Abstract 16872: Characterization of Pediatric Patients With Syndromic Anomalies Listed for Heart Transplant: Waitlist and Post-Heart Transplant Outcomes

Abstract

Introduction: We sought to describe the prevalence, characteristics and outcomes of pediatric patients with a syndromic anomaly (SA) who were accepted for listing for heart transplant (HT). Methods: This is a single center retrospective review of pediatric patients listed for heart transplantation after January 1, 2009 at our center. Patients were analyzed based on the presence of any underlying syndrome, which was heterogeneous and included chromosomal anomalies, skeletal myopathies, connective tissue disorders, mitochondrial disease and other systemic disorders. Waitlist and post-heart transplant outcomes were compared to those without SA. Results: A total of 243 patients were listed for (HT), of whom 21 patients (9%) had an associated SA (Table 1). Of those, 15 (71%) survived to transplant, 3 (14%) died while on the waitlist, 1 (5%) improved and was removed from the waitlist, and 2 (10%) patients are currently listed. Waitlist survival was not different between those with/without an associated syndrome (p = 1.0). Among those who survived to HT, there was no difference in median listing days (70 vs. 90 days, p=0.8), survival to hospital discharge [14 (93%) vs 150 (95%), p = 0.6], post-HT intubation days (2 vs 2 days, p = 0.6), or post-HT hospital length of stay (18 vs 18 days, p = 0.8). Overall survival was equivalent between groups post-HT, with a median follow-up time of 3.3 years (p = 0.7). Conclusions: A syndromic anomaly was present in 9% of pediatric patients waitlisted for heart transplant, but was not associated with an increased waitlist mortality, post-heart transplant hospital morbidity or long-term survival. If children with a syndromic anomaly were decided to be suitable candidates for heart transplant listing, they appear to have similar waitlist times and post-heart transplant survival as non-syndromic children.