Background

Despite therapeutic advances, people with cystic fibrosis (CF) develop progressive worsening and exacerbations of their lung disease, which can lead to acute respiratory failure. Historically, survival after mechanical ventilation (MV) has been poor. Outcomes related to use of extracorporeal membrane oxygenation (ECMO) have not been well described in CF.

Methods

We conducted a retrospective analysis of adult patients with CF admitted to the ICU for acute respiratory failure and requiring invasive MV with or without ECMO between July 1, 2006 and June 30, 2016. Separate analysis for the subgroup of MV patients who were eligible for transplant was conducted.

Results

Mortality for all patients with respiratory failure requiring advanced support was 37%. Ten of 28 (36%) MV patients, 10 of 26 (38%) ECMO+MV patients and 7 of the 21 (33%) transplant eligible MV patients died. Intensive care unit (ICU) length of stay (LOS) was 24.5±16.6 days for ECMO+MV; 12.9±9.0 days for MV (p=0.001), and 12.3 ±10 days for transplant eligible MV patients (p=0.005 for ECMO+MV comparison). Seven transplant eligible MV patients (33%) and 16 ECMO+MV patients (62%) underwent lung transplantation (p<0.001) during the hospital admission. One and 2-year survival for individuals who survived ICU admission was similar regardless of mode of support. Cox-proportional hazards model did not yield any variables that significantly influenced ICU mortality, 1-year or 2-year mortality.

Conclusion

Survival for CF patients with acute respiratory failure requiring MV with or without ECMO has improved over time. ECMO may be an appropriate modality for respiratory support in patients with CF and acute respiratory failure who have greater risk of death from MV alone.

中文翻译：

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体外膜氧合和/或有创机械通气支持急性呼吸衰竭后囊性纤维化的生存

背景

尽管治疗取得了进展，但囊性纤维化 (CF) 患者的肺部疾病会逐渐恶化和恶化，这可能导致急性呼吸衰竭。从历史上看，机械通气 (MV) 后的存活率一直很差。与使用体外膜肺氧合 (ECMO) 相关的结果尚未在 CF 中得到很好的描述。

方法

我们对 2006 年 7 月 1 日至 2016 年 6 月 30 日期间因急性呼吸衰竭入住 ICU 并需要侵入性 MV 联合或不联合 ECMO 的 CF 成年患者进行了回顾性分析。对符合移植条件的 MV 患者亚组进行单独分析进行了。

结果

所有需要高级支持的呼吸衰竭患者的死亡率为 37%。28 名 (36%) MV 患者中的 10 名、26 名 (38%) ECMO+MV 患者中的 10 名和 21 名 (33%) 符合移植条件的 MV 患者中的 7 名死亡。ECMO+MV 的重症监护病房 (ICU) 住院时间 (LOS) 为 24.5±16.6 天；MV 为 12.9±9.0 天（p=0.001），符合移植条件的 MV 患者为 12.3±10 天（对于 ECMO+MV 比较，p=0.005）。7 名符合移植条件的 MV 患者 (33%) 和 16 名 ECMO+MV 患者 (62%) 在住院期间接受了肺移植 (p<0.001)。无论采用何种支持方式，ICU 入院后幸存者的 1 年和 2 年生存率相似。Cox 比例风险模型没有产生任何显着影响 ICU 死亡率、1 年或 2 年死亡率的变量。

结论

随着时间的推移，需要 MV 伴或不伴 ECMO 的急性呼吸衰竭 CF 患者的生存率有所提高。对于 CF 和急性呼吸衰竭患者，ECMO 可能是一种合适的呼吸支持方式，这些患者仅因 MV 而死亡的风险更大。