Outcomes of Lung Transplantation in Patients With Combined Pulmonary Fibrosis and Emphysema: A Single-Center Experience

• Published in: Transplantation proceedings Vol. 55; no. 2; pp. 449 - 455
• Main Authors: Narula, Tathagat, Martin, Archer K., Asif, Abuzar A., Fritz, Ashley V., Li, Zhuo, Erasmus, David B., Alvarez, Francisco, Thomas, Mathew
• Format: Journal Article
• Language: English
• Published: United States Elsevier Inc 01-03-2023
• Subjects: Adult; Emphysema - etiology; Humans; Hypertension, Pulmonary - etiology; Lung Transplantation - adverse effects; Pulmonary Emphysema - complications; Pulmonary Emphysema - diagnosis; Pulmonary Emphysema - surgery; Pulmonary Fibrosis - complications; Pulmonary Fibrosis - surgery; Retrospective Studies
• ISSN: 0041-1345; 1873-2623
• DOI: 10.1016/j.transproceed.2023.01.010

Combined pulmonary fibrosis and emphysema (CPFE) is a distinct clinical entity that can progress to end-stage lung disease. Patients with CPFE may develop pulmonary hypertension and face a predicted 1-year mortality of 60%. Lung transplantation is the only curative therapeutic option for CPFE. This report describes our experience after lung transplantation in patients with CPFE.
This retrospective, single-center study describes short- and long-term outcomes for adult patients who underwent lung transplant for CPFE.
The study included 19 patients with explant pathology-proven diagnosis of CPFE. The patients were transplanted between July 2005 and December 2018. Sixteen recipients (84%) had pulmonary hypertension before transplant. Of the 19 patients, 7 (37%) had primary graft dysfunction at 72 hours post-transplant. 1-, 3-, and 5-year freedom from bronchiolitis obliterans syndrome was 100%, 91% (95% CI, 75%–100%), and 82% (95% CI, 62%–100%), respectively. One-, 3-, and 5-year survival was 94% (95% CI, 84%–100%), 82% (95% CI, 65%–100%), and 74% (95% CI, 54%–100%), respectively.
Our experience demonstrates the safety and feasibility of lung transplant for patients with CPFE. Significant morbidity and mortality without lung transplant coupled with favorable post-transplant outcomes merit prioritization of CPFE in the Lung Allocation Score algorithm for lung transplant candidacy.