The protocol and rationale for the efficacy aNd ToleRability of sacUbitril-valSarTan in adult congenital heart disease patients with heart failure (ENTRUST ACHD HF) registry

Abstract

Background: Heart failure within the adult congenital heart disease (ACHD) population is a significant source of morbidity and mortality. Optimal medical therapy (OMT) remains the mainstay of management for ACHD heart failure patients. Sacubitril/Valsartan is an angiotensin-receptor neprilysin inhibitor (ARNI) which is a relatively novel addition to the pantheon of heart failure therapies with a significant evidence base to support its reduction in heart failure rehospitalisation, improvement in ventricular reverse remodelling and reduction in mortality in the general heart failure population. Its use in the ACHD population is limited to small case series. Objectives: The Efficacy aNd ToleRability of sacUbitril-valSarTan in Adult Congenital Heart Disease patients with Heart Failure (ENTRUST ACHD HF) registry has been developed to address the tolerability and efficacy of an ARNI in the OMT of ACHD HF patients by quantifying its structural, functional and symptomatic outcomes. Methods: ENTRUST ACHD HF is an international multi-centre, open label, prospective, observational cohort registry designed to include ACHD HF patients with NYHA class II-IV symptoms and systemic ventricular ejection fractions <40% or ≥ moderate systolic dysfunction. The registry will seek to recruit 1000 patients over a 5 year period and document clinical, biochemical, functional and echocardiographic data over a 60 month follow-up window. Safety monitoring will be paramount to the registry design and follow-up process. Conclusions: ENTRUST ACHD HF seeks to provide robust evidence regarding the tolerability and efficacy of Sacubitril/Valsartan in the largest cohort of ACHD HF patients to date through a registry design.

Keywords

• Sacubitril
• Valsartan
• Heart failure
• Registry
• Protocol
• Rationale