Sacubitril/valsartan for heart failure in patients with complex adult congenital heart disease – Experience from a tertiary centre in Singapore

Patients with adult congenital heart disease (ACHD) have longer life expectancies with the leading cause of death being heart failure (HF). Angiotensin receptor-neprilysin inhibitors (ARNI) have revolutionised contemporary HF management but is less well studied in the ACHD population. The aim of this paper is to describe the safety of ARNI use in complex ACHD and HF in our tertiary centre in Singapore. A retrospective analysis was conducted at our centre to identify ACHD patients of moderate to severe complexity prescribed with ARNI between December 2015 to November 2020. Demographic and clinical data were obtained from review of clinical documentation, laboratory results and imaging reports before and after ARNI initiation. 16 patients were identified, 62% male, mean age 37 ​± ​9.9 years and mean follow up duration 708 ​± ​238 days. At baseline, majority of patients were NYHA II on appropriate guideline directed medical therapy, mean systolic blood pressure 124 ​± ​17.9 ​mmHg, BMI 23.5 ​± ​3.8 ​kg/m2 and creatinine 68.2 ​± ​12.6 ​μmol/L. ARNI uptitration was limited by symptomatic hypotension. The overall mean tolerated daily dose was 128 ​mg ​± ​68 ​mg. Renal function remained within normal limits and there were no episodes of hyperkalaemia. Although there were no HF hospitalisations for the duration of follow up, only 4 of the 16 patients reported improvement in their symptoms. ARNI use in our cohort of patients with HF secondary to ACHD of moderate to severe complexity was well tolerated at suboptimal doses with symptomatic hypotension limiting uptitration.