Abstract

<h3>Purpose</h3> As the population of adults with congenital heart disease (ACHD) is growing, there is greater need for simultaneous heart-liver transplantation (HLT) in these patients with challenging physiology. The aim of this study was to compare donor characteristics and HLT outcomes between ACHD and other adult cardiomyopathies. <h3>Methods</h3> United Network for Organ Sharing data on adult patients (>18 years) who underwent simultaneous heart-liver transplantation from 2008 through 2018 were analyzed. Patients were assigned to 3 groups according to the underlying cause of heart failure: ACHD, non-ischemic (NICM) and ischemic cardiomyopathy (ICM). The primary outcome was 10-year survival. Cox proportional-hazards modeling was used to assess mortality associations with recipients' and donors' characteristics. <h3>Results</h3> A total of 236 patients underwent HLT during the study period (ICM=27, NICM=138, ACHD=57). ACHD patients were older and had more comparable sex distribution (table). ACHD in-hospital mortality was low but was not significantly different among the 3 groups (4%, 6%, and 8% in NICM, ICM, and ACHD respectively, p=0.74). After adjustment, survival (Kaplan-Meier) was similar between the 3 groups at 5 years (79%, 82%, and 80%) but was higher in the ACHD at 10 years (41%, 63%, and 80%). While ACHD had lower creatinine at transplant (1.05±0.39mg/dL, P=0.03), this group had more postoperative dialysis (31%, P=0.05). Donors for ACHD patients tend to be younger (28±10 years, P=0.01) with a trend towards longer donor heart ischemic times (3.5±1.2 hours, P=0.09). <h3>Conclusion</h3> HLT was performed in ACHD patients with acceptable early mortality and good long-term survival. ACHD donors tend to be younger and more likely to have longer donor ischemic times. Despite better preoperative renal function, ACHD patients had more postoperative dialysis perhaps due to greater complexity of their transplant.

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