Official reprint from UpToDate®
www.uptodate.com ©2018 UpToDate, Inc. and/or its affiliates. All Rights Reserved.

Pregnancy in women with congenital heart disease: Specific lesions

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


Risks associated with pregnancy in women with congenital heart disease affect both the mother and her fetus. The obstetrician and cardiologist are therefore responsible for the welfare of two patients.

The focus here is on management of pregnant women with specific congenital cardiac anomalies, both unrepaired and after reparative surgery. Other issues such as maternal risks, oxygen delivery, and management during labor and postpartum are discussed separately. (See "Pregnancy in women with congenital heart disease: General principles".)

Certain lesions such as coarctation of the aorta, tetralogy of Fallot, and Marfan syndrome are discussed elsewhere. (See "Management of coarctation of the aorta", section on 'Pregnancy' and "Management and outcome of tetralogy of Fallot", section on 'Pregnancy' and "Pregnancy and Marfan syndrome".)

Also covered separately are general cardiac issues concerning the management of arrhythmias, heart failure, valvular heart disease, and anticoagulation during pregnancy. (See "Supraventricular arrhythmias during pregnancy" and "Ventricular arrhythmias during pregnancy" and "Management of heart failure during pregnancy" and "Pregnancy and valve disease" and "Use of anticoagulants during pregnancy and postpartum".)


Reparative surgery has substantially increased the number of females with congenital heart disease who reach childbearing age. A study showed that the number of delivery hospitalizations with congenital heart disease increased significantly from 6.4 to 9 per 10,000 deliveries from 2000 to 2010 and that these deliveries had a greater than expected rate of medical and obstetric complications [1]. A review reported that congenital heart disease now comprises 80 percent of all pregnancies in women with heart conditions in the Western world [2]. Successful operation before gestation is pivotal in reducing maternal and fetal risks. The risks of pregnancy after congenital cardiac surgery are determined chiefly by the presence, type, and degree of cardiac and vascular residua and sequelae.

To continue reading this article, you must log in with your personal, hospital, or group practice subscription. For more information on subscription options, click below on the option that best describes you:

Subscribers log in here

Literature review current through: Dec 2017. | This topic last updated: Nov 03, 2017.
The content on the UpToDate website is not intended nor recommended as a substitute for medical advice, diagnosis, or treatment. Always seek the advice of your own physician or other qualified health care professional regarding any medical questions or conditions. The use of this website is governed by the UpToDate Terms of Use ©2018 UpToDate, Inc.
  1. Thompson JL, Kuklina EV, Bateman BT, et al. Medical and Obstetric Outcomes Among Pregnant Women With Congenital Heart Disease. Obstet Gynecol 2015; 126:346.
  2. Elkayam U, Goland S, Pieper PG, Silverside CK. High-Risk Cardiac Disease in Pregnancy: Part II. J Am Coll Cardiol 2016.
  3. Warnes CA, Williams RG, Bashore TM, et al. ACC/AHA 2008 Guidelines for the Management of Adults with Congenital Heart Disease: Executive Summary: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (writing committee to develop guidelines for the management of adults with congenital heart disease). Circulation 2008; 118:2395.
  4. 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.
  5. 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:e50.
  6. 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.
  7. Presbitero P, Somerville J, Stone S, et al. Pregnancy in cyanotic congenital heart disease. Outcome of mother and fetus. Circulation 1994; 89:2673.
  8. Esscher EB. Congenital complete heart block in adolescence and adult life. A follow-up study. Eur Heart J 1981; 2:281.
  9. Lam D, Cheitlin MD, Popper RW, Szarnicki RJ. Rapid development of biventricular heart failure in corrected transposition of the great arteries during pregnancy. Am Heart J 1988; 116:1111.
  10. Clarkson PM, Wilson NJ, Neutze JM, et al. Outcome of pregnancy after the Mustard operation for transposition of the great arteries with intact ventricular septum. J Am Coll Cardiol 1994; 24:190.
  11. Jatene AD, Fontes VF, Souza LC, et al. Anatomic correction of transposition of the great arteries. J Thorac Cardiovasc Surg 1982; 83:20.
  12. Losay J, Hougen TJ. Treatment of transposition of the great arteries. Curr Opin Cardiol 1997; 12:84.
  13. Gutgesell HP, Massaro TA, Kron IL. The arterial switch operation for transposition of the great arteries in a consortium of university hospitals. Am J Cardiol 1994; 74:959.
  14. Ploeg M, Drenthen W, van Dijk A, Pieper PG. Successful pregnancy after an arterial switch procedure for complete transposition of the great arteries. BJOG 2006; 113:243.
  15. Cataldo S, Doohan M, Rice K, et al. Pregnancy following Mustard or Senning correction of transposition of the great arteries: a retrospective study. BJOG 2016; 123:807.
  16. D'Souza R, Silversides C. Pregnancy following atrial-switch repair. BJOG 2016; 123:814.
  17. Tobler D, Fernandes SM, Wald RM, et al. Pregnancy outcomes in women with transposition of the great arteries and arterial switch operation. Am J Cardiol 2010; 106:417.
  18. Perloff, JK. The Clinical Recognition of Congenital Heart Disease, 6th ed, Elsevier/Saunders, Philadelphia 2012.
  19. Baumann H, Schneider H, Drack G, et al. Pregnancy and delivery by caesarean section in a patient with transposition of the great arteries and single ventricle. Case report. Br J Obstet Gynaecol 1987; 94:704.
  20. Leibbrandt G, Münch U, Gander M. Two successful pregnancies in a patient with single ventricle and transposition of the great arteries. Int J Cardiol 1982; 1:257.
  21. Fong J, Druzin M, Gimbel AA, Fisher J. Epidural anaesthesia for labour and caesarean section in a parturient with a single ventricle and transposition of the great arteries. Can J Anaesth 1990; 37:680.
  22. Sumner D, Melville C, Smith CD, et al. Successful pregnancy in a patient with a single ventricle. Eur J Obstet Gynecol Reprod Biol 1992; 44:239.
  23. Bitsch M, Johansen C, Wennevold A, Osler M. Eisenmenger's syndrome and pregnancy. Eur J Obstet Gynecol Reprod Biol 1988; 28:69.
  24. Hatjis CG, Gibson M, Capeless EL, et al. Pregnancy in a patient with tricuspid atresia. Am J Obstet Gynecol 1983; 145:114.
  25. Canobbio MM, Mair DD, van der Velde M, Koos BJ. Pregnancy outcomes after the Fontan repair. J Am Coll Cardiol 1996; 28:763.
  26. Lao TT, Sermer M, Colman JM. Pregnancy after the fontan procedure for tricuspid atresia. A case report. J Reprod Med 1996; 41:287.
  27. Barber G, Di Sessa T, Child JS, et al. Hemodynamic responses to isolated increments in heart rate by atrial pacing after a Fontan procedure. Am Heart J 1988; 115:837.
  28. Drenthen W, Pieper PG, Roos-Hesselink JW, et al. Pregnancy and delivery in women after Fontan palliation. Heart 2006; 92:1290.