, and there was no significant difference among the different time points (P=0.152). The mean pulmonary artery pressure at 6 months and 2 years after operation was significantly lower than that before operation ［(38±7), (24±5)mmHg vs (68±8)mmHg］, and the pulmonary vascular resistance at 6 months after operation was significantly lower than that before operation［（493±248）dyn·s/cm5 vs （1 635±352）dyn·s/cm5］(all P＜0.05). Conclusions One-stage surgical treatment of patients with IAA and moderate-severe pulmonary hypertension can achieve satisfactory surgical Results . The application of targeted drugs in perioperative period to reduce pulmonary artery pressure and the selection of appropriate surgical Methods  are effective means to reduce mortality and prevent postoperative anastomotic stenosis.