Nature History and Clinical Outcome of Scimitar Syndrome:A Long-Term Study Based on Twenty-One Years'Experience
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.
NETL
NSTL
万方数据
