Outcomes of pediatric heart transplantation in children with selected genetic syndromes

Genetic syndromes (GSs) are often linked to congenital heart disease (CHD) and cardiomyopathy (CM). The effect of GSs on survival following pediatric heart transplant (HT) has not been well described. We aimed to compare outcomes following HT between children with a GS and those without a GS. The Un...

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Bibliographic Details
Published in:JTCVS open 2024-10, Vol.21, p.279-287
Main Authors: Wilkens, Sarah, Trivedi, Jaimin, Kozik, Deborah, Lambert, Andrea Nicole, Alsoufi, Bahaaldin
Format: Article
Language:English
Subjects:
Congenital: Heart Transplantation
genetic abnormalities
heart transplant
outcomes/database
pediatric
syndrome
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Summary:Genetic syndromes (GSs) are often linked to congenital heart disease (CHD) and cardiomyopathy (CM). The effect of GSs on survival following pediatric heart transplant (HT) has not been well described. We aimed to compare outcomes following HT between children with a GS and those without a GS. The United Network for Organ Sharing (UNOS) transplantation database was merged with the Pediatric Health Information System (PHIS) administrative database to identify children with GS who underwent HT between 2009 and 2019. Characteristics and outcomes were compared between children with a GS (GS group) and those without a GS (no GS group). GSs were present in 225 of 2429 HT recipients (9%). The most common GSs were DiGeorge syndrome (n = 28), muscular dystrophy (n = 27), Down syndrome (n = 26), and Turner syndrome (n = 14). The incidence of CHD was higher in the GS group compared to the no GS group (54% vs 38%; P 
ISSN:2666-2736
2666-2736
DOI:10.1016/j.xjon.2024.05.016