Surgical mortality is high in children with
visceral heterotaxy (VH), particularly if atrioventricular valve insufficiency,
ventricular dysfunction, or aortic atresia is present. This study reviews the outcome of
cardiac transplantation (CT) in infants and children with VH and
congenital heart disease who are at high risk for standard palliative or corrective surgery. We reviewed CT outcomes in 29 children with VH,
congenital heart disease, atrioventricular valve insufficiency,
ventricular dysfunction, and/or aortic atresia. Median age at CT was 3.1 years. Cardiac surgery had been performed in 20 patients (69%) before CT. Follow-up since CT has been 8.5 +/- 2.2 years. Outcomes were compared with 45 children who underwent
transplantation for
dilated cardiomyopathy. Actuarial graft survival in the VH group at 30 days and 1, 5, and 10 years was 100%, 86%, 68%, and 50%, respectively, compared with 100%, 96%, 83%, and 68% in children who underwent
transplantation for
dilated cardiomyopathy (p = 0.12). Splenic status, cardiac position, age at CT, number of prior cardiac surgeries, or systemic venous anomalies were not predictors of mortality after CT.
Cardiopulmonary bypass and graft ischemic times were longer in the VH group; time on the
ventilator after CT, length of hospitalization, and rejection,
infection, post-transplant lymphoproliferative disease, and transplant
coronary artery disease rates were equal. Thus, CT is a viable alternative
therapy for high-risk patients with VH, possibly offering improved survival over standard surgical management.