Heart and Heart-Lung Transplantation in Adult Congenital Heart Disease (ACHD): A 4-Year Single-Centre Experience

Purpose Due to impressive improvements in surgical treatment, patients with complex CHD have better survival and may develop end-stage heart failure. The number of patients with CHD that need heart (HT) or heart-lung transplantation (HLT) has been increasing in the last decades. The purpose of our study is (1) to review our experience in these patients and (2) to describe perioperative course and outcomes of ACHD patients who required HT or HLT in our institution in the last 4 years. Methods We performed a retrospective single-centre analysis of HT and HLT performed in ACHD patients in Harefield Hospital from January 2015 to May 2018. Data were obtained from automated data collection systems, National Audit Databases (Mortality Grading and Congenital Surgery), and National Institute for Cardiovascular Outcomes Research (NICOR). Results Seven ACHD patients (mean age 44.4 ± 9.3 y, range 34-61) underwent HT (6) or HLT (1). Original cardiac diagnoses included repaired TOF (n=2), CCTGA (n=1), TGA with pulmonary hypertension (n=1), DILV (n=1), Ebstein's anomaly (n=1), and repaired DORV (n=1). Median duration of mechanical ventilation was 9.1 days. Tracheostomy was performed in 3 cases. All patients required high inotropic and vasopressor support (Adrenaline 6/7, Milrinone 7/7, Noradrenaline 7/7, Vasopressin 2/7, Levosimendan 2/7). iNO was used in all the cases with a median duration of 2.5 days. Mechanical circulatory support was required in 5 patients: 4 VA-ECMO (3 central, 1 post-operative peripheral) and 1 IABP. All patients received transfusion (RBC 6/7, PLT 7/7, Cryo 2/7 and FFP 5/7). The most common complications requiring surgical intervention were re-exploration for bleeding, embolectomy and gastrointestinal bleeding. Two patients died (ischaemic colitis, acute graft dysfunction), 55 and 7 days after transplantation respectively. Conclusion ACHD patients undergoing HT or HLT are high risk group of patients with complex anatomy and physiologic features. Our results were similar to those reported in literature describing elevated post-transplant morbidity and mortality. Nevertheless, the data suggest that transplantation may be successfully performed in carefully selected patients in a highly specialized and multidisciplinary team.