The emergence of a new patient population

The good news is that improvements in diagnosis and surgery have made it possible to repair most congenital heart defects (CHD). These children are now reaching adulthood with the potential to lead full and active lives. Some experts predict that by 2010, there will be more people with CHD over the age of 18 than there are under age 18.

That is great news, but it is also a big challenge for those of us who care for these adults, as the International Journal of Cardiology report indicates. We do not have much substantive clinical research to guide us, but we are beginning to see the benefits of a multidisciplinary approach for CHD adults emerge.

Falling through the cracks
These adult patients have been at risk of falling through the cracks in our health care system. As children, they are treated by pediatric cardiologists who specialize in the repair and treatment of congenital heart defects.

As they become adults, however, their care often transitions to adult cardiologists trained to care for older adults with cardiovascular disease and heart failure. Between the ages of 20 and 40, these adult survivors of CHD may appear to be doing well. Often they are not followed aggressively since they are not exhibiting acute symptoms.

What we have found, however, is that although these patients may report they're doing “okay,” their heart function may be slowly declining. The signs may not be overt, but when physicians question them closely about their lifestyle and activity level, they find these patients are often leading sedentary lives. We know from what we have learned about older patients with heart failure that the disease develops slowly, well before clear symptoms emerge.

Specialized adult CHD centers offer customized care
These patients are ideal candidates for multidisciplinary care by a team that might include pediatric and adult cardiologists, cardiothoracic surgeons, heart failure specialists and intensive care experts, as well as high-risk obstetricians, genetic counselors and a skilled support team.

Our adult CHD program at Yale-New Haven Hospital and a handful of others across the country are now focusing on identifying and treating these patients before they present with advanced heart failure 10 or 15 years down the road when the prognosis may not be as hopeful as it might be with earlier care. There are subtle signs—chemical and biological markers—that tip us off to the existence of progressing cardiac problems.

Each patient at our center undergoes a thorough health history and diagnostic assessment. Some patients need no immediate treatment, but benefit from ongoing vigilant care. Others may need pacemakers or those with pacemakers may need them reprogrammed. Some are good candidates for catheter or surgical interventions or medical management to help the heart pump more effectively, to control blood pressure and other conditions that may increase a patient's risk of developing advanced heart failure.

Preventive care
Our goal is to preserve as much heart function as we can for as long as we can. It may be that a small number of these patients will be candidates for heart transplant eventually, but it is much better to delay that step for as long as possible. Good preventive care can do that.

Specialized adult CHD programs also provide patients with education about their disease, including the chances of passing CHD to the next generation. Pregnant women with CHD may benefit from high-risk ob/gyn programs that can provide specialized care as they encounter the changes to their bodies associated with pregnancy that can affect congenital heart disease. It's often wise as well to have a fetal echocardiogram performed to check the fetus' heart for possible defects so that preparations can be made to provide expert care for the baby.

Adult CHD programs also provide access to social service support to help prepare for the medical expenses that CHD adults frequently encounter. Most significant heart conditions require lifelong, expensive health care, which is why it's important to obtain and maintain adequate health care coverage with a policy that lets you access physicians with special expertise in congenital heart disease.

Dr. Perry is chief of pediatric cardiology at Yale-New Haven Children’s Hospital, director of the Adult Congenital Heart Program and a professor of pediatrics at the Yale University School of Medicine.