New registry offers insights into quality of life for adults with congenital heart disease

For the first time, adults living with congenital heart disease (CHD) now have valuable insights into their long-term quality of life through data from the Congenital Heart Initiative (CHI). CHI is the nation's first and largest patient-focused registry for adults with CHD and released its first study involving over 4,500 participants from all 50 states. 

This research, published today in JAMA Network Open, marks a significant step forward in making better information available for the 1.5 million adults in the United States who were born with CHD. 

Studies like this that leverage actual patient voices and experiences help us get a better sense of how to advise, support and treat people with CHD as they age. Also, researchers get a clearer picture of the questions that need to be answered to make sure they have the best quality of life possible." 

Anitha John, M.D., Ph.D., director of the Washington Adult Congenital Heart program at Children's National Hospital and senior author of the study

The study also demonstrates two of the most successful models of current promising trends in clinical research: 

• The power of patient engagement throughout the research process, including design and implementation. 

• The impact of team science, highlighting the benefits of partnerships between patients, researchers and clinicians. 

Key highlights include: 

• Many participants (88%) reported having one or more additional health issues (comorbidities). 

• 33% had arrhythmias (irregular heartbeat). 

• 35% had mood disorders, including depression or anxiety. 

• Quality of life is good or better for 84% of people who completed quality of life reporting measures, regardless of the type of congenital heart condition. 

• People with more complex congenital conditions were less likely to meet physical activity recommendations -; an important finding with immediate impact. 

Treatments for children born with congenital heart disease have improved so significantly in the last two decades that life expectancy continues to increase as well. 

"There are now more adults living with congenital heart disease than there are children with CHD," says Scott Leezer, patient co-principal investigator for the Congenital Heart Initiative registry and co-author of the study. "However, a significant gap remains in what we know about the adult CHD population. As an adult CHD patient, I was excited to contribute to creating this registry, bringing more answers to people like me who want to know how our unique hearts impact our bodies and quality of life over time." 

The authors note that the study's findings and the registry data currently have a few limitations. First, the registry only contains patient-reported outcomes and no clinical data. The first sub-study of the CHI, the CHI-RON study, addresses this challenge by incorporating additional data sources for a subset of consenting CHI participants. 

Additionally, recall bias, underlying neurocognitive challenges and survey fatigue, may have limited participation in the CHI to a smaller subset of adults with CHD. Efforts are underway to develop methods for people with congenital heart disease who have neurodevelopmental deficits or other disabilities to engage in the registry. The CHI is temporarily closed to new registrants as the study team redesigns the study to better align with the needs of the community. 

"We are grateful for everyone who joined this registry, answered survey questions and shared their experiences," says Thomas Carton, Ph.D., chief data officer at Louisiana Public Health Institute and study co-author. "The CHI registry is a big step forward for adults with CHD, but also can serve as a model for how to bring together physicians, researchers and patients as active participants in care, research and advocacy." 

As the registry grows in the future, it will focus on increasing diversity of participants, developing additional partnerships with other organizations, continued innovation in data usage and improved community engagement, all with the goal of guiding future research that will ultimately improve quality of life for all adults with CHD.