{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,7]],"date-time":"2026-08-07T15:54:23Z","timestamp":1786118063988,"version":"build-2736575974"},"reference-count":19,"publisher":"MDPI AG","issue":"3","license":[{"start":{"date-parts":[[2026,8,6]],"date-time":"2026-08-06T00:00:00Z","timestamp":1785974400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Reports"],"abstract":"<jats:p>Background and Clinical Significance: Budd\u2013Chiari syndrome (BCS) is a rare disorder characterized by hepatic venous outflow obstruction, often associated with underlying hypercoagulable states. Pregnancy represents a physiologic prothrombotic condition that may precipitate disease onset. Case Presentation: We report a case of de novo BCS diagnosed in the second trimester in a previously healthy 36-year-old multiparous patient. Evaluation revealed cirrhotic liver morphology, portal hypertension, and bleeding esophageal varices requiring emergent treatment. The patient\u2019s course included a transjugular intrahepatic portosystemic shunt (TIPS) procedure with complications, anticoagulation, multidisciplinary care, and a work-up revealing a JAK2 mutation consistent with an underlying myeloproliferative disorder. Despite apparent maternal stabilization and reassuring fetal growth, the pregnancy resulted in intrauterine fetal demise at 34 weeks\u2019 due to placental abruption, followed by postpartum hemorrhage. Conclusions: This case highlights the diagnostic and therapeutic challenges of BCS in pregnancy, and demonstrates that favorable maternal stabilization does not preclude severe obstetric complications.<\/jats:p>","DOI":"10.3390\/reports9030259","type":"journal-article","created":{"date-parts":[[2026,8,6]],"date-time":"2026-08-06T14:45:47Z","timestamp":1786027547000},"page":"259","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Budd\u2013Chiari Syndrome Manifesting in Pregnancy: Case Report and Review of Management and Outcomes"],"prefix":"10.3390","volume":"9","author":[{"given":"Hannah S.","family":"Foster","sequence":"first","affiliation":[{"name":"Department of Obstetrics and Gynecology, Hospital of the University of Pennsylvania, Philadelphia, PA 19146, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gregory W.","family":"Kirschen","sequence":"additional","affiliation":[{"name":"Department of Obstetrics and Gynecology, Hospital of the University of Pennsylvania, Philadelphia, PA 19146, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sheri","family":"Bechard","sequence":"additional","affiliation":[{"name":"Department of Obstetrics and Gynecology, Hospital of the University of Pennsylvania, Philadelphia, PA 19146, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2818-4572","authenticated-orcid":false,"given":"Kristin D.","family":"Gerson","sequence":"additional","affiliation":[{"name":"Department of Obstetrics and Gynecology, Hospital of the University of Pennsylvania, Philadelphia, PA 19146, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2026,8,6]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"1307","DOI":"10.1056\/NEJMra2207738","article-title":"Primary Budd-Chiari Syndrome","volume":"388","author":"Valla","year":"2023","journal-title":"N. Engl. J. Med."},{"key":"ref_2","doi-asserted-by":"crossref","unstructured":"American College of Obstetricians and Gynecologists\u2019 Committee on Practice Bulletins\u2014Obstetrics (2018). ACOG Practice Bulletin No. 196: Thromboembolism in Pregnancy. Obstet. Gynecol., 132, e1\u2013e17. Erratum in Obstet. Gynecol.\u00a02018, 132, 1068.","DOI":"10.1097\/AOG.0000000000002706"},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"608","DOI":"10.1111\/1471-0528.16915","article-title":"Pregnancy outcomes in women with Budd-Chiari syndrome or portal vein thrombosis\u2014Amulticentre retrospective cohort study","volume":"129","author":"Wiegers","year":"2022","journal-title":"BJOG"},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"47","DOI":"10.1016\/j.jhep.2009.02.028","article-title":"Pregnancy in women with known and treated Budd-Chiari syndrome: Maternal and fetal outcomes","volume":"51","author":"Rautou","year":"2009","journal-title":"J. Hepatol."},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"318","DOI":"10.1002\/hep.31559","article-title":"Reproductive Health and Liver Disease: Practice Guidance by the American Association for the Study of Liver Diseases","volume":"73","author":"Sarkar","year":"2021","journal-title":"Hepatology"},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"44","DOI":"10.14309\/ajg.0000000000001960","article-title":"Liver Disease During Pregnancy","volume":"117","author":"Reau","year":"2022","journal-title":"Am. J. Gastroenterol."},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"475","DOI":"10.5005\/jp-journals-10006-2093","article-title":"Management of Pregnancy in a Rare Case of Budd\u2013Chiari Syndrome: A Case Report","volume":"14","author":"Mishra","year":"2022","journal-title":"J. South Asian Feder. Obst. Gynaecol."},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"e237761","DOI":"10.1136\/bcr-2020-237761","article-title":"Budd-Chiari syndrome diagnosed with pregnancy in a patient with inherited thrombophilia","volume":"14","author":"Elkhateb","year":"2021","journal-title":"BMJ Case Rep."},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"945","DOI":"10.4254\/wjh.v9.i21.945","article-title":"Outcomes of pregnancy in patients with known Budd-Chiari syndrome","volume":"9","author":"Khan","year":"2017","journal-title":"World J. Hepatol."},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"103176","DOI":"10.1016\/j.jceh.2025.103176","article-title":"Budd-Chiari Syndrome and Pregnancy-A Review","volume":"16","author":"Giri","year":"2026","journal-title":"J. Clin. Exp. Hepatol."},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"1180","DOI":"10.1097\/HEP.0000000000000647","article-title":"AASLD Practice Guidance on risk stratification and management of portal hypertension and varices in cirrhosis","volume":"79","author":"Kaplan","year":"2024","journal-title":"Hepatology"},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"e173","DOI":"10.1097\/CCM.0000000000004192","article-title":"Guidelines for the Management of Adult Acute and Acute-on-Chronic Liver Failure in the ICU: Cardiovascular, Endocrine, Hematologic, Pulmonary, and Renal Considerations","volume":"48","author":"Nanchal","year":"2020","journal-title":"Crit. Care Med."},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"e1912666","DOI":"10.1001\/jamanetworkopen.2019.12666","article-title":"Association of Treatments for Myeloproliferative Neoplasms During Pregnancy With Birth Rates and Maternal Outcomes: A Systematic Review and Meta-analysis","volume":"2","author":"Maze","year":"2019","journal-title":"JAMA Netw. Open"},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"1670","DOI":"10.1016\/S0016-5085(96)70031-8","article-title":"Acute Budd-Chiari syndrome with fulminant hepatic failure in a pregnant woman with factor V Leiden mutation","volume":"111","author":"Fickert","year":"1996","journal-title":"Gastroenterology"},{"key":"ref_15","doi-asserted-by":"crossref","unstructured":"(2021). Antepartum Fetal Surveillance: ACOG Practice Bulletin, Number 229. Obstet. Gynecol., 137, e116\u2013e127.","DOI":"10.1097\/AOG.0000000000004410"},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"1205","DOI":"10.1007\/s00270-015-1053-z","article-title":"Transjugular Intrahepatic Portosystemic Shunt Placement During Pregnancy: A Case Series of Five Patients","volume":"38","author":"Ingraham","year":"2015","journal-title":"Cardiovasc. Interv. Radiol."},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"224","DOI":"10.1097\/HEP.0000000000000530","article-title":"AASLD Practice Guidance on the use of TIPS, variceal embolization, and retrograde transvenous obliteration in the management of variceal hemorrhage","volume":"79","author":"Lee","year":"2024","journal-title":"Hepatology"},{"key":"ref_18","doi-asserted-by":"crossref","unstructured":"Society for Maternal-Fetal Medicine (2021). Consult Series #53: Intrahepatic cholestasis of pregnancy. Am. J. Obstet. 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