Cystic fibrosis: a new challenge for cardiothoracic surgery.

Cystic fibrosis (CF) is the most common fatal inherited disease in caucasian populations. Between 80-120 CF patients die each year in England and Wales, the majority from pulmonary disease. Since October 1985, 17 patients with CF have undergone heart-lung transplantation (HLT) at our institution. Only 3 early deaths have occurred in the first 2 postoperative months. Another patient died 9 months after transplantation. Thirteen patients are now alive 1-46 months (mean 14 month) post transplantation. Actuarial survival at 6 months is 80% for CF patients compared to 73% for non-CF patients. Both groups of patients have similar profiles of rejection and infection. In the Caucasian population, CF patients form the largest potential recipient group for HLT and our experience suggests that those CF patients who undergo HLT have a favourable outcome.