Every year in the United States, more than 1,250 patients—mainly infants—undergo surgery because one of their heart’s two pumping chambers isn’t working properly, a condition known as single ventricle heart defect.
In a healthy heart, the left ventricle pumps blood to the body, and the right ventricle sends blood back to the lungs for oxygen. When one of the ventricles isn’t strong enough or big enough, the body becomes starved of oxygenated blood.
The standard method to correct this defect is the Fontan, a complex multi-stage procedure that involves reconfiguring the heart’s circulatory system so that it will function with one pump instead of two. While the procedure has prolonged the lives of thousands of people, it’s all but guaranteed to eventually fail.
“An estimated half of all children who survive the Fontan operation will have heart failure by adulthood, and all Fontan patients are expected to eventually fail at some point in their lifetime. The only treatment option for these patients is a heart transplant,” said Scott Leezer, who is all too familiar with the lasting complications of the Fontan surgery.
SEEKING A SOLUTION
Diagnosed with a congenital heart defect when he was 6 weeks old, Leezer has undergone two open-heart surgeries, at 2½ and 24 years old, and has had eight pacemakers. Now 32 and married with two children, Leezer is working to raise awareness and funds for a more permanent solution—an effort that connected him in a professional, and very personal way, with Mark Rodefeld, MD, a professor of surgery at Indiana University School of Medicine.
Rodefeld, who performs the Fontan operation on young patients as a pediatric heart surgeon at Riley Hospital for Children at IU Health, has been working for more than 17 years on developing a fix to the failing Fontan.
An operation first performed in 1968, the Fontan is the standard treatment for patients with single ventricle defects. Surveys of past Fontan patients show that nearly 90 percent survive for at least five years after the procedure, with nearly 80 percent surviving for 10 years or more. While the prognosis has improved with more recent forms of the operation, lifelong complications persist, including problems with the kidneys, liver, and ultimately heart failure.
HOPE FOR A CURE
Rodefeld’s research is focused on developing a small pump that could be placed in the hearts of Fontan patients to emulate a normal two-ventricle circulation, halt the progression of disease and reduce the need for a heart transplant. He has been working on the current version of the device for five years.
“In the best-case scenario, in a perfect world, this would normalize their circulations and in theory give them a normal lifespan. Right now it’s fair to say they are going to live at least half a lifespan and at the same time have progressive problems,” said Rodefeld, an Indianapolis native who has been on the faculty at the IU School of Medicine for 18 years.
“So the hope would be for it to be a cure. The operation right now is not curative—it doesn’t 100 percent resolve the problem. It gives them a situation they can live with relatively long-term, but eventually other problems will creep up and become a major problem.”
RAISING FUNDS FOR RESEARCH
Knowing that he, too, faces the certainty of a major health problem, Leezer has worked to raise awareness, and money, for congenital heart defects. He first met Rodefeld at a conference and later offered to help fundraise for the pump project. Initially, Leezer offered to raise $7,000 for the surgeon. Rodefeld’s expectations, however, were set a bit higher.
“He came back to me and said ‘I love the idea, I think that’s great. But do you think you could raise $350,000?’” Leezer laughed. “I told him he was crazy.”
Mark Rodefeld, MD
They settled on a $100,000 goal, and Leezer jumpstarted the campaign with a two-minute video, made with the help of one of Rodefeld’s patients, to generate interest. The response was stunning.
“We launched it with a hope and a prayer that maybe we would get some traction. It went viral—we have had more than 700,000 impressions on Facebook, and hundreds of thousands of views on the video,” Leezer said. “In total we have raised over $400,000 for the project since we launched in November 2016.”
The unanticipated windfall was enough for Rodefeld to finish an advanced prototype that has undergone full feasibility testing. Rodefeld said the next step is optimizing the prototype to function in blood—a challenging prospect since blood is one of the toughest fluids to pump, the surgeon noted.
INSPIRED BY PATIENTS
As Rodefeld moves toward the next phase of development for his blood pump, he and Leezer agree that with more and more Fontan patients entering adulthood, the need for a fix is becoming more urgent.
“I was kind of on the cutting edge of Fontans. At the time, there weren’t very many centers that were doing it, and those that were weren’t always successful. Now we’re seeing a lot more patients, at more than 100 centers, undergoing this procedure. So what’s that wave going to look like for those patients as they reach their 20s and 30s?” Leezer said.
For Rodefeld, this question runs through his mind with each Fontan operation he performs. While the surgery is a necessity for patients to reach adulthood, the need for a permanent fix is never far from his thoughts.
“I can tell you as a doctor I have some conflicting feelings, because I’m beginning to see a potential solution, and I’m putting them into a state that is sort of a chronic illness. But if they don’t have this initial surgery they won’t live to be adults,” Rodefeld said.
“Look at Scott. You talk to him on the phone, he’s a pretty normal guy. Thirty years ago, you wouldn’t have had that conversation with somebody like him, because that person didn’t live. He’s a living example of a person who could be impacted by this blood pump.”
SUPPORT THIS RESEARCH
You can support this research by making a gift to the Cavopulmonary Assist for Single Ventricle Heart Disease Research Fund at the Indiana University Foundation. Visit the foundation’s online giving form and enter “Cavopulmonary” in the “Search all funds field.”
The views expressed in this content represent the perspective and opinions of the author and may or may not represent the position of Indiana University School of Medicine.
Katie Duffey joined the Indiana University School of Medicine Office of Strategic Communications as assistant director in 2018 after a 14-year career in newspaper journalism. Contact Katie at firstname.lastname@example.org.