Children's Hospital of Philadelphia Researchers Spotlight Adults with Hypoplastic Left Heart Syndrome (HLHS) Thriving Without Transplants
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PHILADELPHIA, June 17, 2025 ~ A recent study conducted by researchers at Children's Hospital of Philadelphia (CHOP) has shed light on the long-term outcomes of individuals who underwent complex reconstructive surgery for hypoplastic left heart syndrome (HLHS) as newborns. The study, published in the Journal of the American College of Cardiology, reported that more than 30% of patients in the largest single center cohort survived without a transplant to at least 35 years of age.

Led by Dr. J. William Gaynor, a surgeon in the Cardiac Center at CHOP, the study aimed to bridge the gap in limited data concerning longer-term outcomes for patients with HLHS. The researchers examined long-term survival and the impact of patient factors on survival for newborns with HLHS, as well as functional and health outcomes, including quality-of-life (QOL) in adulthood.

"Our study highlights the impact of CHOP's pioneering surgical approaches to pediatric heart disease," said Dr. Gaynor. "While more research is needed, our findings offer hope for a future where high-functioning adult patients with CHD are the norm."

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The retrospective study analyzed patients with HLHS and associated variants who underwent complex open-heart surgeries between January 1984 and December 2023. The authors noted that while survival has plateaued over the past two decades, they were encouraged by the adult individuals reporting good to excellent health and few Fontan-related complications without a transplant.

The study also emphasized the importance of maintaining cardiac care for adults with congenital heart disease (CHD). CHOP's Philadelphia Adult Congenital Heart Disease Center and Fontan Rehabilitation, Wellness, Activity and Resilience Development program (FORWARD) provide specialized care, evaluation and ongoing monitoring for individuals with single ventricle heart defects and Fontan circulation.

"Moving forward, based on these study findings, we plan to focus more research on adults with CHD, looking at why they are doing well without a transplant," said Dr. Jack Rychik, Director of the FORWARD program at CHOP. "For us, success is seeing our patients thrive as adults. We are committed to finding new, personalized ways to help them heal, grow stronger and live their best lives."

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Dr. Gaynor also stated that they will continue to follow the cohort of adult individuals to better understand their lives in greater depth, including health and wellness, approach to exercise, emotional and cognitive abilities, as well as careers and relationships.

The study was funded by a grant from Big Hearts to Little Hearts, an organization aimed at improving the lives of children and adults with CHD through research, programs, and advocacy. Additional funding includes the Mortimer J. Buckley Jr. M.D. Endowed Chair in Cardiac Surgery, the Thomas L. Spray, MD Endowed Chair in Pediatric Cardiothoracic Surgery and the Daniel M. Tabas Endowed Chair in Pediatric Cardiothoracic Surgery at CHOP.

This groundbreaking study provides valuable insights into the long-term outcomes of individuals with HLHS who underwent complex reconstructive surgery as newborns. With continued research and specialized care for adults with CHD, there is hope for a future where these patients can lead healthy and fulfilling lives without the need for a transplant.
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