Optimizing Long-Term Health for Fontan Circulation Patients: The PittFON Program at UPMC Children's Hospital of Pittsburgh
September 16, 2024
The Fontan procedure is a lifesaving surgery for patients born with single-ventricle congenital heart disease. While the procedure has allowed many individuals to survive and thrive into adulthood, the long-term consequences of Fontan circulation can result in downstream morbidities potentially affecting multiple organ systems. Recognizing the complexity of managing Fontan patients as they age, the Heart Institute at UPMC Children’s Hospital of Pittsburgh, along with colleagues from other divisions has developed PittFON—the Pittsburgh Innovative Therapeutic Approach for Patients with Fontan Circulation. This comprehensive, multidisciplinary initiative is designed to optimize care, enhance outcomes, and address the many health challenges that can be associated with Fontan circulation.
Fontan Circulation: A Multisystem Clinical Challenge
The Fontan procedure creates a unique circulatory path where blood bypasses what would normally be the right ventricle, instead relying on passive flow from the body’s veins directly to the lungs via the pulmonary arteries. While effective in managing single-ventricle congenital heart defects, this abnormal circulation results in increased central venous pressure and decreased cardiac output, both of which can lead to complications affecting the liver, kidneys, lungs, and neurodevelopmental systems over time.
“While the Fontan procedure is primarily a cardiac solution, the long-term effects extend far beyond the heart,” says Tarek Alsaied, MD, MSc, FACC, pediatric cardiologist at the Heart Institute and one of the leaders of the PittFON program. “We can see multiorgan involvement in these patients, and that’s what we’re working to address with PittFON. The goal is to catch morbidities early and prevent them from leading to significant deterioration.”
A Comprehensive Approach to Complex Care
The PittFON program is structured to provide ongoing, comprehensive care for Fontan patients, focusing on both routine surveillance and the early detection of complications. Currently, the program follows approximately 350 to 400 patients across the UPMC system. These patients are closely monitored through standardized testing protocols that focus on cardiac function and the health of other organ systems, particularly the liver, kidneys, lungs, and brain.
“We’ve established screening protocols that allow all physicians, whether they’re at a UPMC facility or a satellite clinic, to follow the same testing guidelines,” says Dr. Alsaied. “This ensures that wherever our patients are, they’re getting the same high level of care.”
The testing protocol includes regular evaluations such as liver ultrasound, cardiac MRI, stress tests, and neurodevelopmental assessments to monitor for early signs of organ dysfunction.
For example, the team monitors liver health with elastography to detect fibrosis, and neurodevelopmental evaluations are performed to identify cognitive or psychological challenges.
Multidisciplinary Collaborations
What sets PittFON apart is its multidisciplinary structure, bringing together specialists from cardiology, hepatology, nephrology, neurodevelopment, and others.
"We have monthly multidisciplinary meetings where we discuss complicated cases and bring in specialists to determine the best course of action for our patients," says Dr. Alsaied.
This collaborative approach ensures that all aspects of a patient’s health are considered and that care plans are comprehensive and tailored to the individual needs of each patient.
“Some patients may need early intervention, whether that’s a heart transplant, a heart-liver transplant, or other surgical procedures,” says Dr. Alsaied. “Our multidisciplinary team works together to identify these patients early and ensure timely care. My colleagues. Lizabeth Lanford, MD, and Evonne Morell, DO, continue to follow many Fontan patients through our comprehensive single ventricle program.”
Veena Venkat, MD, James Squires, MD, MS, and Simon Horslen, MBChB, FRCPCH, from UPMC Children’s Pediatric Hepatology program, are actively involved in the management and treatment of Fontan-associated liver disease. Also collaborating on the PittFON Program are Judy Squires, MD, and Sameh Tadros, MD, from the Department of Pediatric Radiology, who focus on the early detection of liver abnormalities in Fontan patients. This collective expertise ensures that liver-related complications are identified and managed promptly.
The program also benefits from the neurodevelopmental expertise of Lauren Lorenzi, PhD, and her team, who provide comprehensive evaluations and interventions for any neurodevelopmental concerns in Fontan patients.
Given the frequency at which Fontan patients require cardiac catheterization interventions, the PittFON Program works in tandem with the Heart Institute’s interventional cardiology team, including Bryan H. Goldstein, MD, Jacqueline Kreutzer, MD, and Sara Trucco, MD. This collaboration ensures seamless care for patients requiring catheter-based therapies.
“Additionally, we have a close collaboration with our pediatric heart transplant team, referring patients as needed based on their clinical picture for heart transplant evaluation,” says Dr. Alsaied.
Regular “Tune-Ups” and Long-Term Monitoring
Patients are seen in the PittFON clinic every three to five years for a full evaluation, also referred to as a "tune-up." These visits allow the care team to review all test results and make any necessary adjustments to the patient’s care plan.
"We ensure that all necessary testing is done, and we explain to families where their child is in terms of health and potential risks," Dr. Alsaied says.
Between these visits, patients continue to follow up with their primary cardiologists. The program also supports different patterns of care, allowing some patients to co-manage their care between the PittFON clinic and their local providers. This flexible model accommodates patients who live far from UPMC Children’s, ensuring they still receive expert guidance and monitoring even when they cannot visit the clinic regularly.
Supporting Fontan Patients and Families
The program’s commitment to patient education is another key component. The team has developed patient-friendly resources, including a Fontan patient booklet, which provides families with a visually engaging guide to understanding the various organ systems affected by their Fontan circulation and what to expect as the child grows. These resources are designed to empower families to take an active role in their health management.
"We want families to feel confident in their ability to manage their health," says Dr. Alsaied. "By providing resources like the Fontan booklet and personalized testing schedules, we give families the tools they need to stay proactive about their care."
Research and Innovation
Research is another central component of PittFON. The Heart Institute at UPMC Children’s is involved in multiple local and international studies designed to improve care and long-term outcomes for Fontan patients.
For example, the team recently completed a clinical trial on the use of a small respiratory device to improve breathing in Fontan patients. The program also is participating in the FORCE registry, an international effort to use cardiac MRI to identify biomarkers that may help predict health outcomes in Fontan patients.
"Our research helps us better understand the long-term needs of our Fontan patients," says Dr. Alsaied. "By identifying early biomarkers of cardiac or other organ dysfunction or damage, we can develop more effective interventions to prevent complications before they become severe."
A Lifelong Commitment
Fontan patients need lifelong care, and the Heart Institute at UPMC Children’s and the UPMC Heart and Vascular Institute have a robust Adult Congenital Heart Disease (ACHD) program, led by Arvind Hoskoppal, MD, MHS, Anita Saraf, MD, PhD, and Natasha Wolfe, MD, ensuring a seamless transition as patients grow older.
"Once patients reach their mid-20s, they transition to our ACHD team, but we continue to collaborate closely," says Dr. Alsaied. “This is crucial because many complications, particularly involving the liver and kidneys, often manifest later in life.”
Collaborating with the ACHD team ensures that Fontan patients continue to receive expert care tailored to their evolving needs into adulthood.
Looking Ahead
As the Fontan population grows and ages, the result of many decades of advances in surgical techniques and cardiac care, the need for comprehensive, multidisciplinary care is more important than ever. With its structured surveillance protocols, research initiatives, and collaborative care model, the PittFON at UPMC Children’s is optimizing long-term outcomes in this complex cardiac patient population.
“We’re just at the beginning of understanding the full scope of care these patients need as they age,” says Dr. Alsaied. “But with our multidisciplinary approach and commitment to research, we’re committed to improving their quality of life and long-term health outcomes.”
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