弯刀综合征新形态学分型和外科技术改良

New morphologic classifications and modified surgical techniques of scimitar syndrome

目的总结改良心包隧道技术治疗竖直型弯刀综合征的单中心临床结果,优化该病种的手术技术和个体化治疗。方法根据弯刀静脉与下腔静脉的夹角θ对弯刀综合征进行形态分型,定义θ≤45°为竖直型,θ>45°为水平型。2011年6月至2019年6月,上海交通大学医学院附属上海儿童医学中心采用改良心包隧道技术治疗9例竖直型弯刀综合征患者,手术将弯刀静脉与右侧心包壁吻合,再建立心包隧道将其引流入到左心房。手术均同期矫治合并的心内畸形。结果全组9例患儿,年龄为0.3-12.0岁;体质量为3.5-21.0 kg。无住院手术死亡。术后机械通气时间为36-264 h。采用心包隧道技术手术后无残余弯刀静脉回流梗阻。随访8例,随访时间为6-96个月,弯刀静脉均无远期回流梗阻,7例无肺动脉高压,1例轻度肺动脉高压,心功能均有明显改善。结论根据弯刀静脉的解剖形态,对竖直型弯刀综合征患者选择心包隧道技术进行个体化手术治疗可收到良好的效果。

Objectives To optimize the surgical technique and individualized management for scimitar syndrome,we summarized the results of applications of modified pericardial tunnel technique for scimitar vein re-implantation in vertical form in a single center.Methods We established a new morphologic form of scimitar syndrome according to the angulation(θ)measured between scimitar vein and inferior vena cava,θ≤45°was defined as vertical form andθ>45°was defined as horizontal form.Nine patients with vertical form of scimitar syndrome were repaired with modified pericardial tunnel technique from June 2011 to June 2019 in Shanghai Children′s Medical Center,Shanghai Jiaotong University School of Medicine.The modified pericardial tunnel technique refered that the scimitar vein was connected to a pericardial tunnel,inside which the blood from scimitar vein was directed into left atrium through a left atriotomy.All the patients had associated intra-cardiac anomalies repaired concomitantly.Results The ages of the nine patients ranged from 0.3 to 12.0 years,and the weights ranged from 3.5 to 21.0 kg.There was no in hospital mortality.Postop⁃erative mechanical ventilation duration ranged from 36 to 264 h.No residual obstruction in scimitar vein occurred after the modified pericardial tunnel correction.The following up duration of 8 survivors ranged from 6 to 96 months.No late scimitar vein obstruction was noted on echocardiography.Seven patients were free from pulmonary hypertension and 1 patient with mild pulmonary hypertension.Cardiac function was significantly improved in followed up patients.Conclusions According to the anatomical morphology of the scimitar vein,the treatment of patients with vertical scimitar syndrome with pericardial tunnel technique can achieve good results.