Abstract
Background: Adult congenital heart disease (ACHD) patients often require additional interventions or surgeries in adulthood, presenting new clinical challenges. However, clinical research on the current status and outcomes of cardiac procedures in ACHD patients remains limited.
Methods and Results: We analyzed the Japanese Registry of All Cardiac and Vascular Diseases-Diagnosis Procedure Combination (JROAD-DPC) database between April 2013 and March 2021. Patients with ACHD (aged >15 years) who underwent major cardiac surgery and transcatheter procedures were included. We assessed clinical background, treatment, length of hospital stay, and in-hospital mortality. In all, 22,490 patients with ACHD (median age 56 years [interquartile range 36–69 years], 51.1% female) were enrolled. Emergency hospitalizations and in-hospital deaths were observed in 3.7% and 1.1% of cases, respectively. Congenital heart operations with high in-hospital mortality (>5.0%) included aortic arch repair, systemic-to-pulmonary artery shunts, cardiac tumor resection, coronary artery bypass grafting, 3-valve replacement, and ventricular assist device implantation. Although stent graft procedures had the highest in-hospital mortality rate (2.6%), other transcatheter procedures, such as transcatheter patent ductus arteriosus closure, atrial septal defect closure, and catheter ablation, had in-hospital mortality rates of <1.0%.
Conclusions: This study provides fundamental insights into the current clinical characteristics and outcomes associated with procedures in patients with ACHD. The in-hospital mortality rates for both cardiac surgery and transcatheter procedures in Japanese ACHD patients were low, demonstrating acceptable outcomes.
