College of Medicine, Medical University of South Carolina, Charleston, SC, USA.
Section of Pediatric Cardiothoracic Surgery, Department of Surgery, Medical University of South Carolina, 10 McClennan Banks Drive, Charleston, SC, 29425, USA.
Pediatr Cardiol. 2024 Dec;45(8):1838-1840. doi: 10.1007/s00246-023-03289-5. Epub 2023 Sep 2.
Persistent left superior vena cava (PLSVC) draining to the left atrium (LA) is a rare congenital abnormality that is often asymptomatic and found incidentally on imaging. PLSVC is usually described alongside other congenital defects, such as septal defects, tetralogy of fallot, and aortic coarctation. PLSVC to LA with an atrial septal defect is known as Raghib syndrome, but to our knowledge PLSVC to LA without an atrial septal defect or right superior vena cava has not been described in the literature. Here, we report the presentation of a patient with PLSVC-LA without ASD and propose a classification system for this subset of congenital heart defects to help guide clinical and surgical management of these patients.
永存左上腔静脉（PLSVC）引流至左心房（LA）是一种罕见的先天性异常，通常无症状，在影像学检查中偶然发现。PLSVC 通常与其他先天性缺陷一起描述，如间隔缺损、法洛四联症和主动脉缩窄。PLSVC 引流至 LA 并伴有房间隔缺损称为拉吉布综合征，但据我们所知，在文献中尚未描述 PLSVC 引流至 LA 而无房间隔缺损或上腔静脉的情况。在这里，我们报告了一例 PLSVC-LA 无 ASD 的患者，并提出了一种用于此类先天性心脏缺陷的分类系统，以帮助指导这些患者的临床和手术管理。
