INCIDENCE OF OBLITERATIVE BRONCHIOLITIS AFTER HEART-LUNG TRANSPLANTATION IN CHILDREN

Between June 1988 and February 1993, combined heart-lung transplantati on was performed in 30 children and adolescents aged 3.6 to 18.6 years (mean, 12.2 years) at The Hospital for Sick Children in London. Origi nal diagnoses included cystic fibrosis (n = 25), Eisenmenger's syndrom e (n = 4), and chronic graft-versus-host disease of the lung (n = 1). Posttransplantation maintenance immunosuppression comprised a triple r egimen, with methylprednisolone and antithymocyte globulin given perio peratively and for episodes of allograft rejection. Actuarial survival was 63% (95% confidence interval 42% - 78%) at 1 year and 48% (95% co nfidence interval: 27% - 66%) at 3 years. Obliterative bronchiolitis h as been diagnosed in 13 patients (43%). Actuarial freedom from obliter ative bronchiolitis in survivors was 76%, 59%, and 37% at 12, 24, and 36 months after transplantation, respectively. Recipients in whom obli terative bronchiolitis developed within the first year (n = 6) had mor e episodes of pulmonary rejection during the first 6 months after tran splantation (mean, 5.7 episodes per patient) than those in whom ''prem ature'' obliterative bronchiolitis did not develop (mean, 3.2 episodes per patient). Infection of the pulmonary allograft was implicated to a lesser extent in predisposing to obliterative bronchiolitis. At 2, 3 , and 6 months, tracheal stenosis developed in three patients, all of whom died with obliterative bronchiolitis within 10 months of transpla ntation. Noncompliance with therapy was considered a contributory fact or in producing obliterative bronchiolitis in four adolescent recipien ts. The high incidence of obliterative bronchiolitis observed in this pediatric cohort may have a multifactorial cause.