{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,24]],"date-time":"2026-09-24T15:54:04Z","timestamp":1790265244279,"version":"4.1.0"},"reference-count":20,"publisher":"Springer Science and Business Media LLC","issue":"4","license":[{"start":{"date-parts":[[2025,7,4]],"date-time":"2025-07-04T00:00:00Z","timestamp":1751587200000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"},{"start":{"date-parts":[[2025,7,4]],"date-time":"2025-07-04T00:00:00Z","timestamp":1751587200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Pediatr Cardiol"],"published-print":{"date-parts":[[2026,4]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>\n                    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\u2019 age ranged from 5.3\u00a0months to 22.8\u00a0years. Fifteen of the 29 patients (52%) had single ventricle CHD. The median time for follow-up since ARNI initiation was 8\u00a0months (range 6\u00a0days\u20132.4\u00a0years). 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 (\n                    <jats:italic>p<\/jats:italic>\n                    \u2009=\u20090.01), as well as increases in serum creatinine and potassium levels (\n                    <jats:italic>p<\/jats:italic>\n                    \u2009=\u20090.02 and\n                    <jats:italic>p<\/jats:italic>\n                    \u2009=\u20090.03, 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.\n                  <\/jats:p>","DOI":"10.1007\/s00246-025-03938-x","type":"journal-article","created":{"date-parts":[[2025,7,4]],"date-time":"2025-07-04T13:49:32Z","timestamp":1751636972000},"page":"1581-1590","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":4,"title":["Single-Center Experience with Sacubitril\/Valsartan in Patients with Congenital Heart Disease"],"prefix":"10.1007","volume":"47","author":[{"given":"Anusha","family":"Konduri","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ashley","family":"Duimstra","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ray","family":"Lowery","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sunkyung","family":"Yu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ashley","family":"Huebschman","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bronwyn","family":"Crandall","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tiffany","family":"Hunter","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Heang M.","family":"Lim","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Amanda D.","family":"McCormick","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kurt R.","family":"Schumacher","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"David M.","family":"Peng","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2025,7,4]]},"reference":[{"issue":"12","key":"3938_CR1","doi-asserted-by":"publisher","first-page":"982","DOI":"10.1161\/CIRCULATIONAHA.122.061546","volume":"147","author":"N Bergh","year":"2023","unstructured":"Bergh N et al (2023) Risk of heart failure in congenital heart disease: a nationwide register-based cohort study. Circulation 147(12):982\u2013984. https:\/\/doi.org\/10.1161\/CIRCULATIONAHA.122.061546","journal-title":"Circulation"},{"issue":"4","key":"3938_CR2","doi-asserted-by":"publisher","first-page":"569","DOI":"10.1007\/s10741-019-09904-z","volume":"25","author":"SS Vaikunth","year":"2020","unstructured":"Vaikunth SS, Lui GK (2020) Heart failure with reduced and preserved ejection fraction in adult congenital heart disease. Heart Fail Rev 25(4):569\u2013581. https:\/\/doi.org\/10.1007\/s10741-019-09904-z","journal-title":"Heart Fail Rev"},{"issue":"22","key":"3938_CR3","doi-asserted-by":"publisher","first-page":"1756","DOI":"10.1161\/CIRCULATIONAHA.123.066605","volume":"150","author":"R Shaddy","year":"2024","unstructured":"Shaddy R et al (2024) Sacubitril\/valsartan in pediatric heart failure (PANORAMA-HF): a randomized, multicenter, double-blind trial. Circulation 150(22):1756\u20131766. https:\/\/doi.org\/10.1161\/CIRCULATIONAHA.123.066605","journal-title":"Circulation"},{"issue":"18","key":"3938_CR4","doi-asserted-by":"publisher","first-page":"e895","DOI":"10.1161\/CIR.0000000000001063","volume":"145","author":"PA Heidenreich","year":"2022","unstructured":"Heidenreich PA et al (2022) 2022 AHA\/ACC\/HFSA guideline for the management of heart failure: a report of the American college of cardiology\/American heart association joint committee on clinical practice guidelines. Circulation 145(18):e895\u2013e1032. https:\/\/doi.org\/10.1161\/CIR.0000000000001063","journal-title":"Circulation"},{"key":"3938_CR5","doi-asserted-by":"publisher","DOI":"10.3389\/fcvm.2021.754499","volume":"8","author":"D Pascual-Figal","year":"2021","unstructured":"Pascual-Figal D et al (2021) Sacubitril-valsartan, clinical benefits and related mechanisms of action in heart failure with reduced ejection fraction. a review. Front Cardiovasc Med 8:754499. https:\/\/doi.org\/10.3389\/fcvm.2021.754499","journal-title":"Front Cardiovasc Med"},{"key":"3938_CR6","doi-asserted-by":"publisher","unstructured":"Cohn JN, Tognoni G, Valsartan I, Heart Failure Trial (2001) A randomized trial of the angiotensin-receptor blocker valsartan in chronic heart failure. N Engl J Med, 345(23):1667\u20131675. https:\/\/doi.org\/10.1056\/NEJMoa010713","DOI":"10.1056\/NEJMoa010713"},{"issue":"11","key":"3938_CR7","doi-asserted-by":"publisher","first-page":"993","DOI":"10.1056\/NEJMoa1409077","volume":"371","author":"JJ McMurray","year":"2014","unstructured":"McMurray JJ et al (2014) Angiotensin-neprilysin inhibition versus enalapril in heart failure. N Engl J Med 371(11):993\u20131004. https:\/\/doi.org\/10.1056\/NEJMoa1409077","journal-title":"N Engl J Med"},{"issue":"17","key":"3938_CR8","doi-asserted-by":"publisher","first-page":"1609","DOI":"10.1056\/NEJMoa1908655","volume":"381","author":"SD Solomon","year":"2019","unstructured":"Solomon SD et al (2019) Angiotensin\u2013neprilysin inhibition in heart failure with preserved ejection fraction. N Engl J Med 381(17):1609\u20131620. https:\/\/doi.org\/10.1056\/NEJMoa1908655","journal-title":"N Engl J Med"},{"key":"3938_CR9","unstructured":"T. A. C. T. I. O. N. 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Non-financial interests: none.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Conflict of Interest"}},{"value":"This study was reviewed and approved by the University of Michigan Institutional Review Board. As a retrospective chart review, the requirement for informed consent was waived by the IRB in accordance with applicable regulations and institutional guidelines.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Ethical Approval"}}]}}