HFpEF was present in 13% of adults with congenital heart disease and associated with a higher rate of cardiovascular hospitalization compared to normal LV function (IRR 4.6; 95% CI 4.2-5.1; P<0.0001).
Cohort (n=4,507)
Yes
HFpEF is highly prevalent (13%) in adults with congenital heart disease and is associated with significantly increased cardiovascular hospitalizations, yet remains underdiagnosed and undertreated.
Relative Risk: 4.6 (95% CI 4.2–5.1)
p-value: p=<0.0001
Abstract Background and Aims Heart failure with preserved ejection fraction (HFpEF) is increasingly recognized in the general population, but the prevalence among adults with congenital heart disease (ACHD) is not well described. The study aimed to determine the prevalence, characteristics, and hospitalization burden of HFpEF among ACHD patients. Methods Using the electronic medical files, a retrospective, multicentre cohort study was conducted including 4507 ACHD patients with biventricular heart physiology and systemic left ventricle followed at the ACHD centres in Eastern Denmark. Heart failure with preserved ejection fraction was defined as preserved left ventricular ejection fraction (LVEF ≥ 50%) with diuretics use, combined with elevated N-terminal pro-B-type natriuretic peptide and/or echocardiographic signs of HFpEF. Results Among 4507 ACHD patients, 86% had a preserved LVEF. Heart failure with preserved ejection fraction was identified in 13%, with high prevalence across all ACHD severities (13%, 12%, and 18% in mild, moderate, and severe). Compared with patients with normal left ventricular function, HFpEF patients were older median age: 60 interquartile range (IQR) 47–70 vs 41 IQR 29–56 years, P. 001, had more comorbidities age-adjusted odds ratio for diabetes: 15. 60 95% confidence interval (CI) 12. 39–19. 70; obesity: 1. 48 95% CI 1. 17–1. 87, and atrial fibrillation 4. 58 95% CI 2. 22–9. 88, and experienced almost five-fold higher rate of cardiovascular hospitalization incidence rate ratio 4. 6 (95% CI 4. 2–5. 1), P. 0001. Despite this, only 4. 0% had a heart failure diagnosis, and 5. 8% were treated with a sodium-glucose cotransporter 2 (SGLT2) inhibitor. Conclusions Heart failure with preserved ejection fraction is highly prevalent among ACHD patients, regardless of ACHD severity. It is associated with an increased burden of cardiovascular risk factors and hospitalizations. Despite this, only a minority received SGLT2 inhibitors. These findings emphasize the need for increased awareness of HFpEF in the ACHD population.
Eriksen et al. (2026) conducted a cohort in Adults with congenital heart disease (ACHD) (n=4,507). Heart failure with preserved ejection fraction (HFpEF) vs. Normal left ventricular function was evaluated on Cardiovascular hospitalization (IRR 4.6, 95% CI 4.2-5.1, p=<0.0001). HFpEF was present in 13% of adults with congenital heart disease and associated with a higher rate of cardiovascular hospitalization compared to normal LV function (IRR 4.6; 95% CI 4.2-5.1; P<0.0001).