2024 Children's of Alabama/UAB Annual Report

Surgery

How Exercise Testing is Helping Fontan Patients

Children born with single-ventricle heart disease undergo multiple surgeries before age 6, ending with the Fontan procedure, which redirects blood from the inferior and superior vena cava to the pulmonary arteries. This allows blood returning from the body to skip the overworked single ventricle and instead flow directly to the lungs to receive oxygen. While the surgeries have improved survival for these patients, long-term outcomes are still poor. Now, clinicians at Children’s of Alabama are honing in on exercise as an important way to track and improve their quality of life. “The Fontan operation is one of the most complicated congenital heart procedures we do,” said pediatric cardiologist Camden Hebson, M.D. “Even with the best outcomes, there’s a shortened lifespan. Many patients start to have major complications by the time they’re in their 20s and 30s, such that it’s pretty unusual for patients to be still living, especially symptom free, in their 50s.” “Outcomes have dramatically improved over the last 30 years,” added cardiothoracic surgeon Hampton Gray, M.D. “But we have sort of hit a plateau over the last 10 years in our ability to move the needle forward for a patient with Fontan physiology. There are still a significant percentage of patients that end up needing a heart transplant at some point in their life, despite optimal management.” The fundamental challenge lies in the procedure’s physiology. In normal hearts, the right side actively pumps blood to the lungs. Because the Fontan operation eliminates this pump, it creates a passive flow system that, while

Children born with single-ventricle heart disease undergo multiple surgeries before age 6

2024 Academic Annual Report

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