ACC/AHA issue new guideline on managing congenital heart disease in adults

The American College of Cardiology (ACC) and the American Heart Association, along with several other leading medical associations, have issued a new guideline for managing congenital heart disease in adults. The guideline was jointly published today in JACC, the flagship journal of the American College of Cardiology, and Circulation, the flagship journal of the American Heart Association.

Congenital heart disease-being born with defects in the heart’s structure-is the most common birth defect. According to the Centers for Disease Control and Prevention, it affects nearly 1% of births, or about 40,000 babies per year, in the U.S. Advances in surgical treatments over the last few decades have contributed to over 90% of babies born with heart defects in the United States now surviving well into adulthood. This has led to a rapidly growing population of adults with congenital heart disease (ACHD) who remain at increased risk of cardiac complications as they age, despite early interventions.

The new guideline provides updated recommendations for monitoring, counseling and treating adults with congenital heart disease. It includes evidence from research published between 2017-2024 and replaces the previous guidance issued in 2018.

“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, a cardiologist at Boston Children’s Hospital, associate professor of pediatrics at Harvard Medical School and chair of the guideline writing committee. “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.”

The new guideline includes additions related to the role of clinicians with specialized expertise in guiding the care of adults with congenital heart disease; updates regarding mental health, physical activity, pregnancy and heart failure; and new recommendations about specific types of heart defects and treatment approaches.

Congenital heart disease encompasses a wide range of conditions, including more than 30 recognized types of heart defects, which can be classified as simple, moderate, or complex. The symptoms or functional differences that result from these defects include abnormal heart rhythms, diminished exercise capacity, low blood oxygen levels, and signs of heart failure, among others. Many people with congenital heart defects are diagnosed early in life and some may undergo surgeries in infancy or early childhood, while a smaller proportion of individuals with milder defects may only learn about their condition during adulthood.

The guideline authors emphasize the importance of access to ongoing specialized care for adults with congenital heart disease. Even when congenital heart defects are diagnosed and treated in childhood, many patients stop seeing congenital heart disease specialists after they become adults. Common barriers include a lack of awareness about the importance of care continuity, availability of specialists where they live, and socioeconomic or insurance-related challenges. The new guideline provides recommendations about when specialized expertise is warranted and how specialists can partner with other clinicians to broaden access to care.

“We know we need to be diligent in our efforts to get pediatric patients into adult congenital heart disease care,” Gurvitz said. “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. ”

Some adults with congenital heart disease experience mental health or neurocognitive conditions—including mood disorders, as well as cognitive and memory problems—that can worsen if they are not diagnosed and treated.

In the new guideline, Gurvitz said, “we renewed our emphasis on mental health and neurocognitive assessment. Our patients have a lot of challenges with anxiety and depression, so it’s important to discuss that with patients or consider sending them for further evaluation.”

The guideline also includes updated recommendations around exercise. Physical activity is an important part of a heart-healthy lifestyle for everyone, including people with congenital heart disease. However, patients with heart defects are often concerned about what level of activity is safe.

“We looked at using exercise testing to guide physical activity recommendations and spelled this out more broadly than in the prior guidelines,” Gurvitz said. “Many of our patients play sports, and we are now saying that adults with CHD are not just ‘allowed’ to play, they are encouraged to participate in physical activity, after being properly evaluated.”

In addition, the guideline includes new and updated recommendations on birth control options, pregnancy and childbirth. Individuals with ACHD interested in pregnancy should receive preconception counseling, in collaboration with an ACHD cardiologist, about genetic testing, how a pregnancy could affect their health, fetal risks associated with pregnancy and safe birthing options. The majority of pregnant patients with ACHD can have a safe delivery, with appropriate individual risk stratification and monitoring.

Updated guidance on surgical practices, heart failure and treatments for specific forms of congenital heart disease is also provided. The writing committee emphasize that patients with more complex heart defects often require close monitoring and may need additional procedures or medical devices during adulthood, such as valve replacements, ablations or pacemakers to correct arrhythmias (irregular heartbeats), or a heart transplant if heart functioning deteriorates severely. Further research is needed to inform the optimal timing for such interventions, as well as to inform geriatric care approaches as more patients reach older adulthood.

“We’ll continue to push the field to answer the questions because the patient population is not going anywhere—it’s only getting larger,” Gurvitz said. “In particular, our most complex patients are our fastest-growing group, and my hope is that some of the identified gaps will push people to try to answer those questions around what is the most efficient and effective way to deliver care to our patients.”