Heart failure with preserved ejection fraction in adults with congenital heart disease

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.

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.

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.

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.