ORIGINAL CLINICAL SCIENCE
An aging population of patients with cystic fibrosis undergoes lung transplantation: An analysis of the ISHLT Thoracic Transplant Registry

https://doi.org/10.1016/j.healun.2019.06.025Get rights and content

BACKGROUND

Since lung transplantation became a viable option for cystic fibrosis (CF) lung disease, adult transplant recipients with CF have superior survival than all the other major diagnostic indications. However, among adults, recipients with CF have a younger age at transplant than other transplant recipients. Over time, the frequency and proportion of lung transplants for CF has increased for adults compared with children. Using a large international transplant registry, we investigated time trends in numbers of transplants, age at transplant, and post-transplant survival and cause of death for recipients with CF.

METHODS

We conducted a retrospective cohort study of primary lung-alone deceased-donor transplants with a primary diagnostic indication of CF reported to the International Society for Heart and Lung Transplantation Thoracic Transplant Registry from January 2005 through December 2014. We assessed outcomes through December 31, 2015. The study defined the pediatric group as age <18 years at transplant and the adult as ≥18 years at transplant. We assessed time trends (Era I 2005–2009, Era II 2010–2014) in age and compared post-transplant outcomes of age sub-groups with Kruskal–Wallis or chi-square tests. Kaplan–Meier survival analysis estimated the incidence of survival, censoring for loss to follow-up, end of study, and retransplant. In addition, we compared outcomes in age groups and transplant eras with the log-rank test.

RESULTS

Of the 5,613 transplanted recipients with CF, the pediatric group comprised 10.9% and the adult group comprised 89.1%. Of the adults, 73.3% were aged 18 to 39 years and 15.9% were ≥40 years old. During Era I, 2,508 of transplant recipients had CF, whereas 3,105 recipients had CF in Era II (p < 0.001). Comparing Era I with Era II, recipient mean age increased from 28.4 years to 29.5 years (p < 0.001) and the proportion of pediatric CF recipients dropped from 12.4% to 9.6% (p < 0.001), whereas the proportion with age ≥40 years increased from 14.2% to 17.2% (p < 0.001). Mean donor age was significantly lower in children than in recipients aged 18 to 39 years and ≥40 years (17.0 vs 37.0 vs 41.0 years, p < 0.001). Pediatric CF transplant recipients had lower survival in the first 3 years post-transplant than adults (p < 0.0001). Chronic graft failure caused most pediatric deaths, whereas infection was the leading cause of death in adult recipients.

CONCLUSION

As survival of patients with CF has improved in recent decades, the mean age of lung transplant recipients with CF has increased. Currently, an increasing number of adults undergoes lung transplant for this indication. Adult CF transplant recipients continue to have better survival than pediatric recipients, and among adults, older adults have had better survival than younger adults.

Section snippets

Study design

Using data provided via a data sharing agreement by the ISHLT Thoracic Transplant Registry (www.ishlt.org/registries), a multinational database of thoracic organ transplants, we conducted a retrospective cohort study of reported deceased-donor primary lung-alone transplants in patients with CF reported to the Registry from January 1, 2005 through December 31, 2014. The study follow-up termination date of December 31, 2015 allowed for a potential minimum of 12 months post-transplant follow-up.

Study cohort and age sub-groups

A total of 5,613 lung transplant recipients had the primary diagnostic indication of CF-associated lung disease, including 610 (10.9%) pediatric recipients and 5,003 (89.1%) adult recipients. Of the adults, 73.3% were aged 18 to 39 years and 15.9% were aged ≥40 years.

Number transplanted and age of recipients during the 2 study eras

The number of recipients with CF increased significantly from 2,508 in Era I to 3,105 in Era II (p < 0.001), mainly because of an increase in the number of adult transplants (Figure 1). Thus, the proportion of pediatric recipients

Discussion

This study demonstrates that the number of transplants reported for a primary diagnostic indication of CF-associated lung disease has increased during the past decade. During this time, the proportion of transplants for CF in adults has increased, whereas the proportion in children has not. Adult patients from 2 age groups undergoing transplantation for CF-associated lung disease experienced a better survival than pediatric transplant recipients, and adult recipients of at least 40 years of age

Disclosure statement

The authors have no conflicts of interest to disclose.

The authors acknowledge Leah B. Edwards’ tremendous expert input and statistical support with the data analysis and Roger D. Yusen's great assistance with the manuscript revision.

This work was partially presented at the 36th Annual Meeting and Scientific Sessions of the International Society for Heart and Lung Transplantation in Washington D.C., USA.

References (37)

Cited by (21)

  • How Would We Treat Our Own Cystic Fibrosis With Lung Transplantation?

    2024, Journal of Cardiothoracic and Vascular Anesthesia
  • Pathological remodeling of distal lung matrix in end-stage cystic fibrosis patients

    2022, Journal of Cystic Fibrosis
    Citation Excerpt :

    Cystic fibrosis (CF) is characterized by a pathologic cascade of impaired ion transfer, dysregulation of airway surface liquid and mucus, inability to clear infection, chronic inflammation, and ultimately end-stage lung disease necessitating transplantation [1,2].

  • Ethical Dilemma: Elexacaftor-Tezacaftor-Ivacaftor or Lung Transplantation in Cystic Fibrosis and End-Stage Lung Disease?

    2022, Chest
    Citation Excerpt :

    Furthermore, no data on long-term follow-up are currently available and it remains unknown whether elexacaftor-tezacaftor-ivacaftor treatment would provide survival benefits, over lung transplantation, in patients with end-stage lung disease. Data on lung transplantation have a well-demonstrated survival benefit for adult patients with cystic fibrosis.24-26 In pediatric patients, reports demonstrate lower survival when compared with adults,24,25,27,28 with a median survival of about 3.5 years for pediatric patients.25,29

  • Automated antimicrobial susceptibility testing of slow-growing Pseudomonas aeruginosa strains in the presence of tetrazolium salt WST-1

    2021, Journal of Microbiological Methods
    Citation Excerpt :

    Lung transplantation (LTx) remains as a last resort for patients with advanced lung infections due to the limited efficacy of conventional antibiotic therapies. The total number of adult CF patients undergoing LTx has increased by 23.8% within a time-span of 5 years (Benden et al., 2019). These care measures contribute to a significant increase in the costs of CF-related hospitalization (Van Gool et al., 2013; Perkins et al., 2021).

View all citing articles on Scopus
View full text