Mainwaring Richard D, Desai Manan, Punn Rajesh, Felmly L Mac, Hanley Frank L
Division of Pediatric Cardiac Surgery, Lucile Packard Children's Hospital Stanford, Stanford University School of Medicine, Stanford, California.
Division of Pediatric Cardiology, Lucile Packard Children's Hospital Stanford, Stanford University School of Medicine, Stanford, California.
Ann Thorac Surg Short Rep. 2023 Apr 14;1(3):436-440. doi: 10.1016/j.atssr.2023.04.002. eCollection 2023 Sep.
Retroaortic innominate vein (RAIV) is an extremely rare anomaly of systemic venous return. The prevalence of RAIV has been estimated to be 0.02% in individuals without congenital heart disease and 0.5% in those with congenital heart disease. Previous studies have demonstrated an association between RAIV and both conotruncal abnormalities and right aortic arch. Our center specializes in surgical procedures for patients with pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries (PA/VSD/MAPCAs), and we have frequently observed the presence of RAIV in these patients. The purpose of this study was to evaluate the prevalence and anatomic characteristics of RAIV.
This was a single-center retrospective review of medical records to identify patients with an RAIV. The study period was 2002 through 2022, with approximately 20,000 patients evaluated during this time frame.
A total of 99 patients were identified with an RAIV. Of the 99 patients, 64 (65%) had PA/VSD/MAPCAs. Other diagnoses included tetralogy of Fallot (n = 16), tetralogy of Fallot with pulmonary atresia (n = 3), double outlet right ventricle (n = 4), peripheral pulmonary artery stenosis (n = 3), and other (n = 9). In the cohort of patients with PA/VSD/MAPCAs, 69% had a right aortic arch and 30% had completely absent central pulmonary arteries.
The overwhelming majority (90%) of patients with RAIV had conotruncal abnormalities, with PA/VSD/MAPCAs accounting for the preponderance of cases in our center.
主动脉后无名静脉（RAIV）是一种极为罕见的体静脉回流异常。据估计，在无先天性心脏病的个体中，RAIV的患病率为0.02%，而在患有先天性心脏病的个体中为0.5%。既往研究已证实RAIV与圆锥干异常和右主动脉弓均有关联。我们中心专门为患有室间隔缺损和主要体肺侧支动脉的肺动脉闭锁患者（PA/VSD/MAPCAs）实施外科手术，并且我们在这些患者中经常观察到RAIV的存在。本研究的目的是评估RAIV的患病率及解剖学特征。
这是一项单中心回顾性病历审查，以识别患有RAIV的患者。研究期间为2002年至2022年，在此时间段内共评估了约20,000例患者。
共识别出99例患有RAIV的患者。在这99例患者中，64例（65%）患有PA/VSD/MAPCAs。其他诊断包括法洛四联症（n = 16）、法洛四联症合并肺动脉闭锁（n = 3）、右心室双出口（n = 4）、周围肺动脉狭窄（n = 3）以及其他（n = 9）。在PA/VSD/MAPCAs患者队列中，69%有右主动脉弓，30%中央肺动脉完全缺如。
绝大多数（90%）患有RAIV的患者有圆锥干异常，在我们中心PA/VSD/MAPCAs占病例的绝大多数。
