Lifelong Care for Congenital Heart Disease at Penn State Health

By Robin Overbay
Thursday, June 23, 2022
Specialty: 

The children’s hospital treats patients with congenital heart disease for both the heart defect and any sequelae of their condition.

Congenital heart diseases (CHD) and subsequent surgeries required to correct them create a unique cardiac anatomy and physiology that is best managed at a CHD center with specialists who have additional training and expertise in treating heart defects. Penn State Health Milton S. Hershey Medical Center’s commitment to research and innovation has resulted in techniques and devices that allow CHD patients to live longer and more fully.

The Mechanics of Life

John L. Myers, MD, pediatric and congenital heart surgeon at Penn State Health Children’s Hospital and professor of surgery and pediatrics at Penn State College of Medicine, has participated in the development of new approaches to CHD since he began practicing medicine in 1986.

“I’ve been in practice for a long time, and I’ve seen a lot of developments over the years,” says Dr. Myers, who was division chief of Pediatric Cardiac Surgery until six years ago. “These include tremendous changes and improvements in surgical technique, postoperative care and cardiopulmonary bypass circuits.”

Improved mortality rates followed these advancements, Dr. Myers notes.

The Milton S. Hershey Medical Center’s Division of Applied Biomedical Engineering fields a team of scientists dedicated to research and development in cardiology. Biomedical and electrical engineers design and manufacture devices for testing with the goal of eventually using these devices in human clinical trials. In the 1970s, Hershey Medical Center surgeons were the first to implant one of the earliest left ventricular assist devices (LVAD), which received widespread clinical use for adults. Current endeavors include:

  • A novel pump to support the Fontan circulation in patients with a single ventricle
  • A pediatric LVAD
  • A pediatric total artificial heart for children to early teens (older teenagers can typically utilize FDA-approved and clinically available adult devices)

As the devices become ready for human application, physicians participate in trials to vet each new approach or technology to ensure it improves on the standard of care for the condition.

Care from Infancy through Adulthood

Hershey Medical Center coined the phrase “a lifetime of care for congenital heart disease,” Dr. Myers says. Under one roof, providers treat patients with CHD at any stage in their life.

“We treat babies prenatally, following birth and all through childhood into adulthood, with an easy transition from a pediatric cardiologist to adult cardiologist,” Dr. Myers states.

For example: Expectant mothers carrying a fetus with tachycardia receive medication that crosses the placenta to treat arrhythmias. Women whose fetus has CHD can consult a team of specialists prior to delivery. After birth, infants with CHD benefit from the expertise of providers trained in treating CHD, including complex cases requiring surgery.

“Many of the more complex defects need to be followed throughout life,” Dr. Myers explains. “That’s where we get into the adult congenital heart disease practice.”

Sequelae of CHD may occur insidiously and advance to serious conditions by the time patients develop symptoms, so regular monitoring by a CHD specialist is recommended. Patients who had repair of tetralogy of Fallot were born without a pulmonary valve, which is well tolerated in babies and children. However, teens and adults can develop right ventricular enlargement and require valve implantation. This can sometimes be performed via catheterization but often requires open-heart surgery to place a tissue valve — either a pig or cow valve — into the pulmonary position. Those valves last about 10 to 12 years before a new valve is required, Dr. Myers notes.

Some patients with CHD will require pacemakers and/or defibrillators. Others may experience deterioration of heart valves, requiring surgical intervention.

When adult patients with CHD require a heart transplant, Hershey Medical Center’s CHD surgeons will scrub in with the adult heart transplant surgeon to perform any necessary reconstructive surgeries on hearts with unique anatomy so the donor heart can be implanted. To complete the circle, adult CHD specialists monitor women with CHD who become pregnant because, regardless of how well palliated their condition may have been at baseline, their cardiac output increases dramatically during pregnancy.

Seamless Transition to Adulthood

This lifelong care requires collaboration among providers so that patients and families can navigate the demands of CHD with ease. Elisa A. Bradley, MD, director of the Adult Congenital Heart Disease Program, director of Translational Science at Penn State Heart and Vascular Institute and associate professor of medicine at Penn State College of Medicine, says she often knows patients prior to their official transition from pediatric to adult CHD care through consultations with the pediatric team.

Dr. Bradley says it is important for adult CHD specialists to monitor these patients to help differentiate the nuanced variations between acquired and congenital heart disease issues.

“Each underlying congenital anatomic lesion, coupled with the surgery or procedures performed, creates a unique anatomy and physiology,” she explains. “Patients need providers with the expertise to know whether an arrhythmia, for instance, can be treated according to normal guidelines or if it could signify something else, such as a conduit narrowing, an issue with a valve or a weakened heart muscle.”

A Passion for Heart Care

In addition to professional zeal, many providers who care for people with CHD are driven by their own personal experiences. For example, Dr. Myers’ loss of his father to complications following aortic stenosis surgery influenced him to choose a career in heart surgery. Additionally, his newborn granddaughter required heart surgery. He also built a pediatric heart surgery program in Guayaquil, Ecuador. His fellow CHD providers share a similar drive to help patients with CHD thrive into and throughout adulthood.

“It’s a real honor and privilege that a parent entrusts [their child] to us,” Dr. Myers says. “I couldn’t have chosen a more rewarding, interesting and challenging career.”


To learn more about the adult CHD program at Hershey Medical Center, please visit pennstatehealth.org/achd.