Abstract
In December 2025, the American College of Cardiology/American Heart Association (ACC/AHA) Joint Committee on Clinical Practice Guidelines, in collaboration with and consultation from the Heart Rhythm Society (HRS), the International Society for Adult Congenital Heart Disease (ISACHD), and the Society for Cardiovascular Angiography and Interventions (SCAI), released the 2025 guideline for the management of adults with congenital heart disease (ACHD). This guideline represents a significant update from the 2018 version, reflecting the rapid expansion of the ACHD population as well as advances in pathophysiological understanding, structural interventions, arrhythmia management, heart failure therapy, and multisystem care. In this review, we summarize the key components of the guideline, with particular emphasis on practice-changing updates, including the organization of care within specialized ACHD centers, expanded indications for atrial septal defect closure, contemporary approaches to pregnancy and delivery, management of complex arrhythmias, and screening strategies for Fontan-associated liver disease. These recommendations are analyzed in the context of current evidence and compared with the European Society of Cardiology (ESC) guidelines to facilitate their effective implementation in clinical practice.