A new study published in the Journal of the American College of Cardiology on March 11 shows that children born with single-ventricle heart disease face significant health challenges well into adolescence and often throughout their lives. The research, conducted by Duke Health and the Pediatric Heart Network, followed 549 children over a period of 16 years.
The findings are important because they provide families and clinicians with a clearer understanding of what to expect after early surgeries for this rare and serious heart defect. According to the study, 87% of participants either died or developed a major health problem during the follow-up period, while only 12% reached adolescence without a significant complication.
Researchers introduced a new global rank score to evaluate long-term health outcomes. This measure was developed with input from families and considers daily functioning, quality of life, and heart performance. The study found that most children will require ongoing specialized care beyond surgery due to high rates of adaptive behavior delays, quality of life issues, and reduced heart function that can emerge gradually as patients grow.
Premature infants were found to be at particularly high risk: more than 60% died during the study period, and only 3% reached adolescence without major problems. The data suggests that supporting healthy pregnancies could improve outcomes for these patients. Additionally, while two main shunt types used in infancy showed similar long-term results overall, babies with moderate or severe tricuspid valve leakage before surgery had worse outcomes if they received the right ventricle shunt.
The research also highlighted differences among hospitals in early mortality rates and long-term rehabilitation success, suggesting opportunities for sharing best practices across centers. "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 authors hope their findings will help families prepare for future challenges while guiding healthcare teams toward improved outcomes through physical therapy, mental health support, school accommodations, exercise programs, and strong long-term monitoring.
