AMR After Transplant in a Child with Complex CHD and Ruptured LV Aneurysm

Abstract

<h3>Introduction</h3> Antibody mediated rejection (AMR) is a mechanism of allograft dysfunction and a risk factor for transplant outcomes. Left ventricular (LV) aneurysms are rare in children and may be congenital or acquired. This is a unique case of both AMR and acquired LV aneurysm. <h3>Case Report</h3> We present a 1.9 kg Caucasian female born at 35 6/7 weeks gestation with a prenatal diagnosis of D-transposition of the great arteries and a large ventricular septal defect encompassing most of the trabecular ventricular septum. After pulmonary artery banding and balloon atrial septostomy, a Damus-Kaye-Stansel amalgamation (DKS) was performed with de-banding of the pulmonary artery and a hemi-Fontan procedure. After the DKS, she developed ventricular fibrillatory arrest requiring extracorporeal membrane oxygenation (ECMO) for 12 days. During this time her DKS was taken down. She was found to have an intramural course of her right coronary artery causing ischemia and arrhythmia. She developed biventricular dysfunction. After discharge the patient was lost to follow up for 4 years. Upon her return, a computed tomography scan showed interval development of a left ventricular aneurysm with rupture into the right ventricle [Fig 1]. Heart transplantation was pursued. Despite a weakly positive cross match for B flow, she received a heart transplant. One week post-transplant pathological AMR class two and donor specific antibody (DSA) production were identified. Her immunosuppression regimen was increased and she received plasmapheresis, but on post-operative day 16 she suffered a cardiac arrest and required ECMO. Additional steroids, anti-thymocyte globulin, Rituximab and Bortezomib were administered with recovery of function and decreased DSAs. She was decannulated after 8 days and subsequent biopsies were free of rejection. She was discharged home neurologically intact. <h3>Summary</h3> This case illustrates accurate diagnosis and treatment of AMR in a child with complex congenital heart disease and ruptured LV aneurysm status post heart transplant.