Department of Anatomy, Faculty of Medicine, Mersin University, Ciftlikkoy Campus, 33343, Mersin, Turkey.
Department of Pathology, Faculty of Medicine, Mersin University, Mersin, Turkey.
Surg Radiol Anat. 2020 Apr;42(4):391-395. doi: 10.1007/s00276-020-02434-6. Epub 2020 Feb 11.
Persistent left superior vena cava (PLSVC) is one of the cardiac system abnormalities with a 0.3-0.5% incidence and caused by inadequate obliteration of the left anterior cardinal vein during embryonic development. Prognosis of PLSVC is generally assumed to be good if it is not accompanied by other cardiac system abnormalities. During the routine ultrasound control of a patient at 25th week of pregnancy at the Obstetrics and Gynecology Department of Mersin University, PLSVC anomaly was detected in an intrauterine fetus. Then, intrauterine death occurred and after removal of the deceased fetus, PLSVC diagnosis was confirmed by autopsy. According to the autopsy findings, right superior vena cava (SVC) and azygos vein were found in normal course. PLSVC opened into the right atrium via enlarged coronary sinus. There was no connection between the two SVCs. On the left side of posterior mediastinum, instead of hemiazygos or accessory hemiazygos veins, a vein symmetrical to azygos was opened into PLSVC, similar to the one on the right. No other cardiac anomaly associated with PLSVC or any other pathology in the other parts of body that could be responsible for death was discovered during autopsy. There was no evidence indicating that PLSVC played any role in intrauterine exitus of the present case. However, as mentioned in the literature, the ectopic beats in the atrium wall of patients with isolated PLSVC and enlarged coronary sinus may lead to pathologies in the conduction system of the heart. Considering the intrauterine death of an isolated PLSVC case associated with cardiac conduction pathologies, we recommend that the common assumption of 'isolated PLSVC is not associated with death' should be reviewed by studies on large series and even intrauterine cases should be closely monitored for cardiac arrhythmia.
永存左上腔静脉（PLSVC）是一种心脏系统异常，发生率为 0.3-0.5%，是由于胚胎发育过程中左前主静脉不完全闭塞引起的。如果 PLSVC 不伴有其他心脏系统异常，通常预后良好。在梅斯大学妇产科对一名 25 周妊娠的患者进行常规超声检查时，发现胎儿存在 PLSVC 异常。随后，胎儿宫内死亡，在取出死胎后，尸检证实了 PLSVC 诊断。根据尸检结果，发现右上腔静脉（SVC）和奇静脉正常。PLSVC 通过扩大的冠状窦开口于右心房。两条 SVC 之间没有连接。在后纵隔的左侧，没有半奇静脉或副半奇静脉，而是一条与奇静脉对称的静脉开口于 PLSVC，类似于右侧的静脉。尸检未发现与 PLSVC 相关的其他心脏异常或身体其他部位可能导致死亡的任何其他病理变化。没有证据表明 PLSVC 在本例胎儿宫内死亡中起作用。然而，正如文献中提到的，孤立性 PLSVC 和扩大的冠状窦的心房壁异位搏动可能导致心脏传导系统的病理变化。考虑到孤立性 PLSVC 合并心脏传导通路疾病导致胎儿宫内死亡，我们建议对大系列研究甚至宫内病例进行研究，重新审查“孤立性 PLSVC 不与死亡相关”的常见假设，并密切监测心律失常。
