Heart Failure With Improved Ejection Fraction in ACHD With Biventricular Physiology and Systemic Left Ventricle

In patients with heart failure (HF) with reduced ejection fraction (HFrEF) from acquired heart disease, guideline-directed medical therapy (GDMT) for HF is associated with improved left ventricular (LV) ejection fraction (LVEF) (ie, HF with improved EF (HFimpEF), and which in turn is associated with improved survival. Similar data are lacking in patients with congenital heart disease (CHD).

The objective of the study was to describe the prevalence, correlates, and prognostic implications of HFimpEF in adults with CHD, biventricular physiology, and systemic LV.

Retrospective study of adults with CHD, biventricular physiology, and systemic LV presenting with HFrEF (2003-2023). Echocardiogram was performed at baseline and 1-year follow-up encounters. GDMT use was assessed at baseline and 1-year follow-up encounters using GDMT score. GDMT uptitration (ΔGDMT) was calculated as the difference between GDMT scores. HFimpEF was defined as HF with baseline LVEF ≤40%, and a subsequent absolute LVEF increase ≥10%, leading to LVEF >40% at follow-up echocardiogram. HFrEF relapse was defined as decline in absolute LVEF >10% leading to LVEF ≤40% in patients with HFimpEF. Cox regression analysis was used to assess the relationship between HFimpEF, GDMT score, and outcomes (death and cardiovascular events).

Of the 327 patients (age 46 ± 16 years; LVEF 31% ± 6%), 63 (19%) had HFimpEF. GDMT use (higher baseline and ΔGDMT) was associated with great odds of HFimpEF. HFimpEF was associated with a 26% decrease in all-cause mortality, and 28% decrease in cardiovascular events on multivariable analysis compared to patients without improvement in LVEF. Of the 63 patients with HFimpEF, 27% had HFrEF relapse, and HFrEF relapse was associated with higher risk of cardiovascular events.

These data support the use and optimization of GDMT in this subgroup of CHD patients, and the need for ongoing clinical and imaging surveillance.