Toronto Lung Transplant Program, Toronto General Hospital, Toronto, Ontario, Canada; Division of Thoracic Surgery, Toronto General Hospital, Toronto, Ontario, Canada.
Toronto Lung Transplant Program, Toronto General Hospital, Toronto, Ontario, Canada; Division of Respirology, Toronto General Hospital, Toronto, Ontario, Canada; Adult CF Program, St Michael's Hospital, University of Toronto, Toronto, Ontario, Canada.
J Heart Lung Transplant. 2020 Jun;39(6):553-560. doi: 10.1016/j.healun.2020.02.010. Epub 2020 Feb 20.
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.
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.
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.
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.
肺移植治疗终末期囊性纤维化（CF）患者的效果一直存在争议。本研究旨在描述高容量 CF 和肺移植项目的可实现结果。本研究报告了最大的单一中心 CF 成人和儿科患者肺移植经验。它还强调了实践和结果随时间的演变。
对前瞻性收集的多伦多肺移植数据库进行回顾性分析。使用 Kaplan-Meier 方法计算 CF 患者的移植后生存率，并使用对数秩检验进行分析。
1983 年至 2016 年，我院共进行了 1885 例移植手术，其中 364 例（19.3%）为 CF 受者，另有 39 例（2.1%）为 CF 再次移植。首次移植时的平均年龄为 29.5 ± 9.7 岁，其中 56.6%为男性，91.5%为成年人。移植前，88 例（24.2%）患者为洋葱伯克霍尔德菌复合群（BCC）阳性，143 例（39.3%）患有糖尿病，1 秒用力呼气量预测值为 26.0 ± 7.2%。BCC 阴性成人患者的 1、5 和 10 年生存率分别为 94%、70%和 53%。儿童、BCC 阳性和再次移植受者的生存率低于 BCC 阴性的成年患者。改善供体库的策略并未影响生存率，但可能降低了候补名单死亡率。对于整个队列，肺移植后死亡的最常见原因是感染和慢性肺移植物功能障碍。
肺移植治疗 CF 可获得优异的短期和长期效果。这些结果强烈支持将肺移植作为晚期 CF 患者的标准治疗方法。
