无顶冠状静脉窦综合征合并房室通道的临床分析

Clinical analysis of unroofed coronary sinus syndrome with atrioventricular canal defect

目的分析无顶冠状静脉窦综合征（UCSS）合并房室通道的病理学特点和外科手术治疗方法。方法1999年9月至2007年10月，20例UCSS合并房室通道患者接受外科手术治疗。男性10例，女性10例；年龄0．5～38．0岁，平均（11．4±11．0）岁；体重6．7～73．0kg，平均（28．4±21．3）kg。其中合并完全型房室通道2例，部分型房室通道18例，同时合并单心房12例。复杂型UCSS合并左上腔静脉（LSVC）直接汇入左心房，即UCSSI型共11例，其中10例采用行心房内板障术或心内隧道术将LSVC引流入右心房，直接结扎LSVC1例。简单型UCSS9例用其他方法矫治。所有患者同期行房室通道畸形矫治术。结果全组手术死亡1例，死于术后肺部感染；其余19例手术效果满意。随访14例，随访时间4个月～3年，无死亡病例和并发症。结论房室通道合并LSVC及单心房时，要警惕UCSS的存在。根据UCSS的不同病理类型选择不同的手术方法，可获得满意的手术效果。

Objective To analyze symptoms, associated anomalies, diagnostic approach, and surgical procedures in patients with unroofed coronary sinus syndrome and atrivoentricular canal defect. Methods The clinical data of 20 patients with unroofed coroary sinus syndrome from September 1999 to October 2007 were reviewed retrospectively. There were 10 male and 10 female patients. The age ranged from 6 months to 38 years old, with a mean of （11.4±11.0） years old. The body weight ranged from 6. 7 to 73.0 kg, with a mean of （28. 4±21.3） kg. There were 18 cases of patial atrioventricular canal defect, 2 cases of complete atrioventricular canal defect, and 12 cases of common atrium. The initial diagnosis of unroofed coronary sinus syndrome was made by the surgeon at repair of a patial or comple atrioventricular canal defect in 20 patients. Complex unroofed coronary sinus with left superior vena cava （LSVC） directly draining into the left atrium was found in 11 cases, 1 case of LSVC was ligated, 10 cases were reconstructed the intraatrial baffle or the intracardiac tunnel to drain LSVC to right atrium. The other 9 patients with simple unroofed coronary sinus were repaired with other procedures. The associated cardiac lesions were corrected concomitantly. Results Death occurred in 1 patient with complex congenital cardiac disease due to pulmonary infection. In the 14 early survivors, who had been followed up from 4 months to 3 years, there was no death and severe complications. Conclusions When associated with a patial or complete atrioventricular canal defect, LSVC and a common atrium, unroofed coronary sinus syndrome should be considered as a possible additional finding. Repair according to the type of unroofed coronary sinus syndrome is effective.