Congenital heart defects are the most common type of birth defect. While some people end up needing no treatment, others need to address and treat congenital heart disease. The American Heart Association last year noted the importance of exercise for this patient population, but emphasized the importance of individualized care.

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An estimated 40,000 babies are born in the U.S. every year with congenital heart disease—an abnormality of the structure of the heart or blood vessels. Not long ago, many of these children wouldn’t live to reach adulthood. However, advances in treatments are helping people born with congenital heart disease to live longer and with greater quality of life. About 800,000 adults in the U.S. are living with congenital heart disease.

Some of these individuals, because they have been heart patients their whole lives, are mindful of their diet, exercise, and risk factor control. But even with the best of preventive measures in place, conditions such as heart failure continue to challenge these patients and their physicians, explains Yuli Kim, MD, medical director of the Philadelphia Adult Congenital Heart Center at the University of Pennsylvania.

“Adults with congenital heart disease are not just ‘big kids,’” Dr. Kim says. “Many are survivors of pediatric heart disease and others still are diagnosed for the first time in adulthood.” A heart valve, for example, that is the right size for a child’s heart will usually be too small for an adult-sized heart and may need to be replaced one day.

Changing Needs

A person born with congenital heart disease often receives ongoing care from pediatric cardiologists for much of their childhood. They may undergo multiple surgeries and have frequent check-ups to monitor their heart health. But once a congenital heart patient is no longer under a parent’s care, and is experiencing good health, attention to cardiac health may decline.

That’s actually the opposite of what should occur, says Pirooz Eghtesady, cardiothoracic surgeon-in-chief at Washington University-affiliated St. Louis Children’s Hospital. These patients need to be more proactive about heart health. The challenge, unfortunately, is finding doctors who specialize in adults with congenital heart disease.

“Less than 10 percent of adults with CHD are seeing a cardiologist who specializes in their condition,” Dr. Eghtesady says. “Patients are now surviving to adulthood, but it can be difficult for them to find an adult cardiologist with experience in what was once only a childhood disease.” 

Know What’s Ahead

In general, the three main complications that can stem from congenital heart defects are arrhythmias, valve problems, and heart failure—a decline in the heart’s pumping function.

Any one of these issues can also raise the risk of stroke. In fact, a study published last year in the American Heart Association (AHA) journal Circulation found that for individuals up to age 55 with congenital heart defects the rates of ischemic stroke are nine to 12 times higher compared with the general population. The rates are two to four times higher between the ages of 55 and 64.

But it’s heart failure that presents some of the most challenging and life-threatening health issues for many adults with congenital heart disease. Researchers at the Perelman School of Medicine at the University of Pennsylvania presented a study in 2016 at the American College of Cardiology conference that adults with congenital heart disease can often be good candidates for successful heart transplantation.

“We are constantly in search of ways to improve the length and quality of life for those with adult congenital heart disease, and our research indicates that greater consideration should be given to the potential for these patients to receive heart transplants,” says Jonathan Menachem, MD, a fellow in the cardiovascular medicine division at the University of Pennsylvania.

A lack of data on these types of patients, however, means that they often aren’t considered for this potentially life-saving surgery.

“A congenital heart looks, acts, and presents very differently than a normal heart,” Dr. Menachem says. “Due to the complexities of adults with congenital heart disease, the current standards may not assess the needs of this patient population adequately, and therefore these patients in heart failure are listed and transplanted much less frequently than non-congenital heart disease patients with heart failure stemming from other causes.”

Individualized Attention

One of the other great challenges facing adults with congenital heart disease is that the nature of each person’s condition is unique. Potential complications differ depending on the type of congenital heart defect, its severity or complexity, and when it was treated. That’s why it’s especially important to make sure you keep up with your cardiology care throughout your life.

In a first-ever set of recommendations for the treatment of adults with congenital heart disease, the American Heart Association last year noted the importance of exercise for this patient population, but emphasized the importance of individualized care. Some of these patients may have real limitations, so exercise testing with your doctor may be necessary to establish an individualized physical activity plan. And whenever possible, try to get your care at a medical center certified by the Adult Congenital Heart Association (ACHA).

“This ACHA accreditation program is so important because it drives home the fact that these patients require specialized care at certified centers,” says Joseph Billadello, MD, director of the ACHA-certified Washington University Adult Congenital Heart Disease Center. “There are many different types of congenital heart defects, and treatment options are constantly changing. Studies have shown that care at specialized centers saves lives.”