Outcomes Of Heart Transplantation In Children With Heterotaxy Syndrome
Jaimin Trivedi, Sarah Wilkens, Andrea Nicole Lambert, Deborah Kozik, Bahaaldin Alsoufi.
University of Louisville, Louisville, KY, USA.
Objectives: End-stage congenital heart disease (CHD) in children with heterotaxy (HTX) might necessitate heart transplantation (HT). HT for HTX can be associated with several technical (multi-redo, systemic/pulmonary-venous/situs anomalies, PA reconstruction), and extra-cardiac (ciliary dyskinesia, infections, GI) challenges. We aim to examine if HTX is associated with increased early or late HT risks. Methods: The UNOS transplantation database was merged with PHIS administrative database to identify HT children with HTX. Characteristics and outcomes were compared between HTX (n=143) and contemporaneous CHD (n=428) and cardiomyopathy (CMP, n=551) control groups. Results: There were differences in characteristics between the 3 groups, with HTX being comparable to CHD. [Table] The waitlist duration was higher for HTX than CHD and CMP (91 vs. 63 vs. 56 days,p<0.1). Early post-HT complications were similar for all groups except for operative mortality that was 1% for CMP and 4% for CHD and HTX,p<0.1. Post-HT stay was shorter for CMP (57) compared to CHD (99) and HTX (89) days,p<0.1. While rejection before discharge was comparable between groups, 1-year rejection was higher for HTX (22%), than CHD (19%) and CMP (13%),p<0.1. Survival at 5-years was superior for CMP (91%) than CHD (82%) and HTX (81%),p<0.1. On multivariable analysis, no risk factors were identified for HTX group. Conclusion: HT in children with HTX is associated with good outcomes that are comparable to those with other forms of CHD. 
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