Case ReviewsAntibody-Mediated Rejection and Early Cardiac Allograft Vasculopathy in a Child With Congenital Heart DiseaseRevelo, Monica P. MD*†; Everitt, Melanie D. MD*† ‡; Hammond, Elizabeth H. MD*† ‡; Miller, Dylan V. MD*† ‡Author Information From the *University of Utah, Salt Lake City; †Cardiac Transplant Program, Utah Transplantation Affiliated Hospitals; and ‡ Intermountain Medical Center, Murray, UT. Reprints: Monica P. Revelo, MD, Department of Pathology, University of Utah, Circle of Hope, Huntsman Cancer Hospital, Salt Lake City, UT 84112. E-mail: [email protected]. The authors have no funding or conflicts to declare. Pathology Case Reviews: November/December 2012 - Volume 17 - Issue 6 - p 225-228 doi: 10.1097/PCR.0b013e3182759228 Buy Metrics Abstract Antibody-mediated rejection (AMR) is an immunopathological process mediated by antibodies and complement, leading to vascular injury and graft dysfunction. It usually occurs days to weeks after heart transplantation and is associated with a rise in donor-specific antibodies and poor prognosis. Histologically, it is characterized by the presence of interstitial edema, endothelial swelling, and accumulation of macrophages or neutrophils within perimyocyte capillaries, and in more severe cases, interstitial hemorrhage, capillary fragmentation, mixed inflammatory infiltrates, endothelial pyknosis, and/or karyorrhexis can be seen. The pathological features and clinical implications of AMR after heart transplantation are well understood in adults; however, owing to lack of routine biopsy surveillance for AMR as well as fewer pediatric recipients, fewer episodes of rejection, and disparate immune system biology in the pediatric population, there is a limited understanding of this process in children. We present a case of a child receiving a heart transplant who developed AMR in the early posttransplantation period that was associated with the rapid development of cardiac allograft vasculopathy and graft failure despite augmentation of immune suppression. This review addresses the morphological features of AMR, the importance of surveillance biopsies for diagnosis, and the utility of grading these biopsies. A better understanding of the pathogenesis of AMR in children and how it may differ from adults, strategies for early recognition and accurate diagnosis, as well as potential new therapies will all contribute to improved outcomes in pediatric patients. © 2012 Lippincott Williams & Wilkins, Inc.