ORTHOTOPIC HEART-TRANSPLANTATION IN CHILDREN WITH CONGENITAL HEART-DISEASE

The early experience (February 1982 to June 1988) with transplantation for the treatment of congenital heart disease at the University of Pi ttsburgh was disappointing due to an excessively high perioperative mo rtality. From July 1988 to Tune 1992, a further 21 children with conge nital heart disease underwent orthotopic transplantation. Thirteen had undergone multiple prior palliative procedures (mean, 2.8 per patient ). In 12 of these patients, prior procedures involved the pulmonary ar teries on one or more occasions. In contrast to our earlier experience , there were no deaths stemming from inadequate surgical reconstructio n or pulmonary hypertension. The actuarial survival was 71% at both 1 and 3 years. This did not differ significantly from the survival among 18 patients who underwent transplantation for the management of cardi omyopathy over the same period (1-year and 3-year survival, 83%). The perioperative mortality and shortterm survival are now similar for chi ldren undergoing transplantation for the treatment of either congenita l heart disease or cardiomyopathy. These improved results probably ref lect more careful patient selection and an increasing surgical experie nce with complex reconstructive procedures.