Heart Health Check: Are Adults with Congenital Heart Disease at Risk?
"Understanding ASCVD risk in adults with congenital heart disease and how to manage it for a healthier future."
Acquired cardiovascular disease (ASCVD) is a major health concern, even for those who've had heart issues since birth. Myocardial infarction, or heart attack, has become a leading cause of death in adults with non-cyanotic congenital heart disease—where the skin isn't bluish from lack of oxygen. It's important to understand that over 80% of adults with congenital heart disease (CHD) also have other cardiovascular risk factors.
Certain types of CHD and previous surgeries can further increase the risk of ASCVD. This highlights the need for careful assessment and tailored prevention strategies.
A recent study used the Prospective Cardiovascular Munster (PROCAM) 10-year risk score to assess ASCVD risk in adults with CHD. Interestingly, the study found that the absolute 10-year risk was lower in adults with CHD compared to the general population. This may sound reassuring, but it also underscores the complexity of assessing heart health in this specific population.
Risk Assessment in Congenital Heart Disease
Clinical risk assessment aims to identify patients with congenital heart disease (CHD) whose outcomes may be improved, and effective assessment depends on partnership among patients, clinicians, and investigators. A 2024 review notes that CHD spans structural anomalies with distinct natural histories, treatment strategies, and long-term consequences, while current prediction models face challenges with generalizability, limited validation, and use over extended follow-up. For catheterization, the CHARM II model was developed from a large multicenter dataset with contemporary case-type risk categories to support equitable comparisons of adverse events among institutions and operators. A 2025 review set out to assess the characteristics and reliability of clinical practice guidelines for common CHD, but the supplied summary does not report its findings.
From Early Survival to Modern Treatment
The history of CHD care reflects a major transformation: a 2020 review reports that in the 1950s, only approximately 15% of children born with CHD survived. Advances in pediatric cardiology, cardiac surgery, and catheter interventions helped make diagnosis and management a success story of modern medicine. Historical accounts also describe how treatment evolved lesion by lesion, with early surgical outcomes that can seem shocking to readers today. One account suggests CHD may have been identified as early as the fifteenth century by Leonardo da Vinci, while the American College of Cardiology curates key milestones in the field.
Why Standard Risk Calculators Might Fall Short
The usual tools we use to predict heart disease risk, like the Framingham Risk Assessment, Reynolds Risk Score, and ASCVD risk estimator, were developed using data from older adults without CHD. When these scores are applied to younger adults with CHD, they may underestimate their actual risk. None of these calculators, including PROCAM, have been specifically validated for adults with CHD.
- CHD-Specific Factors: The type of congenital heart defect and any previous surgical repairs can significantly impact ASCVD risk.
- Traditional Risk Factors: High blood pressure, high cholesterol, smoking, and diabetes still play a major role.
- New Risk Factors: Research is exploring novel factors, such as coronary anomalies or surgical coronary manipulation, to improve risk prediction.
Psychological Factors and Physical Activity
A study titled “Psychological Factors Related to Physical Activity in Adult Congenital Heart Disease” describes adding five modified items to a validated five-item subscale. The added items were specific to acquired cardiac disease associated with mortality in adults with congenital heart disease (ACHD). Examples given include heart failure and arrhythmia, with the snippet citing Lui and Bhatt (2019). The supplied material does not report the study’s results or establish how the modified items performed.
Risk Factors and Gaps in Care
Evidence in the supplied sources points to important limits in care and risk reduction for people with CHD. A 2024 review reports that controlling modifiable cardiovascular risk factors may lower the risk of heart failure and mortality among CHD survivors of all ages. A review of cardiac care in developing countries notes that a majority of patients with congenital and rheumatic heart diseases do not have access to adequate medical or surgical care. A 2019 meta-analysis of cohort studies evaluated associations between CHD and later cardiovascular disease, including stroke, heart failure, and coronary artery heart disease; the supplied summary does not provide its results.
The Future of Heart Health for Adults with CHD
Better risk assessment could also change how doctors manage their patients' care. Even when patients with CHD meet the guidelines for medications like statins based on ASCVD risk, many don't receive them. By identifying risks earlier, more adults with CHD can reduce morbidity and increased health care utilization.
Long-Term Risk and Limits of Prediction
A 2019 meta-analysis of cohort studies examined the association between CHD and cardiovascular disease risk later in life. Its stated scope included cardiovascular complications such as stroke, heart failure, and coronary artery heart disease, while its background notes that some CHD survivors experience cardiovascular complications over the long term despite advances in surgical and medical management. Separately, a 2024 review describes CHD as a range of structural anomalies with distinct natural histories, evolving treatments, and long-term consequences. That review cautions that current prediction models have challenges with generalizability, limited validation, and application to extended follow-up periods.
Building Global Congenital Heart Care
A 2026 study examined global trends in heart diseases among children and adolescents from 1992 to 2021, focusing on congenital heart disease and non-congenital cardiovascular diseases. The World Heart Federation’s 2026 report addresses the global state of CHD, including epidemiology, care, inequities, and recommendations to close gaps. Its recommendations include developing and scaling centres of excellence and improving referral networks from early detection and diagnosis through surgery, long-term follow-up, and transition to adult care. The report also calls for integrating congenital heart services into broader maternal care.
Care Inequities in Congenital Heart Disease
The World Heart Federation’s 2026 report focuses on the global state of congenital heart disease, covering epidemiology, care, and inequities. It also presents recommendations intended to close gaps in care. The supplied report summary does not specify the size of those gaps or provide individual patient experiences, so the human impact here is reflected in the report’s emphasis on inequity and access to care.
Comprehensive risk modeling, incorporating both traditional ASCVD factors and CHD-specific risks, can help care networks identify high-risk patients and allocate resources effectively. This targeted approach can help ensure that those who need specialized care receive it, while also managing anxiety in stable individuals.
Continued research into risk prediction is critical. By working together and using realistic models, we can create actionable data that improves the lives of adults with CHD as they grow and age.