Children born with single-ventricular heart disease, a rare and serious heart defect, often undergo multiple surgeries during the first few years of life. New research shows that these children’s challenges can continue into adolescence and, in some cases, throughout their lives.
Researchers at Duke Health and Pediatric Heart Network followed 549 children with single-ventricular heart disease for 16 years and found that 87% died or developed serious health problems over time. Only 12% reached puberty without significant complications.
These findings show that Journal of the American College of Cardiology March 11 will give families a clearer picture of what to expect and provide the medical community with new directions to improve care.
Now that we understand these processes and the risk factors for suboptimal outcomes, we can more clearly counsel families and tailor treatment to each child. ”
Kevin D. Hill, MD, corresponding author, Chief of the Division of Pediatric Cardiology, and Professor of Pediatrics, Duke University School of Medicine
This study introduces a new method to assess long-term health status called the Global Rank Score. It is a scale developed with input from families to understand what matters most to them, including daily functioning, quality of life, and cardiac performance.
One of the most important findings for families is that most children continue to require specialized care long after surgery. The study found that many patients had high rates of delayed adaptive behavior, decreased quality of life, and decreased cardiac function, which often appeared gradually as they grew older.
The researchers also found that premature babies faced the steepest climb. More than 60% of the premature babies studied died, and only 3% reached puberty without major complications. This data suggests that supporting healthy pregnancies and preventing preterm birth can make a meaningful difference.
For clinicians, this study provides new clues that may help adjust early surgical decisions. Although the two main shunt types used in infancy had similar long-term outcomes overall, right ventricular shunts were worse in infants who had moderate or severe tricuspid valve leaks before surgery, a finding that may guide future decision-making.
Equally important, this study revealed significant differences between hospitals. Some centers had lower early mortality rates, while others had better long-term follow-up and rehabilitation. This variation highlights the opportunity to share best practices and improve care, regardless of where children are treated.
“These children require comprehensive lifelong care, including medical, developmental and school supports,” Hill said. “They cannot be ‘cured’ by undergoing surgery; ongoing and coordinated services are essential to their growth.”
This study highlights that children with univentricular heart disease often face challenges in multiple areas of life. The research team hopes these findings will help prepare families and provide medical teams with a roadmap to improve outcomes through physical therapy, mental health support, school accommodations, exercise programs, and robust long-term monitoring.
In addition to Hill, study authors include Lillian Kang, Chao Wang, Jane W. Neuberger, J. William Gaynor, Christine M. Burns, Richard Williams, Felicia Trachtenberg, Brian W. McCrindle, Eric M. Graham, Christian Pizarro, Awais Ashfaq, Andrew Souza, James A. Kuo, Dawn Ilardi, Kurt R. Schumacher, Fani Hong, and Karen S. Goldberg.
This study was funded by the National Heart, Lung, and Blood Institute (HL135680, HL135685, HL135683, HL135689, HL135646, HL135665, HL135678, HL135682, HL135666, and HL135691) with additional support from Miracle Miley. Research Fund.
sauce:
Duke University Medical Center
Reference magazines:
Hill, Kentucky; others. (2026). Single-ventricular disease: long-term outcomes and global morbidity in single-ventricular reconstruction trials. jack. DOI: 10.1016/j.jacc.2026.01.049. https://www.sciencedirect.com/science/article/abs/pii/S0735109726002226?dgcid=author
