Top Things to Know: 2025 Guideline for the Management of Adults With Congenital Heart Disease
Published: December 18, 2025
Prepared by Gurvitz, M
- Adults with congenital heart disease (ACHD) benefit from routine care at ACHD centers and in collaboration with ACHD cardiologists. Multidisciplinary teams are useful for complex-care decision-making.
- Patients with anatomic or physiologically moderate or complex ACHD who undergo cardiac or non- cardiac procedures are recommended to have an ACHD cardiologist involved in their care to offer expert guidance on procedures, anesthesia, and postprocedural management.
- The possibility of endocarditis is important to evaluate in acute or subacute malfunction of bioprosthetic pulmonary valves, and endocarditis has been added to the physiological classification system (subacute bacterial endocarditis in the past year is stage D).
- Most pregnant patients with ACHD can undergo vaginal delivery safely, with appropriate risk stratification and monitoring.
- Updated recommendations for tetralogy of Fallot include (a) referral for pulmonary valve replacement in patients with repaired tetralogy of Fallot, according to right ventricular end-systolic volume criteria (>80 mL/m2) and other metrics rather than end-diastolic volume; and (b) new approaches to arrhythmia management, including ablation of ventricular tachycardia.
- Strategies for patients with secundum atrial septal defect and pulmonary arterial hypertension now include recommendations for closure for many patients with a significant left-to-right shunt and pulmonary vascular resistance ≤2 Wood units or >2 to
- Rhythm control is typically preferred over rate control for atrial arrhythmias in complex patients, such as those with a systemic right ventricle or Fontan circulation.
- New recommendations on guideline-directed medical therapy for heart failure in patients with ACHD include people with a systemic right or left ventricle and discussions of pacing strategies for a systemic right ventricle and Fontan circulation.
- Patients with Eisenmenger syndrome can be treated with pulmonary vasodilators, using either phosphodiesterase-5 inhibitors or endothelin receptor antagonists as initial therapy.
- Fontan screening for liver disease includes recommendations for at least annual imaging and laboratory evaluation, including alpha-fetoprotein, and at least 1 consultation with a hepatologist.
Citation
Gurvitz M, Krieger EV, Fuller S, Davis LL, Kittleson MM, Aboulhosn JA, Bradley EA, Buber J, Daniels CJ, Dimopoulos K, Egbe A, Geoffrion TR, John A, Khairy P, Kim YY, Kreutzer J, Lewis MJ, Menachem JN, Moore JP, Osteen KA, Parikh PB, Saidi A, Salciccioli KB, Schunder RL, Valente AM, Wald RM. 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. Circulation. Published online December 18, 2025. doi: 10.1161/CIR.0000000000001402