弯刀综合征的自然病史和临床结果——单中心21年经验回顾  

Nature History and Clinical Outcome of Scimitar Syndrome:A Long⁃Term Study Based on Twenty⁃One Years′Experience

作　　者：刘子娜 李良 张燕搏 李守军 杨克明 常硕 张本青 孙寒松 LIU Zina;LI Liang;ZHANG Yanbo;LI Shoujun;YANG Keming;CHANG Shuo;ZHANG Benqing;SUN Hansong(Department of Cardiovascular Surgery,Fuwai Hospital,National Center for Cardiovascular Diseases,Chinese Academy of Medical Sciences&Peking Union Medical College,Beijing 100037,China)

机构地区：[1]中国医学科学院、北京协和医学院、国家心血管病中心、国家心血管疾病临床医学研究中心、阜外医院心外科,北京100037

出　　处：《中国分子心脏病学杂志》2024年第2期5959-5963,共5页Molecular Cardiology of China

基　　金：中央高水平医院临床科研业务费(2022-GSP-GG-23)。

摘　　要：目的弯刀综合征是指右侧肺静脉部分或全部引流至下腔静脉,其治疗仍有争议,本文旨在阐述弯刀综合征的疾病特点、治疗方案及预后情况,为临床决策提供参考。方法回顾性分析2000年2月至2021年4月在中国医学科学院阜外医院诊断为弯刀综合征的患者病历资料,并通过电话问诊以及门诊进行随访。结果共纳入53例患者,中位年龄6.44岁(1.48岁,24.86岁)。单纯型弯刀综合征16例(30.2%),37例(69.8%)合并其他先天性心脏病。18例(34.0%)合并右肺发育不良。35例患者接受弯刀静脉矫正手术,采用心房内板障法24例(68.6%),弯刀静脉再植法11例(31.4%)。围手术期死亡率为2.9%。全组中位随访时间7.8年(4.2年,11.8年),随访期间手术组死亡2例(5.7%),观察组死亡4例(22.2%)。全组平均估算生存时间(17.55±0.95)年。手术组5例发生肺静脉狭窄(心房内板障法3例,弯刀静脉再植法2例,P=0.640)。合并重度肺高压(HR=30.47,95%CI:2.74~339.02,P=0.005)、合并复杂先天性心脏病(HR=26.79,95%CI:2.82~254.54,P=0.004)为死亡相对危险因素。结论弯刀综合征整体预后良好,手术治疗可以取得满意的治疗效果,合并复杂先天性心脏病和重度肺动脉高压会增加死亡风险。Objective Scimitar syndrome is characterized by anomalous drainage of partial or total right pulmonary vein to the inferior vena cava,and its treatment is still controversial.The purpose of this study is to describe the disease characteristics,treatment and prognosis of scimitar syndrome.Methods The clinical data of patients diagnosed with scimitar syndrome in Fuwai Hospital from February 2000 to April 2021 were retrospectively analyzed.All follow⁃up data were obtained from telephone review and medical record.Results A total of 53 patients were included,the median age was 6.44 years(1.48 years,24.86 years),including 16 cases(30.2%)isolated scimitar syndrome and 37 cases(69.8%)with congenital heart disease(CHD).There were 18 cases(34.0%)complicated with right lung hypoplasia.Thirty⁃five patients underwent scimitar vein corrective surgery,including intra⁃atrial baffle in 24 cases(68.6%)and scimitar vein reimplantation in 11 cases(31.4%).The perioperative mortality was 2.9%.The median follow⁃up time was 7.8 years(4.2 years,11.8 years),2(5.7%)died in surgical group and 4(22.2%)died in clinically monitored group during the follow⁃up period.The mean estimated survival time of the whole group was(17.55±0.95)years.Pulmonary vein stenosis occurred in 5 cases postoperation(3 in intra⁃atrial baffle group,2 in scimitar vein reimplantation group,P=0.640).Multivariable analyses identified severe pulmonary hypertension(PAH)(HR=30.47,95%CI:2.74⁃339.02,P=0.005)and complex congenital heart disease(CHD)(HR=26.79,95%CI:2.82⁃254.54,P=0.004)were the independent risk factors for death.Conclusion The overall prognosis of scimitar syndrome is good.Surgical treatment is beneficial.Combination of complex CHD and severe PAH are associated with poor prognosis.

关 键 词：弯刀综合征 手术治疗 预后 

分 类 号：R54[医药卫生—心血管疾病]