无顶冠状静脉窦综合征的诊断与外科治疗

目的探讨并总结无顶冠状静脉窦综合征(UCSS)的临床表现和外科处理方法。方法1999年1月至2009年6月我科共收治9例UCSS患者,其中Ⅰa型3例、Ⅰb型1例、Ⅱa型1例、Ⅲa型3例、Ⅲb型1例。术前经超声心动图检查明确诊断6例,术中探查明确诊断3例。合并永存左上腔静脉(PLSVC)共7例,其中PLSVC直接汇入左房(Ⅰa型)3例,采取房间隔重建术1例,心内隧道术2例;PLSVC汇入冠状静脉窦后同时开口于左右房(Ⅱa型)1例,开口于左房(Ⅲa型)3例,均处理冠状静脉窦左房开口。合并其他心内畸形或继发性改变同期手术矫治。结果术后早期2例出现并发症,余7例恢复顺利。全组均痊愈出院,1例失访,8例随访2个月至5年,无死亡和晚期并发症发生。结论UCSS是一种罕见的先天性心脏病,术前诊断有一定困难,术中须仔细探查,可根据PLSVC连接心房的方式、冠状静脉窦异常开口的位置、合并其他心内畸形或继发性改变的特点选择手术方法,如能达到生理性矫治则预后良好。

Diagnosis and surgical treatment of unroofed coronary sinus syndrome

Objective To summarize the clinical manifestations and surgical treatment of unroofed coronary sinus syndrome(UCSS).Methods Nine patients underwent surgical repair for UCSS from Jan.1999 to Jun.2009 in our hospital,and the patients included 3 with type Ⅰ a, 1 with type Ⅰ b, 1 with type Ⅱ a, 3 with type Ⅲ a, and 1 with type Ⅲ b UCSS.The diagnosis of UCSS was made by echocardiography in 6 patients and by the surgical exploration in 3 patients.Persistent left superior vena cava (PLSVC) was found in 7 cases; 3 cases with PLSVC directly draining into the left atrium (Ⅰ a) were reconstructed by the intra-atrial routes to guide PLSVC to the right atrium, and 4 cases with PLSVC draining into the coronary sinus and coronary sinus orifice opening to the left atrium and right atrium (1 case, Ⅱ a) and left atrium (3 cases, Ⅲ a) were dealt with the abnormal coronary sinus orifice.Other associated cardiac lesions were corrected concomitantly.Results Two cases had early complications and the other 7 cases had smooth recovery after operation; all the patients were cured and discharged.Eight cases were followed up for 2 months to 5 years and there was no death or late complications.Conclusion UCSS is a rare congenital cardiac anomaly and is difficult to diagnose preoperatively; so careful exploration should be done during operation.Different surgical approaches should be chosen according to the types of communication between PLSVC and cardiac atrium, the positions of abnormal sinus ostium, and other cardiac lesions.Satisfactory prognosis can be obtained if physiological correction is achieved.