Clinical lung and heart/lung transplantation
True Survival Benefit of Lung Transplantation for Cystic Fibrosis Patients: The Zurich Experience

Presented in abstract form at the 2008 European Cystic Fibrosis Society Meeting, Prague, Czech Republic.
https://doi.org/10.1016/j.healun.2008.12.025Get rights and content

Background

Lung transplantation is the ultimate therapy for end-stage cystic fibrosis (CF) lung disease; however, the debate continues as to whether lung transplantation improves survival. We report post-transplant outcome in CF at our institution by comparing 5-year post-transplant survival with a calculated 5-year survival without lung transplantation, using a predictive 5-year survivorship model, and describe pre-transplant parameters influencing transplant outcome.

Methods

CF patients undergoing lung transplantation at our center were included (1992 to 2007). Survival rates were calculated and compared, and univariate and multivariate Cox regression analyses were used for statistical assessment.

Results

Eighty transplants were performed in CF patients, 11 (13.8%) of whom were children. Mean age at transplant was 26.2 years (95% confidence interval: 24.4 to 28.0). The Liou raw score at transplant was −20 (95% confidence interval: −16 to −24), resulting in an estimated 5-year survival without transplantation of 33 ± 14%, compared with a 5-year post-transplant survival of 68.2 ± 5.6%. Further improvement was noted in the recent transplant era (since 2000), with a 5-year survival of 72.7 ± 7.3%. Univariate analysis revealed that later year of transplant and diagnosis of diabetes influenced survival positively. Pediatric age had no negative impact. In the multivariate analysis, only diabetes influenced survival, in a positive manner.

Conclusion

Lung transplantation performed at centers having experience with the procedure can offer a true survival benefit to patients with end-stage CF lung disease.

Section snippets

Study Population

In this study we included all pediatric and adult CF patients undergoing primary lung transplantation at the University Hospital Zurich, Switzerland, between November 1992 and November 2007. Transplant recipients were followed-up until August 2008. Patients' demographic data were collected. Pre-transplant clinical data included forced expiratory volume in 1 second (FEV1), body mass index (BMI), diagnosis of CF-related diabetes mellitus (CFDM) and bone mineral density at the hip/femoral neck

Study Population

Two hundred thirty lung transplant operations have been performed in our program since its establishment in 1992 up to November 2007. Of this cohort, 80 (34.5%) transplant recipients had CF, all of whom were included in this study (13.8% were <18 years of age). All except 1 patient underwent double-lung transplantation. One patient with additional congenital heart disease had a heart–lung transplant operation performed. Two CF patients were chronically infected with Burkholderia cepacia

Discussion

This observational study demonstrates a true survival benefit for CF patients with end-stage lung disease undergoing lung transplantation at our center. Estimated 5-year survival without transplantation in our cohort was 33% compared with a 5-year survival after transplantation of 67%. Moreover, the 1-year post-transplant survival in the recent era (since 2000) improved to >90% in CF transplant recipients, and for 3-year survival was >80%. Furthermore, pediatric age had no negative impact on

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