Single-Center Experience with Sacubitril/Valsartan in Patients with Congenital Heart Disease

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Abstract
Heart failure is a significant cause of morbidity and mortality in patients with congenital heart disease (CHD). While clinical guidelines for acquired cardiovascular diseases exist, evidence-based therapies for heart failure in CHD are lacking. Angiotensin II receptor blocker and neprilysin inhibitor (ARNI) therapy has shown efficacy in adults with heart failure, reducing cardiovascular mortality and hospitalizations, but its use in pediatric CHD patients remains underexplored. This study aimed to evaluate the safety and efficacy of Sacubitril/Valsartan (ARNI) in pediatric and young adult patients with CHD. We conducted a retrospective chart review of 29 patients who received ARNI therapy between August 2021 and December 2023. The patients’ age ranged from 5.3 months to 22.8 years. Fifteen of the 29 patients (52%) had single ventricle CHD. The median time for follow-up since ARNI initiation was 8 months (range 6 days–2.4 years). Twelve (41%) patients experienced hypotension which necessitated dose adjustments, temporary withholding of the medication, or discontinuation. Additionally, 2 patients developed acute kidney injury. The medication had to be discontinued in 7 patients (24%) due to hypotension (3 or 10%), AKI (2 or 7%) and progression of heart failure needing advanced cardiac therapies (2 or 7%). A comparative analysis of clinical and laboratory data before and after ARNI therapy revealed a significant reduction in systolic blood pressure ( p = 0.01), as well as increases in serum creatinine and potassium levels ( p = 0.02 and p = 0.03, respectively). Additionally, there was a trend toward improvement in ventricular systolic function observed on echocardiogram after ARNI therapy; however, this was in the context of patients receiving concomitant other oral heart failure medications. Our findings highlight the need for careful monitoring and individualized management of ARNI therapy in pediatric CHD patients. Larger, well-designed trials are essential to establish clear treatment guidelines and optimize the use of ARNI therapy in this population.

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