{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,23]],"date-time":"2026-06-23T22:42:42Z","timestamp":1782254562642,"version":"3.54.5"},"reference-count":30,"publisher":"Cambridge University Press (CUP)","issue":"10","license":[{"start":{"date-parts":[[2022,6,22]],"date-time":"2022-06-22T00:00:00Z","timestamp":1655856000000},"content-version":"unspecified","delay-in-days":0,"URL":"https:\/\/www.cambridge.org\/core\/terms"}],"content-domain":{"domain":["cambridge.org"],"crossmark-restriction":true},"short-container-title":["Cardiol Young"],"published-print":{"date-parts":[[2022,10]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec id=\"S1047951122001433_as1\"><jats:title>Objectives:<\/jats:title><jats:p>Management of \u201cfailing\u201d and \u201cfailed\u201d Fontan circulation, particularly the indications, timing, and type of re-intervention, currently remains nebulous. Factors contributing to pathogenesis and mortality following Fontan procedure differ between children and adults.<\/jats:p><\/jats:sec><jats:sec id=\"S1047951122001433_as2\"><jats:title>Methods:<\/jats:title><jats:p>Since organ systems in individual patients are affected differently, we searched the extant literature for a \u201cfailing\u201d and \u201cfailed\u201d Fontan reviewing the clinical phenotypes, diagnostic modalities, pharmacological, non-pharmacological, and surgical techniques employed, and their outcomes.<\/jats:p><\/jats:sec><jats:sec id=\"S1047951122001433_as3\"><jats:title>Results:<\/jats:title><jats:p>A total of 410 investigations were synthesised. Although proper candidate selection, thoughtful technical modifications, timely deployment of mechanical support devices, tissue-engineered conduits, and Fontan takedown have decreased the peri-operative mortality from 9 to 15% and 1 to 3% per cent in recent series, pernicious changes in organ function are causing long-term patient attrition. In the setting of a failed Fontan circulation, literature documents three surgical options: Fontan revision, Fontan conversion, or cardiac transplantation. The reported morbidity of 25% and mortality of 8\u201310% among Fontan conversion continue to improve in select institutions. While operative mortality following cardiac transplantation for Fontan failure is 30% higher than for other CHDs, there is no difference in long-term survival with actuarial 10-year survival of around 54%. Mechanical circulatory assistance, stem cells, and tissue-engineered Fontan conduit for destination therapy or as a bridge to transplantation are in infancy for failing Fontan circulation.<\/jats:p><\/jats:sec><jats:sec id=\"S1047951122001433_as4\"><jats:title>Conclusions:<\/jats:title><jats:p>An individualised management strategy according to clinical phenotypes may delay the organ damage in patients with a failing Fontan circulation. At present, cardiac transplantation remains the last stage of palliation with gradually improving outcomes.<\/jats:p><\/jats:sec>","DOI":"10.1017\/s1047951122001433","type":"journal-article","created":{"date-parts":[[2022,6,22]],"date-time":"2022-06-22T07:04:02Z","timestamp":1655881442000},"page":"1554-1563","update-policy":"https:\/\/doi.org\/10.1017\/policypage","source":"Crossref","is-referenced-by-count":12,"title":["Fontan failure: phenotypes, evaluation, management, and future directions"],"prefix":"10.1017","volume":"32","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-1672-1502","authenticated-orcid":false,"given":"Ujjwal Kumar","family":"Chowdhury","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Niwin","family":"George","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lakshmi Kumari","family":"Sankhyan","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Doniparthi","family":"Pradeep","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Chaitanya","family":"Chittimuri","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Abhinavsingh","family":"Chauhan","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6786-8304","authenticated-orcid":false,"given":"Niraj 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