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Pregnancy in women with a bicuspid aortic valve

Carol A Waksmonski, MD
Michael R Foley, MD
Section Editors
Heidi M Connolly, MD, FASE
Charles J Lockwood, MD, MHCM
Deputy Editor
Susan B Yeon, MD, JD, FACC


A bicuspid aortic valve has been estimated to occur in approximately 2 percent of the general population [1,2] with a 4:1 male predominance [2]. A bicuspid aortic valve may be functionally normal or it may be stenotic and/or regurgitant. Bicuspid aortic valve is the most common cause of isolated valvular aortic stenosis (AS) in adults and the most common cause of isolated aortic regurgitation (AR). Dilation of the ascending aorta is likely to coexist independent of valvular function [2,3]. (See "Clinical manifestations and diagnosis of bicuspid aortic valve in adults" and "Clinical manifestations and diagnosis of chronic aortic regurgitation in adults".)

Pregnancy in women with a bicuspid aortic valve will be reviewed here. Issues related to management of bicuspid AS and AR and general principles of congenital heart disease in pregnancy are discussed separately. (See "Management of adults with bicuspid aortic valve disease" and "Natural history and management of chronic aortic regurgitation in adults" and "Pregnancy in women with congenital heart disease: General principles".)


A bicuspid aortic valve is generally detected by echocardiography performed to evaluate the murmur of aortic stenosis (AS) and/or aortic regurgitation (AR) or an ejection click. An isolated, functionally normal bicuspid aortic valve is likely to go unrecognized in women of child-bearing age because auscultatory signs are inconspicuous or unrecognized [4]. Rarely, a bicuspid aortic valve is first identified during pregnancy because of dissection of the ascending aorta [5]. (See "Clinical manifestations and diagnosis of bicuspid aortic valve in adults" and 'Aortic aneurysm and risk of aortic dissection' below.)


Women with a bicuspid aortic valve should be counseled regarding potential risks and treatment prior to and during pregnancy. Potential risks that should be discussed include heritable congenital heart disease, aortic enlargement or dissection, and complications of aortic stenosis (AS) and/or aortic regurgitation (AR).

The specific role of counseling in women with aortic dilation is discussed below (see 'Counseling on aortic dilatation' below).


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Literature review current through: Sep 2016. | This topic last updated: Jul 19, 2016.
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  1. Roberts WC. The congenitally bicuspid aortic valve. A study of 85 autopsy cases. Am J Cardiol 1970; 26:72.
  2. Fedak PW, Verma S, David TE, et al. Clinical and pathophysiological implications of a bicuspid aortic valve. Circulation 2002; 106:900.
  3. Keane MG, Wiegers SE, Plappert T, et al. Bicuspid aortic valves are associated with aortic dilatation out of proportion to coexistent valvular lesions. Circulation 2000; 102:III35.
  4. Perloff, JK. The Clinical Recognition of Congenital Heart Disease, 6th ed, Elsevier/Saunders, Philadelphia 2012.
  5. Anderson RA, Fineron PW. Aortic dissection in pregnancy: importance of pregnancy-induced changes in the vessel wall and bicuspid aortic valve in pathogenesis. Br J Obstet Gynaecol 1994; 101:1085.
  6. Huntington K, Hunter AG, Chan KL. A prospective study to assess the frequency of familial clustering of congenital bicuspid aortic valve. J Am Coll Cardiol 1997; 30:1809.
  7. Cripe L, Andelfinger G, Martin LJ, et al. Bicuspid aortic valve is heritable. J Am Coll Cardiol 2004; 44:138.
  8. Warnes CA, Williams RG, Bashore TM, et al. ACC/AHA 2008 Guidelines for the Management of Adults with Congenital Heart Disease: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (writing committee to develop guidelines on the management of adults with congenital heart disease). Circulation 2008; 118:e714.
  9. Drenthen W, Pieper PG, Roos-Hesselink JW, et al. Outcome of pregnancy in women with congenital heart disease: a literature review. J Am Coll Cardiol 2007; 49:2303.
  10. Immer FF, Bansi AG, Immer-Bansi AS, et al. Aortic dissection in pregnancy: analysis of risk factors and outcome. Ann Thorac Surg 2003; 76:309.
  11. Tadros TM, Klein MD, Shapira OM. Ascending aortic dilatation associated with bicuspid aortic valve: pathophysiology, molecular biology, and clinical implications. Circulation 2009; 119:880.
  12. Hiratzka LF, Bakris GL, Beckman JA, et al. 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. Circulation 2010; 121:e266.
  13. Nishimura RA, Otto CM, Bonow RO, et al. 2014 AHA/ACC guideline for the management of patients with valvular heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines. J Am Coll Cardiol 2014; 63:e57.
  14. European Society of Gynecology (ESG), Association for European Paediatric Cardiology (AEPC), German Society for Gender Medicine (DGesGM), et al. ESC Guidelines on the management of cardiovascular diseases during pregnancy: the Task Force on the Management of Cardiovascular Diseases during Pregnancy of the European Society of Cardiology (ESC). Eur Heart J 2011; 32:3147.
  15. Donnelly RT, Pinto NM, Kocolas I, Yetman AT. The immediate and long-term impact of pregnancy on aortic growth rate and mortality in women with Marfan syndrome. J Am Coll Cardiol 2012; 60:224.
  16. Tzemos N, Silversides CK, Colman JM, et al. Late cardiac outcomes after pregnancy in women with congenital aortic stenosis. Am Heart J 2009; 157:474.
  17. Grewal J, Siu SC, Ross HJ, et al. Pregnancy outcomes in women with dilated cardiomyopathy. J Am Coll Cardiol 2009; 55:45.
  18. Prakash SK, Bossé Y, Muehlschlegel JD, et al. A roadmap to investigate the genetic basis of bicuspid aortic valve and its complications: insights from the International BAVCon (Bicuspid Aortic Valve Consortium). J Am Coll Cardiol 2014; 64:832.
  19. Easterling TR, Chadwick HS, Otto CM, Benedetti TJ. Aortic stenosis in pregnancy. Obstet Gynecol 1988; 72:113.
  20. Lao TT, Sermer M, MaGee L, et al. Congenital aortic stenosis and pregnancy--a reappraisal. Am J Obstet Gynecol 1993; 169:540.
  21. Hameed A, Karaalp IS, Tummala PP, et al. The effect of valvular heart disease on maternal and fetal outcome of pregnancy. J Am Coll Cardiol 2001; 37:893.
  22. Yap SC, Drenthen W, Pieper PG, et al. Risk of complications during pregnancy in women with congenital aortic stenosis. Int J Cardiol 2008; 126:240.
  23. Silversides CK, Kiess M, Beauchesne L, et al. Canadian Cardiovascular Society 2009 Consensus Conference on the management of adults with congenital heart disease: outflow tract obstruction, coarctation of the aorta, tetralogy of Fallot, Ebstein anomaly and Marfan's syndrome. Can J Cardiol 2010; 26:e80.
  24. Task Force on the Management of Cardiovascular Diseases During Pregnancy of the European Society of Cardiology. Expert consensus document on management of cardiovascular diseases during pregnancy. Eur Heart J 2003; 24:761.
  25. Banning AP, Pearson JF, Hall RJ. Role of balloon dilatation of the aortic valve in pregnant patients with severe aortic stenosis. Br Heart J 1993; 70:544.
  26. McIvor RA. Percutaneous balloon aortic valvuloplasty during pregnancy. Int J Cardiol 1991; 32:1.
  27. Lao TT, Adelman AG, Sermer M, Colman JM. Balloon valvuloplasty for congenital aortic stenosis in pregnancy. Br J Obstet Gynaecol 1993; 100:1141.
  28. Sheikh F, Rangwala S, DeSimone C, et al. Management of the parturient with severe aortic incompetence. J Cardiothorac Vasc Anesth 1995; 9:575.
  29. Elkayam U, Bitar F. Valvular heart disease and pregnancy part I: native valves. J Am Coll Cardiol 2005; 46:223.
  30. Dore A, Somerville J. Pregnancy in patients with pulmonary autograft valve replacement. Eur Heart J 1997; 18:1659.
  31. Kari FA, Siepe M, Sievers HH, Beyersdorf F. Repair of the regurgitant bicuspid or tricuspid aortic valve: background, principles, and outcomes. Circulation 2013; 128:854.
  32. Wilson W, Taubert KA, Gewitz M, et al. Prevention of Infective Endocarditis. Guidelines From the American Heart Association. A Guideline From the American Heart Association Rheumatic Fever, Endocarditis, and Kawasaki Disease Committee, Council on Cardiovascular Disease in the Young, and the Council on Clinical Cardiology, Council on Cardiovascular Surgery and Anesthesia, and the Quality of Care and Outcomes Research Interdisciplinary Working Group. Circulation 2007; 115 published online April 19, 2007. www.circ.ahajournals.org/cgi/reprint/CIRCULATIONAHA.106.183095v1 (Accessed on May 04, 2007).