PEDIATRIC CARDIAC TRANSPLANTATION - THE STANFORD EXPERIENCE

Background Cardiac transplantation for children with endstage heart di sease has become an accepted form of therapy and is being practiced wi th increasing frequency and improving short-term outcome. Methods and Results To assess the medium-term outcome of pediatric cardiac transpl antation, we analyzed our experience with 72 patients under the age of 18 (range, 0.1 to 17.7 years; mean, 9+/-6.4 [SD]) who underwent ortho topic cardiac transplantation at Stanford University between 1977 and 1993. There were 38 male and 34 female patients. Preoperative diagnose s included congenital heart disease in 24 (33%), idiopathic cardiomyop athy in 27 (37%), viral cardiomyopathy in 12 (17%), and familial cardi omyopathy in 7 (10%) patients. Immunosuppressive management has evolve d over time and has included a tapering schedule of steroids, azathiop rine, rabbit antithymocyte globulin, cyclosporine in all patients afte r 1980, and induction with OKT3 since 1987. Operative mortality rate w as 12.5+/-4.0% (mean+/-70% confidence intervals). Actuarial survival e stimates at 1, 5, and 10 years are 75+/-7.1%, 60+/-6.4%, and 50+/-8.1% (mean+/-1 SEM), respectively. Causes of death included infection in 8 (28% of deaths), rejection in 7 (24%), graft coronary disease in 5 (1 7%), pulmonary hypertension in 4 (14%), and nonspecific graft failure in 2 (7%) patients. Survival rates were similar for patients over and those under age 10 years (including the infant cohort of 18 patients t ransplanted since 1986). Currently, there are 43 patients alive, all i n New York Heart Association functional class I. Only 22+/-5.6% of pat ients were free of rejection at 1 year, but 86+/-5.4% were free of rej ection-related death at 10 years. At 1 year, only 37+/-6% of patients were free from any infection, but 88+/-4.2% remained free of infection -related death at 5 years. Actuarial freedom from graft coronary arter y disease (angiographic or autopsy proven) was 85+/-6.6% at 5 years an d from coronary artery disease-related death was 91+/-4.7%. Conclusion s These data demonstrate satisfactory medium-term outcome of cardiac t ransplantation in selected pediatric patients with end-stage heart dis ease, but further progress is necessary to more effectively control re jection, infection, and graft coronary disease.