{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,16]],"date-time":"2026-06-16T22:55:23Z","timestamp":1781650523583,"version":"3.54.5"},"reference-count":11,"publisher":"Springer Science and Business Media LLC","issue":"1","license":[{"start":{"date-parts":[[2026,4,17]],"date-time":"2026-04-17T00:00:00Z","timestamp":1776384000000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"},{"start":{"date-parts":[[2026,6,1]],"date-time":"2026-06-01T00:00:00Z","timestamp":1780272000000},"content-version":"vor","delay-in-days":45,"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>\n                  <jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>Aortic dissection and aneurysm rupture are leading causes of death in patients with Marfan syndrome. Due to the risk of such vascular complications, the indication for heart transplantation in this population remains controversial. Previous reports, however, have demonstrated that heart transplantation in patients with Marfan syndrome can result in good long-term survival comparable to that in patients without Marfan syndrome. Here, we present a case of successful orthotopic heart transplantation after total aortic replacement in a patient with Marfan syndrome.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Case presentation<\/jats:title>\n                    <jats:p>A 41-year-old man with Marfan syndrome, who had been managed conservatively for type B aortic dissection, developed acute type A dissection involving both coronary arteries at age 30, resulting in myocardial infarction. He underwent aortic root replacement with a mechanical valve. Thereafter, he was repeatedly hospitalized for heart failure due to ischemic cardiomyopathy. Three years later, at age 33, he was referred to our institution for heart transplant registration because of progressively deteriorating cardiac function and frequent episodes of intractable lethal arrhythmias. He underwent replacement of the ascending aorta and aortic arch, along with bioprosthetic aortic valve replacement. His immediate postoperative course was complicated by hemodynamic decompensation, necessitating support with an extracorporeal left ventricular assist device. One year later, he underwent a thoracoabdominal aortic replacement and bridge-to-bridge implantation of a left ventricular assist device. Three years later, at age 37, he underwent replacement of the durable device because of a pump pocket infection. After 7 years on the transplantation list, he underwent successful orthotopic heart transplantation from a suitable donor at the age of 41.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>This rare case report describes a patient with Marfan syndrome who underwent orthotopic heart transplantation after total aortic replacement, followed by multiple surgical procedures, including extracorporeal and durable left ventricular assist device implantations, over an extended period. Although total aortic replacement was challenging in the context of complex coexisting cardiovascular disease, this approach enabled subsequent heart transplantation after mitigating the potential risk of Marfan syndrome-associated cardiovascular events.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1186\/s44215-026-00249-2","type":"journal-article","created":{"date-parts":[[2026,4,17]],"date-time":"2026-04-17T07:54:35Z","timestamp":1776412475000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Successful orthotopic heart transplantation following total aortic replacement in a patient with Marfan syndrome: case report"],"prefix":"10.1186","volume":"5","author":[{"given":"Tatsuya","family":"Tago","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Masatoshi","family":"Akiyama","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kentaro","family":"Yuda","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kota","family":"Itagaki","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Katsuhiro","family":"Hosoyama","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Koki","family":"Ito","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yusuke","family":"Suzuki","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shintaro","family":"Katahira","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Goro","family":"Takahashi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kiichiro","family":"Kumagai","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yoshikatsu","family":"Saiki","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2026,4,17]]},"reference":[{"issue":"9","key":"249_CR1","doi-asserted-by":"publisher","first-page":"875","DOI":"10.1111\/jocs.14129","volume":"34","author":"Y Ogawa","year":"2019","unstructured":"Ogawa Y, Choi CW, Shudo Y, Woo YJ. Successful orthotopic heart transplantation in a patient with Marfan syndrome. J Card Surg. 2019;34(9):875\u20136.","journal-title":"J Card Surg"},{"issue":"3","key":"249_CR2","doi-asserted-by":"publisher","first-page":"434","DOI":"10.5606\/tgkdc.dergisi.2011.058","volume":"19","author":"K Kirali","year":"2011","unstructured":"Kirali K, Eren E, Mataraci I, \u00c7evirme D. Combined orthotopic heart transplantation and prophylactic ascending aorta replacement in a Marfan patient. Turkish J Thorac Cardiovasc Surg. 2011;19(3):434\u20136.","journal-title":"Turkish J Thorac Cardiovasc Surg"},{"issue":"4","key":"249_CR3","doi-asserted-by":"publisher","first-page":"358","DOI":"10.1016\/j.ejvs.2006.04.014","volume":"32","author":"L Botta","year":"2006","unstructured":"Botta L, Russo V, Grigioni F, Arpesella G, Rocchi G, Di Bartolomeo R, et al. Unusual rapid evolution of type B aortic dissection in a marfan patient following heart transplantation: successful endovascular treatment. Eur J Vasc Endovasc Surg. 2006;32(4):358\u201360.","journal-title":"Eur J Vasc Endovasc Surg"},{"issue":"4","key":"249_CR4","doi-asserted-by":"publisher","first-page":"1174","DOI":"10.1016\/j.transproceed.2012.02.013","volume":"44","author":"J Wei","year":"2012","unstructured":"Wei J, Sue SH, Lee YT, Chang CY. Combined heart transplantation and total replacement of thoracic aorta in Marfan\u2019s syndrome with recurrent aortic dissection: a case report. Transplant Proc. 2012;44(4):1174\u20135.","journal-title":"Transplant Proc"},{"issue":"5","key":"249_CR5","first-page":"899","volume":"13","author":"KA Kesler","year":"1994","unstructured":"Kesler KA, Hanosh JJ, O\u2019Donnell J, Faust S, Turrentine MW, Mahomed Y, et al. Heart transplantation in patients with Marfan\u2019s syndrome: a survey of attitudes and results. 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J Heart Lung Transpl. 2020;39:S225.","journal-title":"J Heart Lung Transpl"},{"issue":"9","key":"249_CR8","doi-asserted-by":"publisher","first-page":"958","DOI":"10.1016\/j.healun.2009.05.012","volume":"28","author":"K Rajagopal","year":"2009","unstructured":"Rajagopal K, Rogers JG, Lodge AJ, Gaca JG, McCann RL, Milano CA, et al. Two-stage total cardioaortic replacement for end-stage heart and aortic disease in Marfan syndrome: case report and review of the literature. J Heart Lung Transpl. 2009;28(9):958\u201363.","journal-title":"J Heart Lung Transpl"},{"issue":"9","key":"249_CR9","doi-asserted-by":"publisher","first-page":"711","DOI":"10.1056\/NEJM200202283460918","volume":"346","author":"M Pasic","year":"2002","unstructured":"Pasic M, Hummel M, Hetzer R. Combined aortic surgery and implantation of a left ventricular assist device. N Engl J Med. 2002;346(9):711.","journal-title":"N Engl J Med"},{"key":"249_CR10","doi-asserted-by":"publisher","first-page":"bcr2015211138","DOI":"10.1136\/bcr-2015-211138","volume":"2015","author":"T Audenaert","year":"2015","unstructured":"Audenaert T, De Pauw M, Fran\u00e7ois K, De Backer J. Type B aortic dissection triggered by heart transplantation in a patient with Marfan syndrome. BMJ Case Rep. 2015;2015:bcr2015211138.","journal-title":"BMJ Case Rep"},{"key":"249_CR11","first-page":"1","volume":"0","author":"S Korukonda","year":"2025","unstructured":"Korukonda S, Byers PH, Kovuri P, Dhanekula AS, Wilcox KT, DeRoo S. Genotype-aortic phenotype correlations in Marfan syndrome: a systematic review and meta-analysis of Fibrillin-1 variants. Heart. 2025;0:1\u20137.","journal-title":"Heart"}],"container-title":["General Thoracic and Cardiovascular Surgery Cases"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/link.springer.com\/article\/10.1186\/s44215-026-00249-2","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1186\/s44215-026-00249-2.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1186\/s44215-026-00249-2.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,6,16]],"date-time":"2026-06-16T22:16:10Z","timestamp":1781648170000},"score":1,"resource":{"primary":{"URL":"https:\/\/link.springer.com\/10.1186\/s44215-026-00249-2"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,4,17]]},"references-count":11,"journal-issue":{"issue":"1","published-online":{"date-parts":[[2026,12]]}},"alternative-id":["249"],"URL":"https:\/\/doi.org\/10.1186\/s44215-026-00249-2","relation":{},"ISSN":["2731-6203"],"issn-type":[{"value":"2731-6203","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,4,17]]},"assertion":[{"value":"16 December 2025","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"6 March 2026","order":2,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"17 April 2026","order":3,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Declarations"}},{"value":"This study was approved by the Ethics Committee of Tohoku University Hospital and was performed in accordance with the 1964 Declaration of Helsinki and its later amendments (Protocol identification number: 42798).","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Ethics approval and consent to participate"}},{"value":"Written informed consent to publish the details of this case was obtained from the patient.","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":"22"}}