For more than two years, Duke Health has been building a comprehensive Fontan program designed for patients with single-ventricle heart disease, by bringing together a team of experts across multiple organ systems for surveillance and monitoring.

Customized to help patients who have often undergone multiple surgeries related to Fontan physiology and circulation, the program helps manage complications that the patients experience as they grow.

The co-directors of the program are Gregory A. Fleming, MD, medical director of Duke’s Pediatric and Congenital Heart Center's cardiac catheterization labs, and Alisha M. Mavis, MD, a pediatric gastroenterologist and hepatologist who helped launch a similar program in Chicago.

“The Duke Fontan program provides surveillance and testing to make sure our team can monitor organ system disorders that may develop in these patients,” Fleming says. “These problems occur most commonly in the liver, but the kidney and other organ systems can also be affected.”

Mavis and Fleming collaborate with a team of specialists that includes several cardiologists, an electrophysiologist, a nephrologist, and a hematologist as well as psychiatrists, dieticians, exercise physiologists, and social workers. As the clinic continues to grow, Fleming and Mavis plan to incorporate other subspecialists.

The multi-disciplinary approach has resulted in rapid growth for a program now unmatched in the Carolinas in terms of multidisciplinary care and monitoring.

Mavis, who launched the Fontan program at the Ann and Robert H. Lurie Children’s Hospital of Chicago before joining Duke, says patient volume almost tripled in 2020 when regional cardiologists began to see the value of multi-organ surveillance and monitoring as patients age. The growth continues.

“When we started the clinic, it required a great deal of effort to bring all the pieces together,” Mavis says. “But we have expanded our team and strengthened our operations to create a well-staffed, talented program serving patients and their families in a convenient setting.”

In the early years of the clinic, encouraging referrals from regional cardiologists while preserving the patients’ existing relationships was a key emphasis.

“We focused on communicating with regional cardiologists to emphasize Duke’s collaborative approach and our commitment to maintaining relationships with the original care team,” Mavis says. One of the advantages for referring cardiologists is the ability to monitor non-cardiac issues associated with Fontan physiology and collaborate with the Fontan specialists.

Another key to growth has been the expansion of the Duke’s Adult Congenital Heart Center lead by Director Richard A. Krasuski, MD. The adult congenital heart center works closely with the Duke Pediatric Heart team, sharing expertise about the most effective management of patients with Fontan circulation and helping with assessment of heart failure and potential transplantation.

“This is a rare, challenging condition,” Fleming says. “In the past, unfortunately, these patients did not survive to adulthood. But surgical outcomes have improved so much decade-to-decade that we are beginning to treat these patients as they grow into adults.” According to public data from the Society of Thoracic Surgeons from July 2012 to July 2020, Duke’s survival rate was 100 percent for patients with Fontan circulatory disorders. Average survival among other U.S. programs was 98.92 percent.

During a single visit to the clinic, a patient and family members may consult with a hepatologist or cardiologist, depending on the patient’s needs. In addition to other advanced specialists such as electrophysiologists or nephrologists, the program offers ancillary guidance from dietitians, exercise physiologists, physical therapists and others about the importance of diet and exercise.

“The care for these patients requires a great deal of team planning and discussion about each step in the process,” Mavis says. “Our team reviews each patient’s needs individually to develop a personalized care plan.”

“Psychiatric and neurological development are typically included within the Fontan program as patients grow older,” Fleming says. “It’s essential that we focus on non-cardiac issues that have an effect on their long-term health and development,” Fleming says.

The Duke team has become part of the Fontan Outcomes Network (FON), a multi-institutional initiative focused on improving quality outcomes and research by sharing patient data.

Launched in 2021, the FON comprises 31 centers collaborating by sharing outcomes and quality-of-life data for individuals with single-ventricle heart disease and Fontan circulation. The 31 centers include 15 hospitals.

Participation in FON, say Mavis and Fleming, allows the programs to share research and patient outcomes. “The mission for this program is, first and foremost, improving the health and long-term well-being of patients with Fontan circulation, but research is also part of our mission as well,” Fleming says. “As part of FON network, we participate in ongoing research to improve therapies and outcomes.”