The 2025 ACC/AHA/HRS/ISACHD/SCAI Guideline for management of adults with congenital heart disease represents a comprehensive, updated replacement of the 2018 guidelines approved by major cardiovascular associations. It uses a refined modular format and rigorous evidence review process to address the growing and increasingly complex population of adult congenital heart disease (ACHD). A main structural feature of this guideline is the updated Anatomic–Physiological (AP) Classification System, which categorizes patients by anatomic complexity (classes I-III) and physiological stage (A-D), and incorporates biomarkers like NT‑proBNP, recent endocarditis, and heart failure-related hospitalizations. This classification guides intensity of surveillance, procedural planning, and risk discussion across lesion types.
The guideline highlights that many patients with congenital heart disease are lost to follow-up during adolescence. To address this gap, structured, developmentally proper transition education and formalized transfer processes from pediatric to adult care are important. Collaborative management with ACHD cardiologists is suggested for all patients with moderate and complex lesions, and even patients with simple anatomy (class IA) must undergo at least one evaluation by an ACHD specialist. Pre- and post-procedural management, whether for noncardiac or cardiac interventions, must include ACHD expertise with anesthesia provided by clinicians trained in congenital heart physiology, particularly for patients in higher AP classifications. Routine mental health screening is recommended, as adults with ACHD have high rates of depression, anxiety, and post-traumatic stress symptoms.
Several major updates distinguish the 2025 guidelines from earlier versions. New dedicated sections address cyanosis, heart failure management, transplantation, and mechanical circulatory support (MCS), genetic screening, reproductive health, and pregnancy counselling. In patients with cyanotic heart disease, annual iron studies are recommended, and urgent neuroimaging is advised for the new neurologic symptoms. Heart failure guidance has expanded to involve the use of guideline-directed medical therapy like sodium–glucose cotransporter 2 (SGLT2) inhibitors and angiotensin receptor–neprilysin inhibitors, as well as cardiac resynchronization therapy (CRT) and implantable cardioverter-defibrillators (ICDs) in adults with systemic left ventricular dysfunction and biventricular circulation.
The guideline addresses reproductive health comprehensively, emphasizing that all adults with congenital heart disease of childbearing age should receive individualized contraceptive counselling and preconception evaluation. Vaginal delivery is generally preferred unless obstetric or cardiac factors need caesarean delivery. Fetal echocardiography is recommended when either parent has congenital heart disease. Women with high-risk conditions like Eisenmenger syndrome, Fontan failure, severe systemic ventricular dysfunction, or severe left-sided obstructive lesions must be counseled to avoid pregnancy because of the high mortality risk. Estrogen-containing contraceptives are contraindicated in patients at increased risk of thromboembolism.
Disease-specific recommendations constitute a substantial portion of the guidelines. Shunt lesions such as atrial septal defect (ASD), ventricular septal defect (VSD), atrioventricular septal defect (AVSD), anomalous pulmonary venous connections (APVC), and patent ductus arteriosus (PDA) are classified using refined criteria based on pulmonary-to-systemic flow ratios (Qp:Qs) and pulmonary vascular resistance (PVR) thresholds. Closure is recommended for most lesions with Qp:Qs ≥1.5 in the absence of prohibitive PVR and is contraindicated in Eisenmenger syndrome. ASD and APVC evaluation relies on cardiac magnetic resonance imaging (CMR) or computed tomography (CT) for detailed anatomic clarity. The updated tetralogy of Fallot guidelines emphasize pulmonary valve replacement based on right ventricular end-systolic volume instead of end-diastolic volume and include contemporary arrhythmia therapy like catheter ablation for ventricular tachycardia. Fontan-associated guidelines include yearly liver imaging and laboratory surveillance, early transplant referral, and multidisciplinary management of complications like arrhythmias, protein-losing enteropathy, and Fontan-associated liver disease.
Eisenmenger management involved pulmonary vasodilator therapy starting with phosphodiesterase‑5 inhibitors or endothelin receptor antagonists and proceeding to combination therapy when indicated. Routine anticoagulation is not recommended, and shunt closure is contraindicated. The guidelines include minimizing radiation exposure by preferring non-ionizing imaging modalities and optimizing fluoroscopic methods when radiation is unavoidable. Exercise testing remains central to functional assessment, with cardiopulmonary exercise testing (CPET) recommended for baseline and longitudinal evaluation. The six-minute walk test is reserved for patients unable to complete CPET, specifically those with pulmonary arterial hypertension. Regular physical activity is encouraged for most ACHD adults, and participation in competitive sports may be considered following individualized specialist evaluation.
Finally, the guideline identifies significant evidence gaps in ACHD care, including mental health management pathways, optimal follow-up intervals, the efficacy of GDMT in specific congenital physiologies, prediction tools for sudden cardiac death, long-term strategies for pulmonary arterial hypertension treatment and repair, and the integration of artificial intelligence into clinical risk stratification.
Reference: Gurvitz M, Krieger EV, Fuller S, et al. 2025 ACC/AHA/HRS/ISACHD/SCAI guideline for the management of adults with congenital heart disease: a report of the American College of Cardiology/American Heart Association Joint Committee on Clinical Practice Guidelines. J Am Coll Cardiol. 2025. doi:10.1016/j.jacc.2025.09.006
