{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,14]],"date-time":"2025-10-14T00:49:42Z","timestamp":1760402982835,"version":"build-2065373602"},"reference-count":44,"publisher":"MDPI AG","issue":"4","license":[{"start":{"date-parts":[[2020,4,15]],"date-time":"2020-04-15T00:00:00Z","timestamp":1586908800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["JCM"],"abstract":"<jats:p>Pregnancy poses a threat to women with aortopathy. Conclusive data on the obstetric and aortic outcome in this risk collective, especially when it comes to aortic complications in the long term, are still missing. This study offers a comparative analysis of pregnancy-associated outcome in 113 consecutive women with Marfan syndrome or bicuspid aortic valve disease, including 46 ever-pregnant and 37 never-pregnant women with Marfan syndrome, and 23 ever-pregnant and 7 never-pregnant females with bicuspid aortic valve disease. The overall obstetric outcome was comparable between ever-pregnant women with Marfan syndrome and with bicuspid aortic valve disease (p = 0.112). Pregnancy-associated aortic dissection occurred in two women with Marfan syndrome (3%) during a total of 62 completed pregnancies, whereas no single case of aortic event occurred in women with bicuspid aortic valve disease during a total of 36 completed pregnancies (p = 0.530). In the long-term follow-up, aortic dissection occurred in 21% of ever-pregnant women with Marfan syndrome, but in none of the women with bicuspid aortic valve disease (p = 0.022). Proximal aortic surgery was performed with similar frequency in ever-pregnant women with Marfan syndrome and with bicuspid aortic valve disease in the long term (p = 0.252). However, ever-pregnant women with Marfan syndrome were younger when surgery was performed (44 \u00b1 9 vs. 59 \u00b1 7 years; p = 0.041). In Marfan syndrome, long-term growth of the aorta was comparable between ever-pregnant and never-pregnant women. Pregnancy thus exhibited an increased immediate aortic risk only in women with Marfan syndrome, but not in women with bicuspid aortic valve disease. Previous pregnancy did not relate to an increased long-term risk of adverse aortic events in women with Marfan syndrome or with bicuspid aortic valve disease.<\/jats:p>","DOI":"10.3390\/jcm9041124","type":"journal-article","created":{"date-parts":[[2020,4,15]],"date-time":"2020-04-15T09:19:50Z","timestamp":1586942390000},"page":"1124","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":3,"title":["Marfan Syndrome Versus Bicuspid Aortic Valve Disease: Comparative Analysis of Obstetric Outcome and Pregnancy-Associated Immediate and Long-Term Aortic Complications"],"prefix":"10.3390","volume":"9","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-5233-0680","authenticated-orcid":false,"given":"Bet\u00fcl","family":"Toprak","sequence":"first","affiliation":[{"name":"Department of Cardiology, University Heart and Vascular Center Hamburg, University Medical Center Hamburg-Hospital Eppendorf, 20246 Hamburg, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5992-4633","authenticated-orcid":false,"given":"Katalin","family":"Sz\u00f6cs","sequence":"additional","affiliation":[{"name":"Department of Cardiology, University Heart and Vascular Center Hamburg, University Medical Center Hamburg-Hospital Eppendorf, 20246 Hamburg, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5351-9162","authenticated-orcid":false,"given":"Elvin","family":"Zengin-Sahm","sequence":"additional","affiliation":[{"name":"Department of Cardiology, University Heart and Vascular Center Hamburg, University Medical Center Hamburg-Hospital Eppendorf, 20246 Hamburg, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7331-8840","authenticated-orcid":false,"given":"Christoph","family":"Sinning","sequence":"additional","affiliation":[{"name":"Department of Cardiology, University Heart and Vascular Center Hamburg, University Medical Center Hamburg-Hospital Eppendorf, 20246 Hamburg, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Amra","family":"Hot","sequence":"additional","affiliation":[{"name":"Department of Medical Biometry and Epidemiology, University Medical Center Hamburg-Hospital Eppendorf, 20246 Hamburg, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7102-534X","authenticated-orcid":false,"given":"Peter","family":"Bannas","sequence":"additional","affiliation":[{"name":"Department of Diagnostic and Interventional Radiology, University Medical Center Hamburg-Hospital Eppendorf, 20246 Hamburg, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kurt","family":"Hecher","sequence":"additional","affiliation":[{"name":"Department of Obstetrics and Fetal Medicine, University Medical Center Hamburg-Hospital Eppendorf, 20246 Hamburg, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Bernd","family":"H\u00fcneke","sequence":"additional","affiliation":[{"name":"Department of Obstetrics and Fetal Medicine, University Medical Center Hamburg-Hospital Eppendorf, 20246 Hamburg, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Thomas S.","family":"Mir","sequence":"additional","affiliation":[{"name":"Department of Pediatric Cardiology, University Heart and Vascular Center Hamburg, University Medical Center Hamburg-Hospital Eppendorf, 20246 Hamburg, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Meike","family":"Rybczynski","sequence":"additional","affiliation":[{"name":"Department of Cardiology, University Heart and Vascular Center Hamburg, University Medical Center Hamburg-Hospital Eppendorf, 20246 Hamburg, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Evaldas","family":"Girdauskas","sequence":"additional","affiliation":[{"name":"Department of Cardiovascular Surgery, University Heart and Vascular Center Hamburg, University Medical Center Hamburg-Hospital Eppendorf, 20246 Hamburg, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Stefan","family":"Blankenberg","sequence":"additional","affiliation":[{"name":"Department of Cardiology, University Heart and Vascular Center Hamburg, University Medical Center Hamburg-Hospital Eppendorf, 20246 Hamburg, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yskert","family":"von Kodolitsch","sequence":"additional","affiliation":[{"name":"Department of Cardiology, University Heart and Vascular Center Hamburg, University Medical Center Hamburg-Hospital Eppendorf, 20246 Hamburg, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2020,4,15]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"3165","DOI":"10.1093\/eurheartj\/ehy340","article-title":"2018 ESC Guidelines for the management of cardiovascular diseases during pregnancy","volume":"39","author":"Bauersachs","year":"2018","journal-title":"Eur. Heart J."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"1126","DOI":"10.1016\/j.lpm.2015.02.024","article-title":"Thoracic aortic aneurysms and pregnancy","volume":"44","author":"Coulon","year":"2015","journal-title":"Presse M\u00e9dicale"},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"797","DOI":"10.1007\/s00404-014-3307-4","article-title":"Management of the pregnant woman with Marfan syndrome complicated by ascending aorta dilation","volume":"290","author":"Cox","year":"2014","journal-title":"Arch. Gynecol. Obstet."},{"key":"ref_4","first-page":"336","article-title":"Histopathologic findings in human aortic media associated with pregnancy","volume":"83","author":"Barker","year":"1967","journal-title":"Arch. Pathol."},{"key":"ref_5","first-page":"1073","article-title":"Maternal hemodynamics and aortic diameter in normal and hypertensive pregnancies","volume":"78","author":"Easterling","year":"1991","journal-title":"Obstet. Gynecol."},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"470","DOI":"10.1016\/0741-5214(88)90113-9","article-title":"Aortic disease associated with pregnancy","volume":"8","author":"Williams","year":"1988","journal-title":"J. Vasc. Surg."},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"318","DOI":"10.1016\/j.tjog.2013.06.003","article-title":"Postpartum aortic dissection","volume":"52","author":"Yuan","year":"2013","journal-title":"Taiwan J. Obstet. Gynecol."},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"120","DOI":"10.1016\/j.ejogrb.2009.10.029","article-title":"Aortic dissection in pregnancy: The incidence of a life-threatening disease","volume":"149","author":"Nasiell","year":"2010","journal-title":"Eur. J. Obstet. Gynecol. Reprod. Biol."},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"337","DOI":"10.1097\/00001703-199710000-00014","article-title":"Marfan syndrome in pregnancy","volume":"9","author":"Simpson","year":"1997","journal-title":"Curr. Opin. Obstet. Gynecol."},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"537","DOI":"10.1016\/j.bpobgyn.2014.03.007","article-title":"Aorta pathology and pregnancy","volume":"28","year":"2014","journal-title":"Best Pract. Res. Clin. Obstet. Gynaecol."},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"112","DOI":"10.1177\/1753495X13496237","article-title":"Marfan\u2019s syndrome and other aortopathies in pregnancy","volume":"6","author":"Stewart","year":"2013","journal-title":"Obstet. Med."},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"699","DOI":"10.1097\/OGX.0b013e31823cdd50","article-title":"Management of pregnancy in women with genetic disorders, Part 1: Disorders of the connective tissue, muscle, vascular, and skeletal systems","volume":"66","author":"Chetty","year":"2011","journal-title":"Obstet. Gynecol. Surv."},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"348","DOI":"10.1016\/j.ijoa.2014.05.001","article-title":"Outcomes in pregnant women with acute aortic dissections: A review of the literature from 2003 to 2013","volume":"23","author":"Rajagopalan","year":"2014","journal-title":"Int. J. Obstet. Anesth."},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"576","DOI":"10.1002\/clc.22165","article-title":"Aortic dissection during pregnancy: A difficult clinical scenario","volume":"36","author":"Yuan","year":"2013","journal-title":"Clin. Cardiol."},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"914","DOI":"10.1093\/eurheartj\/ehi103","article-title":"Pregnancy and aortic root growth in the Marfan syndrome: A prospective study","volume":"26","author":"Meijboom","year":"2005","journal-title":"Eur. Heart J."},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"224","DOI":"10.1016\/j.jacc.2012.03.051","article-title":"The immediate and long-term impact of pregnancy on aortic growth rate and mortality in women with Marfan syndrome","volume":"60","author":"Donnelly","year":"2012","journal-title":"J. Am. Coll. Cardiol."},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"610","DOI":"10.1111\/1471-0528.12515","article-title":"Marfan syndrome and pregnancy: Maternal and neonatal outcomes","volume":"121","author":"Curry","year":"2014","journal-title":"BJOG"},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"53","DOI":"10.1016\/j.ijcard.2005.07.017","article-title":"Obstetric complications in Marfan syndrome","volume":"110","author":"Meijboom","year":"2006","journal-title":"Int. J. Cardiol."},{"key":"ref_19","doi-asserted-by":"crossref","unstructured":"Niwa, K., and Kaemmerer, H. (2017). Bicuspid aortic valve. Aortopathy, Springer.","DOI":"10.1007\/978-4-431-56071-5"},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"81","DOI":"10.1136\/heart.83.1.81","article-title":"Clinical significance of the bicuspid aortic valve","volume":"83","author":"Ward","year":"2000","journal-title":"Heart"},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"831","DOI":"10.1093\/ejcts\/ezu319","article-title":"Bicuspid aortic valve: Phosphorylation of c-Kit and downstream targets are prognostic for future aortopathy","volume":"46","author":"Grewal","year":"2014","journal-title":"Eur. J. Cardiothorac. Surg."},{"key":"ref_22","doi-asserted-by":"crossref","first-page":"96","DOI":"10.1016\/j.amjcard.2010.08.061","article-title":"Frequency of cardiovascular events in women with a congenitally bicuspid aortic valve in a single community and effect of pregnancy on events","volume":"107","author":"McKellar","year":"2011","journal-title":"Am. J. Cardiol."},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"476","DOI":"10.1136\/jmg.2009.072785","article-title":"The revised Ghent nosology for the Marfan syndrome","volume":"47","author":"Loeys","year":"2010","journal-title":"J. Med. Genet."},{"key":"ref_24","doi-asserted-by":"crossref","first-page":"718","DOI":"10.1016\/j.amjcard.2005.04.051","article-title":"Frequency of bicuspid aortic valve in young male conscripts by echocardiogram","volume":"96","author":"Nistri","year":"2005","journal-title":"Am. J. Cardiol."},{"key":"ref_25","doi-asserted-by":"crossref","first-page":"344","DOI":"10.1016\/j.jclinepi.2007.11.008","article-title":"The Strengthening the Reporting of Observational Studies in Epidemiology (STROBE) statement: Guidelines for reporting observational studies","volume":"61","author":"Altman","year":"2008","journal-title":"J. Clin. Epidemiol."},{"key":"ref_26","first-page":"303","article-title":"A formula to estimate the approximate surface area if height and weight be known. 1916","volume":"5","year":"1989","journal-title":"Nutrition"},{"key":"ref_27","doi-asserted-by":"crossref","first-page":"42","DOI":"10.1002\/ajmg.a.34386","article-title":"The 8th international research symposium on the Marfan syndrome and related conditions","volume":"158","author":"Pyeritz","year":"2012","journal-title":"Am. J. Med. Genet. A"},{"key":"ref_28","doi-asserted-by":"crossref","first-page":"1634","DOI":"10.1136\/hrt.2007.132092","article-title":"The bicuspid aortic valve: An integrated phenotypic classification of leaflet morphology and aortic root shape","volume":"94","author":"Schaefer","year":"2008","journal-title":"Heart"},{"key":"ref_29","doi-asserted-by":"crossref","first-page":"2873","DOI":"10.1093\/eurheartj\/ehu281","article-title":"2014 ESC Guidelines on the diagnosis and treatment of aortic diseases: Document covering acute and chronic aortic diseases of the thoracic and abdominal aorta of the adult. The Task Force for the Diagnosis and Treatment of Aortic Diseases of the European Society of Cardiology (ESC)","volume":"35","author":"Erbel","year":"2014","journal-title":"Eur. Heart J."},{"key":"ref_30","doi-asserted-by":"crossref","first-page":"e27","DOI":"10.1016\/j.jacc.2010.02.015","article-title":"2010 ACCF\/AHA\/AATS\/ACR\/ASA\/SCA\/SCAI\/SIR\/STS\/SVM Guidelines for the diagnosis and management of patients with thoracic aortic disease. A Report of the American College of Cardiology Foundation\/American Heart Association Task Force on Practice Guidelines, American Association for Thoracic Surgery, American College of Radiology, American Stroke Association, Society of Cardiovascular Anesthesiologists, Society for Cardiovascular Angiography and Interventions, Society of Interventional Radiology, Society of Thoracic Surgeons, and Society for Vascular Medicine","volume":"55","author":"Hiratzka","year":"2010","journal-title":"J. Am. Coll. Cardiol."},{"key":"ref_31","doi-asserted-by":"crossref","first-page":"617","DOI":"10.1016\/S0022-5223(19)34942-6","article-title":"An aortic valve-sparing operation for patients with aortic incompetence and aneurysm of the ascending aorta","volume":"103","author":"David","year":"1992","journal-title":"J. Thorac. Cardiovasc. Surg."},{"key":"ref_32","doi-asserted-by":"crossref","unstructured":"Hetzer, R., Gehle, P., and Ennker, J. (1995). Management of aortic valve incompetence in patients with Marfan syndrome. Cardiovascular Aspects of Marfan Syndrome, Steinkopff Verlag.","DOI":"10.1007\/978-3-642-72508-1"},{"key":"ref_33","first-page":"1052","article-title":"Comparison of aortic root replacement in patients with Marfan syndrome","volume":"40","author":"Bernhardt","year":"2011","journal-title":"Eur. J. Cardiothorac. Surg."},{"key":"ref_34","doi-asserted-by":"crossref","first-page":"295","DOI":"10.1053\/j.semperi.2014.04.019","article-title":"Aortopathy in pregnancy","volume":"38","author":"Smok","year":"2014","journal-title":"Semin. Perinatol."},{"key":"ref_35","doi-asserted-by":"crossref","first-page":"e004052","DOI":"10.1161\/JAHA.116.004052","article-title":"Aortic Complications Associated With Pregnancy in Marfan Syndrome: The NHLBI National Registry of Genetically Triggered Thoracic Aortic Aneurysms and Cardiovascular Conditions (GenTAC)","volume":"5","author":"Roman","year":"2016","journal-title":"J. Am. Heart Assoc."},{"key":"ref_36","doi-asserted-by":"crossref","first-page":"1599","DOI":"10.1016\/0002-9378(95)90655-X","article-title":"A prospective longitudinal evaluation of pregnancy in the Marfan syndrome","volume":"173","author":"Rossiter","year":"1995","journal-title":"Am. J. Obstet. Gynecol."},{"key":"ref_37","doi-asserted-by":"crossref","first-page":"795","DOI":"10.1007\/s00380-015-0703-z","article-title":"Histopathology of aortic complications in bicuspid aortic valve versus Marfan syndrome: Relevance for therapy?","volume":"31","author":"Grewal","year":"2016","journal-title":"Heart Vessel."},{"key":"ref_38","doi-asserted-by":"crossref","first-page":"126","DOI":"10.1136\/heartjnl-2013-304920","article-title":"Aortic dilatation patterns and rates in adults with bicuspid aortic valves: A comparative study with Marfan syndrome and degenerative aortopathy","volume":"100","author":"Detaint","year":"2014","journal-title":"Heart"},{"key":"ref_39","doi-asserted-by":"crossref","first-page":"780","DOI":"10.21037\/cdt.2018.09.20","article-title":"Bicuspid aortic valve and aortic coarctation in congenital heart disease-important aspects for treatment with focus on aortic vasculopathy","volume":"8","author":"Sinning","year":"2018","journal-title":"Cardiovasc. Diagn. Ther."},{"key":"ref_40","doi-asserted-by":"crossref","first-page":"1048","DOI":"10.1016\/j.amjcard.2010.05.038","article-title":"Frequency and age-related course of mitral valve dysfunction in the Marfan syndrome","volume":"106","author":"Rybczynski","year":"2010","journal-title":"Am. J. Cardiol."},{"key":"ref_41","doi-asserted-by":"crossref","first-page":"251","DOI":"10.1111\/chd.12465","article-title":"Pregnancy-related acute aortic dissection in Marfan syndrome: A review of the literature","volume":"12","author":"Smith","year":"2017","journal-title":"Congenit. Heart Dis."},{"key":"ref_42","doi-asserted-by":"crossref","first-page":"2363","DOI":"10.1016\/j.jacc.2015.03.575","article-title":"Long-Term Risk for Aortic Complications After Aortic Valve Replacement in Patients With Bicuspid Aortic Valve Versus Marfan Syndrome","volume":"65","author":"Itagaki","year":"2015","journal-title":"J. Am. Coll. Cardiol."},{"key":"ref_43","doi-asserted-by":"crossref","first-page":"156","DOI":"10.1016\/j.ijcard.2008.04.035","article-title":"Maternal complication of pregnancy in Marfan syndrome","volume":"136","author":"Pacini","year":"2009","journal-title":"Int. J. Cardiol."},{"key":"ref_44","doi-asserted-by":"crossref","first-page":"1846","DOI":"10.1161\/CIRCULATIONAHA.117.031264","article-title":"Insights From the International Registry of Acute Aortic Dissection: A 20-Year Experience of Collaborative Clinical Research","volume":"137","author":"Evangelista","year":"2018","journal-title":"Circulation"}],"container-title":["Journal of Clinical Medicine"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2077-0383\/9\/4\/1124\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,13]],"date-time":"2025-10-13T14:09:04Z","timestamp":1760364544000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2077-0383\/9\/4\/1124"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2020,4,15]]},"references-count":44,"journal-issue":{"issue":"4","published-online":{"date-parts":[[2020,4]]}},"alternative-id":["jcm9041124"],"URL":"https:\/\/doi.org\/10.3390\/jcm9041124","relation":{},"ISSN":["2077-0383"],"issn-type":[{"type":"electronic","value":"2077-0383"}],"subject":[],"published":{"date-parts":[[2020,4,15]]}}}