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<!DOCTYPE html> <html > <head> <meta charset="utf-8"> <meta rel="search" type="application/opensearchdescription+xml" href="/open_search.xml" title="Academia.edu"> <meta content="width=device-width, initial-scale=1" name="viewport"> <meta name="google-site-verification" content="bKJMBZA7E43xhDOopFZkssMMkBRjvYERV-NaN4R6mrs"> <meta name="csrf-param" content="authenticity_token" /> <meta name="csrf-token" content="A4tDYiEsIwjyp4tKQTk5tC8Uwa4i5GDHB3RKkNlIha0RbOthsTp0UvCWRLRg7wKu4Bef+eaVpYoISm5xBMm9aA==" /> <meta name="twitter:card" content="summary" /> <meta name="twitter:url" content="https://www.academia.edu/81898967/Beyond_a_Broken_Heart_Circulatory_Dysfunction_in_the_Failing_Fontan" /> <meta name="twitter:title" content="Beyond a Broken Heart: Circulatory Dysfunction in the Failing Fontan" /> <meta name="twitter:description" content="The role of ventricular dysfunction in late morbidity and mortality of univentricular hearts has been described previously. However, a significant proportion of adult Fontan patients who die or require heart transplantation do so with preserved" /> <meta name="twitter:image" content="http://a.academia-assets.com/images/twitter-card.jpeg" /> <meta property="fb:app_id" content="2369844204" /> <meta property="og:type" content="article" /> <meta property="og:url" content="https://www.academia.edu/81898967/Beyond_a_Broken_Heart_Circulatory_Dysfunction_in_the_Failing_Fontan" /> <meta property="og:title" content="Beyond a Broken Heart: Circulatory Dysfunction in the Failing Fontan" /> <meta property="og:image" content="http://a.academia-assets.com/images/open-graph-icons/fb-paper.gif" /> <meta property="og:description" content="The role of ventricular dysfunction in late morbidity and mortality of univentricular hearts has been described previously. However, a significant proportion of adult Fontan patients who die or require heart transplantation do so with preserved" /> <meta property="article:author" content="https://independent.academia.edu/AltonFarris" /> <meta name="description" content="The role of ventricular dysfunction in late morbidity and mortality of univentricular hearts has been described previously. However, a significant proportion of adult Fontan patients who die or require heart transplantation do so with preserved" /> <meta name="robots" content="noindex" /> <title>Beyond a Broken Heart: Circulatory Dysfunction in the Failing Fontan | Alton Farris - Academia.edu</title> <link rel="canonical" href="https://www.academia.edu/81898967/Beyond_a_Broken_Heart_Circulatory_Dysfunction_in_the_Failing_Fontan" /> <script async src="https://www.googletagmanager.com/gtag/js?id=G-5VKX33P2DS"></script> <script> window.dataLayer = window.dataLayer || []; function gtag(){dataLayer.push(arguments);} gtag('js', new Date()); gtag('config', 'G-5VKX33P2DS', { cookie_domain: 'academia.edu', send_page_view: false, }); gtag('event', 'page_view', { 'controller': "single_work", 'action': "show", 'controller_action': 'single_work#show', 'logged_in': 'false', 'edge': 'unknown', // Send nil if there is no A/B test bucket, in case some records get logged // with missing data - that way we can distinguish between the two cases. // ab_test_bucket should be of the form <ab_test_name>:<bucket> 'ab_test_bucket': null, }) </script> <script> var $controller_name = 'single_work'; var $action_name = "show"; var $rails_env = 'production'; var $app_rev = '10e3dae8ffd20c8f5a4f7002303d92ad374c73ee'; var $domain = 'academia.edu'; var $app_host = "academia.edu"; var $asset_host = "academia-assets.com"; var $start_time = new Date().getTime(); var $recaptcha_key = "6LdxlRMTAAAAADnu_zyLhLg0YF9uACwz78shpjJB"; var $recaptcha_invisible_key = "6Lf3KHUUAAAAACggoMpmGJdQDtiyrjVlvGJ6BbAj"; var $disableClientRecordHit = false; </script> <script> window.require = { config: function() { return function() {} } } </script> <script> window.Aedu = window.Aedu || {}; window.Aedu.hit_data = null; window.Aedu.serverRenderTime = new Date(1733263183000); window.Aedu.timeDifference = new Date().getTime() - 1733263183000; </script> <script type="application/ld+json">{"@context":"https://schema.org","@type":"ScholarlyArticle","abstract":"The role of ventricular dysfunction in late morbidity and mortality of univentricular hearts has been described previously. However, a significant proportion of adult Fontan patients who die or require heart transplantation do so with preserved ventricular function. The clinical deterioration in patients who have undergone Fontan palliation requires a broader view of circulatory dysfunction, one that takes into account the complex interaction of regulatory systems affecting hepatic, renal, and pulmonary blood flow, in addition to cardiac function. This review focuses primarily on the pathophysiology of multiple organ involvement in this circulatory dysfunction, with particular focus on the consequences of hepatic dysfunction and portal hypertension. The authors discuss hepatic perfusion, both in health and disease, and review the current understanding of liver histopathology and liver disease in adult Fontan patients and similar clinicopathologic states. They compare and contrast features of postsinusoidal portal hypertension with more typical adult cirrhotic disease. Finally, they delineate the related effects of portal hypertensive physiology on the systemic and pulmonary vasculature, the kidney, and the heart itself and discuss how these changes affect the care of the adult Fontan patient.","author":[{"@context":"https://schema.org","@type":"Person","name":"Alton Farris"}],"contributor":[],"dateCreated":"2022-06-20","datePublished":"2014-01-01","headline":"Beyond a Broken Heart: Circulatory Dysfunction in the Failing Fontan","image":"https://a.academia-assets.com/images/blank-paper.jpg","inLanguage":"en","keywords":["Global Health","Medicine","Pediatric Cardiology","Congenital Heart Defects","Humans","Portal hypertension","Hemodynamics","Incidence","Adult","Survival Rate","Heart Ventricles","Treatment Failure","Fontan procedure","Cardiovascular medicine and haematology"],"publication":"Pediatric Cardiology","publisher":{"@context":"https://schema.org","@type":"Organization","name":"Springer Science and Business Media 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window.loswp.willEdgeCache = false; window.loswp.work = {"work":{"id":81898967,"created_at":"2022-06-20T05:59:10.853-07:00","from_world_paper_id":208880609,"updated_at":"2022-07-04T09:30:37.969-07:00","_data":{"abstract":"The role of ventricular dysfunction in late morbidity and mortality of univentricular hearts has been described previously. However, a significant proportion of adult Fontan patients who die or require heart transplantation do so with preserved ventricular function. The clinical deterioration in patients who have undergone Fontan palliation requires a broader view of circulatory dysfunction, one that takes into account the complex interaction of regulatory systems affecting hepatic, renal, and pulmonary blood flow, in addition to cardiac function. This review focuses primarily on the pathophysiology of multiple organ involvement in this circulatory dysfunction, with particular focus on the consequences of hepatic dysfunction and portal hypertension. The authors discuss hepatic perfusion, both in health and disease, and review the current understanding of liver histopathology and liver disease in adult Fontan patients and similar clinicopathologic states. They compare and contrast features of postsinusoidal portal hypertension with more typical adult cirrhotic disease. Finally, they delineate the related effects of portal hypertensive physiology on the systemic and pulmonary vasculature, the kidney, and the heart itself and discuss how these changes affect the care of the adult Fontan patient.","publisher":"Springer Science and Business Media LLC","publication_date":"2014,,","publication_name":"Pediatric Cardiology"},"document_type":"paper","pre_hit_view_count_baseline":null,"quality":null,"language":"en","title":"Beyond a Broken Heart: Circulatory Dysfunction in the Failing Fontan","broadcastable":false,"draft":null,"has_indexable_attachment":false,"indexable":true}}["work"]; window.loswp.workCoauthors = [44516105]; window.loswp.locale = "en"; window.loswp.countryCode = "SG"; window.loswp.cwvAbTestBucket = ""; window.loswp.designVariant = "grid"; window.loswp.fullPageMobileSutdModalVariant = "control"; window.loswp.useOptimizedScribd4genScript = false; window.loswp.appleClientId = 'edu.academia.applesignon';</script><script defer="" src="https://accounts.google.com/gsi/client"></script><div class="loswp-grid--container"><div data-auto_select="false" data-client_id="331998490334-rsn3chp12mbkiqhl6e7lu2q0mlbu0f1b" data-landing_url="https://www.academia.edu/81898967/Beyond_a_Broken_Heart_Circulatory_Dysfunction_in_the_Failing_Fontan" data-login_uri="https://www.academia.edu/registrations/google_one_tap" data-moment_callback="onGoogleOneTapEvent" id="g_id_onload"></div><div class="above-fold js-swp-splash-above-fold"><div class="work-card--container js-swp-control-work-card" data-entity-id="81898967"><div class="work-cover--wrapper"><div class="work-cover--container"><div class="work-cover--no-attachment-container js-swp-splash-paper-cover"><div class="work-cover--file-icon-wrapper"><img alt="paper cover icon" src="//a.academia-assets.com/images/single_work_splash/adobe.icon.svg" /></div><div class="work-cover--title js-swp-splash-paper-cover-page-title">Beyond a Broken Heart: Circulatory Dysfunction in the Failing Fontan</div><br /><div style="margin-top: 170px"><button class="work-cover--request-pdf-button js-request-pdf-button"><svg style="width: 14px; margin-right: 8px;" aria-hidden="true" focusable="false" data-prefix="fas" data-icon="envelope" class="svg-inline--fa fa-envelope fa-w-16" role="img" xmlns="http://www.w3.org/2000/svg" viewBox="0 0 512 512"><path fill="currentColor" d="M502.3 190.8c3.9-3.1 9.7-.2 9.7 4.7V400c0 26.5-21.5 48-48 48H48c-26.5 0-48-21.5-48-48V195.6c0-5 5.7-7.8 9.7-4.7 22.4 17.4 52.1 39.5 154.1 113.6 21.1 15.4 56.7 47.8 92.2 47.6 35.7.3 72-32.8 92.3-47.6 102-74.1 131.6-96.3 154-113.7zM256 320c23.2.4 56.6-29.2 73.4-41.4 132.7-96.3 142.8-104.7 173.4-128.7 5.8-4.5 9.2-11.5 9.2-18.9v-19c0-26.5-21.5-48-48-48H48C21.5 64 0 85.5 0 112v19c0 7.4 3.4 14.3 9.2 18.9 30.6 23.9 40.7 32.4 173.4 128.7 16.8 12.2 50.2 41.8 73.4 41.4z"></path></svg>Request PDF</button></div></div></div></div><div class="work-card--content"><h1 class="work-card--title">Beyond a Broken Heart: Circulatory Dysfunction in the Failing Fontan</h1><div class="work-card--pub-info">Pediatric Cardiology, 2014</div><a class="js-profile-link work-card--single-author" data-has-card-for-user="44516105" href="https://independent.academia.edu/AltonFarris?swp=tc-au-81898967"><div class="work-card--single-author-photo"><img class="Avatar--circle s65 Avatar--xxxs" shape="circle" border="0" alt="" src="//a.academia-assets.com/images/s65_no_pic.png" /></div><div class="work-card--author-name-wrapper" style="max-width: 125px; font-weight: 500; color: #4b4b4b;"><div class="u-textTruncate"><span itemprop="author">Alton Farris</span></div></div></a><script data-card-contents-for-user="44516105" type="text/json">{"id":44516105,"first_name":"Alton","middle_initials":null,"last_name":"Farris","page_name":"AltonFarris","domain_name":"independent","created_at":"2016-03-05T12:30:06.574-08:00","display_name":"Alton Farris","url":"https://independent.academia.edu/AltonFarris","photo":"/images/s65_no_pic.png","has_photo":false,"interests":[{"id":24865,"name":"Robotic Surgery","url":"https://www.academia.edu/Documents/in/Robotic_Surgery"},{"id":39851,"name":"Cardiac Surgery","url":"https://www.academia.edu/Documents/in/Cardiac_Surgery"},{"id":253560,"name":"Newborn Infant","url":"https://www.academia.edu/Documents/in/Newborn_Infant"},{"id":64933,"name":"Child","url":"https://www.academia.edu/Documents/in/Child"}]}</script><div class="work-card--no-attachment-details"><div style="font-weight: 700; font-size: 14px;">Abstract</div><div class="work-card--abstract js-swp-splash-abstract">The role of ventricular dysfunction in late morbidity and mortality of univentricular hearts has been described previously. However, a significant proportion of adult Fontan patients who die or require heart transplantation do so with preserved ventricular function. The clinical deterioration in patients who have undergone Fontan palliation requires a broader view of circulatory dysfunction, one that takes into account the complex interaction of regulatory systems affecting hepatic, renal, and pulmonary blood flow, in addition to cardiac function. This review focuses primarily on the pathophysiology of multiple organ involvement in this circulatory dysfunction, with particular focus on the consequences of hepatic dysfunction and portal hypertension. The authors discuss hepatic perfusion, both in health and disease, and review the current understanding of liver histopathology and liver disease in adult Fontan patients and similar clinicopathologic states. They compare and contrast features of postsinusoidal portal hypertension with more typical adult cirrhotic disease. Finally, they delineate the related effects of portal hypertensive physiology on the systemic and pulmonary vasculature, the kidney, and the heart itself and discuss how these changes affect the care of the adult Fontan patient.</div></div><div class="request-upload--container"><p class="request-upload--title">Alton Farris hasn't uploaded this paper.</p><div class="request-upload--info-text"><svg aria-hidden="true" focusable="false" data-prefix="fas" data-icon="info-circle" class="request-upload--info-icon svg-inline--fa fa-info-circle fa-w-16" role="img" xmlns="http://www.w3.org/2000/svg" viewBox="0 0 512 512"><path fill="currentColor" d="M256 8C119.043 8 8 119.083 8 256c0 136.997 111.043 248 248 248s248-111.003 248-248C504 119.083 392.957 8 256 8zm0 110c23.196 0 42 18.804 42 42s-18.804 42-42 42-42-18.804-42-42 18.804-42 42-42zm56 254c0 6.627-5.373 12-12 12h-88c-6.627 0-12-5.373-12-12v-24c0-6.627 5.373-12 12-12h12v-64h-12c-6.627 0-12-5.373-12-12v-24c0-6.627 5.373-12 12-12h64c6.627 0 12 5.373 12 12v100h12c6.627 0 12 5.373 12 12v24z"></path></svg><p class="no-margin hide-on-small-mobile">Let Alton know you want this paper to be uploaded.</p><p class="no-margin hide-above-small-mobile">Ask for this paper to be uploaded.</p></div><button class="work-cover--request-pdf-button small js-request-pdf-button hide-on-desktop"><svg style="width: 14px; 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