BACKGROUND

The contribution of lung transplantation to the treatment of patients with end-stage cystic fibrosis (CF) has been debated. We aimed to describe achievable outcomes from high-volume CF and lung transplant programs. This study reports on the largest single-center experience of lung transplantation for adult and pediatric patients with CF. It also highlights the evolution of practice and outcomes over time.

METHODS

A retrospective analysis of the prospectively collected Toronto Lung Transplant database was carried out. Post-transplant survival in CF was calculated using the Kaplan–Meier method and analyzed with log-rank tests.

RESULTS

From 1983 to 2016, a total of 1,885 transplants were performed at our institution, where 364 (19.3%) were CF recipients and another 39 (2.1%) were CF retransplants. The mean age at first transplant was 29.5 ± 9.7 years where 56.6% were males and 91.5% were adults. Pre-transplantation, 88 patients (24.2%) were Burkholderia cepacia complex (BCC)-positive, 143 (39.3%) had diabetes mellitus, and the mean forced expiratory volume in one second was 26.0 ± 7.2%, as predicted at listing. The 1-, 5-, and 10-year probabilities of survival in adults who were BCC-negative were 94%, 70%, and 53%, respectively. Pediatric, BCC-positive, and retransplant recipients had worse survival than adult patients who were BCC-negative. Strategies to improve the donor pool did not affect survival but possibly reduced waitlist mortality. For the entire cohort, the most common causes of death after lung transplant were infection and chronic lung allograft dysfunction.

CONCLUSIONS

Lung transplantation for CF provides excellent short- and long-term outcomes. These results strongly support lung transplantation as the standard of care for patients with CF having advanced lung disease.

中文翻译：

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肺移植治疗囊性纤维化。

背景

肺移植对终末期囊性纤维化（CF）患者治疗的贡献已有争议。我们旨在描述大批量CF和肺移植计划可实现的结果。这项研究报告了成人和儿童CF的最大的单中心肺移植经验。它还强调了实践和结果随时间的演变。

方法

对前瞻性收集的多伦多肺移植数据库进行了回顾性分析。使用Kaplan-Meier方法计算CF中的移植后存活率，并通过对数秩检验进行分析。

结果

从1983年到2016年，我们的机构共进行了1885例移植，其中364例（19.3％）是CF接受者，另外39例（2.1％）是CF移植。首次移植的平均年龄为29.5±9.7岁，其中男性为56.6％，成年人为91.5％。移植前88名患者（24.2％）为洋葱伯克霍尔德氏菌如清单所示，复合物（BCC）阳性，有143名（39.3％）患有糖尿病，一秒钟的平均强迫呼气量为26.0±7.2％。BCC阴性的成年人的1年，5年和10年生存率分别为94％，70％和53％。小儿，BCC阳性和再移植接受者的生存率比BCC阴性的成年患者差。改善供体库的策略不会影响生存，但可能会降低候补名单的死亡率。对于整个队列，肺移植后死亡的最常见原因是感染和慢性同种异体移植功能障碍。

结论

CF的肺移植可提供出色的短期和长期结果。这些结果强烈支持将肺移植作为患有晚期肺病的CF患者的护理标准。