RESULTS OF HEART-LUNG TRANSPLANTATION IN CHILDREN WITH CYSTIC-FIBROSIS

Children with cystic fibrosis represent the largest group referred for , and undergoing, heart-lung transplantation at our institute. Between June 1988 and July 1993, 76 patients were accepted for transplantatio n, of whom 25 were transplanted, while a further 36 died waiting. Thos e transplanted ranged from 5-18 years of age and included 13 males and 12 females. Organs were used from donors matched by ABO blood group, size and cytomegalovirus (CMV) status. Post-transplant maintenance imm unosuppression comprised cyclosporin A, azathioprine and prednisolone. Anti-thymocyte globulin and high dose methylprednisolone were given p eri-operatively and for acute rejection episodes. Acturial survival wa s 67% at 1 year, 61% at 2 years and 54% at 3 years. Obliterative bronc hiolitis (OB) has occurred in 13 patients (52%) and was the major caus e of mortality and morbidity. In three patients, OB was associated wit h the development of tracheal anastomotic stenosis. Other complication s included diabetes mellitus (n = 9), pancreatitis (n = 1) and hyperte nsion (n = 8). Despite these problems, those surviving the first year post-transplant showed a mean FEV1 of 71% (compared to 29% pre-transpl ant) and enjoyed an overall improved quality of life.