Cardiology Specialists Keep a Pulse on Adults Born with Heart Defects

By Jean Yaeger

Courtney Peets was born with a rare and complex heart condition that was treated by pediatric cardiologists from her infancy through her teen years.

When Courtney moved to college, the new cardiologists she saw weren’t familiar with her type of defect. Those cardiologists didn’t have experience with young adults like Courtney, who was born with reversed heart chambers and arteries.  

“I ended up in the emergency room a couple times, but it’s so confusing in an adult world,” she said. “When you hook me up for an EKG (electrocardiogram), it looks like you’ve put the leads on incorrectly.”

Courtney was too old for pediatric cardiology at that point. But she didn’t quite fit in with adult cardiology care either. She didn’t know where to find a specialist for treating adults who have heart problems since birth, called congenital heart disease.

That’s when she joined a support group that introduced her to something she didn’t know existed: cardiology geared for adults born with heart defects. Referrals eventually led Courtney to the Adult Congenital Heart Disease (ACHD) program at Cook Children’s.

Now at age 40, Courtney makes an annual visit to see Scott Pilgrim, M.D., the program’s medical director. Dr. Pilgrim and his ACHD team at the Dodson Specialty Clinics in Fort Worth are able to monitor and help manage her ongoing heart issues. She’s proud to be a patient at Cook Children’s. In fact, every appointment is like a homecoming, because:

• The pediatric cardiologist who first saw newborn Courtney in the neonatal intensive care unit was James Allender, M.D., who helped establish the Cook Children’s Heart Center. Dr. Allender treated her through much of her childhood. 
• Courtney underwent open heart surgery at Cook Children’s at age 17.
• She used to be a registered nurse in the Cook Children’s Hematology and Oncology Center, where she connected with the joy and resiliency of her patients. 

Dr. Pilgrim has helped Courtney manage her blood pressure and other concerns so that she can keep up an active lifestyle of exercise, raising her two sons, and working as Chief Health Service Officer for Burleson Independent School District. She knows she might need heart surgery again someday. The ACHD program gives her confidence she’s in good hands. 

This month, we at Cook Children’s are celebrating the 10-year anniversary of our ACHD program. It was started in February 2014 because leaders at Cook Children’s saw a need. The ACHD services provide comprehensive care and support for hundreds of patients in their 20s, 30s and beyond. Here’s the background.

After Pediatrics

About 1% of all newborns have congenital heart disease, ranging from mild to severe. It used to be considered a pediatric condition because many children with severe defects didn’t survive to adulthood. Thanks to advances in diagnosis and surgery, more children born with heart problems are living longer. An estimated 1.4 million U.S. adults have a congenital heart disease. Their underlying congenital problems can lead to unique health challenges.

“This field of adult congenital heart disease has grown primarily because of the success stories we’ve had in pediatric cardiology,” Dr. Pilgrim said. “The incidence of congenital heart disease hasn’t really changed. Bur the lifespan of individuals growing up with congenital heart disease has tremendously improved.”

The ACHD program looks much like what patients often see in pediatric cardiology, such as EKGs and cardiac magnetic resonance imaging (cMRI), Dr. Pilgrim said. The program offers nutrition, social services, physical therapy, occupational therapy, noncardiac surgery, dental care and more.

Dr. Pilgrim also does pre-pregnancy consultations at the patient’s request. He and the patient discuss the risks for mother and baby. He assesses the structure, function and rhythm of the patient’s heart in those consultations.

“Sometimes we do an exercise stress test to see whether or not they have the aerobic capacity to handle the nine-month marathon of pregnancy,” he said. 

For patients who are already pregnant, the ACHD team works with colleagues in obstetrics and fetal medicine to create a plan based on the patient’s specific heart lesion. That plan includes delivery scenarios and the best options for anesthesia.

The future is bright for this growing subspecialty of cardiology, Dr. Pilgrim said. He pointed out that the adult congenital model at Cook Children’s stems from the Promise to improve the wellbeing of every child in our care and community.  

 “We haven’t neglected the fact that by virtue of us doing surgery as a child, now we have a growing population of adults with congenital heart disease who still need specialized care,” he said.

Repair and Continuing Care

Courtney was born in Fort Worth in 1983 with abdominal organs that were reversed, as well as reversed chambers and main arteries in her heart. Her smaller right ventricle pumps blood throughout her body, while her left ventricle pumps blood to her lungs – the opposite of a normal heart. The right ventricle muscle became progressively thicker, causing obstructed blood flow and low oxygen saturation when Courtney was a girl.

“My lips were always blue,” she recalled. “My parents let me do as much as I could. I played basketball, but I could probably play about a minute before I got tired.”

Courtney went in for regular checkups with Dr. Allender, who would draw pictures to explain how her heart worked. By the time she was 17, she needed surgery to replace a valve and to patch the leaky holes that were allowing the oxygenated and non-oxygenated blood in her heart to mingle. 

“I was the only teenager on the heart floor. There were little ones all around me,” she said. “I was kind of an anomaly because I'm one of the first generations that survived into adulthood with congenital heart disease.”

Courtney went through a short bout of depression after surgery, unaware of the link between congenital heart defects and mental health.  She couldn’t go to church camp that summer, or drink Dr Pepper, or hang out with her friends as much as she liked.

Medication after surgery helped keep her blood pressure down and her heartbeat more regular. And her oxygen levels improved. She was able to sing in the school show choir and assist as manager of the school sports teams. She went on to earn a master’s degree in nursing.

In her mid-20s Courtney experienced episodes of chest pain where her heart raced to 200 beats per minute. She and her husband didn’t think she could safely go through a pregnancy. That’s when Courtney found a support group for adults with congenital heart disease, which led her to a local cardiologist who specialized in adults with congenital heart disease and an obstetrician who specialized in heart disease in pregnancy.

Reassured that the obstetrician’s experience with other “heart mamas” would help get her body through the stress of pregnancy and delivery, Courtney became pregnant. It was a tough journey; she went into heart failure midway though, and the leaks inside her heart increased. Three weeks early, she delivered a healthy, 5 pound, 2 ounce baby boy. Courtney and her husband grew their family several years later by adopting their younger son. 

Courtney became Dr. Pilgrim’s patient several years after the ACHD program opened at Cook Children’s. She trusts that she’s in the right place with experts knowledgeable about congenital issues. Under Dr. Pilgrim’s guidance at annual appointments, she has come off of medications. She can run and lift weights.

She advises parents to make life as normal as possible for children with congenital heart disease. She feels fortunate her own parents raised her like she was “just Courtney” and not defined by her heart problem.

“I still needed discipline. I still had high expectations at school. My defect wasn't a crutch,” she said. “Sometimes I feel like we don't push our kids because they have something wrong. But a kid is a kid, and they're resilient. So just treat them like a kid. Let them be a kid, because that's going to make them more successful moving on.”

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