Seven years ago, Jeff Theobald was thrilled to learn that he was going to be a father. Then came the routine 20-week ultrasound. “‘There’s a major problem with the structure of the baby’s heart,’” Theobald recalls the doctor telling him. “It was extremely terrifying.”
The ultrasound identified a single ventricle anomaly in the heart, meaning that only one of their son’s two ventricles was of functional size. Theobald was left with more questions than answers about his son’s future. But a chance conversation with one of Theobald’s professional colleagues at the University of Cincinnati (UC) led the couple to Cincinnati Children’s Heart Institute.
The medical team mapped out a plan for the rest of the pregnancy, as well as the baby’s infancy and early childhood. The baby would need three operations in his first four years of life, and the third surgery, called the Fontan procedure, would reconfigure the circulation. During this surgery, the inferior vena cava, the large vein that carries deoxygenated blood from the lower body into the heart, is disconnected from the heart and attached to the pulmonary artery. Following a successful Fontan operation, all the deoxygenated blood from the body goes to the lungs without passing through the heart. His son would be living with one ventricle, rather than the usual two.
This journey helped Theobald realize that he wanted to be an advocate for his son and others who have the same medical condition. He is now actively involved in the Fontan Outcomes Network (FON), a new learning network of patients, families, clinicians, and researchers working to improve outcomes of single ventricle patients. Cincinnati Children’s is one of 12 care centers nationwide helping the network establish and maintain a data registry that tracks patients over their lifespan. Coordinated through Cincinnati Children’s, the Network rep- resents a true collaboration, with each care center contributing to the mission with their expertise and data. FON also receives support from the Congenital Heart Alliance of Cincinnati and Additional Ventures, a nonprofit foundation striving to help develop a functional cure for single ventricle heart disease.
By sharing data, providers nationwide can better collaborate to improve future outcomes for Fontan patients. Launched in 2021, FON hopes to enroll 10,000 patients over the next three years.
“Cincinnati Children’s has pioneered in this sort of learning network,” says Alexander (Sasha) Opotowsky, M.D., director of the Adult Congenital Heart Disease program at Cincinnati Children’s and executive co-director of FON. “This is a ‘team sport’ approach, applying a different model than most research and quality improvement efforts, integrally involving patients, families and medical professionals as equal partners in co-producing this network. It’s really an amazing effort to leverage all of this information to transform outcomes.”
FON emerged from discussion between centers participating in the National Pediatric Cardiology Quality Improvement Collaborative, known as NPC-QIC, a learning network that focuses on improving outcomes among patients with hypoplastic left heart syn- drome, a specific subset of single ventricle heart disease. NPC-QIC tracks children through their first year of life, and FON expands the effort to all forms of single ventricle heart disease over the patient’s lifespan. It’s a needed effort, as those with Fontan circulation will require specialized, expert care as adults.
Theobald learned about FON and NPC-QIC through Cincinnati Children’s. Because of Theobald’s personal experience as the parent of a child with single ventricle heart disease and his professional background with clinical research at UC, NPC-QIC tapped him for its scientific review committee.
In 2021, Theobald saw a job post for a data analyst at Cincinnati Children’s. As luck would have it, it was for a data specialist with FON. “I felt so indebted to them and wanted to do something,” Theobald says.
One of the most important parts of Theobald’s job is to gather data and ensure its accuracy to help determine the best course of treatment as the children grow older. It’s a role that he knows is as meaningful for families as it is for children and adults with a Fontan. FON represents a partnership between patients, families and health professionals across the country to learn how to better care for individuals with single ventricle heart disease — and dramatically improve outcomes.
Theobald was and is a parent first, but he is now also a key part of the team that will make the lives of these children and their families better.
Can you help the Heart Institute take care of the youngest heart patients? Contact cincinnatichildrens.org/service/h/heart-institute.