HFpEF, HFmEF, HFrEF in adults with congenital heart disease: Time to face it

In a compelling review on heart failure (HF) in adults with congenital heart disease (ACHD), Brida et al. [1] emphasized that its management is challenged by a wide spectrum of anatomic and pathophysiologic substrates. More specifically, the authors distinguished the univentricular from the biventricular circulation and, in this setting, the presence of right and left ventricular failure. Even though HF with preserved ejection fraction (EF) is described as uncommon in ACHD, its prevalence could be underestimated, as patients may consider symptoms they have been used to since infancy as normal. Searching for additional criteria, including raised natriuretic peptides and increased filling pressures of the systemic ventricle may be necessary to apply for a proper diagnosis and tailored management [2]. The prevalence of HF with reduced EF (≤40%) (HFrEF), mildly reduced EF (41–49%) (HFmEF) and preserved EF (≥50%) (HFpEF) is still unknown in ACHD population, although it may reveal clinical implications. For example, the lack of survival impact of renin-angiotensin-aldosterone-system (RAAS) inhibition on patients with systemic right ventricle in the VAL-SERVE trial may partially be related to just a mild degree of ventricular impairment in half of the enrolled patients [3]. In fact, only HFrEF has shown a clear benefit of medical treatment in non-ACHD HF patients, whereas, in HFmEF, β-blockers and RAAS inhibition have a class IIb C recommendation based on current evidence [2]. Although challenging, a characterization of HF based on EF in ACHD, may lead to a better pathophysiological comprehension, risk stratification, and clinical management of this wide-range population.