Outcomes Of Pediatric Heart Transplantation In Children With Major Genetic Syndromes Are Comparable To Those With No Genetic Syndromes
Presented During:
Monday, May 8, 2023: 7:15AM - 7:30AM
Los Angeles Convention Center
Posted Room Name:
403B
Abstract No:
201
Submission Type:
Abstract Submission
Authors:
Bahaaldin Alsoufi (1), Sarah Wilkens (1), Deborah Kozik (2), Andrea Lambert (1), Jaimin Trivedi (2)
Institutions:
(1) University of Louisville and Norton Children's Hospital, Louisville, KY, (2) N/A, N/A
Submitting Author:
*Bahaaldin Alsoufi
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University of Louisville and Norton Children's Hospital
University of Louisville and Norton Children's Hospital
Co-Author(s):
Sarah Wilkens
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University of Louisville and Norton Children's Hospital
University of Louisville and Norton Children's Hospital
Deborah Kozik
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N/A
N/A
Andrea Lambert
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University of Louisville and Norton Children's Hospital
University of Louisville and Norton Children's Hospital
Jaimin Trivedi
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N/A
N/A
Presenting Author:
*Bahaaldin Alsoufi
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Norton Children's Hospital
Norton Children's Hospital
Abstract:
Objective: Genetic syndromes (GS) are often linked to both congenital heart disease (CHD) and cardiomyopathy (CM). While GS are associated with prolonged recovery and increased morbidity and mortality following surgery for CHD, their effect on survival following pediatric heart transplantation (PHT) is not well described. We aim to compare outcomes following PHT between children with and without GS.
Methods: The United Network for Organ Sharing (UNOS) transplantation database was merged with the Pediatric Health Information System (PHIS) administrative database to identify children with major GS who received PHT from 2009-2019. Characteristics and outcomes were compared between the GS (n=225) and non-GS (n=2204) groups.
Results: Major GS were present in 225/2429 (9%) of PHT recipients. The most common syndromes were DiGeorge (n=28), muscular dystrophy (n=27), Down (n=26) and Turner (n=14). The incidence of CHD was higher in the GS compared to non-GS group, 54% vs. 38%, p<0.1. On the other hand, patient demographics, pulmonary artery pressure, incidence of renal and hepatic dysfunction, and requirement for dialysis, mechanical ventilation, ECMO and mechanical circulatory support on listing were not significantly different. [Table] The waitlist duration was also comparable between the GS (55 days, IQR 20-135) and non-GS (53 days, IQR 20-113) groups, p=0.4. The incidence of post-transplant complications were also similar including dialysis (8% vs. 5%, p=0.38), stroke (3% vs. 4%, p=0.34), primary graft dysfunction (2% vs. 2%, p=0.75), pacemaker need (1% vs. 1%, p=0.84) and rejection (3.4% vs. 3.4%, p=0.96). Post-transplant recovery parameters were likewise comparable. Survival at 10 years following PHT for GS and non-GS groups was 71% and 75%, respectively, p=0.59. This comparable survival was present in those with CM and those with CHD.
Conclusions: Children with major GS and end-stage CM or CHD are expected to have similar waitlist and post-transplant outcomes to those without GS. While early and late post-transplant care is individualized to each patient's characteristics including GS, the presence of GS should not be considered contraindication to PHT in well-selected children.
Methods: The United Network for Organ Sharing (UNOS) transplantation database was merged with the Pediatric Health Information System (PHIS) administrative database to identify children with major GS who received PHT from 2009-2019. Characteristics and outcomes were compared between the GS (n=225) and non-GS (n=2204) groups.
Results: Major GS were present in 225/2429 (9%) of PHT recipients. The most common syndromes were DiGeorge (n=28), muscular dystrophy (n=27), Down (n=26) and Turner (n=14). The incidence of CHD was higher in the GS compared to non-GS group, 54% vs. 38%, p<0.1. On the other hand, patient demographics, pulmonary artery pressure, incidence of renal and hepatic dysfunction, and requirement for dialysis, mechanical ventilation, ECMO and mechanical circulatory support on listing were not significantly different. [Table] The waitlist duration was also comparable between the GS (55 days, IQR 20-135) and non-GS (53 days, IQR 20-113) groups, p=0.4. The incidence of post-transplant complications were also similar including dialysis (8% vs. 5%, p=0.38), stroke (3% vs. 4%, p=0.34), primary graft dysfunction (2% vs. 2%, p=0.75), pacemaker need (1% vs. 1%, p=0.84) and rejection (3.4% vs. 3.4%, p=0.96). Post-transplant recovery parameters were likewise comparable. Survival at 10 years following PHT for GS and non-GS groups was 71% and 75%, respectively, p=0.59. This comparable survival was present in those with CM and those with CHD.
Conclusions: Children with major GS and end-stage CM or CHD are expected to have similar waitlist and post-transplant outcomes to those without GS. While early and late post-transplant care is individualized to each patient's characteristics including GS, the presence of GS should not be considered contraindication to PHT in well-selected children.
CONGENTIAL:
Transplant / Mechanical Support
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