Division of Respiratory Medicine, Center of Lung Transplantation, Center of Adult Cystic Fibrosis and CFTR-related disorders, Lausanne University Hospital and University of Lausanne, Lausanne, Switzerland; Division of Respirology, Lung Transplant Program, Toronto General Hospital, University Health Network, Toronto, ON, Canada; Division of Respirology, Adult Cystic Fibrosis Centre, St. Michael's Hospital, Toronto, ON, Canada.
Division of Respirology, Adult Cystic Fibrosis Centre, St. Michael's Hospital, Toronto, ON, Canada.
J Heart Lung Transplant. 2022 Nov;41(11):1617-1627. doi: 10.1016/j.healun.2022.07.017. Epub 2022 Jul 24.
Survival predictors are not established for cystic fibrosis (CF) patients listed for lung transplantation (LT). Using the deficit accumulation approach, we developed a CF-specific frailty index (FI) to allow risk stratification for adverse waitlist and post-LT outcomes.
We studied adult CF patients listed for LT in the Toronto LT Program (development cohort 2005-2015) and the Swiss LT centres (validation cohort 2008-2017). Comorbidities, treatment, laboratory results and social support at listing were utilized to develop a lung disease severity index (LI deficits, d = 18), a frailty index (FI, d = 66) and a lifestyle/social vulnerability index (LSVI, d = 10). We evaluated associations of the indices with worsening waitlist status, hospital and ICU length of stay, survival and graft failure.
We studied 188 (Toronto cohort, 176 [94%] transplanted) and 94 (Swiss cohort, 89 [95%] transplanted) patients. The median waitlist times were 69 and 284 days, respectively. The median follow-up post-transplant was 5.3 and 4.7 years. At listing, 44.7% of patients were frail (FI ≥ 0.25) in the Toronto and 21.3% in the Swiss cohort. The FI was significantly associated with all studied outcomes in the Toronto cohort (FI and post-LT mortality, multivariable HR 1.74 [95%CI:1.24-2.45] per 0.1 point of the FI). In the Swiss cohort, the FI was associated with worsening waitlist status, post-LT mortality and graft failure.
In CF patients listed for LT, FI risk stratification was significantly associated with waitlist and post-LT outcomes. Studying frailty in young populations with advanced disease can provide insights on how frailty and deficit accumulation impacts survival.
对于接受肺移植（LT）的囊性纤维化（CF）患者，尚未建立生存预测指标。我们采用缺陷积累方法，开发了一种 CF 特定的虚弱指数（FI），以对不良等待名单和 LT 后结果进行风险分层。
我们研究了多伦多 LT 计划（开发队列 2005-2015 年）和瑞士 LT 中心（验证队列 2008-2017 年）中列出的成年 CF 患者。在列出名单时，利用合并症、治疗、实验室结果和社会支持来制定肺疾病严重程度指数（LI 缺陷，d=18）、虚弱指数（FI，d=66）和生活方式/社会脆弱性指数（LSVI，d=10）。我们评估了这些指数与等待名单状态恶化、住院和 ICU 住院时间、生存和移植物失败的关联。
我们研究了 188 名（多伦多队列，176 名[94%]接受移植）和 94 名（瑞士队列，89 名[95%]接受移植）患者。等待名单中位数分别为 69 天和 284 天。移植后中位随访时间分别为 5.3 年和 4.7 年。在列出名单时，多伦多队列中 44.7%的患者（FI≥0.25）虚弱，瑞士队列中 21.3%的患者虚弱。FI 在多伦多队列中与所有研究结果均显著相关（FI 和 LT 后死亡率，FI 每增加 0.1 分，多变量 HR 为 1.74[95%CI：1.24-2.45]）。在瑞士队列中，FI 与等待名单状态恶化、LT 后死亡率和移植物失败相关。
在接受 LT 的 CF 患者中，FI 风险分层与等待名单和 LT 后结果显著相关。在患有晚期疾病的年轻人群中研究虚弱可以深入了解虚弱和缺陷积累如何影响生存。
