Caring for a Growing Adult Congenital Heart Disease Population

By: JOHN LANE, M.D.
Thursday, December 13, 2012

Just a few decades ago, many children born with congenital heart disease (CHD), the most common type of birth defect in the United States, were not expected to live past childhood. Thanks to surgical advances, 90 percent of the 40,000 children born each year with CHD now live into adulthood. This medical success story has created a new and growing patient population with extremely specialized health care needs — former pediatric patients who are now grown and living with Adult Congenital Heart Disease (ACHD). It’s estimated there are more than 1 million individuals with ACHD, and that number is growing — fast.

Adults with CHD have very different needs than kids with CHD. The disease spectrum in adults is quite wide, ranging from mild cases that may not impact an individual until late into adulthood, to complex and inoperable disease, to problems associated with prior congenital heart procedures. Some individuals have little or no impairment from ACHD and can live very normal lives, while others have major physical limitations.

Adults with CHD may have holes within the walls of their hearts, narrowed or absent blood vessels, malformed heart valves, or the absence of certain normal heart structures. Patients with these abnormalities can develop congestive heart failure, cardiac rhythm problems, bacterial endocarditis and stroke. There are many surgical and nonsurgical treatment options to help them. Another issue specific to adults with CHD is pregnancy. The concern for women with CHD is whether the additional stresses of pregnancy will cause their condition to worsen. A healthy pregnancy will be easier for some women, while others may be counseled against pregnancy.

Individuals with ACHD need lifelong care by specially trained physicians who will monitor their repairs and identify potential problems. The challenge is, if pediatric cardiologists are trained to care for children with CHD and adult cardiologists are trained to treat adult-onset heart diseases, what physicians are best suited to care for adults with CHD? The answer is physicians trained in both congenital heart disease and the care of adult patients, and unfortunately, there is a severe shortage of cardiologists with this specialized training.

According to the Adult Congenital Heart Association, there are only 92 ACHD clinics in the United States. An ACHD specialist understands the nature of CHD, as well as the problems that can develop throughout a person’s lifetime. Also, ACHD patients have needs that aren’t just medical in nature, and these physicians can counsel patients to deal with psychosocial issues such as employment, marriage, insurability and disability

Without training more ACHD specialists, the number of ACHD patients will continue to far exceed the capacity of medical specialists. A greater focus is needed on the development of training programs, as well as the collaboration among adult and pediatric hospitals.

Dr. John Lane is board certified in pediatric and adult cardiology and is the director of Pediatric Cardiology and the Adult Congenital Heart Service in the Akron Children’s Hospital Heart Center.