Adult Congenital Heart Disease

2025 Guidelines for Management of Adult Congenital Heart Disease

New 2025 Guidelines For Management of Adult Congenital Heart Disease

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In December 2025, the most current guidelines for management of adults with congenital heart disease were published. Explore the complete guidelines. The American College of Cardiology (ACC) and the American Heart Association (AHA) Joint Committee on Clinical Practice Guidelines are responsible for providing the foundation of evidence-based practice in the field of ACHD. 

The 2025 guidelines were also endorsed by the Society for Cardiovascular Angiography and Interventions (SCAI), the Heart Rhythm Society (HRS) and the International Society of Adult Congenital Heart Disease (ISACHD). 

UCLA Health expertise was well represented in the current guidelines, with past UCLA Health ACHD cardiologist Michelle Gurvitz, MD, as chair and Dr. Jamil Aboulhosn and Dr. Jeremy Moore on the writing committee and Jeannette Lin, MD, on the peer review committee.

Key updates in 2025 include: 

  1. 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. 
  2. Patients with anatomic or physiologically moderate or complex ACHD who undergo cardiac or noncardiac procedures are recommended to have an ACHD cardiologist involved in their care to offer expert guidance on procedures, anesthesia and postprocedural management. 
  3. 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). 
  4. Most pregnant patients with ACHD can undergo vaginal delivery safely, with appropriate risk stratification and monitoring. 
  5. Updated recommendations for tetralogy of Fallot include (a) referral for pulmonary valve replacement in patients with repaired tetralogy of Fallot according to right ventricular endsystolic 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. 
  6. 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 <5 Wood units. 
  7. 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. 
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  1. 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. 
  2. Patients with Eisenmenger syndrome can be treated with pulmonary vasodilators using either phosphodiesterase-5 inhibitors or endothelin receptor antagonists as initial therapy. 
  3. 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.