无顶冠状静脉窦综合征的外科治疗

目的分析无顶冠状静脉窦综合征（UCSS）的临床特点和外科手术治疗方法，为术中处理此类疾病提供借鉴。方法回顾性分析1998年5月至2008年1月在阜外心血管病医院手术治疗的44例UCSS患者的临床资料，术前诊断12例，术中诊断32例。根据Kirklin分型，其中Ⅰ型15例，Ⅱ型9例，Ⅲ型5例，Ⅳ型15例。44例均合并其他心脏畸形。合并左上腔静脉（LSVC）直接引流入左心房15例，其中手术采用心内隧道引流LSVC至右心房14例，直接结扎LSVC1例。手术同期矫治合并的其他心脏畸形。结果本组手术死亡3例，其中1例死于低心排血量综合征，2例死于肺部感染。1例因术后发生右侧膈肌麻痹行膈肌折叠术，术后70d出院。其余40例患者术后早期恢复顺利，术后呼吸机辅助时间11．7±12．1h；住院时间8．1±2．8d。随访32例，随访时间4个月～10年，其中9例左心房内隧道术处理LSVC患者，无死亡和并发症发生。结论先天性心脏病合并LSVC时，要警惕UCSS的存在。根据LSVC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。

Surgical Treatment of Unroofed Coronary Sinus Syndrome

Objective To analyze symptoms, associated anomalies, diagnostic approach, and surgical procedures in patients with unroofed coronary sinus syndrome （UCSS）, a rare congenital disorder. Methods The clinical, echocardiographic, operative, and follow-up data on 44 patients with unroofed coronary sinus syndrome （type Ⅰ in 15,type Ⅱ in 9,type Ⅲ in 5 and type Ⅳ in 15） between May 1998 and January 2008 were reviewed retrospectively. The initial diagnosis of unroofed coronary sinus syndrome was made by echocardiography in 12 patients, by the surgeon at repair of other congenital cardiac anomalies in 32 patients. The patients underwent cardiovascular surgery at Fu Wai hospital. Left superior vena cava（LSVC） directly drains into the left atrium （LA） were found in 15 cases, LSVC was ligated in 1 case, the intracardiac tunnel to drain LSVC to right atrium （RA） were reconstructed in 14 cases. The associated cardiac lesions were corrected concomitantly. Results There were 3 hospital deaths. One patient died of low cardiac output syndrome and 2 patients died of pulmonary infection. One case was applied diaphragm plication because of right diaphragm paralysis after the heart operation and the hospital stay was 70 days. The other 40 cases had good surgical result. The average time of extubation was 11.7 ± 12. 1 h and hospital stay was 8.1±2.8 d. In the 32 cases, who have been followed up from 4 months to 10 years, there was no death and severe complications. Conclusion Unroofed coronary sinus syndromes are often missed in the preoperative evaluation of congenitally malformed hearts. When associated with LSVC, unroofed coronary sinus syndrome should be considered as a possible additional finding. We performed different surgical approaches to deal with the different kinds of LSVC with a good result.