Abstract：
This study aimed to compare long-term morbidity in patients with pulmonary atresia with intact ventricular septum (PA-IVS) treated with catheter-based intervention (group A) versus those undergoing heart surgery (group B) as initial intervention. Additionally, we conducted a systematic literature review on PA-IVS treatment. All neonates born in Sweden with PA-IVS between 2007 and 2019 were screened for inclusion. The inclusion criterion was decompression of the right ventricle for initial intervention. Medical records were reviewed, as well as the initial preoperative angiogram, and the diagnostic echocardiogram. Comparisons between groups were performed with Mann-Whitney U-test and Fisher´s exact test. A systematic literature review of original studies regarding treatment of PA-IVS (2002 and onward) was conducted following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines, to assess the outcomes of patients with PA-IVS. 34 (11 females) patients were included, 18 in group A and 16 in group B. There was no mortality in either group. Follow-up time ranged from 2 to 15 years (median 9). All attempted perforations in group A were successful, and 16 out of 18 patients reached biventricular circulation. In the surgical group 15 out of 16 patients reached biventricular circulation. The literature review presented heterogeneity in standards for treatment. This retrospective population-based multicenter study demonstrates that both catheter-based intervention and heart surgery are safe procedures. Our results are comparable to, or exceed, those in the systematic literature review. The systematic literature review displays a great heterogeneity in study design, with no definitive golden standard treatment.
摘要：
本研究旨在比较接受导管介入治疗的室间隔完整的肺动脉闭锁（PA-IVS）患者（A组）与接受心脏手术（B组）作为初始干预的患者的长期发病率。此外,我们对PA-IVS治疗进行了系统的文献综述.所有在2007年至2019年期间在瑞典出生的PA-IVS新生儿都进行了筛查。纳入标准为初始干预右心室减压。检查了医疗记录,以及最初的术前血管造影,还有诊断超声心动图.组间比较采用Mann-WhitneyU检验和Fisher精确检验。根据系统评价和荟萃分析指南的首选报告项目，对有关PA-IVS治疗的原始研究（2002年及以后）进行了系统文献综述。评估PA-IVS患者的预后。包括34名（11名女性）患者，A组18例，B组16例。两组均无死亡。随访时间为2至15年（中位数9）。A组所有尝试穿孔均成功,18例患者中有16例达到双心室循环。在手术组中，16例患者中有15例达到了双心室循环。文献综述显示了治疗标准的异质性。这项基于人群的回顾性多中心研究表明，基于导管的介入和心脏手术都是安全的手术。我们的结果与，或超过，系统文献综述中的那些。系统的文献综述在研究设计中表现出很大的异质性，没有明确的金标准治疗。
