Why Haven’t We Seen This Before? The Importance of Reporting Experience to Improve Access and Equity

ecently, our team was asked to evaluate a unique patient for pediatric heart transplant. She was a 5- month- old female patient with trisomy 21 and a tetralogy of Fallot– type atrioventricular septal defect status post complete surgical repair 2 months earlier. The surgery was complicated complete heart block and pacemaker dependence, but she recovered un-eventfully and was discharged home after several weeks. After 3 weeks at home, she presented to the emergency department with lethargy and was found to be in cardiogenic shock. Her echocardiogram demonstrated a severely dilated left ventricle and severely de-pressed biventricular function. She required support with venoarterial extracorporeal membrane oxygen-ation. After 4 days, she was able to be weaned from mechanical circulatory support, but her systolic function demonstrated no signs of recovery despite aggres-sive medical management, prompting referral for heart transplant evaluation. While perhaps an unusual case given the specific congenital heart disease lesion, none of the patient’s cardiac course was particularly impact-ful in terms of our team’s decision making on transplant candidacy. However, her Down syndrome made her extremely unique as a heart transplant candidate at our center. We had never been asked to consider transplant in a patient with trisomy 21 before. We were completely ignorant of the ramifications of Down syndrome on transplant outcomes. Was the increased risk of pulmonary vascular disease present in these patients risk long- term graft function survival? 1 the incidence of leukemia in individuals with Down syndrome, posttransplant lymph-oproliferative