Giant Coronary Aneurysms After Pediatric Heart Transplant: Giant Problem

Abstract

<h3>Introduction</h3> Cardiac allograft vasculopathy (CAV) is a leading cause of death and retransplant after heart transplantation (HTx). Coronary aneurysms are not typically seen in CAV. We present a case of atypical CAV with coronary aneurysms resulting in premature death. <h3>Case Report</h3> A 4-year-old male underwent HTx after failed single ventricle palliation. His first year after HTx was uneventful with no cellular rejection score greater than 1R and no antibody mediated rejection on multiple surveillance endomyocardial biopsies. Coronary angiography was normal at 3 months. At 1-year post-HTx, surveillance coronary angiography demonstrated a diffusely dilated appearance with multiple aneurysmal segments of the right and left coronary systems (Figure). No filling defects or areas of focal stenosis were appreciated. His pulmonary capillary wedge pressure was elevated at 15 mmHg, but stable from previous assessments. There was no known history of Kawasaki disease in the donor. The patient had no history of prolonged fevers or other symptoms of vasculitis. He was placed on enoxaparin and aspirin for anticoagulation. He remained asymptomatic with no regional wall abnormalities and normal systolic function by echocardiogram. He denied episodes of palpitations and had a Holter without any ectopy or arrhythmias. While awaiting further evaluation with additional advanced imaging, he had a sudden cardiac arrest and died 10 months after coronary aneurysms were found. His autopsy revealed segmental fusiform aneurysmal expansion with severe concentric intimal fibrosis and luminal narrowing of multiple segments of the right and left coronary systems consistent with severe CAV. Left ventricular histopathology exhibited chronic ischemic changes to the subendocardium. <h3>Summary</h3> Our patient with severe CAV presented with coronary aneurysm formation within 1 year of HTx. Coronary aneurysm formation may be an atypical and more severe form of CAV that should prompt close monitoring and early consideration for retransplant.