目的:探讨弯刀综合征的优化治疗策略及影响其预后的危险因素。方法:回顾性分析2013年1月至2018年12月收治的34例弯刀综合征患儿的临床资料、治疗结果和随访资料。结果:34例患儿，男16例，女18例；年龄(21.16± 33.19)个月；随访(50.30 ± 26.99)个月；婴儿型(1岁)更多合并肺动脉高压及肺静脉狭窄。8例介入治疗(包括封堵侧支血管及其他合并心脏畸形)后未行外科手术。15例接受手术纠治弯刀静脉，术后4例弯刀静脉狭窄，采用直接吻合法和心包补片隧道法术后弯刀静脉狭窄发生率差异无统计学意义(n P＝0.569)，更多见于术前有弯刀静脉狭窄的患者(n P＝0.009)。7例(20.5%)死亡，死亡的高风险因素包括诊断时年龄(n P＝0.000)、肺动脉高压(n P＝0.007)及肺静脉狭窄(n P＝0.014)。n 结论:肺动脉压力基本正常且不伴有房间隔缺损以外心脏畸形的弯刀综合征患者可不需手术。术后弯刀静脉狭窄与术前狭窄有关，而与术式无关。弯刀综合征死亡的高风险因素包括诊断时年龄、显著肺动脉高压、肺静脉狭窄。“,”Objective:To evaluated the clinical optimization of treatment strategies for the disease and risk factors for prognosis.Methods:The records of 34 patients with scimitar syndrome evaluated at our medical center between Jan 2013 and Dec 2018 were reviewed, including clinical data, treatment outcome and follow-up.Results:Scimitar syndrome was identified in 34 patients, 16 males and 18 females, aged (21.16±33.19) months. The mean length of follow-up after diagnosis was (50.30±26.99) months. Compared with adult form patients, patients presenting less than 1 year of age had a higher incidence of pulmonary hypertension and pulmonary veins stenosis. 8 patients didn’t require surgical treatment after interventional coil embolization of aortopulmonary collaterals or other interventional maneuvers in associated congenital heart diseases. 15 patients had scimitar vein surgery, 4 patients had postoperative pulmonary vein obstruction that occurred with similar frequency after baffle or reimplantation procedures(n P=0.569), and tended to be more common in patients had preoperative obstruction of scimitar vein(n P=0.009). Overall, 7(20.5%) of 34 died. High risk factors for death included age at diagnosis(n P=0.000), obvious pulmonary hypertension(n P=0.007) and pulmonary vein stenosis(n P=0.014).n Conclusion:Patients with pulmonary artery systolic pressure near normal and absence of congenital heart disease excluding atrial septal defect do not require surgery. Postoperative pulmonary vein obstruction is related with scimitar vein stenosis before surgery regardless of redirection technique. Age at diagnosis, obvious pulmonary hypertension and pulmonary vein stenosis are risk factors for death in patients with scimitar syndrome.