Exceptional Lifelong Care for Congenital Heart Disease Takes a Trusted, Team Approach

Kilan McCardie’s first memories are the moments before having heart surgery at Lucile Packard Children’s Hospital Stanford when she was 3 years old. She remembers cheerful nurses using puppets to explain what was about to happen and then hearing a calm voice count down as an anesthesiologist sent her off to sleep.

Kilan was born with a form of congenital heart disease that physicians commonly refer to as complex single ventricle condition, in which the left side of the heart doesn’t develop properly before birth. Doctors in the hospital where she was born quickly discovered the defect and transferred Kilan to Packard Children’s Hospital, where she had her first heart surgery when she was just a few days old. At specialized centers like the Betty Irene Moore Children’s Heart Center at Packard Children’s, children with this type of heart abnormality are often treated with a highly intricate, three-part surgery, and so Kilan’s second surgery followed when she was 3 months old. When Kilan was 3 years old, cardiothoracic surgeons performed the final operation, a Fontan procedure, to reroute blood flow so that her single ventricle could pump blood to her body.

Taking the long view

Kilan, now a recent college graduate, is in excellent health.

This is in part because she’s never had to leave the Stanford hospital system. Even successfully treated congenital heart defects like hers require lifelong monitoring, preventive care, and specialized treatment—orchestrated by a team with expertise in how congenital heart disease affects patients both when they’re children and as adults. “Some malformations require a unique knowledge base that adult cardiologists aren’t regularly trained in, so there’s usually a gap when children grow up and see adult cardiac providers who didn’t have that training,” says Daniel Murphy, MD, medical director, Outpatient Heart Center, Packard Children’s Hospital.

Thanks to technological advances, few heart defects in babies have been deemed inoperable in the past several decades. This has created a large group of people who have survived, for the first time, well into adulthood. As infant mortality continues to drop, pediatric cardiology has moved from a paradigm of “Can we get kids to survive?” to a paradigm of “Knowing what we know, what can we do differently for children now that makes them healthy later on?” says Dr. Murphy.

To meet these needs, he started what eventually became the Adult Congenital Heart Program, a one-of-a-kind collaboration that brings together a team of cardiologists at both Packard Children’s Hospital and Stanford Health Care that specializes in caring for children of all ages as well as adolescents and adults with congenital heart disease. The program was designated one of the first accredited comprehensive care centers for adult congenital heart disease in the country by the Adult Congenital Heart Association (ACHA) in recognition of this high-quality care. Dr. Murphy and his Stanford colleagues regularly consult with other hospitals around the country to help them create similar programs to meet the needs of these unique patients.

“The communication between doctors caring for today’s generation of and those who will care for tomorrow’s generation of adults is critical,” says Dr. Murphy. Working together has enabled them to consider how childhood experiences and interventions affect patients decades later. For example, Frank Hanley, MD, chief of pediatric cardiac surgery at the hospital’s Moore Children’s Heart Center, pioneered an approach to treating the heart condition called tetralogy of Fallot that spares the pulmonary valve, with the goal of avoiding the need for a further intervention later on. This is where collaboration among the team plays a crucial role, aided by the fact that Lucile Packard Children’s Hospital Stanford and Stanford Hospital are literally—as well as figuratively—connected.

Transitioning with trust

When Kilan entered middle school, her relationship with Dr. Murphy changed. Kilan began to get more involved in her yearly appointments, with his encouragement. “When I was younger, my mom asked all the questions but I was quiet,” she says. She found that as she entered adolescence, she was able to open up with Dr. Murphy and talk more. Once it became clear to her that he cared about her personally, they formed a connection based on more than her heart health. “He’d always remember what we talked about the last time, like small details about my life and sports, and about things going on in my life,” she says. “I could just tell how much he cared for me as a person.”

Kilan benefited from Dr. Murphy’s early—and controversial—conviction about the benefits of exercise in patients with congenital heart disease. “Dr. Murphy explained to me how sports were important to keep me healthy,” she says. “He always said, ‘You can do it! I know because I’ve taken care of you for all these years.’” With Dr. Murphy’s enthusiastic support, Kilan went on to become an athlete in middle school and high school, which she loved. “Looking back, I think he helped me focus on all on the things I could do rather than feel like I could never be the kid who could run up and down the basketball court.”

This feeling of support and encouragement carried over into Kilan’s successful transition to an adult provider in the Stanford ACHD program. She went through a formalized, yearlong transition, working with a transition specialist, and learned details about her condition, how to explain it in an emergency, and how to take charge of her health and medical care. “They helped me put all the pieces together and reassured me that even though it sounded scary to move to a new side of the hospital, the same team would be there with me every step of the way,” she says.

A range of adult care

Kilan was apprehensive before her first appointment with her new adult cardiologist, Anitra Romfh, MD. But her concerns dissipated the moment Dr. Romfh breezed into the room. “She was so bright and bubbly and engaging,” says Kilan. “I remember it was very early in the morning and she’d just gotten off a plane from India, but she was wholly present. At that moment, I knew she was a doctor who was willing to show up for their patients no matter what.”

Like Dr. Murphy, Dr. Romfh championed the idea that Kilan’s condition was a reality but not necessarily a limitation. And fostering a warm connection with Kilan has helped her understand Kilan’s big-picture needs. “She never just focuses on my heart,” says Kilan. “One of the first things she said to me was, ‘Have you thought about having kids?’ Coming right out and saying it alleviated the wonder and worry I felt.”

“It’s easy to stick with the status quo but harder to ask, ‘What is it that would help this person thrive?’” says Dr. Romfh, who trained with the specific goal of caring for adult survivors of congenital heart disease. “Dr. Murphy’s team did the hard work to get these kids to adulthood, and I get to take them into the next several decades,” she says. “It’s uncharted territory, but promising, and I feel honored.”

As part of treating people ages 12 to 75, Dr. Romfh not only maintains wonderful care for her patients’ congenital heart disease but also implements prevention methods to keep them healthy. This includes prescribing exercise plans and having patients work with a nutritionist to prevent acquired diseases that all adults can easily fall victim to, such as diabetes, obesity, and hypertension. Her patients are at higher risk because of their heart histories and because at least half of them have an organ system other than their heart that’s negatively affected. While many patients have been seen at Stanford since they were infants—in some cases even earlier as fetuses—other patients enter the program as adults having never been diagnosed as children, so they have never had any kind of cardiac care.

Patients in the program with severe forms of congenital heart defects have had multiple interventions, some of them as many as six heart surgeries, before reaching adolescence. “You could think of these as separate events, but it’s actually cumulative surgical baggage, as each brings scarring as well as psychological trauma,” says Dr. Romfh.

Rewards that last a lifetime

Being able to help patients with their unique, lifelong challenges is a large part of what Dr. Romfh appreciates about this program. “I love my job because I can see patients through the evolution of their lives—when they’re well, not so well, if they need more surgeries,” she says. “I get to be part of their weddings and have witnessed the birth of my patients’ children. It feels like family.” It’s also what draws patients to her. “I know I can go to Dr. Romfh at any point and say, I’m not doing too great, I’m feeling depressed or worried, what can I do?” says Kilan.

Dr. Romfh and Dr. Murphy are proud that Kilan is so independent. “They’ve always told me that I could do it, whatever that was,” Kilan says. “So when I face a big situation now, I think, ‘You can do this.’ It’s probably where my personal drive now comes from. And most important is knowing that I’m not alone. When it gets really difficult, I know I always have a support system to turn to.”