Cardiac Remodeling, Prognostic markers, and Cardiovascular Outcomes Across Heart Failure Phenotypes in Adults with Congenital Heart Disease.

BACKGROUND

Data comparing cardiac remodeling, prognostic markers, and cardiovascular outcomes across heart failure (HF) phenotypes in adults with congenital heart disease (CHD) are limited.

METHODS

In this study, adults with CHD and HF were classified based on left ventricular ejection fraction (LVEF): HF with reduced EF (HFrEF; LVEF <40%), HF with mildly reduced EF (HFmrEF; LVEF 41%-49%), and HF with preserved EF (HFpEF; LVEF ≥50% with pulmonary artery wedge pressure ≥15 mmHg). Cardiac function (strain imaging), prognostic markers (NT-proBNP and peak oxygen consumption), and cardiovascular outcomes (HF hospitalization and cardiovascular death/heart transplantation) were compared across groups.

RESULTS

Among 1,303 patients, 304 (23%) had HFrEF, 551 (42%) had HFmrEF, and 448 (34%) had HFpEF. There was a graded worsening of biatrial and right ventricular function from HFpEF to HFmrEF to HFrEF (all p<0.001). Despite less severe chamber dysfunction, HFpEF was characterized by higher NT-proBNP levels and lower peak oxygen consumption compared with HFmrEF. HFrEF was associated with the highest adjusted risk of HF hospitalization (hazard ratio, 1.52; p=0.005) and cardiovascular death/heart transplantation (hazard ratio, 1.74; p=0.002), compared to HFmrEF and HFpEF. HF therapy was associated with lower event rates overall, with the strongest association in HFrEF, a more modest association in HFmrEF, and no significant association in HFpEF.

CONCLUSIONS

In adults with CHD and HF, LVEF-defined HF phenotypes identified distinct patterns of cardiac remodeling and prognosis, with worse cardiac remodeling and adverse outcomes observed in the HFrEF phenotype. These findings support use of EF phenotype-based approach to HF management in CHD.