Lung transplantation for cystic fibrosis

There have been several recent articles which have attempted to refine the appropriate timing of listing for lung transplantation and the identification of factors which increase the risk with particular emphasis on pretransplant microbiology in patients with cystic fibrosis. Moreover, there are several series describing long-term outcomes and demonstrating improved long-term survival following lung transplantation for patients with cystic fibrosis. This review attempts to summarize the main points.

Although forced expiratory volume in 1 s still plays an important role in timing for listing for lung transplantation, other factors such as arterial PaCO2, number of exacerbations, sex, weight, development of pneumothorax and microbiology of colonizing bacteria with particular relevance to Burkholderia all have been shown to influence survival in patients with advanced cystic fibrosis. The median survival should now be around 10 years, and strategies to reduce renal damage and chronic allograft dysfunction including preventing gastro-oesophageal reflux and airway infection receive greater emphasis.

This article attempts to provide an overview of the current important topics for the clinician involved in cystic fibrosis lung transplantation.