Researchers from Duke Health and the Pediatric Heart Network reported on Mar. 11 that a 16-year study following 549 children with single-ventricle heart disease found that 87% either died or developed a major health problem over time, with only 12% reaching adolescence without significant complications.
The findings, published in the Journal of the American College of Cardiology, provide families and clinicians with new information about the long-term outlook for children born with this rare and serious heart defect. The study offers guidance for counseling families and tailoring therapies to individual patients.
“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 the division of pediatric cardiology and professor in the Department of Pediatrics at Duke University School of Medicine.
The research introduced a global rank score to evaluate long-term health, focusing on daily functioning, quality of life, and heart performance based on input from families. The study found that most children require ongoing specialized care after surgery due to high rates of adaptive behavior delays, quality-of-life challenges, and reduced heart function emerging as they grow. Premature infants faced particularly poor outcomes; more than 60% died during the study period, while only 3% reached adolescence without major problems.
For clinicians making early surgical decisions, researchers noted that babies with moderate or severe tricuspid valve leakage before surgery had worse outcomes if they received a right ventricle shunt. Additionally, differences among hospitals were observed: some centers had lower early mortality rates while others excelled in long-term follow-up and rehabilitation. This variation suggests opportunities for sharing best practices across institutions.
“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.”
Duke University Hospital is located in Durham, North Carolina. It was founded in 1925 and its current president is Craig T. Albanese. The hospital has a pediatric ward and serves as a training hospital; according to their 2022 annual report, it admitted 41,549 patients during that year.
