This retrospective cohort study aimed to explore the long-term outcomes of an individualized strategy in patients with pulmonary atresia and intact ventricular septum (PAIVS). We analyzed survival and reintervention rates and identified risk factors for outcomes in patients with PAIVS treated based on individual right heart structures between 1979 and 2019. Ninety-five patients were included in this study. The z-scores of the pulmonary annulus, tricuspid annulus, and right ventricular end-diastolic volume were - 3.30 (- 15.15 to 1.83), - 0.70 (- 4.65 to 2.33), and - 1.51 (- 6.35 to 1.18), respectively. Right ventricular-dependent coronary circulation occurred in 15% of the patients. Among the 63 patients attempting biventricular strategy at first, 55 patients achieved biventricular circulation, 3 patients had one-and-a-half circulation, and 4 patients died perioperatively. Among the 33 patients attempting univentricular strategy at first, 10 patients died before the completion of Fontan operation, 17 patients (48%) accomplished Fontan operation, and 5 patients waited for Fontan operation. In one patient, conversion to biventricular circulation occurred. During the follow-up period of 720 person-years, the 20-year survival rate was significantly higher in patients with biventricular circulation than in those patients with univentricular circulation (93% vs. 67%, P < 0.001). Freedom from reintervention rates at 20 years was significantly lower in patients with biventricular circulation than in those patients with univentricular circulation (29% vs. 72%, P < 0.001). The pulmonary annulus z-score was an independent risk factor for reintervention in patients with biventricular circulation. Patients with biventricular circulation had an acceptable survival rate, but a high reintervention rate. Meanwhile, patients with univentricular circulation had high mortality before the completion of Fontan operation, although the reintervention rate was relatively low.

中文翻译：

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肺闭锁和完整室间隔患者个体化策略后的长期结果。

这项回顾性队列研究旨在探索个体化策略在肺闭锁和完整室间隔 (PAIVS) 患者中的长期结果。我们分析了 1979 年至 2019 年间基于个体右心结构治疗的 PAIVS 患者的生存率和再干预率，并确定了预后的危险因素。本研究包括 95 名患者。肺环、三尖瓣环和右心室舒张末期容积的 z 值分别为 - 3.30（- 15.15 至 1.83）、- 0.70（- 4.65 至 2.33）和 - 1.51（- 6.35 至 1.18）。15%的患者出现右心室依赖性冠状动脉循环。在最初尝试双心室策略的 63 例患者中，55 例患者实现了双心室循环，3 例患者进行了一个半循环，4例患者围手术期死亡。在最初尝试单心室策略的33例患者中，10例患者在Fontan手术完成前死亡，17例（48%）患者完成了Fontan手术，5例患者等待Fontan手术。在一名患者中，发生了向双心室循环的转变。在 720 人年的随访期间，双心室循环患者的 20 年生存率明显高于单心室循环患者（93% vs. 67%，P < 0.001）。与单心室循环患者相比，双心室循环患者的 20 年免于再干预率显着降低（29% 对 72%，P < 0.001）。肺环 z 评分是双心室循环患者再干预的独立危险因素。双心室循环患者的存活率可接受，但再干预率高。同时，单心室循环患者在Fontan手术完成前死亡率较高，但再介入率较低。