13例弯刀综合征的诊治分析  

Analysis of diagnosis and treatment of scimitar syndrome in 13 cases

作　　者：谭玉玉 孙云霞 温树生 庄建 潘微 Tan Yuyu;Sun Yunxia;Wen Shusheng;Zhuang Jian;Pan Wei(Department of Neonatology,Guangdong General Hospital(Guangdong Academy of Medical Sciences),Guangzhou 510100,China;Department of Congenital Cardiac Surgery,Guangdong General Hospital(Guangdong Academy of Medical Sciences),Guangzhou 510100,China;Department of Maternal-Fetal Cardiology,Guangdong General Hospital(Guangdong Academy of Medical Sciences),Guangzhou 510100,China)

机构地区：[1]广东省人民医院(广东省医学科学院)新生儿重症监护室,广州510100 [2]广东省人民医院(广东省医学科学院)心外小儿科,广州510100 [3]广东省人民医院(广东省医学科学院)胎儿心脏诊疗科,广州510100

出　　处：《中华胸心血管外科杂志》2021年第10期595-599,共5页Chinese Journal of Thoracic and Cardiovascular Surgery

基　　金：国家重点研发计划项目(2018YFC1002602)。

摘　　要：目的探讨弯刀综合征的临床表现、诊断、治疗和预后。方法收集2013年1月至2020年11月广东省人民医院超声心动图诊断后经手术、心脏CT或心导管检查证实的13例弯刀综合征患儿的临床表现、X线胸片、超声心动图、心脏CT及心导管检查、手术方法、预后和随访资料。结果13例患儿中男7例,女6例。年龄21天~10岁,平均17个月。体质量3.2~29.5 kg,平均7.9 kg。婴儿型10例,临床表现为反复呼吸道感染、心力衰竭、生长发育迟缓,其中8例合并重度肺动脉高压。成人型3例,均因发现心脏杂音检查确诊。合并心脏畸形包括房间隔缺损12例,动脉导管未闭2例,右心室双出口/室间隔缺损1例,右肺及右肺动脉发育不良4例,右肺发育不良2例,侧支循环供应右肺6例。10例行右肺静脉异位引流矫治术,1例仅行右心室双出口矫治术,其中3例术后因严重肺动脉高压死亡;1例因梗阻型肺动脉高压失去手术机会,1例因合并神经系统病变放弃治疗。出院时1例出现右肺静脉狭窄,血流速度2 m/s。随访8例患儿,最长7年7个月。随访期间2例出现右肺静脉狭窄,血流速度分别为1.8 m/s、2.4 m/s。结论如发现中位心或右位心,反复呼吸道感染,不明原因的肺动脉高压,需考虑弯刀综合征的可能。可联合超声心动图及心脏CTA尽早明确诊断。婴儿型、术前肺动脉高压重度是弯刀综合征的死亡危险因素。术后仍需长期终身的随访,必要时需再次手术干预。Objective To investigate the clinical features,diagnosis,treatment and prognosis of Scimitar syndrome.Methods A retrospective analysis of clinical data of 13 children with scimitar syndrome from January 2013 to November 2020,including clinical symptoms,chest X-ray,echocardiography,cardiac CT and cardiac catheterization prognosis and follow-up.Results 13 children with scimitar syndrome were diagnosed,including 7 girls and 6 boys with a average age of 17 months(21 days to 10 years).3 cases<5 kg in weight.Ten patients presented with the infantile form and 3 with the adult form of scimitar syndrome.13 infantile form had lower respiratory tract infections,heart failure,and growth retardation,of which 8 cases were with severe pulmonary arterial hypertension.3 adult form were diagnosed because of heart murmur.12 cases had coexisting cardiac lesions,including 12 atrial septal defect,2 patent ductus arteriosus,1 right ventricular double outlet/ventricular septal defect,4 right lung and right pulmonary artery dysplasia,2 right lung dysplasia,6 additional systemic arterial supply to the right lung.Ten patients had pulmonary venous drainage correction surgery,one patient only underwent right ventricular double-outlet correction,three patients died of severe pulmonary hypertension;one patient lost the opportunity of surgery due to obstructive pulmonary hypertension,and one patient was complicated by nervous system The disease gave up treatment.One corrected case was stenosed during discharge and 2 corrected children became stenotic during follow-ups.Conclusion Found with the median or dextrocardial heart,recurrent respiratory infections,or unexplained pulmonary hypertension,the possibility of scimitar syndrome should be considered.The combination of echocardiography and cardiac CTA can confirm the diagnosis as soon as possible.The risk factors for mortality included infantile form and severe preoperative pulmonary hypertension.Long-term follow-up is still required after operation,and surgical intervention is required again if

关 键 词：弯刀综合征 部分型肺静脉异位引流 肺动脉高压 

分 类 号：R726.5[医药卫生—儿科]