Guideline Update

ACC/AHA Update Guideline on Adult Congenital Heart Disease Management

Anthony Calabro, MA

Key Highlights

  • New ACC/AHA guideline updates 2018 recommendations for adult congenital heart disease (ACHD), integrating evidence from 2017–2024 and emphasizing lifelong, specialized cardiovascular care.
  • Major focus areas include continuity of ACHD specialty care, mental health and neurocognitive screening, individualized physical activity recommendations, and reproductive counseling.
  • Additional updates address pregnancy management, heart failure therapies, arrhythmia and device management, and guidance on timing of reintervention for moderate and complex congenital heart defects.

The American College of Cardiology (ACC) and the American Heart Association (AHA), in collaboration with several specialty organizations, have released an updated clinical practice guideline for the management of adults with congenital heart disease (ACHD). Published in JACC and Circulation, the update replaces the 2018 guideline and incorporates evidence generated between 2017 and 2024. With more than 90% of individuals born with congenital heart defects now living into adulthood, the ACHD population continues to expand, creating increasing demand for structured, lifelong cardiovascular care.

“We have moved the field forward, in that we have more evidence than we did with the last set of guidelines,” said Michelle Gurvitz, MD, cardiologist, Boston Children’s Hospital, associate professor of pediatrics, Harvard Medical School, and chair of the guideline writing committee, said in a press release. “While we always want more evidence, we are doing better. The growth of the field has been almost exponential in the amount of data being gathered and research being published.”

A core theme of the new guideline is the necessity of ongoing access to clinicians with specialized ACHD expertise. Although many patients receive early intervention in childhood, a significant proportion stop receiving congenital-focused care once they become adults. This discontinuity increases the risk of late complications, including arrhythmias, heart failure, and the need for reintervention. The guideline outlines indications for referral to ACHD specialists and emphasizes collaborative care models intended to improve access across geographic and socioeconomic barriers.

“We know we need to be diligent in our efforts to get pediatric patients into adult congenital heart disease care,” Dr Gurvitz said in a press release. “We still have a lot of patients who stop receiving specialized care as they transition and transfer from pediatric to adult care, and a lot of patients who can’t access physicians for other reasons like insurance or geography.”

The guideline also expands recommendations for mental health and neurocognitive screening. Adults with congenital heart disease experience elevated rates of anxiety, depression, and cognitive impairment, conditions that may influence adherence, functional status, and quality of life. The authors encourage clinicians to incorporate routine mental health assessments and to refer patients for formal evaluation when appropriate.

Additionally, using updated evidence, the writing committee recommends exercise testing to better determine safe and appropriate activity levels. The guideline encourages adults with ACHD to participate in regular physical activity once appropriately evaluated, moving away from historically restrictive or overly cautious approaches.

Reproductive health receives comprehensive updates as well, including guidance on contraceptive counseling, genetic risk assessment, preconception evaluation, and pregnancy management. With proper monitoring and individualized risk stratification, most individuals with ACHD can safely pursue pregnancy and childbirth.

Additional sections address heart failure therapies, surgical and catheter-based reinterventions, arrhythmia management, and device therapy. The authors note that adults with moderate or complex defects may require repeated procedures, valve replacements, ablations, or pacemaker implantation, and in rare cases, heart transplantation.

The guideline concludes by identifying persistent evidence gaps, particularly around optimal timing for interventions and the evolving needs of aging ACHD populations. The writing committee underscores the importance of continued research to support this growing and increasingly complex patient group.

Reference
American College of Cardiology; American Heart Association. ACC/AHA issue new guideline on managing congenital heart disease in adults. Press release. December 18, 2025. Accessed December 18, 2025.