RESUMO
BACKGROUND: An improvement in life expectancy in patients suffering from adult congenital heart disease (ACHD) has corresponded with a rise in heart failure incidence within this group. An area that has not been addressed in ACHD heart failure guidelines has been the use of combined inhibition of angiotensin receptor-neprolysin pathways. This case series sought to demonstrate tolerability and 6-month outcome measures of sacubitril/valsartan use in ACHD patients with a severely impaired systemic ventricle. METHODS: A prospective cohort analysis of ACHD patients between December 2016 and September 2017 with severe systemic ventricular systolic dysfunction, New York Heart Association (NYHA) class II-III symptoms and eligible for commencement on a sacubitril/valsartan regime was undertaken. RESULTS: Five (5) consecutive patients were included in this cohort review, 80% male, mean age 41.8 (±19) years and mean systemic ventricular ejection fraction 27% (±3.6%). Two (2) patients with pre-existing D-TGA and atrial baffle repair, one patient with Tetralogy of Fallot repair and pulmonary valve replacement (PVR), one patient with left atrial isomerism and partial atrioventricular (AV) canal defect repair and mitral valve replacement (MVR) and the last patient had biventricular repair for pulmonary atresia with MVR and PVR. Forty per cent (40%) of patients had a systemic right ventricle. All patients had NYHA functional class of ≥II, were on optimal tolerated doses ACE-I or ARB prior to sacubitril/valsartan combination. Six (6) months post commencement of sacubitril/valsartan patients experienced a mean improvement of one functional class. CONCLUSIONS: Our experience suggests that sacubitril/valsartan therapy is well tolerated in ACHD heart failure patients and is associated with improvement in functional class.