A New Era of Cardiothoracic Transplantation in Adults With Congenital Heart Disease

Background: A growing number of children with congenital heart disease survive to adulthood and require heart transplantation. This study evaluates the trends and outcomes of cardiothoracic transplantation for patients with adult congenital heart disease (ACHD) and describes our institution's 3 decades of experience. Methods: We identified all patients with ACHD who underwent first-time cardiothoracic transplantation at our institution between December 1987 and December 2024. We compared characteristics and outcomes between a historical and contemporary cohort of transplant recipients. Results: In total, 196 patients with ACHD underwent cardiothoracic transplantation. The annual rate of cardiothoracic transplantation in patients with ACHD doubled after 2018. The first era of heart transplantation in patients with ACHD from 1987 to 2018 is defined predominantly by heart-lung transplantation, most commonly for isolated septal defect. The second era since 2018 is defined by sicker, more complex patients. Following the United Network for Organ Sharing heart allocation policy change, recipients were older, underwent more prior cardiac surgeries, were more likely to be hospitalized, and were more likely to have single-ventricle anatomy with Glenn/Fontan palliation. Comparing the 2 eras, 1-year survival rates for all patients with ACHD were comparable (81% versus 81%, P=0.939) and improved for heart-only transplant recipients (72.5% versus 90.7%, P=0.066). Conclusions: The number and complexity of patients with ACHD undergoing cardiothoracic transplantation has grown, with a significant increase in patients who previously underwent palliative surgeries. With increased use of medical and surgical adjuncts, excellent long-term survival for transplant recipients is achievable.