P3473Outcome following assessment for orthotopic heart transplant in adults with congenital heart disease

Abstract

Background: Adults with congenital heart disease (CHD) are a growing group at risk of end stage heart failure. A proportion will need to be considered for orthotopic heart transplantation (OHT). Although outcomes from OHT and listing for OHT are well documented in this population there is no data as to the outcome of patients undergoing assessment for OHT. Purpose: To describe the outcome of adults with CHD undergoing assessment for OHT. Methods: All CHD adults (>16 years) referred to our unit for OHT between 1st January 2000 and 2016 were included. Case notes were retrospectively reviewed and outcomes reported until 1st January 2017. Patients were divided into three groups: LV (balanced ventricles with systemic left ventricle), RV (balanced ventricles with systemic right ventricle) and SV (unbalanced ventricles leading to a physiological single ventricle circulation). Results: 196 patients (67% male, 32 years [IQR 23-41], LV: n=53, RV: n=57, SV: n=86) were assessed for OHT. In the SV group, 70% had a systemic LV and 67% a Fontan repair. The RV group had fewer previous sternotomies but higher pulmonary pressures (37±23mmHg vs. LV 29±17mmHg, p=0.092 and SV 17±7mmHg, p<0.001). Greater co-morbidity occurred in the SV group. 89/196 (45%) patients were listed for OHT with 67/196 (34%) transplanted. Two patients were listed for dual organ transplant (heart/liver and heart/lung). There were no differences in rates of listing or OHT between groups. Time from assessment to listing was longest in the SV group (4.7 months vs RV:2.3 and LV:1.3, p=0.058). Mechanical support (MCS) was more frequent in the RV group (23% vs LV:8% and SV:1%, p<0.05). 5 patients (4 RV, 1 LV) were successfully bridged to OHT and one (RV) to heart-lung transplant. Conventional surgery was performed in 13 (7 alive and 1 transplanted at latest follow-up). Survival following assessment for the whole group was 84.2±2.6% (one year), 69.7±3.6% (five years) and 68.5±3.8% (ten years). Survival was not influenced by diagnostic group (Fig. 1a) and varied by decision (Fig. 1b). Survival after OHT for the whole group was 85.0±4.4% (30 days), 80.4±4.9% (one year), 75.9±5.6% (five years) and 75.9±5.6% (ten years). There was no difference between diagnostic groups on pooled comparison. Conclusions: Less than 50% of CHD adults assessed for OHT are suitable for listing with only 35% transplanted. Assessment should be carried out by specialist teams who can provide adjunctive management including high-risk conventional surgery and long term MCS. Underlying diagnosis does not affect likelihood of listing or OHT. Those with systemic RVs are more frequently bridged to candidacy and OHT with MCS. Despite greater comorbidity in the SV group, survival following assessment is comparable with other diagnoses. Early assessment should be considered to ensure the opportunity to transplant is not missed. Listing criteria and donor organ allocation should reflect the unique problems of this population.