Background:Pulmonary vein stenosis is a progressive disease associated with a high rate of mortality in children. If left untreated, myofibroblastic proliferation can lead to pulmonary vein atresia (PVA). In our experience, transcatheter recanalization has emerged as a favorable interventional option. We sought to determine the acute success rate of recanalization of atretic pulmonary veins and mid-term outcomes of individual veins after recanalization.Methods:We reviewed all patients with PVA at our institution between 2008 and 2020 diagnosed by either catheterization or cardiac computed tomography. All veins with successful recanalization were reviewed and procedural success rate and patency rate were noted. Competing risk analysis was performed to demonstrate outcomes of individual atretic veins longitudinally.Results:Between 2008 and 2020, our institution diagnosed and treated 131 patients with pulmonary vein stenosis. Of these, 61 patients developed atresia of at least one pulmonary vein. In total, there were 97 atretic pulmonary veins within this group. Successful recanalization was accomplished in 47/97 (48.5%) atretic veins. No atretic pulmonary veins were successfully recanalized before 2012. The majority of veins were recanalized between 2017 and 2020—39/56 (70%). The most common intervention after recanalization was drug-eluting stent placement. At 2-year follow-up 42.6% of recanalized veins (20.6% of all atretic veins) remained patent with a median of 4 reinterventions per person.Conclusions:Transcatheter recanalization of PVA can result in successful reestablishment of flow to affected pulmonary veins in many cases. Drug-eluting stent implantation was the most common intervention performed immediately post-recanalization. Vein patency was maintained in 42.6% of patients at 2-year follow-up from recanalization with appropriate surveillance and reintervention. Overall, only a small portion of atretic pulmonary veins underwent successful recanalization with maintained vessel patency at follow-up. Irrespective of successful recanalization, there was no detectable survival difference between the more recently treated PVA cohort and non-PVA cohort.

中文翻译：

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婴儿和儿童闭锁肺静脉的经导管再通

背景：肺静脉狭窄是一种与儿童高死亡率相关的进行性疾病。如果不及时治疗，肌纤维母细胞增殖可导致肺静脉闭锁 (PVA)。根据我们的经验，经导管再通已成为一种有利的介入选择。我们试图确定闭锁肺静脉再通的急性成功率和再通后单个静脉的中期结果。方法：我们回顾了我们机构 2008 年至 2020 年间通过导管插入术或心脏计算机断层扫描诊断出的所有 PVA 患者。回顾了所有成功再通的静脉，并记录了手术成功率和通畅率。进行竞争风险分析以纵向证明单个闭锁静脉的结果。结果：2008 年至 2020 年之间，我院诊治肺静脉狭窄患者131例。其中，61 名患者出现至少一根肺静脉闭锁。本组共有 97 条闭锁肺静脉。47/97 (48.5%) 的闭锁静脉成功再通。2012 年之前无闭锁肺静脉成功再通。2017 年至 2020 年大部分静脉再通—39/56（70%）。再通后最常见的干预措施是放置药物洗脱支架。在 2 年的随访中，42.6% 的再通静脉（占所有闭锁静脉的 20.6%）保持通畅，每人中位数为 4 次再介入治疗。结论：PVA 的经导管再通可以成功地重建许多受累肺静脉的血流案例。药物洗脱支架植入是再通后立即进行的最常见干预措施。通过适当的监测和再干预，在 2 年的随访中，42.6% 的患者维持静脉通畅。总体而言，只有一小部分闭锁肺静脉成功再通，并在随访中保持血管通畅。无论是否成功再通，最近治疗的 PVA 队列和非 PVA 队列之间没有可检测到的生存差异。在随访中，只有一小部分闭锁肺静脉成功再通并保持血管通畅。无论是否成功再通，最近治疗的 PVA 队列和非 PVA 队列之间没有可检测到的生存差异。在随访中，只有一小部分闭锁肺静脉成功再通并保持血管通畅。无论是否成功再通，最近治疗的 PVA 队列和非 PVA 队列之间没有可检测到的生存差异。