Initial Experience in Adults with Complex Congenital Heart Disease Treated with Sacubitril/Valsartan for Heart Failure

T L Goodwin, C R Broda, A Opina, W Lam, P R Ermis
Journal of Heart and Lung Transplantation 2020, 39 (4S): S238-S239

PURPOSE: Adults with congenital heart disease (ACHD) are an emerging population. Heart failure (HF) is a major cause of morbidity and mortality for ACHD. Sacubitril/valsartan has been shown to reduce the risk of hospitalizations and death from HF in acquired cardiovascular disease but there is little information available on its effect on HF in ACHD.

METHODS: We retrospectively studied patients with complex congenital heart disease (CHD) who were prescribed sacubitril/valsartan to treat HF. Data collected includes New York Heart Association (NYHA) classification, laboratory data, vital signs, and imaging by echocardiogram.

RESULTS: Eight ACHD patients with HF with a median age of 40.6 [IQR 33.3-52.8] years were treated with sacubitril/valsartan for a median duration of 269 [IQR 102-473] days. Five (62.5%) had a systemic right ventricle and 3 (37.5%) had single ventricle anatomy. Five (62.5%) had severe systemic systolic ventricular dysfunction. At time of last follow-up, 3 (37.5%) patients at NYHA III had no change in functional class, 4 (50%) patients at NYHA IV improved to NYHA II. One patient has not yet returned for follow-up to evaluate functional status. No patients were noted to have a substantial change in renal function or potassium levels. Median brain natriuretic peptide values pre and post- sacubitril/valsartan were 2517 pg/mL and 1040 pg/mL, respectively. Median mean arterial pressures pre and post-sacubitril/valsartan were 83 mmHg and 77 mmHg, respectively. One (12.5%) patient required dose reduction due to hypotension. At time of last follow-up, 2 (25%) achieved 97 mg/100 mg dose of sacubitril/valsartan. No patients experienced symptoms related to angioedema.

CONCLUSION: In a small cohort of adults with complex CHD and HF, sacubitril/valsartan was well-tolerated, with some patients experiencing improvement in NYHA class. More studies and a registry of outcomes of sacubitril/valsartan use in ACHD are needed.

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