Hepatology. 2020 Jun 19.
BACKGROUND: There are more adults than children living with congenital heart disease (CHD) in the US, with a growing proportion requiring heart-liver transplantation (HLT). Our aim was to ascertain the frequency, outcomes and prognostic factors in this patient population.
METHODS: UNOS data on adult patients who underwent heart transplantation (HT) from 2009 through March 2020 were analyzed. The primary study outcome was patient survival. Cox proportional-hazards modeling assessed for mortality associations.
RESULTS: There were 1084 HT recipients: 817 (75.4%) CHD HTs-only; 74 (6.8%) CHD HLTs; 179 (16.5%) non-CHD HLTs; 14 (1.3%) heart-liver-kidney transplants. The number of CHD HLTs increased from a prior rate of 4/year to 21/year in 2019. Among CHD patients, the 5-year survival rates were 74.1% and 73.6% in HTs-only and HLTs, respectively (p=0.865). There was a higher rate of allograft failure attributable to rejection in CHD HTs-only compared with CHD HLTs (3.2% vs. 0.4%; p=0.014). Only 25 out of 115 HT-performing hospitals undertook CHD HLTs. Higher-volume centers (averaging 1 CHD HLT per year) had a 5-year patient survival rate of 83.0% compared with 61.3% in lower-volume centers (p=0.079). Among HLT recipients, total bilirubin [HR=1.06, 95% CI=1.01-1.12] and diabetes [HR=2.97, 95% CI=1.21-7.31] were independently associated with increased mortality risk, whereas CHD and age were not.
CONCLUSIONS: The rate of HLT for adult CHD in the US is rising dramatically. The survival outcomes between CHD HTs-only and CHD HLT groups are comparable, however, the HLT group had lower rates of acute rejection. Among HLT recipients, diabetes and elevated bilirubin are associated with increased posttransplant mortality risk. An average of 1 CHD HLT per year could be considered as a minimum quality metric at transplant centers.
Keywords: Acute rejection; Cardiac cirrhosis; Fontan; Graft survival; Patient survival