The good news for patients with congenital heart disease (CHD) is that advances in diagnosis and surgical treatment have led to dramatic increases in the survival rate, especially for adolescents. In 1985, for example, the median age for patients with severe CHD was 11 years; in 2000 it was 17 years (Circulation 2007;115:163-172). The troubling news is that as these patients age they face increasing psycho-social issues ranging from drug interactions for co-morbid conditions to risks related to exercise, sports, contraception and pregnancy, placing greater emphasis on their transition to independent care as young adults. Yet too few successfully make that transition, noted pediatric resident Tosin Fatusin at a recent Hopkins Children’s Grand Rounds, in large part because many of them lack access to a physician familiar with their condition.

“Only 48 percent of adolescents with congenital heart disease undergo a successful transition,” said Fatusin, explaining that success means the adolescent with CHD has shown an ability to meet his or her own healthcare needs independent of the family.

So, what can pediatricians do? What is their role in transition of care for patients with CHD, the most common type of birth defect, affecting nearly 1 percent of births each year?

Guidelines from the American Heart Association, Fatusin said, target the teen’s emotional and physical developmental status. The transition should foster personal and medical independence and a greater sense of control over health, healthcare decisions and psychosocial environment. Pediatricians should introduce the idea of self-management during adolescence—helping patients learn how to handle health insurance and to schedule routine-care and follow-up visits with specialists—while counseling the patient about diet and exercise and high-risk behaviors.

“They can provide a curriculum in which teens can educate themselves about their medical history, diagnosis and how their hearts are different,” Fatusin said. “Understanding which symptoms are cause for concern, as well as different treatment options, is important.”

But patients should not transition to independent care during a medical crisis or complications such as pregnancy, mental illness or noncompliance. That, Fatusin said, would only impose additional psychological stress on the patient.

For more information, see the American Heart Association’s “Best Practices in Managing Transition to Adulthood for Adolescents With Congenital Heart Disease” (Circulation 2011;123:1454-1485). Also, the Helen B. Taussig Congenital Heart Center at Johns Hopkins offers care for infants, children, adolescents and adults with congenital heart disease.