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目的:总结无顶冠状静脉窦综合征(UCSS)的手术方法,为术中处理此类疾患提供借鉴.方法:入选UCSS 患者14例,男 8例,女6例,年龄2个月～35(10.55±10.63)岁,体重3.5～60(26.17±19.60)kg,其中完全型心内膜垫缺损4例,部分型心内膜垫缺损6例,室间隔缺损1例,法洛四联症2例,镜像右位心4例,伴中至重度肺动脉高压11例.14例均为永存左上腔静脉(PLSVC)直接汇入左心房,即完全型冠状静脉窦间隔缺损(Ⅰ型).心脏彩超明确诊断3例,提示伴左上腔静脉(LSVC)5例.14例中未行手术处理1例,采用心外管道连结PLSVC至右心耳5例,心内隧道重建冠状窦顶4例,建立心房内隔板2例,限制性环缩LSVC 1例,直接结扎LSVC 1例.结果:全组死亡3例,出现脑部并发症2例,延迟关胸致伤口延迟愈合1例,其余患者远期效果满意.结论:心内膜垫缺损或镜像右位心伴PLSVC时,要警惕UCSS的存在.根据PLSVC汇入左心房的位置选择不同的手术方法,可获得满意的手术效果.限制性环缩LSVC可能是处理该类畸形的又一方法.“,”Objective:Unroofed coronary sinus syndrome is a rare cardiac anomaly in which communication occurs between the coronary sinus and the left atrium due to the partial or complete absence of the roof of the coronary sinus. It is usually associated with other cardiovascular anomalies, especially persistent left superior vena cava. It is difficult to dignosis preoperatively.Method:Fourteen patients underwent surgical repair for the unroofed coronary sinus syndrome from Jan,2001 to Aug,2008 in our hospital.There were 8 males and 6 females with age range from 2 months to 35(10.55±10.63) years old, and weight 3.5-60(26.17±19.60)kg.Congenital heart malformation were partial endocardial cushion defect in 6 cases,complete endocardial cushion defect in 4 cases, tetralogy of fallot in 2 cases, mirror image dextrocardia in 4cases,complicated with moderate-severe pulmonary hypertension in 11cases. Persistent left superior vena cava (PLSVC) dircetly draining into the left atrium(LA) occurs in all of them. One case was not treated ,1 case was ligated PLSVC and repaired ostium primum septal defects. 1 case were used restricted constraction of PLSVC, 5 cases were used extracardiac conduit made from pericardial patch or artificial vessel to connect left superior vena cava to right arium by anastomosis in an end-to-side fashion.4 cases were created an intra-atrial tunnel by pericardial patch from PLSVC to the right atrium, 2 cases were reconstructed the intra-atrial septal baffle with pericardial or Dacron patch. Result:Three patients died in this group,cerebral complication occurred in 2 cases and delayed sternal closure and wound healing delay occurred in 1 case.The others were no severe postoperative complications and their follow-up results were satisfactory. Conclusion:Unroofed coronary sinus syndrome is always accompanied with endocardial cushion defect and/or mirror image dextrocardia,it should be careful to explore PLSVC in these malformations. We can use different methods to deal with such malformation. Restricted constraction of PLSVC may be another choice for unroofed coronary sinus syndrome.