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Years of Specialized Support Essential with Rare Heart Defects
- March 12, 2026
- Deanna Neff HealthDay Reporter
For children born with a single-ventricle heart — a rare defect in which the heart has only one functional pumping chamber — the first few years of life are often defined by a series of high-stakes surgeries.
However, a landmark 16-year study reveals that these operations are only the beginning of a lifelong medical journey.
Researchers from Duke Health and the Pediatric Heart Network tracked 549 children with the condition from infancy into their teens.
The study — published Mar. 11 in the Journal of the American College of Cardiology — found that 87% of these children either died or developed a major health complication over time. Only 12% reached their teenage years without experiencing a significant medical setback.
The study provides a much-needed roadmap for parents and doctors.
By using a new evaluation method called a "global rank score," which measures daily functioning and heart performance based on family feedback, researchers can now better predict how a child will fare over the long term.
The score pinpoints the severity of complications by ranking within nine tiers of possible outcomes by age 16.
Death was the most common global rank outcome (39%). About 24% were ranked based on severely impaired quality of life, and 7% based on severely impaired adaptive behavior.
“Now that we understand these trajectories and the risk factors for suboptimal outcomes, we can counsel families more clearly and tailor therapies to each child,” lead author Dr. Kevin Hill said in a news release. He’s chief of pediatric cardiology at Duke University School of Medicine in Durham, North Carolina.
The data highlighted a difficult path for babies born prematurely with the heart defect.
More than 60% of preterm infants in the study did not survive, and a mere 3% reached adolescence without major issues. This suggests that preventing preterm births could be a vital strategy for improving survival rates for children with heart defects.
For many years, the medical community focused heavily on the success of the initial surgery and shunt types.
The two main shunt types used in infancy showed similar long-term outcomes in the study, except in the cases where babies had moderate to severe leakage of the valve between the heart's right chambers. These babies seemed to fare worse with the right ventricle shunt, a finding that could guide more research and care pathways.
This study shifts focus from surgery toward whole-person care that continues long after the child leaves the operating room. Issues like developmental delays, reduced heart function and quality-of-life struggles often emerge slowly as the child matures.
“It’s not like they get surgery and are 'fixed,’ " Hill said. "Ongoing, coordinated services are essential to help them thrive.”
He noted that these children often require physical therapy, exercise programs and special accommodations in school to reach their full potential.
The researchers also found that outcomes varied significantly between hospitals. Some centers were better at preventing early deaths, while others were more successful at long-term rehabilitation, suggesting that sharing best practices between hospitals could save more lives.
More information
The National Heart, Lung, and Blood Institute provides detailed information on congenital heart defects and ongoing research.
SOURCES: Duke University Medical Center, news release, Mar. 11, 2026; Journal of the American College of Cardiology, Mar. 11, 2026
