O The Bntah Camel] 1992 Lung transplantation for cystic fibrosis

Significant progress has been made since the first successful human heart-lung transplantation (HLT) for pulmonary vascular disease performed in 1981.1 The refinement of surgical techniques, use of cyclosporin as the main immunosuppressant, technique of distant organ procurement to expand the donor organ pool, and improved diagnosis and management of pulmonary infection and rejection have all contributed to this accomplishment. This has inevitably coincided with the extension of this procedure to other groups of patients with end stage heart and lung disease. Initially, HLT was offered to patients with cardiac disease associated with pulmonary hypertension. Because of the success, consideration was given to transplantation for parenchymal pulmonary diseases, initially pulmonary fibrosis and emphysema, and then suppurative lung disease such as in cystic fibrosis (CF). However, the application of HLT to patients with CF lagged behind because of concern related to the risk of sepsis, the systemic nature of the disease, malnourishment, and fear of recurrence of the epithelial CF defect in the transplanted lungs. The first successful heart-lung transplants for CF were performed in the UK at Harefield and Papworth Hospitals in 1985.2 ^ Approximatley 150 CF patients have undergone this procedure worldwide with encouraging results. During this time, other options of lung transplantation have developed. Single lung transplantation would not be suitable for CF patients as the remaining native lung would be a source of infection. En bloc double lung transplantation without tracheal revascularisation has given poor results due to tracheal anastomotic dehiscence, and bilateral single lung transplantation requires further evaluation. at Pennsylvania State U niversity on February 20, 201