{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,14]],"date-time":"2026-07-14T05:46:12Z","timestamp":1784007972053,"version":"3.55.0"},"reference-count":10,"publisher":"Springer Science and Business Media LLC","issue":"1","license":[{"start":{"date-parts":[[2024,12,27]],"date-time":"2024-12-27T00:00:00Z","timestamp":1735257600000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"},{"start":{"date-parts":[[2024,12,27]],"date-time":"2024-12-27T00:00:00Z","timestamp":1735257600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Gen Thorac Cardiovasc Surg Cases"],"abstract":"<jats:title>Abstract<\/jats:title><jats:sec>\n                <jats:title>Background<\/jats:title>\n                <jats:p>Lung transplantation is a viable lifesaving option for patients with diffuse pulmonary arteriovenous malformations (AVMs). We present a case of diffuse pulmonary AVMs associated with juvenile polyposis and hereditary hemorrhagic telangiectasia (JP-HHT) that was successfully managed by lung transplantation.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Case presentation<\/jats:title>\n                <jats:p>A 19-year-old woman developed severe hypoxemia due to pulmonary AVMs diagnosed at 4\u00a0years of age. She also had epistaxis, hemangioma of the tongue, and numerous polyps in the gastrointestinal tract, leading to the JP-HHT diagnosis. Although she had undergone transcatheter embolization for pulmonary AVMs four times, all lesions became recanalized, and her hypoxemia never improved. She also had hepatic AVMs that did not result in portal hypertension or required any interventions. She underwent bilateral lung transplantation from a brain-dead donor at 3\u00a0years after registration. Given that she had severe hypoxemia caused by intrapulmonary shunting, venoarterial extracorporeal membrane oxygenation (V-A ECMO) support was initiated from the femoral vessels under local anesthesia. Then, she was anesthetized and intubated. Peripheral V-A ECMO was switched to central cardiopulmonary bypass during the transplant procedure to prevent persistent hypoxia of the upper body and thromboembolic event due to severe polycythemia. The total graft ischemic time was\u2009&gt;\u200911\u00a0h, which resulted in ischemia\u2013reperfusion injury immediately after transplantation. Furthermore, the patient\u2019s postoperative course was complicated by acute cellular rejection and right heart failure due to hepatic AVM progression. She was finally discharged home without oxygen therapy on postoperative day 68. At 1-year post-transplantation, she is currently enjoying college life. However, she still has to undergo periodic endoscopic examinations to monitor her numerous polyps, which are known to carry a risk of cancer development.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Conclusions<\/jats:title>\n                <jats:p>Lung transplantation can be a viable treatment option for diffuse pulmonary AVMs in patients with JP-HHT. However, meticulous perioperative management is mandatory to prevent the development of multiple organ disorders.<\/jats:p>\n              <\/jats:sec>","DOI":"10.1186\/s44215-024-00183-1","type":"journal-article","created":{"date-parts":[[2024,12,27]],"date-time":"2024-12-27T13:03:29Z","timestamp":1735304609000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":3,"title":["Lung transplantation for diffuse pulmonary arteriovenous malformations associated with juvenile polyposis\u2013hereditary hemorrhagic telangiectasia overlap syndrome: a case report"],"prefix":"10.1186","volume":"3","author":[{"given":"Taiki","family":"Ryo","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Daisuke","family":"Nakajima","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Satoshi","family":"Kimura","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hiroshi","family":"Date","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2024,12,27]]},"reference":[{"key":"183_CR1","doi-asserted-by":"publisher","first-page":"787","DOI":"10.1016\/S1051-0443(96)70851-5","volume":"7","author":"RI White Jr","year":"1996","unstructured":"White RI Jr, Pollak JS, Wirth JA. Pulmonary arteriovenous malformations: diagnosis and transcatheter embolotherapy. J Vasc Interv Radiol. 1996;7:787\u2013804.","journal-title":"J Vasc Interv Radiol"},{"key":"183_CR2","doi-asserted-by":"publisher","first-page":"506","DOI":"10.1016\/S1053-2498(01)00365-5","volume":"21","author":"G Svetliza","year":"2002","unstructured":"Svetliza G, De la Canal A, Beveraggi E, Giacoia A, Ruiz C, Caruso E, et al. Lung transplantation in a patient with arteriovenous malformations. J Heart Lung Transplant. 2002;21:506\u20138.","journal-title":"J Heart Lung Transplant"},{"key":"183_CR3","doi-asserted-by":"publisher","first-page":"E364","DOI":"10.1111\/j.1399-3046.2012.01717.x","volume":"16","author":"MV Misra","year":"2012","unstructured":"Misra MV, Mullen MP, Vargas SO, Kim HB, Boyer D. Bilateral lung transplant for hereditary hemorrhagic telangiectasia in a pediatric patient. Pediatr Transplant. 2012;16:E364\u20137.","journal-title":"Pediatr Transplant"},{"key":"183_CR4","doi-asserted-by":"publisher","first-page":"3278","DOI":"10.1111\/ajt.12499","volume":"13","author":"H Fukushima","year":"2013","unstructured":"Fukushima H, Mitsuhashi T, Oto T, Sano Y, Kusano KF, Goto K, et al. Successful lung transplantation in a case with diffuse pulmonary arteriovenous malformations and hereditary hemorrhagic telangiectasia. Am J Transplant. 2013;13:3278\u201381.","journal-title":"Am J Transplant"},{"key":"183_CR5","doi-asserted-by":"publisher","first-page":"795","DOI":"10.1007\/s00535-012-0545-8","volume":"47","author":"F Schwenter","year":"2012","unstructured":"Schwenter F, Faughnan ME, Gradinger AB, Berk T, Gryfe R, Pollett A, et al. Juvenile polyposis, hereditary hemorrhagic telangiectasia, and early onset colorectal cancer in patients with SMAD4 mutation. J Gastroenterol. 2012;47:795\u2013804.","journal-title":"J Gastroenterol"},{"key":"183_CR6","doi-asserted-by":"publisher","first-page":"852","DOI":"10.1016\/S0140-6736(04)15732-2","volume":"363","author":"CJ Gallione","year":"2004","unstructured":"Gallione CJ, Repetto GM, Legius E, Rustgi AK, Schelley SL, Tejpar S, et al. A combined syndrome of juvenile polyposis and hereditary haemorrhagic telangiectasia associated with mutations in MADH4 (SMAD4). Lancet. 2004;363:852\u20139.","journal-title":"Lancet"},{"key":"183_CR7","doi-asserted-by":"publisher","first-page":"232470961455366","DOI":"10.1177\/2324709614553669","volume":"2","author":"D Robinson","year":"2014","unstructured":"Robinson D, Rogers B, Kapoor R, Swan J, Speas G, Gutmann R, et al. Anesthetic considerations for a patient with hereditary hemorrhagic telangiectasia (Osler-Weber-Rendu syndrome) undergoing a five-box thoracoscopic maze procedure for atrial fibrillation. J Investig Med High Impact Case Rep. 2014;2:2324709614553669.","journal-title":"J Investig Med High Impact Case Rep"},{"key":"183_CR8","doi-asserted-by":"publisher","first-page":"6","DOI":"10.1186\/s40981-023-00600-4","volume":"9","author":"T Hiyoshi","year":"2023","unstructured":"Hiyoshi T, Shimizu K, Kimura S, Naritani T, Morimatsu H. Anesthetic management of a patient with Osler-Weber-Rendu syndrome with multiple pulmonary arteriovenous malformations and pheochromocytoma for femoral artificial bone replacement: a case report. JA Clin Rep. 2023;9:6.","journal-title":"JA Clin Rep"},{"key":"183_CR9","doi-asserted-by":"publisher","first-page":"159","DOI":"10.1016\/0034-5687(94)00133-K","volume":"100","author":"N Weissmann","year":"1995","unstructured":"Weissmann N, Grimminger F, Walmrath D, Seeger W. Hypoxic vasoconstriction in buffer-perfused rabbit lungs. 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Chest. 2022;161:e23\u20138.","journal-title":"Chest"}],"container-title":["General Thoracic and Cardiovascular Surgery Cases"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1186\/s44215-024-00183-1.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/article\/10.1186\/s44215-024-00183-1\/fulltext.html","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1186\/s44215-024-00183-1.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2024,12,27]],"date-time":"2024-12-27T14:03:03Z","timestamp":1735308183000},"score":1,"resource":{"primary":{"URL":"https:\/\/gtcscases.biomedcentral.com\/articles\/10.1186\/s44215-024-00183-1"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2024,12,27]]},"references-count":10,"journal-issue":{"issue":"1","published-online":{"date-parts":[[2024,12]]}},"alternative-id":["183"],"URL":"https:\/\/doi.org\/10.1186\/s44215-024-00183-1","relation":{},"ISSN":["2731-6203"],"issn-type":[{"value":"2731-6203","type":"electronic"}],"subject":[],"published":{"date-parts":[[2024,12,27]]},"assertion":[{"value":"3 October 2024","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"18 December 2024","order":2,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"27 December 2024","order":3,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Declarations"}},{"value":"Not applicable.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Ethics approval and consent to participate"}},{"value":"Written informed consent was obtained from the patient for the publication of this case report and accompanying images.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Consent for publication"}},{"value":"The authors declare that they have no competing interests.","order":4,"name":"Ethics","group":{"name":"EthicsHeading","label":"Competing interests"}}],"article-number":"57"}}