Double lung transplantation is a feasible treatment approach for end-stage lung disease due to cystic fibrosis (CF), according to a new study published in Transplantation Proceedings. Although this approach is technically challenging, the researchers found significantly better survival among transplant recipients with CF than among those with other underlying conditions.

“The main goal is to perform lung transplant only when all other therapeutic options have reached their limits and the risks from not only such a procedure but also lifelong immunosuppressive treatment are outweighed by the benefits,” the researchers explained.

“Lung transplantation among CF patients should be considered if CFTR [cystic fibrosis transmembrane conductance] modulators are not providing expected results.”

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The research team assessed survival, reoperations, and intraoperative death among 56 patients with CF undergoing primary lung transplantation via minithoracotomy between 2018 and 2021. There was only 1 intraoperative patient death.

Two patients needed reoperation, and among all patients studied, 1-year survival was 87%, 2-year survival was 85%, and 3-year survival was 75%. All patients reported a significant improvement in spirometry results after the transplant compared with before the procedure.

The authors note that CFTR modulators are changing the landscape of CF therapy because they appear to offer a safer and more therapeutic option for patients than other treatments and do not entail the risks of transplantation. However, until CFTR modulators are more affordable and available for all patients with CF, double lung transplantation remains the main treatment option for patients with end-stage respiratory failure.

The researchers also reported that between the 2 possible surgical approaches to double lung transplantation, the clam-shell incision or bilateral sequential minithoracotomy, the latter offered more effective rehabilitation and wound healing.

Reference

Urlik M, Stącel T, Latos M, et al. Results of lung transplantations among cystic fibrosis patients: a single-center study. Transplant Proc. Published online April 29, 2022. doi:10.1016/j.transproceed.2022.02.056