{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,10]],"date-time":"2025-10-10T01:39:16Z","timestamp":1760060356386,"version":"build-2065373602"},"reference-count":18,"publisher":"MDPI AG","issue":"3","license":[{"start":{"date-parts":[[2025,8,26]],"date-time":"2025-08-26T00:00:00Z","timestamp":1756166400000},"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: Left ventricular hypoplasia is often repaired surgically in sequence to a Fontan circulation, which is a physiologic state that presents unique challenges during pregnancy. Although women with Fontan physiology can achieve successful pregnancy outcomes, they remain at elevated risk for cardiac, thrombotic, and obstetric complications. Case Presentation: We describe a 38-year-old woman with Fontan physiology and acquired von Willebrand syndrome (AVWS) who was admitted at 23 weeks gestation for preterm premature rupture of membranes. The patient had history of prior classical cesarean delivery and two previous miscarriages. Her pregnancy was further complicated by abnormal placental vasculature and uterine arteriovenous malformation. Given her bleeding diathesis, hematology advised against anticoagulation or antiplatelet therapy, and she ultimately underwent a successful low transverse cesarean delivery under general anesthesia at 24 weeks. Postpartum hemorrhage was managed with clotting factor replacement and supportive care. Conclusions: This case illustrates how AVWS may mitigate thrombotic risk in Fontan physiology and how early activation of a cardio-obstetrics team can enable tailored planning. As more patients with complex congenital heart disease reach reproductive age, multidisciplinary coordination, shared infrastructure, and individualized birth plans will be essential to achieving optimal maternal\u2013fetal outcomes.<\/jats:p>","DOI":"10.3390\/reports8030157","type":"journal-article","created":{"date-parts":[[2025,8,26]],"date-time":"2025-08-26T10:35:17Z","timestamp":1756204517000},"page":"157","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Managing High Risk Pregnancy in Single Ventricle Physiology with Acquired von Willebrand Disease: A Case Report"],"prefix":"10.3390","volume":"8","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-7777-7131","authenticated-orcid":false,"given":"Yash","family":"Nagpal","sequence":"first","affiliation":[{"name":"Adult Congenital Heart Disease Center, Memorial Cardiac and Vascular Institute, Joe DiMaggio Children\u2019s Hospital, 3501 Johnson St., Hollywood, FL 33021, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0009-0000-6918-773X","authenticated-orcid":false,"given":"Nisha","family":"Chachad","sequence":"additional","affiliation":[{"name":"Adult Congenital Heart Disease Center, Memorial Cardiac and Vascular Institute, Joe DiMaggio Children\u2019s Hospital, 3501 Johnson St., Hollywood, FL 33021, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Paola Andrea","family":"Benito","sequence":"additional","affiliation":[{"name":"Adult Congenital Heart Disease Center, Memorial Cardiac and Vascular Institute, Joe DiMaggio Children\u2019s Hospital, 3501 Johnson St., Hollywood, FL 33021, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Todd Stuart","family":"Roth","sequence":"additional","affiliation":[{"name":"Adult Congenital Heart Disease Center, Memorial Cardiac and Vascular Institute, Joe DiMaggio Children\u2019s Hospital, 3501 Johnson St., Hollywood, FL 33021, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1896-6423","authenticated-orcid":false,"given":"Joshua","family":"Saef","sequence":"additional","affiliation":[{"name":"Adult Congenital Heart Disease Center, Memorial Cardiac and Vascular Institute, Joe DiMaggio Children\u2019s Hospital, 3501 Johnson St., Hollywood, FL 33021, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2025,8,26]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"142","DOI":"10.1038\/pr.2016.194","article-title":"Hypoplastic left heart syndrome: From comfort care to long-term survival","volume":"81","author":"Yabrodi","year":"2017","journal-title":"Pediatr. Res."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"945","DOI":"10.1016\/j.pcl.2020.06.008","article-title":"Outcomes in Hypoplastic Left Heart Syndrome","volume":"67","author":"Metcalf","year":"2020","journal-title":"Pediatr. Clin. N. Am."},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"S1","DOI":"10.1016\/j.jacc.2011.09.022","article-title":"Hypoplastic left heart syndrome: Current considerations and expectations","volume":"59","author":"Feinstein","year":"2012","journal-title":"J. Am. Coll. Cardiol."},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"1290","DOI":"10.1136\/hrt.2005.085407","article-title":"Pregnancy and delivery in women after Fontan palliation","volume":"92","author":"Drenthen","year":"2006","journal-title":"Heart"},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"132445","DOI":"10.1016\/j.ijcard.2024.132445","article-title":"Pregnancy in women with a Fontan circulation: Short and long-term outcomes","volume":"415","author":"Breviario","year":"2024","journal-title":"Int. J. Cardiol."},{"doi-asserted-by":"crossref","unstructured":"Bartczak-Rutkowska, A., Tomkiewicz-Paj\u0105k, L., Kawka-Paciorkowska, K., Bajorek, N., Ciep\u0142ucha, A., Ropacka-Lesiak, M., and Trojnarska, O. (2023). Pregnancy Outcomes in Women after the Fontan Procedure. J. Clin. Med., 12.","key":"ref_6","DOI":"10.3390\/jcm12030783"},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"93","DOI":"10.1017\/S1047951111001661","article-title":"Longer-term issues for young adults with hypoplastic left heart syndrome: Contraception, pregnancy, transition, transfer, counselling, and re-operation","volume":"21","author":"Said","year":"2011","journal-title":"Cardiol. Young"},{"doi-asserted-by":"crossref","unstructured":"Panebianco, M., Perrone, M.A., Gagliardi, M.G., Galletti, L., and Bassareo, P.P. (2023). Pregnancy in Patients with Moderate and Highly Complex Congenital Heart Disease. Healthcare, 11.","key":"ref_8","DOI":"10.3390\/healthcare11111592"},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"e026087","DOI":"10.1161\/JAHA.122.026087","article-title":"Impact of Fontan fenestration on long-term outcomes: A propensity score\u2013matched analysis","volume":"11","author":"Daley","year":"2022","journal-title":"J. Am. Heart Assoc."},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"97","DOI":"10.1016\/j.ijcard.2016.01.180","article-title":"Fertility and pregnancy in the Fontan population","volume":"208","author":"Zentner","year":"2016","journal-title":"Int. J. Cardiol."},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"739","DOI":"10.1016\/j.ijcard.2014.07.104","article-title":"Acquired von Willebrand syndrome in adult patients with congenital heart disease","volume":"176","author":"Waldow","year":"2014","journal-title":"Int. J. Cardiol."},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"711","DOI":"10.1177\/2150135116658011","article-title":"Acquired von Willebrand Syndrome: An Under-Recognized Cause of Major Bleeding in the Cardiac Intensive Care Unit","volume":"7","author":"Jones","year":"2016","journal-title":"World J. Pediatr. Congenit. Heart Surg."},{"key":"ref_13","first-page":"100328","article-title":"Thromboembolic characteristics and role of anticoagulation in long-standing Fontan circulation","volume":"7","author":"Klages","year":"2022","journal-title":"Int. J. Cardiol. Congenit. Heart Dis."},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"374","DOI":"10.1016\/j.jacc.2022.10.037","article-title":"Thromboprophylaxis in Patients with Fontan Circulation","volume":"81","author":"Eynde","year":"2023","journal-title":"JACC"},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"84","DOI":"10.1016\/j.bjae.2020.11.004","article-title":"Regional anaesthesia in patients at risk of bleeding","volume":"21","author":"Ashken","year":"2021","journal-title":"BJA Educ."},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"e26","DOI":"10.1002\/bjs.11393","article-title":"Strategies to minimize intraoperative blood loss during major surgery","volume":"107","author":"Shah","year":"2020","journal-title":"Br. J. Surg."},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"144","DOI":"10.1093\/icvts\/ivt110","article-title":"Is vaginal delivery or caesarean section the safer mode of delivery in patients with adult congenital heart disease?","volume":"17","author":"Asfour","year":"2013","journal-title":"Interact. Cardiovasc. Thorac. Surg."},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"100921","DOI":"10.1016\/j.ajogmf.2023.100921","article-title":"Delivery trends and obstetric outcomes in patients with Fontan circulation","volume":"5","author":"Sobhani","year":"2023","journal-title":"Am. J. Obstet. Gynecol. MFM"}],"container-title":["Reports"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2571-841X\/8\/3\/157\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,9]],"date-time":"2025-10-09T18:32:52Z","timestamp":1760034772000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2571-841X\/8\/3\/157"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,8,26]]},"references-count":18,"journal-issue":{"issue":"3","published-online":{"date-parts":[[2025,9]]}},"alternative-id":["reports8030157"],"URL":"https:\/\/doi.org\/10.3390\/reports8030157","relation":{},"ISSN":["2571-841X"],"issn-type":[{"type":"electronic","value":"2571-841X"}],"subject":[],"published":{"date-parts":[[2025,8,26]]}}}