Researchers at the Children’s Hospital of Philadelphia (CHOP) have released findings on the long-term clinical outcomes for individuals who underwent reconstructive surgery for hypoplastic left heart syndrome (HLHS) as newborns. The study, published in the Journal of the American College of Cardiology, reports that over 30% of these individuals survived without a transplant to at least 35 years old. Many adults from this group reported good to excellent health and quality-of-life, indicating a “high-performing” Fontan phenotype.
The study addresses a gap in data concerning long-term outcomes for HLHS patients by examining survival rates and patient factors affecting survival, as well as functional and health outcomes in adulthood.
Dr. J. William Gaynor, a lead author and surgeon at CHOP’s Cardiac Center, stated, “Our study highlights the impact of CHOP’s pioneering surgical approaches to pediatric heart disease.” He added that while more research is needed, the findings offer hope for high-functioning adult patients with congenital heart disease (CHD).
The retrospective study analyzed patients with HLHS who underwent complex surgeries between January 1984 and December 2023. Although survival rates have plateaued over two decades, many adults report good health without transplants.
The study emphasizes maintaining cardiac care for adults with congenital heart disease. CHOP’s Adult Congenital Heart Disease Center and FORWARD program provide specialized care and monitoring for those with single ventricle defects and Fontan circulation. Historically, research focused on risk factors associated with worse outcomes for HLHS patients. In contrast, CHOP aims to understand what drives patient success through its innovative programs.
Dr. Jack Rychik, Director of the FORWARD program at CHOP, noted plans to focus future research on adults thriving without transplants: “For us, success is seeing our patients thrive as adults.”
Dr. Gaynor mentioned ongoing efforts to follow adult individuals from the cohort to gain deeper insights into their lives including health, wellness, exercise habits, emotional and cognitive abilities, careers, and relationships.
Funding for this study was provided by Big Hearts to Little Hearts along with several endowed chairs in cardiac surgery at CHOP.
