Department of Perinatology, Zeynep Kamil Gynecologic and Pediatric Training Research Hospital, Health Sciences University, Istanbul, Turkey.
Department of Pediatric Cardiology, Dr. Siyami Ersek Thoracic and Cardiovascular Surgery Training and Research Hospital, Health Sciences University, Istanbul, Turkey.
Echocardiography. 2023 Nov;40(11):1259-1268. doi: 10.1111/echo.15706. Epub 2023 Oct 25.
To assess the accuracy of prenatal echocardiography in defining pulmonary vasculature in pulmonary atresia with VSD (PAVSD). The second aim is to compare the perinatal and postnatal outcomes of different pulmonary blood supply types.
The cases prenatally diagnosed with PAVSD between 2017 and 2022 in a single tertiary fetal medicine center were identified on the electronic database. Fetal echocardiography reports and images were reviewed retrospectively. Postnatal outcomes were acquired from the hospital records of relevant pediatric cardiology and cardiovascular surgery clinics. Fetal echocardiography results were compared with postnatal results. Perinatal and postnatal outcomes were compared between the different pulmonary vascular supply types.
Among the 24 PAVSD cases, six were diagnosed with major aortopulmonary collateral arteries (MAPCA) dependent, eleven were diagnosed with ductus arteriosus (DA) dependent pulmonary supply, and seven were diagnosed with double pulmonary supply (MAPCA + DA) on prenatal echocardiography. Seventeen cases were live-born and have undergone postnatal investigations. Fetal echocardiography was 88.2% accurate about the type of pulmonary supply. The accuracy of fetal echocardiography regarding pulmonary vascular anatomy was 82.3%. Postoperative survival was 69.2%. Mortality before surgery and postoperative survival did not differ between pulmonary supply groups. Survival was impaired by the extracardiac anomalies. The need for early interventions was significantly higher in the DA group.
Pulmonary vascularization in PAVSD can be defined precisely on fetal echocardiography. The source of pulmonary blood supply does not impact postnatal short-term outcomes significantly but it impacts the management. The associated anomalies highly contribute to postnatal mortality.
评估产前超声心动图在肺动脉瓣闭锁伴室间隔缺损（PAVSD）中定义肺血管的准确性。第二个目的是比较不同肺血供应类型的围产期和围生期结局。
在单个三级胎儿医学中心，通过电子数据库确定了 2017 年至 2022 年期间产前诊断为 PAVSD 的病例。回顾性审查胎儿超声心动图报告和图像。从相关儿科心脏病学和心血管外科诊所的医院记录中获取围生期结局。将胎儿超声心动图结果与产后结果进行比较。比较不同肺血管供应类型之间的围生期和围生期结局。
在 24 例 PAVSD 病例中，6 例被诊断为主要体肺侧支循环动脉（MAPCA）依赖性，11 例被诊断为动脉导管依赖性肺供应，7 例被诊断为双肺供应（MAPCA + DA）在产前超声心动图上。17 例为活产并接受了产后检查。胎儿超声心动图对肺供应类型的准确率为 88.2%。胎儿超声心动图对肺血管解剖结构的准确率为 82.3%。术后存活率为 69.2%。手术前和手术后的死亡率在肺供应组之间没有差异。生存受到心脏外畸形的影响。DA 组早期干预的需求明显更高。
PAVSD 中的肺血管化可以在胎儿超声心动图上精确定义。肺血供应的来源不会显著影响产后短期结局，但会影响管理。相关的畸形对产后死亡率有很大的影响。
