Abstract

<h3>Purpose</h3> Children with Fontan-related protein-losing enteropathy (PLE) demonstrate immunodeficiency due to lymphatic-intestinal losses of immunoglobulins and lymphocytes. Use of lymphocyte depleting agents such as rabbit antithymocyte globulin (RATG) at time of orthotopic heart transplant (OHT) may result in excessive immunosuppression and post-transplant complications. Our aim was to compare post-transplant outcomes such as rejection, infections, and malignancies between PLE patients who received RATG for induction opposed to a RATG-sparing protocol. <h3>Methods</h3> Retrospective, single center study of pediatric OHT recipients with PLE transplanted from 1/2014 to 12/2019. Multi-organ transplant and heart re-transplant recipients were excluded. Data collected included: demographic variables, immunosuppression type and levels, immune cell function assays, CD3 levels, CMV and EBV viremia, clinical rejection/biopsy results, and diagnoses of malignancy. Minimum follow-up time included 6 months from time of OHT. <h3>Results</h3> Twelve patients were identified of whom 6 received RATG with a median dose of 7.4 (4.4-7.6) mg/kg total. Median age at the time of OHT was 12.5 (11.5-16.5) years. One RATG patient had clinical rejection requiring ECMO support. Otherwise, all biopsies were grade 0 except for one 2R/3A and one 1R/1A in the RATG group and two 1R/1A in the non-RATG group. One patient in each group experienced AMR warranting intervention. Infections were more frequent in the RATG group with 2 patients with low-grade CMV viremia despite adequate prophylaxis and 3 RATG patients with EBV viremia compared to 1 EBV viremia in non-RATG group. Two diagnoses of PTLD occurred during the follow-up period, both of whom were in the RATG group. Trends of immune cell function assays and CD3 counts for the two groups are shown in Fig. 1. <h3>Conclusion</h3> Based on this pilot study, induction with RATG in PLE pediatric OHT patients may predispose them to higher frequency of post-transplant complications.

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