University of Utah
Shaji Menon, MD, Primary Investigator
Division of Pediatrics
Salt Lake City, UT
Research project: Effect of Sildenafil on Exercise Capacity in Pediatric Fontan Patients
Amount funded: $24,106
About 35,000 infants are born with congenital heart disease each year in the United States. In the 1940’s, many of these children failed to survive to adulthood. Tremendous progress has been made in the management of even the most complex forms of congenital heart disease and there are now 1,300,000 adults with congenital heart disease in the United States. As more and more children born with congenital heart disease survive in to adulthood, we are seeing a myriad of complex medical issues in the survivors that has skyrocketed the resource utilization and cost of health care. This is particularly true for patients born with single ventricle (essentially “half a heart”) who continue to have the highest morbidity, mortality and health care costs.
The standard treatment for single ventricle patients requires several surgeries with the final stage being the Fontan operation. The Fontan operation allows the only ventricle the child has to pump blood to the body. Venous blood must flow into the lungs without a pump. The Fontan procedure is largely responsible for the improvement in survival of these patients. Along with the improvement in survival, however, Fontan patients are at risk for developing multiple medical problems and this risk increases with age. One of the main health problems that interfere with the patients’ quality of life is the progressive decrease in the ability to perform exercise or work. There are several factors responsible for this but two main causes: 1) absence of a pump to push blood into the lungs and 2) poor pump function that tends to develop over time.