{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,14]],"date-time":"2026-08-14T20:29:16Z","timestamp":1786739356338,"version":"build-2736575974"},"reference-count":47,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"2","license":[{"start":{"date-parts":[[2026,3,1]],"date-time":"2026-03-01T00:00:00Z","timestamp":1772323200000},"content-version":"unspecified","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0"}],"content-domain":{"domain":["lww.com","ovid.com"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2026,3]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:p>Patients with Fontan circulation face progressive multisystem dysfunction driven by venous hypertension, lymphatic failure, and metabolic derangements. This state-of-the-art review synthesizes evidence across hemodynamic, lymphatic, pulmonary vascular, and metabolic domains to outline emerging therapies and a pragmatic care framework. We summarize data on exercise rehabilitation; pulmonary vasodilators; lymphatic imaging\u2013guided interventions; metabolic modulation including bile acid sequestration and SGLT2 inhibition; arrhythmia prevention; mechanical circulatory support; and transplantation, including combined heart\u2013liver strategies. Recognizing limitations of conventional randomized trials in this heterogeneous, small population, we propose adaptive, registry-embedded, and platform designs with harmonized phenotypes and core outcomes. This review aims to bridge mechanistic insight and clinical application, offering clinicians a concise roadmap and researchers a set of testable hypotheses to improve survival, function, and quality of life.<\/jats:p>","DOI":"10.4103\/apc.apc_296_25","type":"journal-article","created":{"date-parts":[[2026,5,26]],"date-time":"2026-05-26T05:00:21Z","timestamp":1779771621000},"page":"205-214","update-policy":"https:\/\/doi.org\/10.1097\/lww.0000000000001000","source":"Crossref","is-referenced-by-count":1,"title":["Emerging therapeutic strategies for the failing Fontan circulation"],"prefix":"10.4103","volume":"19","author":[{"given":"Ashish H.","family":"Shah","sequence":"first","affiliation":[{"name":"Department of Internal Medicine, Max Rady College of Medicine, University of Manitoba, Winnipeg, MB, Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"276","published-online":{"date-parts":[[2026,5,25]]},"reference":[{"key":"R1-20260527","doi-asserted-by":"crossref","first-page":"e234","DOI":"10.1161\/CIR.0000000000000696","article-title":"Evaluation and management of the child and adult with Fontan circulation: A scientific statement from the American Heart Association","volume":"140","author":"Rychik","year":"2019","journal-title":"Circulation"},{"key":"R2-20260527","doi-asserted-by":"crossref","first-page":"790","DOI":"10.1016\/j.ijcard.2016.11.121","article-title":"Isolated heart transplant and combined heart-liver transplant in adult congenital heart disease patients:Insights from the united network of organ sharing","volume":"228","author":"Bradley","year":"2017","journal-title":"Int J Cardiol"},{"key":"R3-20260527","doi-asserted-by":"crossref","first-page":"2161","DOI":"10.1016\/j.jacc.2023.03.422","article-title":"Morbidity and mortality in adult Fontan 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