Pediatric waitlist and heart transplant outcomes in patients with syndromic anomalies.

Abstract

We sought to determine whether the presence of a systemic SA with potential complicating factors affects waitlist and post-HT outcomes in pediatric patients. This is a single-center retrospective review of pediatric patients listed for HT between January 1, 2009, and July 1, 2018. Patients were selected based on the presence of any underlying syndromes, which included chromosomal anomalies, skeletal myopathies, connective tissue disorders, mitochondrial disease,and other systemic disorders. Waitlist and post-HT outcomes were compared to those without SA. A total of 243 patients were listed for HT, of which 21 (9%) patients had associated SA. Of those, 16 (76%) survived to transplant, 3 (14%) died while on the waitlist, 1 (5%) improved and was removed from the waitlist, and 1 (5%) patient is 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 listing days (70 vs 90, P=.8), survival to hospital discharge [14 (93%) vs 150 (95%), P=.6], post-HT intubation days (2 vs 2days, P=.6), or post-HT hospital length of stay (18 vs 18days, P=.8). Overall survival during the study period post-HT was not different between groups (P=.8). A SA was present in 9% of pediatric patients wait-listed for HT, but was not associated with an increased waitlist mortality or post-HT hospital morbidity or long-term survival. For several anomalies, HT is safe and feasible.