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.”

RELATED STORIES:

• Diving Heart First 
• Heart to Heart: Why This Mother and Daughter Share the Same Cardiologist

At the Cook Children's Heart Center, many of our patients grow up with us. We care for the needs of infants with congenital heart disease, and we stay with them all the way into adulthood. Our groundbreaking technologies and expert team of cardiologists, cardiac surgeons, diagnosticians, technicians and health care professionals make up our Adult Congenital Heart Disease program, which provides inpatient and outpatient services. Teenagers or adults with a previously confirmed or newly suspected diagnosis should be referred for a formal evaluation. Because when it comes to patients with congenital heart disease, we're here for life. 

Home is many things for different people, but where most people feel ‘at home’ usually comes with a sense of safety, security, and love. That is how Amber feels about Cook Children’s Medical Center. Although she lives more than 500 miles away from the castle with the blue peaks in downtown Fort Worth, she has a story that began (and ended) right where she felt the safest.

In early June, Amber, 40, found herself on the road traveling from Kansas to Texas. However, this was no vacation. She was headed to have open-heart surgery at Cook Children’s. You may be thinking, “But that’s a children’s hospital, how is that possible?”

Amber worked as a nurse at Cook Children’s from 2006-2014 on several different units at the medical center. In 2014, she and her family moved to Kansas, but she still had ties to Cook Children’s. Amber’s sister, Kathleen Powderly, M.D., is a pediatrician at the Cook Children’s Magnolia Clinic.

In the fall of 2020, Amber went for a routine checkup with her primary care physician and after running some tests, she learned she had a heart murmur. After getting an abnormal echo test and being referred to a cardiologist, she learned she had a subaortic membrane that had gone undetected.

“The symptoms I'd been having, I attributed it to turning 40,” Amber said. “I had just started exercising, so I thought some of those symptoms were just part of getting older.”

Knowing she would need surgery, Amber began her search for a cardiologist in Kansas. After that search led to a dead end, she called her sister in Fort Worth for help.

“I was talking to my sister, Kathleen, and she told me that there was a congenital heart program for adults at Cook Children’s, which I didn't know,” Amber explained. “I had been trying to find a surgeon in Kansas and didn’t have any luck.”

Amber’s sister connected her with Scott Pilgrim, M.D., medical director of adult congenital heart disease at Cook Children’s, who performed an evaluation and determined she was a candidate for surgery.

“Amber’s case was a little unique in that she was not diagnosed with her heart problem until later in life,” Dr. Pilgrim said. “Her diagnosis was a subaortic membrane or tissue that had developed underneath the level of the aortic valve. While not necessarily truly congenital, it is something that we do see in children as little as infants, all the way up into adulthood. Our surgeons are very versed in taking care of these types of problems. Her case fell right within our purview and right within our expertise of being able to handle that problem.”

Phillip Burch, M.D., a cardiothoracic surgeon at Cook Children’s, performed Amber’s surgery. On that early Monday morning in June, he met Amber in her hospital room and explained what would take place detail by detail.

“It feels just like coming home,” Amber said. “I loved it when I worked here. I thought it was one of the best places to work. I have always had every confidence in my fellow nurses and the physicians, and I honestly feel more at ease being here than even at the adult hospital back home. I know how skilled and compassionate they are here and I am very confident and happy to be back.”

There was not a dry eye in Amber’s hospital room as the medical staff prepared to wheel her back to the operating room. Amber embraced her husband tightly, as the nerves of being minutes away from open-heart surgery began to get the best of her. Next, she would embrace her sister. As her sister handed her a rosary, they prayed.

“Can I take this into surgery with me?” Amber asked. The medical staff agreed and moments later Amber was on her way. Her family was only able to walk so far before the waiting game began.

“Kathleen let me take her rosary because I’d left mine in my bag,” Amber said. “Faith has always been important to me. We have lost our parents and a sister, and I knew they were there with me. Kathleen even had holy oil with her that she put on me right before surgery and it was automatically calming to me.”

Amber’s successful surgery was followed by a short Cardiac Intensive Care Unit (ICU) stay before she was released to recover at her sister’s home. She spent four weeks healing, but the process was hard on her physically and mentally.

“I have my good days and bad days,” Amber said. “With open-heart surgery, Dr. Pilgrim explained there would be a great occurrence of depression and emotional instability, and I’ve noticed that. My emotions go up and down, one moment I'll be fine and all of a sudden I'll be crying. It's been a lot to take in and I find myself frustrated with not being able to do what I’m used to right now.”

She says while it was nice to be able to visit her sister and spend time with the family in Texas, it was hard to be away from her five children. She spent a lot of time on FaceTime with her children and played games with them on her phone to make the time pass by.

Amber was able to reunite with her family just in time for the Fourth of July, but being back home came with a hard adjustment period.

“It feels so good to be home,” Amber said. “I am still taking it easy. I’ve been using my arms more to push myself up and I even tried driving recently. I have been trying to use those muscles that I haven’t used in a while, I’ve just been sore.”

She says the best part of being back in the comfort of her own home was reuniting with her husband and children. The younger children were fearful and careful not to ‘hurt’ their mommy as they knew she was still recovering.

For now, Amber will continue to take it slow and regain her strength before she gets back to taking care of patients and working long nursing shifts. She says she needs more strength and endurance before she can be on her feet for a long time.

Amber and her sister, Dr. Powderly, say that while Amber gains her strength to get back to work, they will always reflect on the strong team of employees at Cook Children’s that made what could have been a scary experience, seamless.

“From the time that we walked in for registration that morning to when we walked out about nine days later, I can't even tell you the level of exceptional care that we received,” Dr. Powderly explained. “When you work at a place, you have a sense that what you provide is good, compassionate care. When you are then a patient or a family and you see it from the other side, it is just a total validation of why I’ve spent my whole career at Cook Children’s.”

Amber says she agrees with that sentiment, from being an employee to becoming a patient, she is thankful her story started and ended at home.

“Thank you would never be enough,” Amber said. “Every single person was amazing. They are responsible for giving me my life back, and there is no amount of gratitude in the world that I think would cover it. I am very blessed to have been a patient and been a part of an amazing team.”