Clinical Characteristics and Outcomes of Adults with Congenital Heart Disease listed for Heart and Heart-Lung Transplantation in the Eurotransplant Region

Abstract Background The therapeutic success in patients with congenital heart disease leads to a growing number of adults with congenital heart disease (ACHD) who develop end-stage heart failure. We aimed to determine patient characteristics and outcomes of ACHD listed for heart transplantation. Methods Using data from all ACHD patients in 20 transplanting centers in the Eurotransplant region from 1999-2015, we analyzed patient characteristics, waiting list and post transplantation outcomes. Results A total of 204 patients with ACHD were listed during the study period. Median age was 38 years and 62.3% of the patients were listed in HU (high-urgency) and 37.7% of the patients in T (transplantable) listing status. 23.5% of the patients died or were delisted due to clinical worsening, 75% of the patients were transplanted. Median waiting time for patients with HU listing status was 4.18 months and with T listing status 9.07 months. There was no difference in crude mortality or delisting between HU- and T-status listed patients (p = 0.65). In multivariable regression analysis, markers for respiratory failure (mechanical ventilation, HR 1.41, 95% CI 1.11-1.81, p=0.006) and arrhythmias (antiarrhythmic medication, HR 1.42, 95% CI 1.01-2.01, p=0.044) were associated with higher risk of death or delisting. In the overall cohort, post transplantation mortality was 26.8% after 1 year and 33.4% after 5 years. Conclusions Listed patients are at high risk of death without differences between urgency of listing. Respiratory failure requiring invasive ventilation and possibly arrhythmias requiring antiarrhythmic medication indicate worse outcome on waiting list.