{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,10]],"date-time":"2026-09-10T02:03:22Z","timestamp":1789005802563,"version":"build-2803163510"},"reference-count":21,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"1","content-domain":{"domain":["www.ahajournals.org"],"crossmark-restriction":true},"short-container-title":["JAHA"],"published-print":{"date-parts":[[2022,1,4]]},"abstract":"<jats:sec xml:lang=\"en\">\n            <jats:title>Background<\/jats:title>\n            <jats:p xml:lang=\"en\">The Fontan circulation is a successful operative strategy for abolishing cyanosis and chronic volume overload in patients with congenital heart disease with single ventricle physiology. \u201cFontan failure\u201d is a major cause of poor quality of life and mortality in these patients. We assessed the number and clinical characteristics of adult patients with Fontan physiology receiving pulmonary arterial hypertension (PAH) therapies across specialist centers in the United Kingdom.<\/jats:p>\n          <\/jats:sec>\n          <jats:sec xml:lang=\"en\">\n            <jats:title>Methods and Results<\/jats:title>\n            <jats:p xml:lang=\"en\">\n              We identified all adult patients with a Fontan\u2010type circulation under active follow\u2010up in 10 specialist congenital heart disease centers in England and Scotland between 2009 and 2019. Patients taking PAH therapies were matched to untreated patients. A survey of experts was also performed. Of 1538 patients with Fontan followed in specialist centers, only 76 (4.9%) received PAH therapies during follow\u2010up. The vast majority (90.8%) were treated with a phosphodiesterase\u20105 inhibitor. In 33% of patients, PAH therapies were started after surgery or during hospital admission. In the matched cohort, treated patients were more likely to be significantly limited, have ascites, have a history of protein\u2010losing enteropathy, or receive loop diuretics (\n              <jats:italic>P<\/jats:italic>\n              &lt;0.0001 for all), also reflecting survey responses indicating that failing Fontan is an important treatment target. After a median of 12\u00a0months (11\u201315\u00a0months), functional class was more likely to improve in the treated group (\n              <jats:italic>P<\/jats:italic>\n              =0.01), with no other changes in clinical parameters or safety issues.\n            <\/jats:p>\n          <\/jats:sec>\n          <jats:sec xml:lang=\"en\">\n            <jats:title>Conclusions<\/jats:title>\n            <jats:p xml:lang=\"en\">PAH therapies are used in adult patients with Fontan circulation followed in specialist centers, targeting individuals with advanced disease or complications. Follow\u2010up suggests stabilization of the clinical status after 12\u00a0months of therapy.<\/jats:p>\n          <\/jats:sec>","DOI":"10.1161\/jaha.121.023035","type":"journal-article","created":{"date-parts":[[2021,12,20]],"date-time":"2021-12-20T12:48:26Z","timestamp":1640004506000},"update-policy":"https:\/\/doi.org\/10.1161\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":23,"title":["Use of Pulmonary Arterial Hypertension Therapies in Patients with a Fontan Circulation: Current Practice Across the United Kingdom"],"prefix":"10.1161","volume":"11","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-7899-463X","authenticated-orcid":false,"given":"Andrew","family":"Constantine","sequence":"first","affiliation":[{"name":"Adult Congenital Heart Centre and Centre for Pulmonary Hypertension, Royal Brompton HospitalRoyal Brompton and Harefield HospitalsGuy's and St Thomas' NHS Foundation Trust London United Kingdom"},{"name":"National Heart and Lung InstituteImperial College London London United Kingdom"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1300-0504","authenticated-orcid":false,"given":"Konstantinos","family":"Dimopoulos","sequence":"additional","affiliation":[{"name":"Adult Congenital Heart Centre and Centre for Pulmonary Hypertension, Royal Brompton HospitalRoyal Brompton and Harefield HospitalsGuy's and St Thomas' NHS Foundation Trust London United Kingdom"},{"name":"National Heart and Lung InstituteImperial College London London United Kingdom"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Petra","family":"Jenkins","sequence":"additional","affiliation":[{"name":"North West ACHD ServiceLiverpool Heart and Chest HospitalLiverpool Heart and Chest Hospital NHS Foundation Trust Liverpool United Kingdom"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3180-6993","authenticated-orcid":false,"given":"Robert M. R.","family":"Tulloh","sequence":"additional","affiliation":[{"name":"Bristol Heart InstituteUniversity Hospital Bristol and Weston NHS Foundation Trust Bristol United Kingdom"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Robin","family":"Condliffe","sequence":"additional","affiliation":[{"name":"Pulmonary Vascular Disease Unit Royal Hallamshire HospitalSheffield Teaching Hospitals NHS Foundation Trust Sheffield United Kingdom"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Katrijn","family":"Jansen","sequence":"additional","affiliation":[{"name":"Adult Congenital and Paediatric Heart Unit Freeman HospitalNewcastle Upon Tyne Hospitals NHS Foundation Trust Newcastle Upon Tyne United Kingdom"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Natali A. Y.","family":"Chung","sequence":"additional","affiliation":[{"name":"Adult Congenital Heart Disease Service, St Thomas\u2019 HospitalGuy\u2019s and St Thomas\u2019 NHS Foundation Trust London United Kingdom"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"James","family":"Oliver","sequence":"additional","affiliation":[{"name":"Yorkshire Heart Centre, Leeds General InfirmaryLeeds Teaching Hospitals NHS Trust Leeds United Kingdom"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Helen","family":"Parry","sequence":"additional","affiliation":[{"name":"Yorkshire Heart Centre, Leeds General InfirmaryLeeds Teaching Hospitals NHS Trust Leeds United Kingdom"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Samantha","family":"Fitzsimmons","sequence":"additional","affiliation":[{"name":"Congenital Cardiac Centre, Southampton General HospitalUniversity Hospital Southampton NHS Foundation Trust Southampton United 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ServiceLiverpool Heart and Chest HospitalLiverpool Heart and Chest Hospital NHS Foundation Trust Liverpool United Kingdom"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kate","family":"von Klemperer","sequence":"additional","affiliation":[{"name":"Grown\u2010Up Congenital Heart Disease Service Barts Heart CentreSt Bartholomew\u2019s HospitalBarts Health NHS Trust London United Kingdom"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8906-7647","authenticated-orcid":false,"given":"Paul","family":"Clift","sequence":"additional","affiliation":[{"name":"Department of Cardiology Queen Elizabeth Hospital Birmingham United Kingdom"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Paul","family":"Clift","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Robin","family":"Condliffe","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Konstantinos","family":"Dimopoulos","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Katrijn","family":"Jansen","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shahin","family":"Moledina","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andrew","family":"Constantine","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"276","reference":[{"key":"e_1_3_1_2_2","doi-asserted-by":"publisher","DOI":"10.1161\/CIRCHEARTFAILURE.117.004515"},{"key":"e_1_3_1_3_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-5223(19)35174-8"},{"key":"e_1_3_1_4_2","doi-asserted-by":"publisher","DOI":"10.1161\/CIRCULATIONAHA.106.676445"},{"key":"e_1_3_1_5_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-5223(98)70406-4"},{"key":"e_1_3_1_6_2","doi-asserted-by":"publisher","DOI":"10.1111\/j.1747-0803.2007.00116.x"},{"key":"e_1_3_1_7_2","doi-asserted-by":"publisher","DOI":"10.1136\/hrt.2006.094516"},{"key":"e_1_3_1_8_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.ijcard.2014.06.015"},{"key":"e_1_3_1_9_2","doi-asserted-by":"publisher","DOI":"10.1161\/CIRCULATIONAHA.115.017202"},{"key":"e_1_3_1_10_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.jtcvs.2012.11.031"},{"key":"e_1_3_1_11_2","doi-asserted-by":"publisher","DOI":"10.1136\/heartjnl-2015-307930"},{"key":"e_1_3_1_12_2","doi-asserted-by":"publisher","DOI":"10.1093\/eurheartj\/ehn215"},{"key":"e_1_3_1_13_2","doi-asserted-by":"publisher","DOI":"10.1161\/CIRCULATIONAHA.110.981746"},{"key":"e_1_3_1_14_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.ijcard.2013.03.014"},{"key":"e_1_3_1_15_2","doi-asserted-by":"publisher","DOI":"10.1161\/CIRCULATIONAHA.113.008441"},{"key":"e_1_3_1_16_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.amjcard.2016.02.024"},{"key":"e_1_3_1_17_2","doi-asserted-by":"publisher","DOI":"10.1161\/CIRCULATIONAHA.119.044352"},{"key":"e_1_3_1_18_2","doi-asserted-by":"publisher","DOI":"10.1093\/eurheartj\/ehv317"},{"key":"e_1_3_1_19_2","first-page":"e698","article-title":"2018 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Circulation. 2018;139:e698\u2013e800. doi: 10.1161\/CIR.0000000000000603","journal-title":"Circulation"},{"key":"e_1_3_1_20_2","doi-asserted-by":"publisher","DOI":"10.1093\/eurheartj\/ehaa554"},{"key":"e_1_3_1_21_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.healun.2015.01.005"},{"key":"e_1_3_1_22_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.jtcvs.2004.07.013"}],"container-title":["Journal of the American Heart Association"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.ahajournals.org\/doi\/full\/10.1161\/JAHA.121.023035","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2024,5,13]],"date-time":"2024-05-13T11:09:33Z","timestamp":1715598573000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.ahajournals.org\/doi\/10.1161\/JAHA.121.023035"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2022,1,4]]},"references-count":21,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2022,1,4]]}},"alternative-id":["10.1161\/JAHA.121.023035"],"URL":"https:\/\/doi.org\/10.1161\/jaha.121.023035","relation":{},"ISSN":["2047-9980"],"issn-type":[{"value":"2047-9980","type":"electronic"}],"subject":[],"published":{"date-parts":[[2022,1,4]]},"assertion":[{"value":"2021-06-27","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2021-11-15","order":1,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2021-12-20","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}],"article-number":"e023035"}}