Success in treating congenital heart disease prompted need for new adult guidelines
Congenital heart disease is diagnosed in approximately 40,000 babies per year. Treatment options were once quite limited, so it was relatively rare to see adult patients with congenital defects. Now, however, care has advanced enough that more than 90% of U.S. newborns with these heart defects surviving into adulthood. Adults with congenital heart disease (ACHD) face a much higher risk of cardiac complications as they age, and there have been concerns over the fact that not many cardiologists have experience treating them.
The American College of Cardiology (ACC) and American Heart Association, in collaboration with several other medical associations, have issued a new guideline for the management of congenital heart disease in adults. It was published in both JACC and Circulation.[1,2]
The new guideline replaces an earlier guidance from 2018. It also includes updated recommendations for monitoring, counseling and treating ACHD based on clinical evidence from research published between 2017 and 2024.
“We have moved the field forward, in that we have more evidence than we did with the last set of guidelines,” writing committee chair Michelle Gurvitz, MD, a cardiologist at Boston Children’s Hospital, associate professor of pediatrics and program director of adult congenital heart disease fellowships at Harvard Medical School, said in a statement. “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.”
There are more than 30 types of congenital heart defects, and each is classified as simple, moderate or complex. The defects cause symptoms or functional issues that include arrhythmias, lower exercise tolerance, low blood oxygen levels, neurocognitive issues and they may present with what appears to be heart failure. The new guideline includes recommendations about specific types of heart defects and treatment approaches and the role of specialists in guiding care of these patients. The documents also include updates on mental health, physical activity, pregnancy and heart failure.
The writing committee emphasized that patients with more complex heart defects may require closer monitoring. Their condition also may require additional procedures as adults, including valve replacements, electrophysiology ablations, pacemakers or heart transplants.
Access to adult congenital specialists is a barrier to care
Gurvitz said a lot of patients stop receiving specialized cardiac care as they transition from pediatric to adult physicians. Many patients also have issues accessing physicians because of issues with insurance or geography. These individuals are often not aware of the cardiac risks that face later in life.
For these reasons, the new guideline provides recommendations about when specialized expertise is needed and how these specialists can partner with other clinicians to broaden the access to care.
“We know we need to be diligent in our efforts to get pediatric patients into adult congenital heart disease care,” Gurvitz added.
Adult congenital heart patients often experience neurocognitive conditions
Some adults with congenital heart disease experience mental health or neurocognitive issues such as mood disorders and memory issues that can worsen if not treated. Gurvitz said the new guideline renewed the emphasis on mental health and neurocognitive assessments in these patients.
"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,” she explained.
Exercise recommendations for congenital heart patients
The guideline also includes updated recommendations about 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 physical 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 ACHD are not just ‘allowed’ to play, they are encouraged to participate in physical activity, after being properly evaluated.”
New guidance for women with congenital heart disease
The guideline has updated recommendations for women on pregnancy, childbirth and the use of birth control. This includes preconception counseling for women who want to come pregnant in collaboration with an ACHD cardiologist. There are also recommendations on genetic testing, how a pregnancy could impact their health, associated risks to the fetus during pregnancy and safe birthing options.
The majority of pregnant patients with ACHD can have a safe delivery, but the writing committee said there is a need for appropriate risk stratification and regular monitoring to ensure safety.
Questions still remain on adult congenital heart patients
As this patient population continues to increase and age, new research and data is needed to inform future updates to this guideline. Data is also needed to better understand geriatric care in these patients and if there are any special considerations.
“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.”
The writing committee included specialists in ACHD cardiology and interventional cardiology, congenital cardiac surgery, heart failure and electrophysiology, advanced practice nurses and a patient advocate. The guideline was developed in collaboration and endorsement from the Heart Rhythm Society, the International Society for Adult Congenital Heart Disease and the Society for Cardiovascular Angiography and Interventions.
Dave Fornell has covered healthcare for more than 17 years, with a focus in cardiology and radiology. Fornell is a 5-time winner of a Jesse H. Neal Award, the most prestigious editorial honors in the field of specialized journalism. The wins included best technical content, best use of social media and best COVID-19 coverage. Fornell was also a three-time Neal finalist for best range of work by a single author. He produces more than 100 editorial videos each year, most of them interviews with key opinion leaders in medicine. He also writes technical articles, covers key trends, conducts video hospital site visits, and is very involved with social media. E-mail: [email protected]