Gabe Ezell visits with Fontan team members Anita Pai, MD, left, and Angela Weingarten, MD. Photo by Donn Jones.

New clinic ensures comprehensive care for Fontan patients

Published on November 11th, 2022 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 his 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 oxygenated 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 mom, 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 they have a quality life well into adulthood. Monroe Carell 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 needed 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 long term. Monroe Carell has everything we need in one place to take good care of a patient, and we have the expertise across multiple areas.”

Sandra Ezell is thrilled with the experience of the clinic.

“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.”