By Tanner Lambson

How many doctors does it take to heal a heart? Paula Peyton has been on a lifelong quest to find out.

Paula’s medical journey began nearly 40 years ago, during what should have been a relaxing day trip to a beach in Virginia’s Tidewater region. Suddenly, out of nowhere — she fainted.

Paula was rushed to a nearby hospital, revived, evaluated, and admitted, then released a few days later. While she was in the hospital, doctors said something might be wrong with her heart. Paula’s father, a dentist in Newport News at the time, encouraged her to follow up with a family friend who was a local cardiologist.

What Paula learned next changed her life as she knew it.

The cardiologist informed Paula that she was born with a with a rare defect in her tricuspid valve known as Ebstein’s anomaly, a type of congenital heart disease. The tricuspid valve acts as a sort of doorway between the two chambers on the right side of the heart, opening as the heart pumps blood into the lungs and then closing snugly to prevent any backflow. Paula’s tricuspid valve was neither the right shape, nor in the right location, allowing blood to move backward in her heart.

Ebstein’s can cause a variety of symptoms. For Paula, those symptoms were an elevated heart rate, or arrythmia, that caused her fainting spell.

Even though she had lived with Ebstein’s for her entire life, prior to her fainting spell, Paula had always felt perfectly healthy.

“When I was coming up, in elementary school, sometimes my heart would have a little ache in it,” she said. “I never paid any attention to it. I just kept doing what I was doing until that day at the beach in my forties.”

In fact, many people, just like Paula, live with congenital heart disease long into adulthood before they realize something is wrong.

“So often, adult congenital heart disease patients don’t complain [about their symptoms] because either they don’t know another norm, or the symptoms come on gradually – so they make physical accommodations slowly and don’t realize the change in their health over time,” said Sangeeta Shah, M.D., director of the adult congenital heart disease and cardio-OB programs at VCU Health Pauley Heart Center.

Accessing complex adult congenital heart disease care

After her diagnosis of Ebstein’s, Paula would learn that her heart had other problems. Wolff-Parkinson-White syndrome is a malfunction in the electrical system of the heart that causes an elevated heartbeat, and an atrial septal defect is a hole in critical structures of the heart that is common in those with Ebstein’s.

Paula was diagnosed with both.

After three open-heart surgeries in the span of two years, Paula knows a lot about her conditions. She encourages those living with adult congenital heart disease to stay active in their own care and work closely with their cardiologists.

“You always have to be aware of what's going on with you. [If] anything changes or you notice anything — you should always give your doctors a call and let them know,” she said. “Keep [your] appointments and do the things that the doctors tell you to do. Learn about all your medicines and their side effects. If you're taking something and it's not working for you, you need to let them know.”

As time passed, Paula moved her cardiology care to VCU Health, which was closer to her Richmond home. In 2014, Paula and her Pauley care team discovered that the valve repair she received in the 1990s was beginning to fail — a common occurrence for valve repairs ten or more years old. As a result, Paula received a bioprosthetic valve replacement from Pauley cardiothoracic surgeon Vigneshwar Kasirajan, M.D., that same year.

Then, in 2022, Paula began experiencing shortness of breath and excessive sweating — both of which can be symptoms of heart failure. Her Pauley care team diagnosed her with right ventricular heart failure.

“Paula is a tough woman who always tries her best to stay active,” Shah said. “But she was having worsening heart failure that was affecting her liver.”

The needs of adults with congenital heart defects are complex – and caring for patients with adult congenital heart disease requires additional training on top of standard cardiology training.

“If you don't have exposure to a good volume of these very unique conditions, you will not be expert enough to treat them,” said Inna Tchoukina, M.D., a heart failure specialist on Paula’s care team. “The best a general cardiologist can do and should do is to recognize and know when to refer to an adult congenital heart specialist.”

Knowing Paula’s history of congenital heart disease (CHD), Tchoukina recognized the importance of bringing Shah into her care team as well. Shah came to VCU Health in 2020 to establish Pauley’s ACHD program, in order to serve the growing population of adults with congenital heart disease.

Heart defects are the most common form of birth defect and are diagnosed in about 1% of births. Advances in medicine have greatly increased survival rates for babies born with any type of heart defect, which means that today, there are an estimated 1.4 million people living with CHD in the United States, and each year, an estimated 20,000 CHD patients enter adulthood.

Even after successful treatment in infancy or childhood, people born with CHD have an increased risk of developing cardiovascular issues later in life. People living with CHD, like Paula, need lifelong medical care that includes regular checkups and imaging of the heart, and may also require medications, surgery, or even heart transplant.

“As an ACHD team, we spend a lot of time listening to our patients and using the advanced diagnostic testing available at Pauley to look for possible complications,” Shah said. “Each of our patients are unique, and that’s why we develop a personalized treatment plan for each.”

Pursuing novel treatments for congenital heart disease

In 2023, during Paula’s annual clinic visit at Pauley, Shah noticed that the bioprosthetic valve Kasirajan had installed almost ten years earlier was beginning to narrow. Replacement valves can become calcified over time, which thickens the valve and limits their motion, making it more difficult for blood to pass through the heart.

Shah consulted with Samuel Casella, M.D., pediatric cardiologist and director of invasive congenital cardiology at Children’s Hospital of Richmond at VCU, to determine a path forward.

“You could go back to surgery, open up the chest, then take out the old valve and put a new one in,” Casella explained, “but obviously that's a pretty invasive thing to do. It's another open-heart surgery. So we asked ourselves: could we do valve replacement through a catheter instead? And the long and the short [answer] to that is — yes.”

Shah regularly collaborates with CHoR’s pediatric cardiology team as part of her holistic and personalized approach to patient care. Pediatric cardiologists can offer valuable insights for ACHD patients with lifelong cardiovascular medical histories, or patients who may not have discovered their congenital heart defects until much later in life. Paula fit into both categories.

“Over time, congenital heart patients will often be seen by several different medical providers and receive several different diagnoses involving multiple aspects of the heart,” Shah said. “As a congenital heart team, we try to empathize with their concerns and fear, while conveying their complicated medical diagnoses and options in a way both they and their families can understand.”

To repair Paula’s valve for the third time, Casella and Shah added one more person to her care team: Zachary Gertz, M.D., Pauley’s director of structural heart disease. Together, they proposed a novel technique of replacing Paula’s tricuspid valve with a SAPIEN valve, which is a biological valve implanted via catheter rather than open-heart surgery. The SAPIEN valve was developed to replace the aortic valve, not the tricuspid valve. Using a SAPIEN valve to replace a tricuspid valve is considered “off-label,” and though done rarely, has a proven track record as a successful treatment.

“With congenital heart disease, a lot of what we do is ‘off-label’ because [ACHD patients are] a unique patient population that it takes a long time for new treatments to get approved,” Casella said.

“Adults with congenital heart disease have to see a cardiologist for their lifetime, but until more recently, many of these patients were not living into their fifties, let alone seventies,” said Pauley ACHD team member Jessica Hallam, MSN, FNP. “New ACHD procedures are always under development – and great advances have been made in recent years. Paula is living proof of that.”

After a review of the literature and some careful decision-making between Shah, Casella, Gertz, and the manufacturers of the valve – Paula was ready to set a date for the surgery.

“[Paula] asked great questions and did not take anything at face value. She wanted to make sure that we were making the right decisions and that everybody was in agreement,” Casella said. “You need to ask your providers the tough questions and then make sure that you're happy with the answers that they give and that you trust them. And [Paula] trusted us and worked with us every step of the way.”

“A lot of congenital cardiac patients have limited knowledge of their cardiac history,” Hallam said. “This was not the case with Paula. She was an active participant in determining how to best move forward."

The procedure was performed over the span of a few hours, and Paula spent one day and one night in the hospital. She was home the next day.

“We've just been thrilled to see her flourish afterwards, to see her feeling better and getting back to the life that she had before this valve went bad,” Casella added. “It is just wonderful to watch.”

Paula, too, has enjoyed her collaborations with her large care team at Pauley Heart Center.

“They’re always willing to help me,” she said. “When you call them, they aways call you back quick as they can, and they try to assist you in the best way they can. You always need somebody to stand with you at those times that you really are having a hard time, and [the doctors at Pauley] do that. They don’t just talk at you. They’re genuinely concerned about their patients, and they meet people where they are.”

“At Pauley, our ACHD team is vesting in being the medical home for our patients,” Shah concluded. “We take our understanding of the congenital anomaly and then communicate and collaborate with our colleagues to obtain the best results – and we used that collaboration to show Paula what we could do to make her feel better.”

“I've been doing well,” Paula said. “I just get up every morning and say, ‘Gosh, thank you Lord! I'm up! Now, let me continue.’”