镶嵌治疗室间隔完整型肺动脉膜性闭锁及重度肺动脉瓣狭窄  

Hybrid treatment of pulmonary atresia with intact ventricular septum and critical pulmonary stenosis by transesophageal echocardiographic guidance

作　　者：卢绪宁 文平 刘宇航 刘启龙 赵烨 崔琳 刘大为 朱全伟 王宁 Lu Xuning;Wen Ping;Liu Yuhang;Liu Qilong;Zhao Ye;Cui Lin;Liu Dawei;Zhu Quanwei;Wang Ning(Heart Center, Affiliated Children's Hospital, Dalian Medical University, Dalian 116012, China)

机构地区：[1]大连医科大学附属大连市儿童医院心脏中心,116012

出　　处：《中华小儿外科杂志》2019年第9期801-805,共5页Chinese Journal of Pediatric Surgery

摘　　要：目的探讨镶嵌治疗室间隔完整型肺动脉膜性闭锁及重度肺动脉瓣狭窄的安全性及有效性。方法回顾性分析大连市儿童医院心脏中心2015年3月至2018年2月收治的室间隔完整型肺动脉膜性闭锁及重度肺动脉瓣狭窄新生儿14例的临床资料。其中,男8例,女6例;年龄1h^23d;入院体重(3.6±0.7)kg,范围在2.8~4.9kg。所有患儿均经胸部正中部分切开胸骨,于右室肺动脉瓣环下方约15~20mm处缝一荷包,在食道超声引导下穿刺通过肺动脉瓣建立轨道,选择1~1.3倍于肺动脉瓣环的球囊,进行瓣膜扩张。附加手术为非体外循环下改良体肺动脉分流术、动脉导管结扎术。结果术后所有患儿均入重症监护室继续治疗,呼吸机辅助时间为(1.7±1.6)d,监护室停留时间为(3.9±2.0)d,术后住院时间为(6.9±2.2)d。全组死亡1例,考虑由于结扎动脉导管,未行改良体肺动脉分流术;余者无围手术期并发症发生。术后经皮氧饱和度为(89.2±7.0)%。随访2~23个月,4例患儿残存轻度肺动脉瓣狭窄,2例肺动脉瓣轻度反流,三尖瓣反流较术前明显减轻,8例已完成双心室根治手术。结论镶嵌治疗室间隔完整型肺动脉膜性闭锁及重度肺动脉瓣狭窄安全有效,无论动脉导管是否开放,在新生儿期进行,可以促进右心室的发育,为二期双心室根治创造条件。Objective To explore the efficacy and safety of hybrid treatment of pulmonary atresia with intact ventricular septum and critical pulmonary stenosis under transesophageal echocardiographic guidance. Methods A total of 14 neonates with pulmonary atresia with intact ventricular septum and critical pulmonary stenosis were selected. There were 8 boys and 6 girls with a body weight of (2.8-4.9)(3.6±0.7) kg and an age range of 1 hour to 23 days. All right ventricles grew well and tricuspid valve Z score -2-0.5 (-0.8±0.8) on transthoracic and transesophageal echocardiography. The diameter of pulmonary annulus was (6.5-8.0)(7.3±0.6) mm and the balloon sizes were 1.0-1.3 folds greater than those of annellus diameters. Hybrid treatment was offered solely under transesophageal echocardiographic guidance. A minimal median incision was performed after hybrid treatment of balloon valvuloplasty under transesophageal echocardiographic guidance. Results After operations, all patients were transported into intensive care unit (ICU). The mean durations of mechanical ventilation, ICU stay and postoperative hospitalization were (1.7±1.6),(3.9±2.0) and (6.9±2.2) days respectively. Except for one death, there were no postoperative complications. The postoperative transcutaneous oxygen saturation was (89.2±7.0)%. During a follow-up period of 2 to 23 months, there were mild pulmonary stenosis (n=4) and mild pulmonary insufficiency (n=2). Tricuspid insufficiency significantly improved. Conclusions Hybrid treatment of pulmonary atresia with intact ventricular septum and critical pulmonary stenosis under transesophageal echocardiographic guidance is both safe and effective in neonates. It may accelerate right ventricle growth and achieve biventricular repair after modified Blalock-Taussig shunting and ligation of ductus arteriosus.

关 键 词：肺动脉闭锁 肺动脉瓣狭窄 新生儿 

分 类 号：R726.5[医药卫生—儿科]