Cardiac Retransplantation for Graft Vasculopathy in Children

Abstract

Background: Cardiac transplantation (CTx) has been established as an effective therapy for a variety of inoperable cardiac conditions in infants and children. However, graft vasculopathy (GV) has emerged as the main limiting factor to long-term survival of CTx recipients. The only treatment of severe GV is cardiac retransplantation (re-Tx). Controversy exists regarding the use of scarce donor organs for cardiac re-Tx.Objective: To compare the outcome of cardiac re-Tx for GV with that of primary CTx in children.Design: A 12-year retrospective cohort review.Setting: A university-affiliated children's hospital.Patients: All infants and children who underwent CTx (group 1, n = 322) had complete follow-up of 1389.7 patient-years. Graft vasculopathy was confirmed in 32 recipients (1.1-8.2 years after undergoing CTx). Thirteen patients died suddenly, 3 died waiting for cardiac re-Tx (1-17 days after relisting), 4 are pending cardiac re-Tx, and 12 (group 2) underwent cardiac re-Tx.Intervention: Cardiac re-Tx at a mean (± SD) interval from the first CTx of 6.3 ± 1.8 years (range, 2.2-9.4 years). Two patients required additional aortic arch aneurysm repair with cardiac re-Tx.Results: When group 1 was compared with group 2, there was no significant difference in operative mortality (9.0% vs 8.3%; P = .9), rejection rate (0.98 vs 0.86; P = .1), and hospital stay (23.0 ± 18.8 days vs 20.5 ± 11.6 days; P = .65). Actuarial survival for groups 1 and 2 at 1 and 4 years was 84.3% vs 83.3% (P = .59) and 74.4% vs 83.3% (P = .85), respectively. Conclusions:The surgical outcome and intermediate survival of cardiac re-Tx for GV and primary CTx are similar. Children with severe cardiac GV are at risk of sudden death and can benefit from early cardiac re-Tx.

International Society for Heart and Lung Transplantation working formulation of a standardized nomenclature for cardiac allograft vasculopathy—2010

“…There are variable anatomic classifications/scoring systems with lack of consistency and prognostic value. 70,[73][74][75][76][77][78] There was a relatively low incidence of any degree of angiographic abnormality in the reported multicenter cohort, ranging from 2.5% at 1 year and plateaus at less than 10% from 3 to 8 years after transplant in the patients evaluated. 70 Comparable with the adult experience but notably with a lower prevalence, the data suggest that moderate to severe CAV by angiography is associated with cardiac events, death, and retransplant.…”

Risk Factor Analysis in Pediatric Heart Transplantation

“…4 Deaths are mainly due to acute rejection and early and late allograft failure. 5,6 The shortage of donor hearts, increasing demand, and constraint of financial or medical resources necessitate optimal organ utilization. Steady assessment of risk factors of mortality will enable identification of patients at higher risk for post-transplant death, and may thus improve organ utilization and outcomes.…”

Perspectives on cardiac allograft vasculopathy

“…Razzouk et al [44] also suggested that repeat transplantation may not carry quite as dismal a prognosis as the registry suggests. This group evaluated a 12-year retrospective cohort of pediatric heart transplant patients.…”

“…This probably sets the stage for worse outcomes after a second heart transplant. Reports from single centers seem more encouraging, but are flawed by the obvious bias of ascertainment associated with this type of publication [44].…”