{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,7,26]],"date-time":"2025-07-26T08:48:47Z","timestamp":1753519727901,"version":"3.40.5"},"reference-count":20,"publisher":"Cambridge University Press (CUP)","issue":"6","license":[{"start":{"date-parts":[[2020,5,22]],"date-time":"2020-05-22T00:00:00Z","timestamp":1590105600000},"content-version":"unspecified","delay-in-days":0,"URL":"https:\/\/www.cambridge.org\/core\/terms"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Cardiol Young"],"published-print":{"date-parts":[[2020,6]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec id=\"S1047951120001110_as1\"><jats:title>Background:<\/jats:title><jats:p>Pulmonary vasodilators improve the functional capacity of some patients with pulmonary arterial hypertension. However, pulmonary vasodilators frequently fail to improve unequivocal endpoints of efficacy in patients with lower pulmonary arterial pressures who have been palliated with a Fontan procedure.<\/jats:p><\/jats:sec><jats:sec id=\"S1047951120001110_as2\"><jats:title>Objective:<\/jats:title><jats:p>Haemodynamic measurements and the results of acute vasodilator testing in a subset of patients were reviewed to determine whether some patients acutely respond more favourably to sildenafil and might be candidates for precision care with a phosphodiesterase V inhibitor long term.<\/jats:p><\/jats:sec><jats:sec id=\"S1047951120001110_as3\"><jats:title>Materials and Methods:<\/jats:title><jats:p>Heart catheterisation was performed in 11 patients with a Fontan procedure. Haemodynamic measurements were performed before and after treatment with intravenous sildenafil (mean 0.14, range 0.05\u20130.20 mg\/kg). Results (mean\u00a0\u00b1\u00a0standard deviation) were compared by paired and unpaired t-tests to identify statistically significant changes.<\/jats:p><\/jats:sec><jats:sec id=\"S1047951120001110_as4\"><jats:title>Results:<\/jats:title><jats:p>Sildenafil was acutely associated with changes in mean pulmonary arterial pressure, transpulmonary gradient, indexed blood flow, and indexed vascular resistance. Changes in mean pulmonary arterial pressure were greater for patients with a mean pulmonary arterial pressure greater than 14\u00a0mmHg compared to patients with a lower mean pulmonary arterial pressure. Changes in transpulmonary gradient were greater for patients with a transpulmonary gradient greater than 5\u00a0mmHg compared to patients with a lower transpulmonary gradient.<\/jats:p><\/jats:sec><jats:sec id=\"S1047951120001110_as5\"><jats:title>Conclusion:<\/jats:title><jats:p>Sildenafil acutely decreases mean pulmonary arterial pressure and transpulmonary gradient and causes greater acute changes in patients with higher mean pulmonary arterial pressures and transpulmonary gradients. Haemodynamic measurements and vasodilator testing might help to guide precision care following Fontan palliation.<\/jats:p><\/jats:sec>","DOI":"10.1017\/s1047951120001110","type":"journal-article","created":{"date-parts":[[2020,5,22]],"date-time":"2020-05-22T11:23:53Z","timestamp":1590146633000},"page":"829-833","source":"Crossref","is-referenced-by-count":3,"title":["Acute vasodilator testing following Fontan palliation: an opportunity to guide precision care?"],"prefix":"10.1017","volume":"30","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-5333-7844","authenticated-orcid":false,"given":"Ronald W.","family":"Day","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"56","published-online":{"date-parts":[[2020,5,22]]},"reference":[{"key":"S1047951120001110_ref14","doi-asserted-by":"publisher","DOI":"10.1017\/S1047951109990023"},{"key":"S1047951120001110_ref8","doi-asserted-by":"publisher","DOI":"10.1016\/j.ijcard.2013.03.014"},{"key":"S1047951120001110_ref17","doi-asserted-by":"publisher","DOI":"10.1017\/S1047951115002747"},{"key":"S1047951120001110_ref5","first-page":"233","article-title":"Pulmonary artery size is associated with functional clinical status in the Fontan circulation","volume":"106","author":"Ridderbos","year":"2020","journal-title":"Heart"},{"key":"S1047951120001110_ref15","doi-asserted-by":"publisher","DOI":"10.1007\/s00246-017-1716-4"},{"key":"S1047951120001110_ref10","doi-asserted-by":"crossref","first-page":"265","DOI":"10.1161\/CIRCIMAGING.113.001243","article-title":"Sildenafil improves exercise hemodynamics in Fontan patients","volume":"7","author":"Van de Bruaene","year":"2014","journal-title":"Circ Cardiovasc Imag"},{"key":"S1047951120001110_ref13","doi-asserted-by":"crossref","first-page":"690","DOI":"10.1093\/eurjhf\/hft017","article-title":"Impact of Bosentan on exercise capacity in adults after the Fontan operation: a randomized study","volume":"15","author":"Schuuring","year":"2013","journal-title":"Eur J Heart Fail"},{"key":"S1047951120001110_ref2","doi-asserted-by":"publisher","DOI":"10.1161\/01.CIR.85.2.469"},{"key":"S1047951120001110_ref1","first-page":"559","volume-title":"Paediatric Cardiology","author":"Choussat","year":"1977"},{"key":"S1047951120001110_ref6","doi-asserted-by":"publisher","DOI":"10.1136\/hrt.2010.193912"},{"key":"S1047951120001110_ref19","doi-asserted-by":"publisher","DOI":"10.1177\/2045894018790450"},{"key":"S1047951120001110_ref3","doi-asserted-by":"publisher","DOI":"10.1016\/j.ijcard.2005.01.039"},{"key":"S1047951120001110_ref4","doi-asserted-by":"publisher","DOI":"10.1016\/j.healun.2015.01.005"},{"key":"S1047951120001110_ref7","doi-asserted-by":"crossref","first-page":"651","DOI":"10.1111\/apa.15098","article-title":"Providing a framework of principles for conceptualising the Fontan circulation","volume":"109","author":"Gewillig","year":"2020","journal-title":"Acta Pediatr"},{"key":"S1047951120001110_ref9","doi-asserted-by":"publisher","DOI":"10.1093\/eurheartj\/ehn215"},{"key":"S1047951120001110_ref11","doi-asserted-by":"publisher","DOI":"10.1161\/CIRCULATIONAHA.110.981746"},{"key":"S1047951120001110_ref12","doi-asserted-by":"publisher","DOI":"10.1161\/CIRCULATIONAHA.113.008441"},{"key":"S1047951120001110_ref16","doi-asserted-by":"publisher","DOI":"10.1016\/j.ijcard.2016.06.322"},{"key":"S1047951120001110_ref18","doi-asserted-by":"publisher","DOI":"10.1007\/s00392-014-0694-2"},{"key":"S1047951120001110_ref20","article-title":"Results of the Fontan udenafil exercise longitudinal (FUEL) trial","author":"Goldberg","year":"2019","journal-title":"Circulation"}],"container-title":["Cardiology in the Young"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.cambridge.org\/core\/services\/aop-cambridge-core\/content\/view\/S1047951120001110","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2020,10,8]],"date-time":"2020-10-08T13:18:03Z","timestamp":1602163083000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.cambridge.org\/core\/product\/identifier\/S1047951120001110\/type\/journal_article"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2020,5,22]]},"references-count":20,"journal-issue":{"issue":"6","published-print":{"date-parts":[[2020,6]]}},"alternative-id":["S1047951120001110"],"URL":"https:\/\/doi.org\/10.1017\/s1047951120001110","relation":{},"ISSN":["1047-9511","1467-1107"],"issn-type":[{"type":"print","value":"1047-9511"},{"type":"electronic","value":"1467-1107"}],"subject":[],"published":{"date-parts":[[2020,5,22]]}}}