{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,25]],"date-time":"2026-08-25T13:18:46Z","timestamp":1787663926428,"version":"build-2736575974"},"reference-count":14,"publisher":"Springer Science and Business Media LLC","license":[{"start":{"date-parts":[[2026,8,25]],"date-time":"2026-08-25T00:00:00Z","timestamp":1787616000000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"},{"start":{"date-parts":[[2026,8,25]],"date-time":"2026-08-25T00:00:00Z","timestamp":1787616000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"}],"funder":[{"DOI":"10.13039\/501100004332","name":"Seoul National University Hospital","doi-asserted-by":"crossref","id":[{"id":"10.13039\/501100004332","id-type":"DOI","asserted-by":"crossref"}]}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Pediatr Cardiol"],"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>Thrombosis arising from a main pulmonary artery (MPA) stump or related blind pouch remains a potentially devastating but under-recognized complication in patients after the Fontan operation. We aimed to evaluate the contemporary clinical characteristics, management strategies, and outcomes of patients with MPA stump\u2013related pathology.\u00a0We retrospectively reviewed patients treated between 2003 and 2023 who either developed MPA stump thrombosis after Fontan completion or underwent pulmonary valve obliteration for MPA stump\u2013related problems. Nine patients were identified, including three previously reported cases and six newly identified patients. Clinical characteristics, imaging findings, management strategies, and long-term outcomes were analyzed.\u00a0MPA stump pathology was diagnosed at a median age of 18.2 (15.5\u201329.3) years, occurring a median of 15.9 (14.5\u201328.7) years after MPA ligation. Thrombus size ranged from 8\u2009\u00d7\u20096\u00a0mm to 37\u2009\u00d7\u200917\u00a0mm, and supraventricular arrhythmia was present in seven patients at diagnosis. Three patients experienced systemic thromboembolic events, including cerebral and renal infarction. Seven patients underwent surgical intervention, whereas two were managed conservatively. During a median follow-up of 14.6 (4.5\u201319) years after treatment for MPA thrombus, no recurrent thromboembolic events or deaths occurred.\u00a0MPA stump\u2013related pathology can cause late systemic thromboembolism after Fontan completion. Both overt thrombosis and severe blood stasis represent high-risk substrates, and surgical elimination of the MPA stump may represent a potential preventive strategy. These findings represent a hypothesis-generating observation supporting future evaluation of proactive strategies such as concomitant pulmonary valve obliteration at the time of MPA ligation.<\/jats:p>","DOI":"10.1007\/s00246-026-04412-y","type":"journal-article","created":{"date-parts":[[2026,8,25]],"date-time":"2026-08-25T12:22:23Z","timestamp":1787660543000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Main Pulmonary Artery Stump Thrombosis After Fontan Completion: Anatomic Predisposition, Clinical Impact, and Management"],"prefix":"10.1007","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-1518-6034","authenticated-orcid":false,"given":"Eun Chae","family":"Kim","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2837-7929","authenticated-orcid":false,"given":"Woong-Han","family":"Kim","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6375-1210","authenticated-orcid":false,"given":"Jae Gun","family":"Kwak","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2026,8,25]]},"reference":[{"issue":"3","key":"4412_CR1","doi-asserted-by":"publisher","first-page":"513","DOI":"10.1016\/j.jpeds.2012.03.004","volume":"161","author":"C Manlhiot","year":"2012","unstructured":"Manlhiot C, Brand\u00e3o LR, Kwok J, Kegel S, Menjak IB, Carew CL et al (2012) Thrombotic complications and thromboprophylaxis across all three stages of single ventricle heart palliation. J Pediatr 161(3):513\u2013519e3. https:\/\/doi.org\/10.1016\/j.jpeds.2012.03.004","journal-title":"J Pediatr"},{"issue":"5","key":"4412_CR2","doi-asserted-by":"publisher","first-page":"1563","DOI":"10.1016\/S0022-5223(95)70080-3","volume":"110","author":"DN Rosenthal","year":"1995","unstructured":"Rosenthal DN, Bulbul ZR, Friedman AH, Hellenbrand WE, Kleinman CS (1995) Thrombosis of the pulmonary artery stump after distal ligation. J Thorac Cardiovasc Surg 110(5):1563\u20131565. https:\/\/doi.org\/10.1016\/S0022-5223(95)70080-3","journal-title":"J Thorac Cardiovasc Surg"},{"issue":"4","key":"4412_CR3","doi-asserted-by":"publisher","first-page":"396","DOI":"10.1136\/heart.88.4.396","volume":"88","author":"N Madan","year":"2002","unstructured":"Madan N, Robinson BW, Jacobs ML (2002) Thrombosis in the proximal pulmonary artery stump in a Fontan patient. Heart 88(4):396. https:\/\/doi.org\/10.1136\/heart.88.4.396","journal-title":"Heart"},{"issue":"4","key":"4412_CR4","doi-asserted-by":"publisher","first-page":"870","DOI":"10.1007\/s00246-007-9190-z","volume":"29","author":"K Yie","year":"2008","unstructured":"Yie K, Lee CH, Kim SJ (2008) Thrombi in the main pulmonary artery stump after a Fontan operation. Pediatr Cardiol 29(4):870\u2013871. https:\/\/doi.org\/10.1007\/s00246-007-9190-z","journal-title":"Pediatr Cardiol"},{"issue":"1","key":"4412_CR5","doi-asserted-by":"publisher","first-page":"42","DOI":"10.1007\/s00246-011-0074-x","volume":"33","author":"SY Lee","year":"2012","unstructured":"Lee SY, Baek JS, Kim GB, Kwon BS, Bae EJ, Noh CI et al (2012) Clinical significance of thrombosis in an intracardiac blind pouch after a Fontan operation. Pediatr Cardiol 33(1):42\u201348. https:\/\/doi.org\/10.1007\/s00246-011-0074-x","journal-title":"Pediatr Cardiol"},{"issue":"4","key":"4412_CR6","doi-asserted-by":"publisher","first-page":"734","DOI":"10.1093\/ejcts\/ezs043","volume":"41","author":"SA Nashef","year":"2012","unstructured":"Nashef SA, Roques F, Sharples LD, Nilsson J, Smith C, Goldstone AR et al (2012) EuroSCORE II. Eur J Cardiothorac Surg 41(4):734\u2013744 discussion 744-5. https:\/\/doi.org\/10.1093\/ejcts\/ezs043","journal-title":"Eur J Cardiothorac Surg"},{"issue":"5","key":"4412_CR7","doi-asserted-by":"publisher","first-page":"484","DOI":"10.1177\/1076029613520464","volume":"20","author":"M Firdouse","year":"2014","unstructured":"Firdouse M, Agarwal A, Chan AK, Mondal T (2014) Thrombosis and thromboembolic complications in Fontan patients: a literature review. Clin Appl Thromb Hemost 20(5):484\u2013492. https:\/\/doi.org\/10.1177\/1076029613520464","journal-title":"Clin Appl Thromb Hemost"},{"issue":"2","key":"4412_CR8","doi-asserted-by":"publisher","first-page":"242","DOI":"10.1093\/cvr\/cvt044","volume":"99","author":"V Peiffer","year":"2013","unstructured":"Peiffer V, Sherwin SJ, Weinberg PD (2013) Does low and oscillatory wall shear stress correlate spatially with early atherosclerosis? A systematic review. Cardiovasc Res 99(2):242\u2013250. https:\/\/doi.org\/10.1093\/cvr\/cvt044","journal-title":"Cardiovasc Res"},{"issue":"12","key":"4412_CR9","doi-asserted-by":"publisher","first-page":"1312","DOI":"10.1016\/j.jacc.2016.06.056","volume":"68","author":"AC Egbe","year":"2016","unstructured":"Egbe AC, Connolly HM, McLeod CJ, Ammash NM, Niaz T, Yogeswaran V et al (2016) Thrombotic and embolic complications associated with atrial arrhythmia after Fontan operation: role of prophylactic therapy. J Am Coll Cardiol 68(12):1312\u20131319. https:\/\/doi.org\/10.1016\/j.jacc.2016.06.056","journal-title":"J Am Coll Cardiol"},{"issue":"6","key":"4412_CR10","doi-asserted-by":"publisher","first-page":"e234","DOI":"10.1161\/CIR.0000000000000696","volume":"140","author":"J Rychik","year":"2019","unstructured":"Rychik J, Atz AM, Celermajer DS, Deal BJ, Gatzoulis MA, Gewillig MH et al (2019) Evaluation and management of the child and adult with Fontan circulation: a scientific statement from the American Heart Association. Circulation 140(6):e234\u2013e284. https:\/\/doi.org\/10.1161\/CIR.0000000000000696","journal-title":"Circulation"},{"issue":"12","key":"4412_CR11","doi-asserted-by":"publisher","first-page":"2107","DOI":"10.1002\/ejhf.3071","volume":"25","author":"W Doehner","year":"2023","unstructured":"Doehner W, B\u00f6hm M, Boriani G, Christersson C, Coats AJS, Haeusler KG et al (2023) Interaction of heart failure and stroke: a clinical consensus statement of the ESC Council on Stroke, the Heart Failure Association (HFA) and the ESC Working Group on Thrombosis. Eur J Heart Fail 25(12):2107\u20132129. https:\/\/doi.org\/10.1002\/ejhf.3071","journal-title":"Eur J Heart Fail"},{"issue":"25","key":"4412_CR12","doi-asserted-by":"publisher","first-page":"3173","DOI":"10.1016\/j.jacc.2017.10.045","volume":"70","author":"CJ Daniels","year":"2017","unstructured":"Daniels CJ, Bradley EA, Landzberg MJ, Aboulhosn J, Beekman RH 3rd, Book W et al (2017) Fontan-associated liver disease: proceedings from the American College of Cardiology Stakeholders Meeting, October 1 to 2, 2015, Washington DC. J Am Coll Cardiol 70(25):3173\u20133194. https:\/\/doi.org\/10.1016\/j.jacc.2017.10.045","journal-title":"J Am Coll Cardiol"},{"issue":"6","key":"4412_CR13","doi-asserted-by":"publisher","first-page":"1260","DOI":"10.1016\/S0022-5223(02)73605-2","volume":"125","author":"KC Odegard","year":"2003","unstructured":"Odegard KC, McGowan FX Jr, Zurakowski D, Dinardo JA, Castro RA, del Nido PJ et al (2003) Procoagulant and anticoagulant factor abnormalities following the Fontan procedure: increased factor VIII may predispose to thrombosis. J Thorac Cardiovasc Surg 125(6):1260\u20131267. https:\/\/doi.org\/10.1016\/S0022-5223(02)73605-2","journal-title":"J Thorac Cardiovasc Surg"},{"issue":"1","key":"4412_CR14","doi-asserted-by":"publisher","first-page":"85","DOI":"10.1161\/CIRCULATIONAHA.107.738559","volume":"117","author":"P Khairy","year":"2008","unstructured":"Khairy P, Fernandes SM, Mayer JE Jr, Triedman JK, Walsh EP, Lock JE et al (2008) Long-term survival, modes of death, and predictors of mortality in patients with Fontan surgery. Circulation 117(1):85\u201392. https:\/\/doi.org\/10.1161\/CIRCULATIONAHA.107.738559","journal-title":"Circulation"}],"container-title":["Pediatric Cardiology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s00246-026-04412-y.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/article\/10.1007\/s00246-026-04412-y","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s00246-026-04412-y.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,8,25]],"date-time":"2026-08-25T12:22:24Z","timestamp":1787660544000},"score":1,"resource":{"primary":{"URL":"https:\/\/link.springer.com\/10.1007\/s00246-026-04412-y"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,8,25]]},"references-count":14,"alternative-id":["4412"],"URL":"https:\/\/doi.org\/10.1007\/s00246-026-04412-y","relation":{},"ISSN":["0172-0643","1432-1971"],"issn-type":[{"value":"0172-0643","type":"print"},{"value":"1432-1971","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,8,25]]},"assertion":[{"value":"12 March 2026","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"27 July 2026","order":2,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"25 August 2026","order":3,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"The authors declare no competing interests.","order":1,"name":"Ethics","label":"Conflict of interest","group":{"name":"EthicsHeading","label":"Declarations"}},{"value":"2405-149-1539 (approved on 2024-11-06).","order":2,"name":"Ethics","label":"IRB Approval Number","group":{"name":"EthicsHeading","label":"Declarations"}}]}}