Complex congenital heart patients live longer, need new treatment paradigm

“Sometime in the next decade,” said James C. Perry, M.D., professor of pediatrics (cardiology) and chief of the Section of Pediatric Cardiology, “there will be, for the first time, more people over 18 living with congenital heart disease than people under 18.”

Congenital heart disease (CHD), the most common of birth defects, affects 0.8 percent of all live births in any given year in the United States. Thanks to advances in surgical and medical management techniques for treating children born with structural abnormalities of the heart, many children for whom CHD would once have been an early death sentence can now expect to live well into adulthood.

That fact underlies the work of the Yale-New Haven Adult Congenital Heart Program, the first of its kind in Connecticut and one of only two dozen in the country. The outpatient clinic is staffed by Perry and coordinator Nicole K. Boramanand, A.P.R.N. ’99, and the program provides a collaborative and multidisciplinary approach to monitoring and treating adult survivors of pediatric CHD. The most common problems experienced by these patients are arrhythmias and heart failure.

“Pediatric heart patients in the past were often discharged from pediatric care based on age, but adult cardiologists are not usually trained to manage congenital heart disease. Those patients had nowhere to go,” Perry said. “Our program offers access to pediatric and adult cardiologists, heart failure specialists, pulmonologists, transplant specialists, specialists in high-risk obstetrics and other medical staff with essential expertise.”

Patient education is also critical. “Our focus is on preventive maintenance,” Boramanand said. “What treatments and lifestyle adjustments can increase the length and quality of life? For instance, we emphasize to our patients that the old notion that all people with adult CHD should avoid exercise is no longer accepted.”

Boramanand notes that another aspect of education and prevention is obtaining extensive diagnostic information up front. “If we get readings early and track them regularly, we can see situations that may require treatment.”

Perry points out that individuals who survive into adulthood with CHD also experience the full range of health concerns, from catching the flu to developing arthritis to managing pregnancy and childbirth, and notes that the center helps clinicians learn more about this population.

As of this spring the Yale-New Haven Adult Congenital Heart Program, which began in July of 2004, had seen nearly 100 patients ranging in age from 17 to their late 50s. “Life expectancies vary,” said Perry, “and this is a group of patients we are just beginning to learn about. But to see people with complex congenital heart defects going strong into middle age is remarkable.”