Pregnancy in Adults with Congenital Heart Disease

  • Evin YucelEmail author
Part of the In Clinical Practice book series (ICP)


  • As a result of medical and surgical advances, there is an increasing number of women with congenital heart disease (CHD) surviving to childbearing age [1].

  • Maternal mortality is uncommon in patients with CHD, except in the setting of Eisenmenger syndrome, severe systemic cardiomyopathy, and Marfan syndrome with aortopathy.

  • Maternal hypoxemia is associated with high incidence of fetal loss, even in the absence of Eisenmenger physiology. In a series of 90 patients, the rate of live births decreased to below 20% when maternal resting oxygen was <85% [2].

  • A multidisciplinary approach to the pregnant woman with CHD is essential for favorable maternal and fetal outcomes.

  • In general, for women with CHD, cesarean delivery is only recommended for obstetric reasons and for women who are on full anticoagulation during delivery, due to the risk of fetal intracranial hemorrhage [3].

  • The recent American Heart Association (AHA) [4] and European Society of Cardiology [5] guidelines do not support the use of antibiotic prophylaxis during vaginal delivery, owing to the limited data. However, for those at highest risk of an adverse outcome, such as patients with Eisenmenger syndrome or cyanosis, it can be considered at the time of rupture of membranes [2].



American Heart Association


Canadian Cardiac Disease in Pregnancy


Congenital heart disease


Cardiac output


Heart rate


World Health Organization


Red blood cell


Systemic vascular resistance


Zwangerschap bij Aangeboren HARtAfwijkingen


  1. 1.
    Bhatt AB, DeFaria Yeh D. Pregnancy and adult congenital heart disease. Cardiol Clin. 2015;33:611–23, ix.CrossRefGoogle Scholar
  2. 2.
    Presbitero P, Somerville J, Stone S, Aruta E, Spiegelhalter D, Rabajoli F. Pregnancy in cyanotic congenital heart disease. Outcome of mother and fetus. Circulation. 1994;89(6):2673.CrossRefGoogle Scholar
  3. 3.
    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). Developed in Collaboration With the American Society of Echocardiography, Heart Rhythm Society, International Society for Adult Congenital Heart Disease, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons. J Am College Cardiol. 2008;52:e143–263.CrossRefGoogle Scholar
  4. 4.
    Canobbio MM, Warnes CA, Aboulhosn J, et al. Management of pregnancy in patients with complex congenital heart disease: a scientific statement for healthcare professionals from the American Heart Association. Circulation. 2017;135(8):e50–87.CrossRefGoogle Scholar
  5. 5.
    European Society of Gynecology, Association for European Paediatric Cardiology, German Society for Gender Medicine, 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;32:3147–97.CrossRefGoogle Scholar
  6. 6.
    Fujitani S, Baldisseri MR. Hemodynamic assessment in a pregnant and peripartum patient. Crit Care Med. 2005;33:S354–61.CrossRefGoogle Scholar
  7. 7.
    Robson SC, Hunter S, Moore M, Dunlop W. Haemodynamic changes during the puerperium: a Doppler and M-mode echocardiographic study. Br J Obstet Gynaecol. 1987;94:1028–39.CrossRefGoogle Scholar
  8. 8.
    Hankins GD, Wendel GD Jr, Leveno KJ, Stoneham J. Myocardial infarction during pregnancy: a review. Obstet Gynecol. 1985;65:139–46.PubMedGoogle Scholar
  9. 9.
    Hansen SL, Clark SL, Foster JC. Active pushing versus passive fetal descent in the second stage of labor: a randomized controlled trial. Obstet Gynecol. 2002;99:29–34.PubMedGoogle Scholar
  10. 10.
    Drenthen W, Pieper PG, Roos-Hesselink JW, et al. Outcome of pregnancy in women with congenital heart disease: a literature review. J Am College Cardiol. 2007;49:2303–11.CrossRefGoogle Scholar
  11. 11.
    Siu SC, Sermer M, Colman JM, et al. Prospective multicenter study of pregnancy outcomes in women with heart disease. Circulation. 2001;104:515–21.CrossRefGoogle Scholar
  12. 12.
    Drenthen W, Boersma E, Balci A, et al. Predictors of pregnancy complications in women with congenital heart disease. Eur Heart J. 2010;31:2124–32.CrossRefGoogle Scholar
  13. 13.
    Balci A, Sollie-Szarynska KM, van der Bijl AG, et al. Prospective validation and assessment of cardiovascular and offspring risk models for pregnant women with congenital heart disease. Heart. 2014;100:1373–81.CrossRefGoogle Scholar
  14. 14.
    van Hagen IM, Roos-Hesselink JW, Donvito V, et al. Incidence and predictors of obstetric and fetal complications in women with structural heart disease. Heart. 2017;103(20):1610–8.CrossRefGoogle Scholar
  15. 15.
    Zomer AC, Ionescu-Ittu R, Vaartjes I, et al. Sex differences in hospital mortality in adults with congenital heart disease: the impact of reproductive health. J Am College Cardiol. 2013;62:58–67.CrossRefGoogle Scholar
  16. 16.
    Greutmann M, Pieper PG. Pregnancy in women with congenital heart disease. Eur Heart J. 2015;36:2491–9.CrossRefGoogle Scholar
  17. 17.
    I. van De Laar MW. Inheritance of congenital heart disease. In: Roos-Hesselink JW, Johnson MR, editors. Pregnancy and congenital heart disease. Basel: Springer; 2017. p. 51–66.CrossRefGoogle Scholar
  18. 18.
    Cotrufo M, De Feo M, De Santo LS, et al. Risk of warfarin during pregnancy with mechanical valve prostheses. Obstet Gynecol. 2002;99:35–40.PubMedGoogle Scholar
  19. 19.
    Vitale N, De Feo M, De Santo LS, Pollice A, Tedesco N, Cotrufo M. Dose-dependent fetal complications of warfarin in pregnant women with mechanical heart valves. J Am College Cardiol. 1999;33:1637–41.CrossRefGoogle Scholar
  20. 20.
    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. Circulation. 2014;129:e521–643.PubMedGoogle Scholar
  21. 21.
    Ackerman MJ, Priori SG, Willems S, et al. HRS/EHRA expert consensus statement on the state of genetic testing for the channelopathies and cardiomyopathies: this document was developed as a partnership between the Heart Rhythm Society (HRS) and the European Heart Rhythm Association (EHRA). Europace. 2011;13:1077–109.CrossRefGoogle Scholar
  22. 22.
    Mahli A, Izdes S, Coskun D. Cardiac operations during pregnancy: review of factors influencing fetal outcome. Ann Thorac Surg. 2000;69:1622–6.CrossRefGoogle Scholar
  23. 23.
    Thorne S, MacGregor A, Nelson-Piercy C. Risks of contraception and pregnancy in heart disease. Heart. 2006;92:1520–5.CrossRefGoogle Scholar

Copyright information

© Springer International Publishing AG, part of Springer Nature 2018

Authors and Affiliations

  1. 1.Corrigan Minehan Heart Center, Massachusetts General HospitalBostonUSA

Personalised recommendations