Okumori M, Hyuga M, Ogata S, Akamatsu T, Otomi S, Ota S
Jpn J Surg. 1982;12(5):356-61. doi: 10.1007/BF02469635.
We treated two patients with a rare developmental complex. The persistent left superior vena cava draining into the left atrium (PLSVC into LA) was associated with an absent coronary sinus and an atrial septal defect. Ligation of PLSVC and patch-repair of the atrial septal defect were successfully performed in one stage. The atrial septal defect was located in the upper and posterior aspect of the interatrial septum and appeared to be an unique type of atrial septal defect. In the other patient, additional multiple cardiac defects were associated with this syndrome, including ventricular septal defect, pulmonary stenosis, tricuspid insufficiency, and complete transposition of the great arteries. Palliative Blalock procedure was used for this patient. The PLSVC into LA was discovered accidentally in both cases during heart catheterization and it was clearly demonstrated by venography. For a preoperative recognition of PLSVC, computed tomograms of the heart are of great assistance. Surgical correction of the persistent superior vena cava was emphasized for treatment of this syndrome.
我们治疗了两名患有罕见发育性综合征的患者。持续存在的左上腔静脉引流至左心房（PLSVC至LA）与冠状窦缺如和房间隔缺损相关。一期成功进行了PLSVC结扎和房间隔缺损补片修补术。房间隔缺损位于房间隔的上部和后部，似乎是一种独特类型的房间隔缺损。在另一名患者中，该综合征还伴有其他多种心脏缺陷，包括室间隔缺损、肺动脉狭窄、三尖瓣关闭不全和大动脉完全转位。该患者采用了姑息性布莱洛克手术。两例患者均在心脏导管检查期间意外发现PLSVC至LA，静脉造影清晰显示了这一情况。对于术前识别PLSVC，心脏计算机断层扫描有很大帮助。强调对持续性上腔静脉进行手术矫正以治疗该综合征。
