{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,10]],"date-time":"2026-08-10T12:34:10Z","timestamp":1786365250853,"version":"build-2736575974"},"reference-count":30,"publisher":"Cambridge University Press (CUP)","issue":"6","license":[{"start":{"date-parts":[[2026,6,23]],"date-time":"2026-06-23T00:00:00Z","timestamp":1782172800000},"content-version":"unspecified","delay-in-days":22,"URL":"https:\/\/www.cambridge.org\/core\/terms"}],"content-domain":{"domain":["cambridge.org"],"crossmark-restriction":true},"short-container-title":["Cardiol Young"],"published-print":{"date-parts":[[2026,6]]},"abstract":"Abstract<\/jats:title>\n \n Background:<\/jats:title>\n Sacubitril\/valsartan is widely used in adults with heart failure with reduced ejection fraction, but paediatric experience, especially across ventricular dysfunction phenotypes, remains limited.<\/jats:p>\n <\/jats:sec>\n \n Aim:<\/jats:title>\n To evaluate the efficacy and tolerability of sacubitril\/valsartan in children with cardiomyopathy and compare outcomes in patients\u00a0with predominantly right or left ventricular dysfunction.<\/jats:p>\n <\/jats:sec>\n \n Methods:<\/jats:title>\n This single-centre retrospective study included 33 patients aged 1 month to 18 years who received sacubitril\/valsartan\u00a0between October 2020 and October 2024. Diagnoses included arrhythmogenic right ventricular cardiomyopathy, left ventricular non-compaction, and dilated cardiomyopathy. Patients were stratified by predominant ventricular dysfunction. Echocardiographic parameters, N-terminal pro B-type natriuretic peptide, six-minute walk test, NYHA class, blood pressure, creatinine, and potassium were recorded at baseline and at 1, 3, and 6 months, and at final follow-up. Clinical outcomes included rehospitalisation, death, and heart transplantation or listing.<\/jats:p>\n <\/jats:sec>\n \n Results:<\/jats:title>\n \n The cohort included 21 male patients (63.6%), and the median age was 8.6 years. Arrhythmogenic right ventricular cardiomyopathy was present in 45.5%, left ventricular non-compaction in 15.2%, and dilated cardiomyopathy in 39.4%. Left ventricular ejection fraction >45% increased from 39.4% to 72.7%. Mean N-terminal pro B-type natriuretic peptide decreased from 6309 to 3137 pg\/mL (\n p<\/jats:italic>\n < 0.001). NYHA class improved or remained stable in 93.3% of patients, with greater improvement in left ventricular dysfunction. During a median 17.3-months follow-up, rehospitalisation occurred in 42.4%,\u00a0and 5 patients died or underwent heart transplantation\/listing. Hypotension occurred in 18.2% but was manageable.\n <\/jats:p>\n <\/jats:sec>\n \n Conclusion:<\/jats:title>\n Sacubitril\/valsartan was generally well tolerated and associated with improved ventricular function, biomarkers, and functional status in paediatric cardiomyopathy, particularly in left ventricular dysfunction.<\/jats:p>\n <\/jats:sec>","DOI":"10.1017\/s1047951126113742","type":"journal-article","created":{"date-parts":[[2026,6,23]],"date-time":"2026-06-23T10:53:29Z","timestamp":1782212009000},"page":"1187-1196","update-policy":"https:\/\/doi.org\/10.1017\/policypage","source":"Crossref","is-referenced-by-count":1,"title":["Sacubitril\/valsartan in paediatric cardiomyopathy: a retrospective evaluation of ventricular response and clinical outcomes"],"prefix":"10.1017","volume":"36","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-1708-4007","authenticated-orcid":false,"given":"Aslihan","family":"Karaman","sequence":"first","affiliation":[{"name":"University of Health Sciences, Istanbul Mehmet Akif Ersoy Thoracic and Cardiovascular Surgery Training and Research Hospital"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4530-2948","authenticated-orcid":false,"given":"Buse","family":"G\u00fcnyel","sequence":"additional","affiliation":[{"name":"University of Health Sciences, Istanbul Mehmet Akif Ersoy Thoracic and Cardiovascular Surgery Training and Research Hospital"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7521-2313","authenticated-orcid":false,"given":"Bahar","family":"Caran","sequence":"additional","affiliation":[{"name":"University of Health Sciences, Istanbul Mehmet Akif Ersoy Thoracic and Cardiovascular Surgery Training and Research Hospital"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Erman","family":"Cilsal","sequence":"additional","affiliation":[{"name":"University of Health Sciences, Istanbul Mehmet Akif Ersoy Thoracic and Cardiovascular Surgery Training and Research Hospital"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fatma","family":"Sevinc Sengul","sequence":"additional","affiliation":[{"name":"University of Health Sciences, Istanbul Mehmet Akif Ersoy Thoracic and Cardiovascular Surgery Training and Research Hospital"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Pelin","family":"Ayy\u0131ld\u0131z","sequence":"additional","affiliation":[{"name":"University of Health Sciences, Istanbul Mehmet Akif Ersoy Thoracic and Cardiovascular Surgery Training and Research Hospital"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3812-2081","authenticated-orcid":false,"given":"Selman","family":"Gokalp","sequence":"additional","affiliation":[{"name":"University of Health Sciences, Istanbul Mehmet Akif Ersoy Thoracic and Cardiovascular Surgery Training and Research Hospital"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1924-9036","authenticated-orcid":false,"given":"Alper","family":"Guzeltas","sequence":"additional","affiliation":[{"name":"University of Health Sciences, Istanbul Mehmet Akif Ersoy Thoracic and Cardiovascular Surgery Training and Research Hospital"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7319-4139","authenticated-orcid":false,"given":"Sezen","family":"Atik Ugan","sequence":"additional","affiliation":[{"name":"University of Health Sciences, Istanbul Mehmet Akif Ersoy Thoracic and Cardiovascular Surgery Training and Research Hospital"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"56","published-online":{"date-parts":[[2026,6,23]]},"reference":[{"key":"S1047951126113742_ref3","unstructured":"3. U.S. Food and Drug Administration (FDA). Approval Letter for Entresto (sacubitril\/valsartan), Pediatric Supplement. March 2019. Link to Letter (PDF)."},{"key":"S1047951126113742_ref14","first-page":"704","article-title":"Sacubitril\/valsartan in pediatric patients with heart failure: clinical experience in a single center","volume":"43","author":"Tan","year":"2021","journal-title":"Clin Ther"},{"key":"S1047951126113742_ref19","first-page":"905","article-title":"PANORAMA-HF part 2: efficacy and safety of sacubitril\/valsartan in pediatric heart failure","volume":"79","author":"Giglia","year":"2022","journal-title":"J Am Coll Cardiol"},{"key":"S1047951126113742_ref27","first-page":"733","article-title":"Improved outcomes in children with cardiomyopathy over time","volume":"140","author":"Singh","year":"2019","journal-title":"Circulation"},{"key":"S1047951126113742_ref26","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa021715"},{"key":"S1047951126113742_ref12","first-page":"1079","article-title":"Real-world experience with sacubitril\/valsartan in pediatric heart failure","volume":"29","author":"Chen","year":"2025","journal-title":"Cardiol Young"},{"key":"S1047951126113742_ref4","first-page":"e003396","article-title":"Pediatric heart failure: current state and future directions","volume":"10","author":"Kantor","year":"2017","journal-title":"Circ Heart Fail"},{"key":"S1047951126113742_ref6","doi-asserted-by":"publisher","DOI":"10.1016\/j.jacc.2021.12.012"},{"key":"S1047951126113742_ref2","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1409077"},{"key":"S1047951126113742_ref23","first-page":"1208","article-title":"Mechanical circulatory support as bridge to pediatric heart transplantation: a multicenter study","volume":"162","author":"Morales","year":"2021","journal-title":"J Thorac Cardiovasc Surg"},{"key":"S1047951126113742_ref17","first-page":"E9","article-title":"Natriuretic peptides and cardio-renal disease","volume":"18","author":"Volpe","year":"2016","journal-title":"Eur Heart J Suppl"},{"key":"S1047951126113742_ref13","first-page":"1199","article-title":"Sacubitril\/valsartan in children with dilated cardiomyopathy: a case series","volume":"41","author":"Bruns","year":"2020","journal-title":"Pediatr Cardiol"},{"key":"S1047951126113742_ref18","first-page":"H916","article-title":"Effects of sacubitril\/valsartan on cardiac structure and function in animal models","volume":"318","author":"Sharifi Kia","year":"2020","journal-title":"Am J Physiol Heart Circ Physiol"},{"key":"S1047951126113742_ref22","first-page":"375","article-title":"Hemodynamic effects of sacubitril\u2013valsartan in heart failure with preserved ejection fraction","volume":"72","author":"Reddy","year":"2018","journal-title":"J Am Coll Cardiol"},{"key":"S1047951126113742_ref15","first-page":"673","article-title":"Sacubitril\/valsartan:Evolving evidence in HFrEF","volume":"79","author":"Docherty","year":"2022","journal-title":"J Am Coll Cardiol"},{"key":"S1047951126113742_ref7","doi-asserted-by":"publisher","DOI":"10.1093\/eurheartj\/ehad194"},{"key":"S1047951126113742_ref28","first-page":"1190","article-title":"Prevalence, morbidity, and mortality of heart failure\u2013related hospitalizations in children","volume":"59","author":"Rossano","year":"2012","journal-title":"J Am Coll Cardiol"},{"key":"S1047951126113742_ref5","first-page":"725","article-title":"Pharmacologic strategies in pediatric heart failure: extrapolation and evidence","volume":"8","author":"Fudim","year":"2020","journal-title":"JACC Heart Fail"},{"key":"S1047951126113742_ref9","first-page":"879","article-title":"Reference intervals for NT-proBNP in healthy pediatric population: data from a multicenter study","volume":"52","author":"Cantinotti","year":"2014","journal-title":"Clin Chem Lab Med"},{"key":"S1047951126113742_ref29","first-page":"1223","article-title":"Sacubitril\/valsartan modulates molecular remodeling in pediatric heart failure","volume":"34","author":"Miyamoto","year":"2013","journal-title":"Pediatr Cardiol"},{"key":"S1047951126113742_ref11","first-page":"905","article-title":"Sacubitril\/Valsartan in pediatric heart failure: a multicenter dose-finding and PK\/PD study (PANORAMA-HF)","volume":"79","author":"Giglia","year":"2022","journal-title":"J Am Coll Cardiol"},{"key":"S1047951126113742_ref21","first-page":"2774","article-title":"Sacubitril\/valsartan alleviates right ventricular remodeling in pulmonary arterial hypertension","volume":"10","author":"Zhao","year":"2023","journal-title":"ESC Heart Fail"},{"key":"S1047951126113742_ref30","first-page":"917","article-title":"Nutritional outcomes following heart failure therapy in children","volume":"41","author":"Alvarez","year":"2022","journal-title":"J Heart Lung Transplant"},{"key":"S1047951126113742_ref16","first-page":"688","article-title":"Soluble neprilysin and prognosis in heart failure with reduced ejection fraction","volume":"22","author":"Bayes-Genis","year":"2020","journal-title":"Eur J Heart Fail"},{"key":"S1047951126113742_ref24","first-page":"317","article-title":"Sacubitril\/valsartan in children with heart failure: a systematic review","volume":"10","author":"Wang","year":"2023","journal-title":"ESC Heart Fail"},{"key":"S1047951126113742_ref1","first-page":"e895","article-title":"AHA\/ACC\/HFSA guideline for the management of heart failure","volume":"145","author":"Heidenreich","year":"2022","journal-title":"Circulation"},{"key":"S1047951126113742_ref8","doi-asserted-by":"publisher","DOI":"10.1016\/j.echo.2010.03.019"},{"key":"S1047951126113742_ref20","first-page":"1230","article-title":"Role of ARNI in right heart failure secondary to pulmonary hypertension","volume":"10","author":"Jain","year":"2023","journal-title":"ESC Heart Fail"},{"key":"S1047951126113742_ref10","doi-asserted-by":"publisher","DOI":"10.1016\/j.jchf.2019.02.015"},{"key":"S1047951126113742_ref25","first-page":"H1041","article-title":"Safety and efficacy of ARNI in infants with cardiomyopathy","volume":"323","author":"Chen","year":"2022","journal-title":"Am J Physiol Heart Circ Physiol"}],"updated-by":[{"DOI":"10.1017\/s1047951126123749","type":"correction","label":"Correction","source":"publisher","updated":{"date-parts":[[2026,6,23]],"date-time":"2026-06-23T00:00:00Z","timestamp":1782172800000}}],"container-title":["Cardiology in the Young"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.cambridge.org\/core\/services\/aop-cambridge-core\/content\/view\/S1047951126113742","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,8,10]],"date-time":"2026-08-10T12:24:55Z","timestamp":1786364695000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.cambridge.org\/core\/product\/identifier\/S1047951126113742\/type\/journal_article"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,6]]},"references-count":30,"journal-issue":{"issue":"6","published-print":{"date-parts":[[2026,6]]}},"alternative-id":["S1047951126113742"],"URL":"https:\/\/doi.org\/10.1017\/s1047951126113742","relation":{},"ISSN":["1047-9511","1467-1107"],"issn-type":[{"value":"1047-9511","type":"print"},{"value":"1467-1107","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,6]]},"assertion":[{"value":"\u00a9 The Author(s), 2026. Published by Cambridge University Press","name":"copyright","label":"Copyright","group":{"name":"copyright_and_licensing","label":"Copyright and Licensing"}}]}}