Fenestration in the total cavopulmonary connection system may improve the outcome of patients with significant risk factors for Fontan haemodynamics. Our study aims to analyse the difference in long-term survival between non-fenestrated and fenestrated patients.

All consecutive patients (n = 351) who underwent total cavopulmonary connection between 1992 and 2016 were identified. Six early deaths were excluded resulting in a group of 345 patients. Median (interquartile range,) length of follow-up was 14.4 (7.1–19.7) years. Freedom from the composite endpoint of death, total cavopulmonary connection take-down or indication for a heart transplant was analysed.

Fenestration was absent in 237 patients (68.7%, Group 1), was created and closed later in 79 patients (22.9%, Group 2), and remained open in 29 patients (8.4%, Group 3). Mean survival probability until composite endpoint was 97.1 and 92.9% at 10 and 20 years, respectively. Patients with patent fenestration had worse survival (p < 0.001) as compared to both the non-fenestrated and fenestration closure groups. Despite a similar outcome, exercise capacity was lower in Group 2 than 1 (p = 0.013). In 58 patients with interventional fenestration closure, Nakata index was lower at the time of closure than pre-operatively, and both the pressure in the circuit and oxygen saturation in the aorta increased significantly (p < 0.001).

Patients with persisting risk factors preventing fenestration closure are at higher risk of reaching the composite endpoint. Patients after fenestration closure have the worse functional outcome; their survival is, however, not different from the non-fenestrated group.

全腔静脉肺连接系统中的开窗可以改善具有显着 Fontan 血流动力学危险因素的患者的预后。我们的研究旨在分析未开窗和开窗患者之间长期生存率的差异。

确定了 1992 年至 2016 年期间接受全腔肺连接的所有连续患者 (n = 351)。六名早期死亡被排除在外，导致一组 345 名患者。中位（四分位距）随访时间为 14.4（7.1-19.7）年。分析了免于死亡、完全腔肺连接断开或心脏移植指征的复合终点。

237 名患者（68.7%，第 1 组）没有开窗，79 名患者（22.9%，第 2 组）后来创建并关闭，29 名患者（8.4%，第 3 组）保持开放。在 10 年和 20 年，直到复合终点的平均生存概率分别为 97.1% 和 92.9%。与非开窗组和开窗闭合组相比，开放开窗患者的生存率更差（p < 0.001）。尽管结果相似，但第 2 组的运动能力低于第 1 组（p = 0.013）。58例介入性开窗封堵患者，封堵时中田指数低于术前，回路压力和主动脉血氧饱和度均显着升高（p < 0.001）。

具有阻止开窗关闭的持续风险因素的患者达到复合终点的风险更高。开窗关闭后的患者功能结果较差；然而，他们的生存与未开窗组没有什么不同。