In June, U.S. News took an in-depth look at children born with serious heart defects. With earlier detection, evolving surgical techniques and treatment at specialized centers, these kids can do better than ever before. But how do their grown-up lives look?
Even with the most complex congenital heart defects, kids are surviving far later into adulthood than they used to, says Dr. Anushree Agarwal, a cardiologist with the adult congenital heart disease clinic at the University of California–San Francisco Medical Center. An estimated 1.6 million American adults live with congenital heart disease, Agarwal says, with this group growing at the rate of 40,000 to 50,000 people yearly.
Unfortunately, some young adults — believing they were cured after surgery as kids — abandon cardiology health care for years. It can take decades and dire emergencies for these patients to reconnect to providers with the cardiology expertise they’ll need throughout their lives.
[See: 10 Seemingly Innocent Symptoms You Shouldn’t Ignore.]
For a long time, Russell Tenofsky, 50, of San Francisco, assumed his heart was “fixed.” Born with Tetralogy of Fallot, a complex combination of heart defects, he underwent open-heart surgery as an 8-year-old. He received pediatric heart care throughout his school years. But once he left college, his cardiac care took a long detour.
Tenofsky’s route back started with a 2009 bike ride. Stopping to pump the tires, he felt “a little pinch” in his chest that soon passed. In the next few weeks, however, he began to feel winded and lightheaded. Eventually, he was hospitalized to undergo open-heart surgery. In another three months, specialists placed an implantable cardioverter defibrillator in his chest, to detect and treat potentially life-threatening abnormal heart rhythms.
Just a few weeks ago, Tenofsky underwent a catheterization procedure to replace a faulty heart valve. Despite all this, he says, “If you looked at me, you would never be able to know I have these issues.”
Because of his preexisting condition, Tenofsky says he has been denied insurance through employers multiple times. Currently covered through Medi-Cal’s working disabled program, he says, “Without that insurance, I obviously wouldn’t be alive.” He now receives treatment through the UCSF program.
As a volunteer with the Adult Congenital Heart Association, Tenofsky stresses the importance of keeping up with cardiac care. “I was the poster child for what not to do as an adult congenital heart disease patient,” he says. “Now I’m the poster child for exactly what to do.”
At the UCSF adult-CHD program, some patients are in their 40s and 50s, Agarwal says. And a few are even in their 70s. These patients have unique needs, of which general cardiologists may not be aware.
“With all the surgical techniques available, [patients] have survived the initial phase of their disease,” Agarwal says. “But those surgeries and procedures are palliative — not a cure.” Most adults with CHD will need cardiac evaluations at least yearly, she says, to identify problems before they become too late to treat or repair. Today, families are told that a congenital heart diagnosis means a lifetime of cardiac care, and groups like the Pediatric Congenital Heart Association are improving awareness.
[See: 17 Ways Heart Health Varies in Women and Men.]
“She’ll grow into her heart.” That’s what doctors told the parents of Pamela Thomas when she was diagnosed with an enlarged heart at age 5 in 1975. It wasn’t until 2008 that heart disease caught up with Thomas, now 46 and a manager in the automotive industry in Florida.
Thomas began experiencing sensations of heavy pressure on her chest and suffered fainting episodes as well. In an all-too-common scenario for women, she describes having her heart symptoms discounted by doctors.
When she was finally evaluated by a cardiologist, Thomas learned she had atrial fibrillation, a problem with her heart’s rhythm. She was also diagnosed with valve defects and congestive heart failure. It turned out her chest pressure stemmed from the whopping 1.7 quarts of fluid she was retaining around her heart. Even now, it’s unclear exactly how much her childhood and adult heart conditions are connected.
Eventually, Thomas required open-heart surgery, including procedures to replace two valves. Diagnosis and recovery were physically and emotionally devastating, she says. Always strong and physically active, she had also spent 18 years in the modeling industry, where “they look for skin that’s clear and beautiful,” she points out. “Scars are not considered as such, right?” A deeper pain was being told she should never have a child, because pregnancy was just too risky for her heart.
Thomas found support through the nonprofit WomenHeart, which advocates for women with heart disease. Now, as a volunteer WomenHeart Champion, Thomas encourages others to take care of their hearts, just as they do with preventive breast cancer care. Her surgical scars don’t bother her anymore. In rare instances where people might point them out, she says, “I do feel like I have a story to tell.”
Critical Treatment Choices
Parents of babies with congenital heart defects make treatment choices that have an enormous impact on their child’s health and survival. When these children grow up, seeking care from appropriate centers and clinicians can be just as critical as the initial treatment decisions, says Dr. Gareth Morgan, an interventional cardiologist on the adult congenital heart disease team at Children’s Hospital Colorado and University of Colorado Hospital.
An estimated 30 percent or more of adult CHD patients who require ongoing care aren’t getting it, Morgan says. But the problem goes beyond patients who are not getting critical ongoing care. “Outside of that are patients being seen by people who have absolutely no expertise, education or training in adult congenital heart disease.” However well-intentioned such providers might be, he says, they’re not equipped to give the right care to patients with highly complex heart conditions.
For example, he says, patients born with a condition that involves narrowing of the heart’s major artery, called coarctation of the aorta, may have undergone major repair surgery in early childhood. However, he says, even as adults, they still have the underlying heart problem. Clinicians must understand subtle treatment distinctions regarding timing of surgical procedures, medication regimens, recommendations for physical activity and more. As children, he says, many patients received overly pessimistic prognoses from doctors unaware of treatment and survival advances.
Only a handful of dedicated centers exist in this country with specifically trained and experienced multidisciplinary teams focused on adults with congenital heart disease. “It’s not enough,” Morgan says. “The U.S. population probably needs several dozens of these centers to make sure we have the capacity to look after these patients properly.”
[See: 9 Extra Safety Assignments for Hospital Patients.]
With proper care, most adult CHD patients can live full, active lives. Active, successful careers are quite possible. “I have patients in my practice that are lawyers, doctors and interior designers,” Agarwal says. She’d like to see more research on the educational impact of congenital heart disease as it can disrupt kids’ schooling when they’re sick.
Huge strides have been made in helping adult CHD patients raise families of their own. “If managed with a team of experts in high-risk obstetrics and adult cardiologists who know the management of these patients, we realize that more and more are able to get through pregnancy safely,” she says.
Coming to terms with psychological issues of being an adult with CHD is important. Agarwal strongly advises that patients connect with the Adult Congenital Heart Association to find support and learn how to advocate for their own care as adults.
More from U.S. News
10 Seemingly Innocent Symptoms You Shouldn’t Ignore
17 Ways Heart Health Varies in Women and Men
9 Extra Safety Assignments for Hospital Patients
Life for Adults With Congenital Heart Disease originally appeared on usnews.com
