Survival after lung transplantation lags behind outcomes of other solid organ transplants, and complications from lung transplant are the second most common cause of death in cystic fibrosis. Evolving surgical techniques, therapeutics, and perioperative management have improved short-term survival after lung transplantation, yet have not translated into significant improvement in long-term mortality. Areas covered: We review risk factors for poor long-term outcomes among patients with cystic fibrosis undergoing lung transplantation to highlight areas for improvement. This includes reasons for organ dysfunction, complications of immunosuppression, further exacerbation of extrapulmonary complications of cystic fibrosis, and quality of life. A literature search was performed using PubMed-indexed journals. Expert commentary: There are multiple medical and socioeconomic barriers that threaten long-term survival following lung transplant for patients with cystic fibrosis. An understanding of the causes of each could elucidate treatment options. There is a lack of prospective, multicenter, randomized control trials due to cost, complexity, and feasibility. Ongoing prospective studies should be reserved for the most promising interventions identified in retrospective studies in order to improve long-term outcomes.
Keywords: Cystic fibrosis; lung transplant; quality of life.