Myths and Facts: Adult Congenital Heart Disease
Oct 18, 2021 Cedars-Sinai Staff
Congenital heart disease is usually diagnosed in babies—and often requires time-sensitive surgery. But did you know that even after a childhood procedure to correct the heart's structure, most patients will need specialized treatment as adults?
"Congenital heart conditions are not something that end with childhood—all patients should be in lifelong specialty care by physicians who understand their unique needs," says Dr. Rose Tompkins, associate director of the Adult Congenital Heart Program at the Smidt Heart Institute at Cedars-Sinai. "Yes, you can lead a long and productive life—it's just going to be something that requires ongoing surveillance."
It's easy to understand why as many as three-quarters of adults with a history of congenital heart disease are not getting the right care: Cardiologists specialized in treating such adults weren't formally credentialed by the American Board of Internal Medicine until 2015. It's harder to find a cardiologist who treats adult congenital heart disease—there are fewer of them than there are care cardiologists who treat kids with the condition.
At Cedars-Sinai, comprehensive care teams encourage all congenital heart patients to stay in specialized care, especially during the transition from childhood to adulthood, so they stay informed about their condition and remain healthy for life. Here, Dr. Tompkins shares common myths and facts about congenital heart disease.
"Yes, you can lead a long and productive life—it's just going to be something that requires ongoing surveillance."
Myth: After surgery for a congenital heart defect, a patient no longer needs a specialist
Procedures to correct congenital heart defects have improved in the last 50 years, dramatically expanding patients' lifespans and improving quality of life. Even still, most surgical fixes will likely need to be maintained into adulthood.
"We think about surgeries as repairs, not cures," Dr. Tompkins says. "Complications later in life are more common than not."
Though heart defects range in severity, and all patients are unique, many patients who have undergone procedures will eventually need further attention for their heart valves. Others will need stenting to correct narrowing arteries, and corrections for arrhythmias and abnormal heart rhythms.
Much of Dr. Tompkins' work is preventive and aimed at keeping patients healthy in hopes they can avoid future procedures.
"Patients can be so focused on their heart condition that they miss other things," she says. "Our comprehensive program focuses on helping patients maintain things like healthy cholesterol and blood pressure, screening for diabetes, encouraging healthy lifestyle, guiding family planning and promoting engagement with primary care to ensure they are undergoing age-appropriate cancer screening and vaccines."
Fact: Adults with congenital heart disease now outnumber children with congenital heart disease
Thanks to recent advances in surgeries and treatments, more than 90% of kids with congenital heart disease are anticipated to live to adulthood.
Cedars-Sinai's program supports patients as they make the transition from pediatric to adult care—providing young adults education to prepare them to take ownership of their medical matters.
"This is such a huge step for a lot of people whose parents have handled their healthcare their whole lives," Dr. Tompkins says. "As young adults, they need to be able to communicate about their diagnosis and history, make appointments, refill prescriptions and learn how their lifestyles can impact their underlying condition."
Myth: Congenital heart disease is only diagnosed in infancy or childhood
Though a person with a congenital heart defect is always born that way, some people don't know about their condition until they develop symptoms in adulthood.
"Congenital heart disease presents at all spectrums of life, even if you didn't have an early operation," Dr. Tompkins says. "We even see patients who aren't diagnosed until they're in their 60s or 70s."
And it's never too late for patients to see a specialist who can help manage their health—the right interventions can even help patients of any age avoid a heart transplant.
"Sometimes we see really sick patients who had surgery as kid but is not sure what their history is and hasn't seen a doctor in a decade. If we had the opportunity, we could've intervened along the way and kept them healthy," she says. "What we strive to do is prevent their hearts from failing."
Fact: Adult congenital heart disease physicians are specialized cardiologists
The American College of Cardiology and the American Heart Association guidelines recommend that all adults with a history of congenital heart disease, no matter how simple, should see a specialized cardiologist at least once.
"Adult congenital heart disease physicians recognize the unique needs of the population and know enough history to be comfortable treating the interplay of aging and disease," Dr. Tompkins says. "No matter whether we think something is simple and cured, we are finding out that there are sometimes opportunities where we can re-intervene to optimize people's health. If nothing else, we can provide reassurance to a patient that everything is still working well."