Heart failure with preserved ejection fraction (HFpEF) in children

Introduction

Heart failure (HF) with preserved ejection fraction (HFpEF) is a complex cardiac condition that results from a structural or functional impairment of filling of the heart. It has a high prevalence in elderly but has rarely been analyzed in children.

Objective

To identify HFpEF in children with biventricular physiology without shunt or outflow tract obstruction, through a dedicated algorithm applied to a large tertiary hospital database, and to describe the causes, hemodynamic profiles, treatment, and hard outcomes of this population.

Methods

We applied a combination of clinical, echocardiographic, and hemodynamic measurements to confirm the diagnosis of HFpEF to identify children with a confirmed HFpEF phenotype extracted from a pediatric hospital data warehouse.

Results/Expected results

In total, 137 HFpEF cases were included. Mean age was 7 ± 5.7 years. The cardiovascular causes of HFpEF were hypertrophic cardiomyopathy (44%), restrictive cardiomyopathy (RCM) (32%), cardiomyopathy due to repeated cardiac surgeries (10%), dilated cardiomyopathy (7%), and constrictive pericarditis (7%) (Figure 1). Children with RCM had the worst prognosis (27% ten years transplant-free survival, P < 0.005). Mean patient follow-up was 5.3 years. 43% of the patients were hospitalized for acute HF, 12% were transplanted and 31% died. NT-proBNP increase was found in all groups and was associated with mortality and transplantation, HR 1.91 (95% CI 1.31, 2.76, p-value < 0.001). Based on echocardiographic evaluation, 56% of the patients were diagnosed with pulmonary hypertension (PH), associated with mortality and transplantation, HR 2.84 (95% CI 1.58, 5.10, P < 0.001). In the 48 patients who underwent right heart catheterization, post-capillary or combined PH were the most frequent but the 15% with isolated pre-capillary PH had poorer outcomes, HR 4.60 (95% CI, 1.46, 14.5, P = 0.009).

Conclusion/Perspectives

HFpEF is a rare and concerning condition in children. Younger age, RCM, acute HF episodes, increased NT-proBNP are associated with hard outcomes. A precapillary component in patients with PH is associated with poorer outcomes.