MINNEAPOLIS, MN- December 19, 2018 – University of Minnesota Medical School researchers evaluated the long-term survival rate of patients with a very serious cardiac defect called tetralogy of Fallot. This project was in collaboration with researchers at Emory University.

Tetralogy of Fallot is a birth defect that affects normal blood flow through the heart. This heart defect can cause reduced flow of oxygen in the blood to the rest of the body. Infants with tetralogy of Fallot can have a bluish-looking skin color, called cyanosis, because their blood doesn’t carry enough oxygen. It is treated surgically. The University of Minnesota was one of the first centers to perform this procedure.

In a cohort study funded by a grant from the National Heart, Lung, and Blood Institute (NHLBI), researchers studied nearly 3,300 patients who had surgery which repaired their tetralogy of Fallot. The database, the Pediatric Cardiac Care Consortium, was founded in 1983 at the University of Minnesota to collect surgical outcomes among children with congenital heart disease throughout the country. With NHLBI funding the investigators linked the database to the National Death Index and the United Network for Organ Sharing.

“This study shows adults who had a congenital heart disease as a child that they may have ongoing medical needs as a result of their status and they should seek out a specialist who can tell them what their risks are,” said Logan Spector, PhD, professor in the Department of Pediatrics and the Director of the Division of Epidemiology and Clinical Research at the University of Minnesota Medical School.

Researchers found the overall survival of patients in the database of tetralogy of Fallot surgery was almost 95 percent over 25 years, but there were survival differences among patients. Children who received surgery in stages had nearly twice the mortality rate as children whose heart was repaired at once in infancy, as did children who received “non-valve-sparing” operations.

“A potential cause for higher mortality in non valve sparing surgeries is that with this operation a big incision is made in the right pumping chamber, which could lead to later complications,” explained Julia Steinberger, MD, MS, Director of the Division of Cardiology in the Department of Pediatrics at the University of Minnesota Medical School. Steinberger also said that staged repair consists of two operations, in which the first is a smaller procedure to provide blood flow to the lungs and the second is complete repair. Staged repairs are often done in more severe disease or in patients who have other severe medical problems. These factors in addition to having to undergo two surgeries at a young age, may explain the higher mortality rate.

Steinberger believes this study, which encompasses large numbers of patients from multiple centers, brings them closer to what is needed in this field of research.

“Long-term Outcomes of Tetralogy of Fallot: A Study From the Pediatric Cardiac Care Consortium,” published in JAMA Cardiology, is one of several papers published looking at pediatric mortality. Another such paper is, “Trends in Long-Term Mortality After Congenital Heart Surgery”- and researchers say there are more to come.

“Hundreds of Minnesota children are born with heart defects each year. There is an urgent need to understand their health after surgery,” said Spector.

About the University of Minnesota Medical School
The University of Minnesota Medical School is at the forefront of learning and discovery, transforming medical care and educating the next generation of physicians. Our graduates and faculty produce high-impact biomedical research
and advance the practice of medicine. Visit med.umn.edu to learn how the University of Minnesota is innovating all aspects of medicine.