Background: Cardiac transplantation for patients with complex congenital heart disease poses several anatomic and physiologic challenges for the transplant surgeon. We undertook the current single center study to evaluate surgical outcomes and lessons learned through a nearly twenty year experience with cardiac transplantation for complex congenital heart disease.
Methods: A retrospective review was performed to evaluate all patients undergoing cardiac transplantation from January 1, 1984 through January 1, 2004. Donor and recipient demographic and intraoperative and postoperative variables were acquired and correlated with perioperative (30-day) and late mortality in both univariate and multivariate analyses, and with Kaplan-Meier survival estimates.
Results: One hundred and six patients underwent transplantation for complex congenital heart disease and were followed for a median of 56 months. Thirty-seven (34.9%) patients died. Male gender and later year of transplantation were protective, and neonatal age and pulmonary artery reconstruction detrimental in multivariable modeling of overall mortality. Transplantation to a physiologic or anatomic single lung did not impact on survival. Patients in the study cohort had comparable survival estimates when compared with all those in the entire cohort without complex congenital heart disease. When comparing patients by era of transplantation, both cohorts demonstrated improved survival with later transplantation.
Conclusions: Outcomes with transplantation for complex congenital heart disease have improved annually over the past twenty years. Transplantation to an anatomic or physiologic single lung did not impair overall survival. Pulmonary artery reconstruction imparted an increase in mortality both short and long term, a finding which merits further investigation.