Years ago, children with congenital heart defects often didn’t survive into adulthood.
Now, with new pediatric surgical techniques perfected over the past 50 years, about 90 percent live to be adults.
Despite corrective surgery, it is important for adults with congenital heart defects to have regular follow-up care with cardiologists familiar with the most common birth defect — even when they are considered “cured.”
“The number of adults with congenital heart disease is growing exponentially,” said John Kugler, M.D., the D.B. & Paula Varner Professor of Pediatrics at UNMC’s College of Medicine, chief of the UNMC and Creighton University Medical Center Joint Division of Pediatric Cardiology, and director of cardiology at Children’s Hospital. “There are so many more survivors, especially with complex defects.”
On Friday, during UNMC’s 11th annual Cardiovascular Research Symposium, Roberta Williams, M.D., a pioneer in the field of echocardiography, will give the keynote address on the transition to adult health care for patients with congenital heart disease. Her presentation is at 12:05 p.m. in the Durham Research Center Auditorium. She also is presenting pediatric grand rounds at Children’s Hospital on Friday at 8 a.m.
Dr. Williams is professor and chairwoman of pediatrics at the University of Southern California Keck School of Medicine and the vice president of pediatrics and academic affairs at Children’s Hospital in Los Angeles. In 2000, she was named to the Cardiac Advisory Committee of the National Heart, Lung, and Blood Institute.
Survivors of childhood heart defects and heart surgery have an increased risk for developing cardiac problems, including arrhythmias or congestive heart failure in later years, Dr. Kugler said. “Some have weakened function and require medical management or become transplant candidates,” he said.
About one in 120 babies are born with some kind of heart defect ranging from a hole in the heart to half the heart missing. There are about one million adults in the United States living with heart defects. Of those, about 500,000 have more complex defects. At least 10 percent of all congenital heart defects are first found in adults.
Individuals with congenital heart defects should not become complacent in thinking they are “cured,” Dr. Kugler said. “Congenital heart defects are different than regular adult heart problems, so survivors need to maintain regular scheduled check-ups with a cardiologist specialist who has interest and expertise in congenital heart disease in adults,” he said.
Unlike pediatric cardiologists, adult cardiologists lack the in-depth training on the complexity and management of congenital heart defects. Pediatric cardiologists, meanwhile, have little experience in managing adult care.
“Nationally, there’s a gap,” Dr. Kugler said. “We’re trying to close the shortage of adult congenital heart defect programs by having pediatric cardiologists partner with adult medical specialists.”
Survivors of heart defects need to more closely watch their health habits and get regular heart checkups, Dr. Kugler said. In addition, females with heart defects should have perinatal care during pregnancy.
Medical and life insurance issues also must be considered, Dr. Kugler said.
“There are huge issues when they leave their parents’ coverage and don’t really think about what they need for coverage as adults,” he said.
Dr. Kugler encourages congenital heart patients to join the Adults Congenital Heart Association (ACHA), a nonprofit organization that seeks to improve the quality of life and extend the lives of adults with congenital heart defects.
UNMC’s Cardiovascular Research Symposium also will feature researchers from UNMC and The Nebraska Medical Center presenting original ongoing research in the area of developmental cardiology. The symposium includes poster presentations from clinical fellows, basic science postdoctoral fellows and graduate students currently involved in the investigation of all aspects of cardiovascular research. The poster presentations begin at 3:15 p.m. in the Durham Outpatient Center West Atrium. An award ceremony follows at 4 p.m.
Dr. Williams was a pioneer in the field of echocardiography, beginning in 1973. She developed the initial clinical correlations with echo findings that formed the bases of non-invasive diagnosis of a wide variety of cardiac anomalies.
She established the accuracy of diagnosis that allowed echo to supplant cardiac catheterization prior to surgical correction for many types of defects. These observations changed management of congenital heart disease around the world.
She was one of the few worldwide leaders who advanced the development of two-dimensional Doppler and fetal echocardiography.
More recently, her research interests have focused on clinical outcomes of medical and surgical management of congenital heart disease and physician workforce studies.
She is currently serving as co-chair of the 32nd Bethesda Conference on the Care of the Adult with Congenital Heart Disease.