静脉窦型房间隔缺损的外科治疗

Surgical Treatment of Sinus Venosus Atrial Septal Defect

目的总结静脉窦型房间隔缺损(SVASD)外科治疗的方法和经验。方法32例SVASD患者均伴有右侧部分或全部的肺静脉异位引流。上腔SVASD 25例,其中22例采用双片心包补片修补,3例将异位引流的肺静脉改道、上腔静脉与右心耳吻合(Warden法)进行手术纠治。7例下腔SVASD患者中,3例为弯刀综合征,关闭房间隔缺损(ASD)的同时将异位引流的肺静脉离断后与左心房后壁吻合;其余4例采用单片心包补片修补ASD。结果手术效果满意,无手术死亡。随访28例,心电图检查均为窦性心律;心脏超声心动图检查提示:心内无残余分流、右肺静脉及腔静脉回流无梗阻。结论SVASD特殊的解剖结构决定了其多伴右侧肺静脉异位引流,纠治上腔SVASD时应注意避免损伤窦房结,防止上腔静脉回流梗阻;但弯刀综合征多伴右肺发育不良,心功能情况还须长期随访。

Objective To summarize the experiences of surgical treatment of sinus venosus atrial septal defect （SVASD）. Methods There were 32 patients of SVASD, all of them were associated with totally or partially right anomalous pulmonary venous connection. There were 25 cases of superior SVASD, 22 cases underwent double-patch procedure, 3 cases underwent Warden procedure. In 7 cases of inferier SVASD, 3 cases of Scimitar syndrome underwent ASD repair by pericardial patch and at the same time the anomalous pulmonary vein was divided and reimplanted to the posterior wall of left atrium, 4 cases of them underwent single-patch ASD repair. Results The surgical results were satisfying and no patient died, 28 patients were in sinus rhythm and echocardiography showed neither obstruction of caval vein and right pulmonary vein nor residual interatrial shunt. Conclusion SVASD mostly associated with right anomalous pulmonary venous connection according to its special anatomic structure. Preserving the function of sinus node and avoiding of obstruction of caval vein should be routinely considered when superior SVASD was repaired. The heart function of Scimitar syndrome should be followed-up for a long time for it usually associated with right lung hypoplasia.