Outcomes of Lung Transplantation in Patients With Combined Pulmonary Fibrosis and Emphysema: A Single-Center Experience |
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| Institution: | 1. Department of Transplantation, Mayo Clinic, Jacksonville, Florida;2. Department of Anesthesiology and Perioperative Medicine, Mayo Clinic, Jacksonville, Florida;3. Internal Medicine, University of Illinois College of Medicine, Peoria, Illinois;4. Department of Biostatistics, Mayo Clinic, Jacksonville, Florida;5. Division of Allergy, Pulmonary, and Critical Care Medicine and The Vanderbilt Lung Institute;6. Department of Cardiothoracic Surgery, Mayo Clinic, Jacksonville, Florida;1. Section of Renal Transplantation, Edward Hines VA Jr Hospital, Hines, Illinois;2. Department of Surgery, Division of Intra-Abdominal Transplantation, Stritch School of Medicine, Maywood, Illinois;3. Department of Medicine, Division of Transplant Nephrology, Stritch School of Medicine, Maywood, Illinois;1. Private Inova Hospital, Urology, Aksaray, Turkey;2. Asklepios Klinik Triberg, Urology, Triberg, Germany;3. University of Health Sciences, Ankara City Hospital, Department of Urology, Ankara, Turkey;4. Bingol State Hospital, Department of Urology, Bingol, Turkey;1. Clinic for Nephrology, Clinical Center of Montenegro, Podgorica, Montenegro;2. Department of Nephrology, Arterial Hypertension, Dialysis and Transplantation, University Hospital Centre Zagreb, Zagreb, Croatia;3. Department of Pathology and Cytology, University Hospital Centre Zagreb, Zagreb, Croatia;4. Clinic for Urology, University Hospital Center Zagreb, Zagreb, Croatia;1. Department of Liver Transplantation and Hepato-pancreatico-biliary Surgery, Pakistan Kidney and Liver Institute & Research Centre, Lahore, Pakistan;2. Supernova School Islamabad, Islamabad, Pakistan;3. Benazir Income Support Program, Islamabad, Pakistan;1. Ajmera Transplant Center, University Health Network, Toronto, Ontario, Canada;2. Center for Mental Health, University Health Network, Toronto, Ontario, Canada;3. Multi-Organ Transplant & Medical Specialties, The Hospital for Sick Children, Toronto, Ontario, Canada;4. Temerty Faculty of Medicine, University of Toronto, Toronto, Ontario, Canada;1. Center for Stem Cell and Regenerative Medicine, Tokyo Medical and Dental University, Tokyo, Japan;2. Japan Tissue Engineering Co., Ltd., Aichi, Japan;3. Fujifilm Corporation, Tokyo, Japan;4. Department of Joint Surgery and Sports Medicine, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University, Tokyo, Japan |
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| Abstract: | BackgroundCombined 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.MethodsThis retrospective, single-center study describes short- and long-term outcomes for adult patients who underwent lung transplant for CPFE.ResultsThe 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.ConclusionOur 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. |
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