{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,22]],"date-time":"2026-08-22T20:21:27Z","timestamp":1787430087221,"version":"build-2736575974"},"reference-count":15,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2022,6,9]],"date-time":"2022-06-09T00:00:00Z","timestamp":1654732800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["frontiersin.org"],"crossmark-restriction":true},"short-container-title":["Front. Pediatr."],"abstract":"<jats:sec><jats:title>Background<\/jats:title><jats:p>The Fontan operation is a surgical procedure used in children with univentricular hearts. Pulmonary arteriovenous fistulae (PAVF) is a major complication after a Fontan operation. However, the incidence and related clinical pathophysiology of PAVF remain unclear.<\/jats:p><\/jats:sec><jats:sec><jats:title>Purpose<\/jats:title><jats:p>This study aimed to clarify the incidence of PAVF, its clinical characteristics, and its influence on all-cause mortality.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods and Results<\/jats:title><jats:p>We serially assessed the presence of PAVF using pulmonary artery angiography and\/or contrast echocardiography during catheterization in 391 consecutive patients who underwent the Fontan procedure and compared the results with the Fontan pathophysiology and all-cause mortality. PAVF developed in 36 patients (9.2%), including 30 diffuse- and six discrete-PAVF types. The PAVF-free rates at 1, 5, 10, 15, 20, and \u226525 years after Fontan operation were 97, 96, 93, 88, 87, and 83%, respectively. The mean arterial blood oxygen saturation (SaO<jats:sub>2<\/jats:sub>) in patients with diffuse PAVF at each corresponding postoperative stage were 90, 91, 91, 91, 89, and 88%, respectively, indicating lower SaO<jats:sub>2<\/jats:sub> levels than those in patients without PAVF (all <jats:italic>p<\/jats:italic> &amp;lt; 0.01). However, there was no difference in the SaO<jats:sub>2<\/jats:sub> levels between patients with discrete PAVF and those without PAVF. During a median follow-up period of 2.9 years after the last catheterization, 31 patients, including 12 patients with PAVF, died. Patients with PAVF, especially those with diffuse PAVF, had a higher mortality rate (<jats:italic>p<\/jats:italic> = 0.01) than those without PAVF (hazard ratio: 3.6, 95% confidence interval: 1.6\u20137.8, <jats:italic>p<\/jats:italic> = 0.0026).<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>Patients who underwent Fontan surgery had an increased incidence of PAVF as they aged. Discrete PAVF did not influence SaO<jats:sub>2<\/jats:sub> or mortality, whereas the presence of diffuse PAVF caused hypoxia and was associated with all-cause mortality.<\/jats:p><\/jats:sec>","DOI":"10.3389\/fped.2022.713219","type":"journal-article","created":{"date-parts":[[2022,6,9]],"date-time":"2022-06-09T05:10:40Z","timestamp":1654751440000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":9,"title":["Pulmonary Arteriovenous Fistulae After Fontan Operation: Incidence, Clinical Characteristics, and Impact on All-Cause Mortality"],"prefix":"10.3389","volume":"10","author":[{"given":"Hideo","family":"Ohuchi","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Aki","family":"Mori","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michikazu","family":"Nakai","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kazuto","family":"Fujimoto","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Toru","family":"Iwasa","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Heima","family":"Sakaguchi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kenichi","family":"Kurosaki","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Isao","family":"Shiraishi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1965","published-online":{"date-parts":[[2022,6,9]]},"reference":[{"key":"B1","doi-asserted-by":"publisher","first-page":"181","DOI":"10.1111\/chd.12570","article-title":"Five decades of the Fontan operation: a systematic review of international reports on outcomes after univentricular palliation.","volume":"13","author":"Kverneland","year":"2018","journal-title":"Congenit Heart Dis."},{"key":"B2","doi-asserted-by":"publisher","first-page":"703","DOI":"10.1586\/14779072.2014.912132","article-title":"Pulmonary arteriovenous malformations after the superior cavopulmonary shunt: mechanisms and clinical implications.","volume":"12","author":"Kavarana","year":"2014","journal-title":"Expert Rev Cardiovasc Ther."},{"key":"B3","doi-asserted-by":"publisher","first-page":"1362","DOI":"10.1016\/j.jtcvs.2010.08.088","article-title":"Cavopulmonary pathway modification in patients with heterotaxy and newly diagnosed or persistent pulmonary arteriovenous malformations after a modified Fontan operation.","volume":"141","author":"McElhinney","year":"2011","journal-title":"J Thorac Cardiovasc Surg."},{"key":"B4","doi-asserted-by":"publisher","first-page":"835","DOI":"10.1016\/j.diii.2013.03.014","article-title":"Diagnosis and treatment of pulmonary arteriovenous malformations in hereditary hemorrhagic telangiectasia: an overview.","volume":"94","author":"Lacombe","year":"2013","journal-title":"Diagn Interv Imaging."},{"key":"B5","doi-asserted-by":"publisher","first-page":"9","DOI":"10.1016\/j.ahj.2017.03.020","article-title":"Hemodynamic determinants of mortality after Fontan operation.","volume":"189","author":"Ohuchi","year":"2017","journal-title":"Am Heart J."},{"key":"B6","doi-asserted-by":"publisher","first-page":"1288","DOI":"10.1378\/chest.08-1266","article-title":"Grading of pulmonary right-to-left shunt with transthoracic contrast echocardiography: does it predict the indication for embolotherapy?","volume":"135","author":"van Gent","year":"2009","journal-title":"Chest."},{"key":"B7","doi-asserted-by":"publisher","first-page":"7","DOI":"10.1007\/BF02505805","article-title":"Measurement and validity of the ventilatory threshold in patients with congenital heart disease.","volume":"17","author":"Ohuchi","year":"1996","journal-title":"Pediatr Cardiol."},{"key":"B8","doi-asserted-by":"publisher","first-page":"980","DOI":"10.1053\/j.semtcvs.2020.03.004","article-title":"Hepatic vein blood increases lung microvascular angiogenesis and endothelial cell survival-toward an understanding of univentricular circulation.","volume":"32","author":"Spearman","year":"2020","journal-title":"Semin Thorac Cardiovasc Surg."},{"key":"B9","doi-asserted-by":"publisher","first-page":"1805","DOI":"10.3390\/cancers12071805","article-title":"Risk of liver cirrhosis and hepatocellular carcinoma after Fontan operation: a need for surveillance.","volume":"12","author":"Yoon","year":"2020","journal-title":"Cancers (Basel)."},{"key":"B10","doi-asserted-by":"publisher","first-page":"425","DOI":"10.1136\/heart.83.4.425","article-title":"Intrapulmonary arteriovenous shunting may be a universal phenomenon in patients with the superior cavopulmonary anastomosis: a radionuclide study.","volume":"83","author":"Vettukattil","year":"2000","journal-title":"Heart."},{"key":"B11","doi-asserted-by":"publisher","first-page":"548","DOI":"10.1016\/j.athoracsur.2008.10.024","article-title":"Inclusion of hepatic venous drainage in patients with pulmonary arteriovenous fistulas.","volume":"87","author":"Kim","year":"2009","journal-title":"Ann Thorac Surg."},{"key":"B12","doi-asserted-by":"publisher","first-page":"69","DOI":"10.1016\/j.ejcts.2009.02.046","article-title":"Pulmonary arteriovenous malformations after a Fontan operation in the leftisomerism and absent inferior vena cava.","volume":"36","author":"Nakamura","year":"2009","journal-title":"Eur J Cardiothorac Surg."},{"key":"B13","first-page":"480","article-title":"Surgical improvement of hepatic venous mixing to resolve systemic arterial hypoxemia associated with post-Fontan pulmonary arteriovenous fistulae.","volume":"36","author":"Sernich","year":"2009","journal-title":"Tex Heart Inst J."},{"key":"B14","doi-asserted-by":"publisher","first-page":"1359","DOI":"10.1016\/j.jtcvs.2013.02.036","article-title":"Hemodynamics and arteriovenous malformations in cavopulmonary anastomosis: the case for residual antegrade pulsatile flow.","volume":"146","author":"Henaine","year":"2013","journal-title":"J Thorac Cardiovasc Surg."},{"key":"B15","doi-asserted-by":"publisher","first-page":"456","DOI":"10.1016\/j.amjcard.2015.11.014","article-title":"Liver disease in patients after the Fontan operation.","volume":"117","author":"Pundi","year":"2016","journal-title":"Am J Cardiol."}],"container-title":["Frontiers in Pediatrics"],"original-title":[],"link":[{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fped.2022.713219\/full","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2022,6,9]],"date-time":"2022-06-09T05:10:43Z","timestamp":1654751443000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fped.2022.713219\/full"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2022,6,9]]},"references-count":15,"alternative-id":["10.3389\/fped.2022.713219"],"URL":"https:\/\/doi.org\/10.3389\/fped.2022.713219","relation":{},"ISSN":["2296-2360"],"issn-type":[{"value":"2296-2360","type":"electronic"}],"subject":[],"published":{"date-parts":[[2022,6,9]]},"article-number":"713219"}}