{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,21]],"date-time":"2026-08-21T13:02:09Z","timestamp":1787317329264,"version":"build-2736575974"},"reference-count":105,"publisher":"MDPI AG","issue":"5","license":[{"start":{"date-parts":[[2022,5,12]],"date-time":"2022-05-12T00:00:00Z","timestamp":1652313600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"National Institutes of Health","award":["NIH\/NHLBI HL 156670"],"award-info":[{"award-number":["NIH\/NHLBI HL 156670"]}]},{"name":"National Institutes of Health","award":["1HL156670-01S1"],"award-info":[{"award-number":["1HL156670-01S1"]}]},{"name":"Jack Cooper Millisor Chair in Heart Disease","award":["NIH\/NHLBI HL 156670"],"award-info":[{"award-number":["NIH\/NHLBI HL 156670"]}]},{"name":"Jack Cooper Millisor Chair in Heart Disease","award":["1HL156670-01S1"],"award-info":[{"award-number":["1HL156670-01S1"]}]},{"name":"Additional Ventures Foundation","award":["NIH\/NHLBI HL 156670"],"award-info":[{"award-number":["NIH\/NHLBI HL 156670"]}]},{"name":"Additional Ventures Foundation","award":["1HL156670-01S1"],"award-info":[{"award-number":["1HL156670-01S1"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["JCDD"],"abstract":"<jats:p>Significant surgical and medical advances over the past several decades have resulted in a growing number of infants and children surviving with hypoplastic left heart syndrome (HLHS) and other congenital heart defects associated with a single systemic right ventricle (RV). However, cardiac dysfunction and ultimately heart failure (HF) remain the most common cause of death and indication for transplantation in this population. Moreover, while early recognition and treatment of single ventricle-related complications are essential to improving outcomes, there are no proven therapeutic strategies for single systemic RV HF in the pediatric population. Importantly, prototypical adult HF therapies have been relatively ineffective in mitigating the need for cardiac transplantation in HLHS, likely due to several unique attributes of the failing HLHS myocardium. Here, we discuss the most commonly used medical therapies for the treatment of HF symptoms in HLHS and other single systemic RV patients. Additionally, we provide an overview of potential novel therapies for systemic ventricular failure in the HLHS and related populations based on fundamental science, pre-clinical, clinical, and observational studies in the current literature.<\/jats:p>","DOI":"10.3390\/jcdd9050152","type":"journal-article","created":{"date-parts":[[2022,5,12]],"date-time":"2022-05-12T05:10:32Z","timestamp":1652332232000},"page":"152","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["Medical Therapies for Heart Failure in Hypoplastic Left Heart Syndrome"],"prefix":"10.3390","volume":"9","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-2833-4697","authenticated-orcid":false,"given":"Angela N.","family":"Baybayon-Grandgeorge","sequence":"first","affiliation":[{"name":"Department of Medicine, Division of Cardiology, University of Colorado Anschutz Medical Campus, Aurora, CO 80045, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6988-7899","authenticated-orcid":false,"given":"Ashley E.","family":"Pietra","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Section of Cardiology, University of Colorado Anschutz Medical Campus, Children\u2019s Hospital Colorado, Aurora, CO 80045, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shelley D.","family":"Miyamoto","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Section of Cardiology, University of Colorado Anschutz Medical Campus, Children\u2019s Hospital Colorado, Aurora, CO 80045, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2431-2212","authenticated-orcid":false,"given":"Anastacia M.","family":"Garcia","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Section of Cardiology, University of Colorado Anschutz Medical Campus, Children\u2019s Hospital Colorado, Aurora, CO 80045, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2022,5,12]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"e20593","DOI":"10.1097\/MD.0000000000020593","article-title":"Incidence and Mortality Trend of Congenital Heart Disease at the Global, Regional, and National Level, 1990-2017","volume":"99","author":"Wu","year":"2020","journal-title":"Medicine"},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"2241","DOI":"10.1016\/j.jacc.2011.08.025","article-title":"Birth Prevalence of Congenital Heart Disease Worldwide: A Systematic Review and Meta-Analysis","volume":"58","author":"Konings","year":"2011","journal-title":"J. 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