16-year study finds major health burden in single‑ventricle heart disease

Children born with single-ventricle heart disease, a rare and serious heart defect, often undergo multiple surgeries in their first years of life. A new study shows the challenges for these children can last well into adolescence and sometimes throughout their lives.

Researchers from Duke Health and the Pediatric Heart Network followed 549 children with single ventricle heart disease for 16 years and found that 87% either died or developed a major health problem over time. Only 12% reached adolescence without a significant complication.

These findings, published in the Journal of the American College of Cardiology, give families a clearer picture of what to expect and offer the medical community new direction for improving care.

“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,” said Kevin D. Hill, M.D., corresponding author, chief of division of pediatric cardiology and professor in the Department of Pediatrics at Duke University School of Medicine.

The study introduces a new way of evaluating long-term health called a global rank score. It’s a measure developed with input from families that captures what matters most to them, including daily functioning, quality of life, and heart performance.

One of the most important findings for families is that most children will continue to need specialized care long after surgery. The study found high rates of adaptive behavior delays, quality of life challenges, and reduced heart function in many patients, often emerging gradually as they grow.

Researchers also found that premature babies faced the steepest uphill climb. More than 60% of premature infants in the study died, and only 3% reached adolescence without major problems. The data suggests that supporting healthy pregnancies and preventing preterm birth could make a meaningful difference.

For clinicians, the study provides new clues that may help tailor early surgical decisions. Although the two main shunt types used in infancy showed similar long-term outcomes overall, babies who had moderate or severe tricuspid valve leakage before surgery did worse with the right ventricle shunt, a finding that could guide future decision-making.

Just as importantly, the study revealed substantial differences among hospitals. Some centers had lower early mortality, while others excelled in long-term follow-up and rehabilitation. This variation highlights opportunities to share best practices and improve care no matter where a child is treated.

“These children need lifelong comprehensive care, including medical, developmental, and school support,” Hill said. “It’s not like they get surgery and are ‘fixed.’ Ongoing, coordinated services are essential to help them thrive.”

The study emphasizes that children with single ventricle heart disease often face challenges in multiple areas of life. The research team hopes these findings will help families feel better prepared, while giving health care teams a roadmap for improving outcomes through physical therapy, mental health support, school accommodations, exercise programs, and strong long-term monitoring.