Mortality and morbidity in pre-sensitized pediatric heart transplant recipients with a positive donor crossmatch utilizing peri-operative plasmapheresis and cytolytic therapy.

BACKGROUND: The difficulty in obtaining a prospective negative donor/recipient crossmatch limits the ability to successfully transplant pediatric heart transplant candidates who show evidence of antibodies to multiple human leukocyte antigens (pre-sensitized patients). METHODS: We utilized a protocol that included peri-operative plasmapheresis, thymoglobulin and cyclophosphamide in 17 pre-sensitized (panel-reactive antibodies [PRA] >10%) pediatric patients to accept donors for these patients without a prospective crossmatch between 1995 and 2005. A retrospective review of survival, rejection and infection was performed, comparing the frequency of rejection and infection in our patients who were transplanted with a complement-dependent cytotoxic (CDC)-positive donor/recipient crossmatch to those patients transplanted with a negative crossmatch. RESULTS: Thirteen of 17 patients were found to have a CDC-positive crossmatch. Actuarial survival after transplantation was 85% at 1 year and 73% at 3 years. Twelve of 13 (92%) of these patients experienced rejection, and 5 of 13 (38%) had recurrent rejection, generally in the first 2 months after transplantation. Rejection was associated with hemodynamic compromise in 58% of first rejection episodes and 67% of episodes of recurrent rejection. The frequency of rejection in these patients was significantly greater than the frequency in patients with a negative crossmatch in the first 6 months after transplantation, but not afterward. The frequency of infection episodes was not significantly different between the groups. CONCLUSIONS: Heart transplantation in pre-sensitized pediatric recipients with a CDC-positive donor/recipient crossmatch may result in reasonable short-term survival, but with a high frequency of early rejection, often with hemodynamic compromise.

Primary pulmonary vein stenosis.

Primary pulmonary vein stenosis (PVS) is a rare entity with a high mortality rate. Relatively little is known regarding predictors of outcome or the appropriate timing of intervention. The Pediatric Cardiac Care Consortium database (n = 98,126) was searched for patients who had undergone cardiac catheterization or surgical procedures with primary diagnoses of PVS or atresia from 1982 to 2002. Patients with total or partial anomalous pulmonary venous return, cor triatriatum, previous atrial switch, or previous lung transplantation were excluded. Additional data were obtained through questionnaires sent to each Pediatric Cardiac Care Consortium institution. A total of 31 patients were identified with primary PVS. Excluding lung transplantation, 16 of 31 patients underwent intervention to relieve PVS. Univariate predictors of lung death, defined as death or lung transplantation, included younger age at diagnosis (16.2 vs 52.5 months, p = 0.0221), higher initial mean pulmonary arterial pressure (46.4 vs 26.8 mm Hg, p = 0.0003), and bilateral vessel involvement (lung death in 17 of 19 vs 0 of 9 patients, p <0.0001). Patients diagnosed at 18 months of age and those with initial mean pulmonary arterial pressures >33 mm Hg had incidences of lung death of 76% and 88%, respectively. In conclusion, primary PVS carries a high mortality rate despite attempts at palliation. Patients diagnosed at 18 months of age, having initial mean pulmonary arterial pressures >33 mm Hg, or with bilateral vessel involvement are at significantly increased risk for death or lung transplantation. In these high-risk groups, death occurs rapidly despite intervention, and lung transplantation should be an early consideration.