Lung Transplantation for Cystic Fibrosis: Results, Indications, Complications, and Controversies

Abstract

Survival in patients with cystic fibrosis (CF) has improved dramatically over the past 30 to 40 years, with mean survival now approximately 40 years. Nonetheless, progressive respiratory insufficiency remains the major cause of mortality in CF patients, and lung transplantation (LT) is eventually required. Timing of listing for LT is critical, because up to 25 to 41% of CF patients have died while awaiting LT. Globally, approximately 16.4% of lung transplants are performed in adults with CF. Survival rates for LT recipients with CF are superior to other indications, yet LT is associated with substantial morbidity and mortality (~50% at 5-year survival rates). Myriad complications of LT include allograft failure (acute or chronic), opportunistic infections, and complications of chronic immunosuppressive medications (including malignancy). Determining which patients are candidates for LT is difficult, and survival benefit remains uncertain. In this review, we discuss when LT should be considered, criteria for identifying candidates, contraindications to LT, results post-LT, and specific complications that may be associated with LT. Infectious complications that may complicate CF (particularly Burkholderia cepacia spp., opportunistic fungi, and nontuberculous mycobacteria) are discussed.

Incidence and risk factors for respiratory tract bacterial colonization and infection in lung transplant recipients

“…We included in the analysis only those subjects who had at least 18 years of age, had an available 1-month post-transplant follow-up, and complete clinical and microbiological data after transplantation. We excluded patients who underwent LT for cystic fibrosis since this disease has its own clinical features in terms of either recipient typology or pre-transplant colonization and infection etiologies, and therefore it is less comparable to the other lung transplant indications [14]. Patients were followed from the time of LT to a 1 month of follow-up and evaluated for the occurrence of bacterial respiratory colonization or infection, as below defined.…”

Novel therapeutic approaches for the management of cystic fibrosis

“…This would depend upon how the transplant team have evaluated their condition, but also on the availability of a donor that is a good match for the patient. For most, this takes several months where only two in three will be successful in having a transplant [ 107 ]. Survival rates at 5 years after the procedure are approximately 60% among CF patients, with many of these living for at least 10 years [ 108 ].…”

Host-Pathogen Interactions after Lung Transplant: Are Cystic Fibrosis Patients Unique?

“… 2 Pseudomonas aeruginosa (PsA) colonization and infection are risk factors for the development of CLAD; 3 however, the role of PsA on lung transplant outcomes in patients with CF is less clear. 4 , 5 , 6 , 7 Cystic fibrosis transmembrane conductance regulator ( CFTR ) gene mutations may lead to distinct immune responses in CF lung transplant recipients, 8 especially when coupled with the unique airway microbiome that CF patients harbor. In this issue of Cell Reports Medicine , Dugger et al.…”

“… 9 Pan-resistant PsA in CF lung transplant recipients has been associated with worse outcomes in some, 10 but not all, reports. 4 , 5 , 7 Confounding factors such as age and center-specific management protocols have made registry-based epidemiologic studies comparing CF versus non-CF lung transplant recipients difficult.…”