by Christina Echegaray
By age 2, Gabe Ezell had two open heart surgeries to repair his congenital heart condition — tricuspid atresia with a hypoplastic right ventricle.
Diagnosed in utero during a 20-week ultrasound, doctors found that the right side of Gabe’s heart was underdeveloped, resulting in him having only one functional pumping chamber rather than two. Gabe’s two complex heart surgeries, a Glenn and a Fontan, were part of a two-staged approach to help reroute deoxygenated blood coming back from the body to the lungs without using a pump. His one functional pump is used to pump oxygenation blood to the body. The surgeries, however, don’t fully correct the defect, but make it more manageable.
“We had a fetal echocardiogram, and our cardiologist, Dr. David Parra, was very compassionate. We were very, very upset (when we found out the diagnosis in utero),” said Gabe’s mother, Sandra Ezell. “We didn’t know what it meant for Gabe short term or even long term.”
As children like Gabe, now 11, live longer following these palliative heart procedures, cardiologists at Monroe Carell Jr. Children’s Hospital at Vanderbilt want to ensure these patients have a quality life well into adulthood. Children’s Hospital recently launched a new multidisciplinary Fontan Clinic, which will provide an ongoing, coordinated care plan for children and teens, ages 10-19, who have had the Fontan procedure for single ventricle heart disease.
Led by cardiologist Angela Weingarten, MD, the comprehensive team coordinates care to address the long-term impact that these congenital heart conditions have on children and adolescents beyond the original diagnosis.
The team, which works in consultation with a child’s primary cardiologist, consists of two cardiologists, a cardiac nurse and nurse practitioner, a liver expert, a pulmonologist, a psychologist, a physical therapist and a nutritionist. Patients will be seen once every three years, with follow ups as need for various evaluations and care plans.
“We don’t fully know the real long-term effects of the Fontan. In the span of medical history, it’s still a relatively new procedure,” said Weingarten. “We want to be able to optimize people’s outcomes while they’re adolescents and still doing well, so that hopefully it will help them longterm. Children’s Hospital has everything we need in one place to take good care of a patient, and we have the expertise across multiple areas.”
Children’s Hospital was also recently selected to join the Fontan Outcomes Network (FON), a group of 31 care centers from around the United States and Canada that work together with “a shared vision to improve outcomes and quality of life for all individuals with single ventricle heart disease and Fontan circulation.”
FON estimates that about 70,000 people worldwide live with single ventricle heart disease and Fontan circulation.
“While there are increasing numbers of patients who have undergone Fontan, at any single center there are not enough patients for high impact research. FON will allow us to answer big questions with larger patient numbers to improve outcomes and maximize quality of life in people with Fontan,” Weingarten said.
Patients in the Children’s Hospital Fontan Clinic will undergo a series of medical examinations to determine any underlying health issues so the medical team can intervene early. Also, because patients with chronic heart conditions commonly have depression, anxiety and learning disabilities, each patient will also meet with the team psychologist. Liver and pulmonary issues can also occur with single ventricle heart disease, so specialists will evaluate any potential concerns.
Other specialists on the team, like physical therapists and nutritionists, will provide care plans as need for exercise and nutrition management.
“The benefits are obvious,” said Ezell. “My husband and I have thought, ‘We’ve worked so much on his heart function, but what damage has it done to the rest of his body?’ We hadn’t really thought much about that until this Fontan Clinic — that this abnormal heart function was potentially going to affect his other organs.”
Sandra Ezell is already thrilled with the experience of the clinic. While the information she is learning about potential areas of concerns can be stressful, she recognizes that not knowing would be worse.
“We’ve met with several specialists, and we’re going back for some further testing,” she said. “I love knowing we can be aware of areas of concern and be proactive in trying to fix them. Vanderbilt has needed this for so long, and I can’t wait to see it expand and help others. These kids are basically pioneers, as more of them are living longer. We need to explore more ways to help them.”
Gabe said he loves the clinic too and the chance to see his doctors at Children’s Hospital. He also doesn’t mind when a visit to the hospital means he gets a special treat in the food court. Ultimately, Gabe hopes to help other children. “I want to be able to give them advice,” he said.