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Pregnancy in women with congenital heart disease: General principles

Authors
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

INTRODUCTION

Progress in medical and surgical treatment has resulted in larger numbers of women with congenital heart disease surviving to child-bearing years and proceeding with pregnancy [1].

The general principles of management of pregnancy and contraception in women who have unrepaired or repaired congenital malformations of the heart or great vessels will be reviewed here. Pregnancy in women with specific congenital cardiac anomalies, the management of valvular heart disease during pregnancy, and the management of heart failure and arrhythmias during pregnancy are discussed separately. (See "Pregnancy in women with congenital heart disease: Specific lesions" and "Pregnancy and valve disease" and "Management of heart failure during pregnancy" and "Supraventricular arrhythmias during pregnancy" and "Ventricular arrhythmias during pregnancy".)

EPIDEMIOLOGY

Successful cardiac surgery improves fertility and reduces the maternal and fetal risk of pregnancy in women with congenital heart disease [2,3]. Accordingly, women are now presenting for obstetric and cardiologic care after reparative cardiac surgery [1]. In a registry of the European Society of Cardiology, congenital heart disease was the most prevalent form of structural heart disease (66 percent) affecting pregnancy outcomes worldwide [4].

Congenital heart disease remains an important cause of maternal mortality and morbidity during pregnancy. The magnitude of risk is illustrated by the following observations:

In a report of maternal deaths from 2000 to 2002 in the United Kingdom, cardiac disease was the second most common cause, with congenital heart disease accounting for 20 percent of cardiac deaths [5].

                                            

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Literature review current through: Nov 2016. | This topic last updated: Mon Nov 14 00:00:00 GMT+00:00 2016.
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References
Top
  1. Child, JS.. Management of Pregnancy and contraception in Congenital Heart Disease. In: Congenital Heart Disease in Adults, 3rd ed, Perloff, JK, Child, JS, Aboulhosn, J (Eds), Saunders/Elsevier, 2009.
  2. Whittemore R, Hobbins JC, Engle MA. Pregnancy and its outcome in women with and without surgical treatment of congenital heart disease. Am J Cardiol 1982; 50:641.
  3. Shime J, Mocarski EJ, Hastings D, et al. Congenital heart disease in pregnancy: short- and long-term implications. Am J Obstet Gynecol 1987; 156:313.
  4. Roos-Hesselink JW, Ruys TP, Stein JI, et al. Outcome of pregnancy in patients with structural or ischaemic heart disease: results of a registry of the European Society of Cardiology. Eur Heart J 2013; 34:657.
  5. Lewis G, Drife JO. Why mothers die 2000-2002—The sixth report of confidential enquiries into maternal deaths in the United Kingdom, RCOG Press, London 2004.
  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. Kuklina E, Callaghan W. Chronic heart disease and severe obstetric morbidity among hospitalisations for pregnancy in the USA: 1995-2006. BJOG 2011; 118:345.
  8. Greutmann M, Pieper PG. Pregnancy in women with congenital heart disease. Eur Heart J 2015; 36:2491.
  9. Toglia MR, Weg JG. Venous thromboembolism during pregnancy. N Engl J Med 1996; 335:108.
  10. Joint Task Force on the Management of Valvular Heart Disease of the European Society of Cardiology (ESC), European Association for Cardio-Thoracic Surgery (EACTS), Vahanian A, et al. Guidelines on the management of valvular heart disease (version 2012). Eur Heart J 2012; 33:2451.
  11. 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.
  12. Thorne S, MacGregor A, Nelson-Piercy C. Risks of contraception and pregnancy in heart disease. Heart 2006; 92:1520.
  13. 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.
  14. Drenthen W, Boersma E, Balci A, et al. Predictors of pregnancy complications in women with congenital heart disease. Eur Heart J 2010; 31:2124.
  15. Siu SC, Sermer M, Harrison DA, et al. Risk and predictors for pregnancy-related complications in women with heart disease. Circulation 1997; 96:2789.
  16. Siu SC, Sermer M, Colman JM, et al. Prospective multicenter study of pregnancy outcomes in women with heart disease. Circulation 2001; 104:515.
  17. Khairy P, Ouyang DW, Fernandes SM, et al. Pregnancy outcomes in women with congenital heart disease. Circulation 2006; 113:517.
  18. Elkayam U, Goland S, Pieper PG, Silverside CK. High-Risk Cardiac Disease in Pregnancy: Part I. J Am Coll Cardiol 2016; 68:396.
  19. Elkayam U, Goland S, Pieper PG, Silverside CK. High-Risk Cardiac Disease in Pregnancy: Part II. J Am Coll Cardiol 2016.
  20. Siu S, Chitayat D, Webb G. Pregnancy in women with congenital heart defects: what are the risks? Heart 1999; 81:225.
  21. Kahn ML. Eisenmenger's syndrome in pregnancy. N Engl J Med 1993; 329:887.
  22. Bédard E, Dimopoulos K, Gatzoulis MA. Has there been any progress made on pregnancy outcomes among women with pulmonary arterial hypertension? Eur Heart J 2009; 30:256.
  23. Presbitero P, Somerville J, Stone S, et al. Pregnancy in cyanotic congenital heart disease. Outcome of mother and fetus. Circulation 1994; 89:2673.
  24. Weiss BM, Atanassoff PG. Cyanotic congenital heart disease and pregnancy: natural selection, pulmonary hypertension, and anesthesia. J Clin Anesth 1993; 5:332.
  25. McFaul PB, Dornan JC, Lamki H, Boyle D. Pregnancy complicated by maternal heart disease. A review of 519 women. Br J Obstet Gynaecol 1988; 95:861.
  26. Selzer A. Risks of pregnancy in women with cardiac disease. JAMA 1977; 238:892.
  27. Kampman MA, Balci A, van Veldhuisen DJ, et al. N-terminal pro-B-type natriuretic peptide predicts cardiovascular complications in pregnant women with congenital heart disease. Eur Heart J 2014; 35:708.
  28. Hameed AB, Chan K, Ghamsary M, Elkayam U. Longitudinal changes in the B-type natriuretic peptide levels in normal pregnancy and postpartum. Clin Cardiol 2009; 32:E60.
  29. Tanous D, Siu SC, Mason J, et al. B-type natriuretic peptide in pregnant women with heart disease. J Am Coll Cardiol 2010; 56:1247.
  30. Perloff, JK. The Clinical Recognition of Congenital Heart Disease, 6th ed, Elsevier/Saunders, Philadelphia 2012.
  31. Schmaltz AA, Neudorf U, Winkler UH. Outcome of pregnancy in women with congenital heart disease. Cardiol Young 1999; 9:88.
  32. Burn J, Brennan P, Little J, et al. Recurrence risks in offspring of adults with major heart defects: results from first cohort of British collaborative study. Lancet 1998; 351:311.
  33. Gill HK, Splitt M, Sharland GK, Simpson JM. Patterns of recurrence of congenital heart disease: an analysis of 6,640 consecutive pregnancies evaluated by detailed fetal echocardiography. J Am Coll Cardiol 2003; 42:923.
  34. Whittemore R, Wells JA, Castellsague X. A second-generation study of 427 probands with congenital heart defects and their 837 children. J Am Coll Cardiol 1994; 23:1459.
  35. Kovacs AH, Harrison JL, Colman JM, et al. Pregnancy and contraception in congenital heart disease: what women are not told. J Am Coll Cardiol 2008; 52:577.
  36. Weiss BM, von Segesser LK, Alon E, et al. Outcome of cardiovascular surgery and pregnancy: a systematic review of the period 1984-1996. Am J Obstet Gynecol 1998; 179:1643.
  37. Parry AJ, Westaby S. Cardiopulmonary bypass during pregnancy. Ann Thorac Surg 1996; 61:1865.
  38. Strickland RA, Oliver WC Jr, Chantigian RC, et al. Anesthesia, cardiopulmonary bypass, and the pregnant patient. Mayo Clin Proc 1991; 66:411.
  39. Chambers CE, Clark SL. Cardiac surgery during pregnancy. Clin Obstet Gynecol 1994; 37:316.
  40. John AS, Gurley F, Schaff HV, et al. Cardiopulmonary bypass during pregnancy. Ann Thorac Surg 2011; 91:1191.
  41. Willcourt RJ, King JC, Queenan JT. Maternal oxygenation administration and the fetal transcutaneous PO2. Am J Obstet Gynecol 1983; 146:714.
  42. 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).
  43. ACOG Committee on Practice Bulletins. ACOG Practice Bulletin No. 74. Antibiotic prophylaxis for gynecologic procedures. Obstet Gynecol 2006; 108:225.
  44. Sawaya GF, Grady D, Kerlikowske K, Grimes DA. Antibiotics at the time of induced abortion: the case for universal prophylaxis based on a meta-analysis. Obstet Gynecol 1996; 87:884.
  45. Ramsey PS, Hogg BB, Savage KG, et al. Cardiovascular effects of intravaginal misoprostol in the mid trimester of pregnancy. Am J Obstet Gynecol 2000; 183:1100.
  46. Bagga R, Choudhary N, Suri V, et al. First and second trimester induced abortions in women with cardiac disorders: a 12-year analysis from a developing country. J Obstet Gynaecol 2008; 28:732.
  47. Hackney DN, Creinin MD, Simhan H. Medical management of early pregnancy failure in a patient with coronary artery disease. Fertil Steril 2007; 88:212.e1.
  48. Guiahi M, Davis A, Society of Family Planning. First-trimester abortion in women with medical conditions: release date October 2012 SFP guideline #20122. Contraception 2012; 86:622.
  49. Campuzano K, Roqué H, Bolnick A, et al. Bacterial endocarditis complicating pregnancy: case report and systematic review of the literature. Arch Gynecol Obstet 2003; 268:251.
  50. Armson BA, Samuels P, Miller F, et al. Evaluation of maternal fluid dynamics during tocolytic therapy with ritodrine hydrochloride and magnesium sulfate. Am J Obstet Gynecol 1992; 167:758.
  51. 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.
  52. 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; 116:1736.
  53. 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.
  54. Canobbio MM, Rapkin AJ, Perloff JK, et al. Menstrual patterns in women with congenital heart disease. Pediatr Cardiol 1995; 16:12.
  55. Sciscione AC, Callan NA. Congenital heart disease in adolescents and adults. Pregnancy and contraception. Cardiol Clin 1993; 11:701.
  56. Leonard H, O'Sullivan JJ, Hunter S. Family planning requirements in the adult congenital heart disease clinic. Heart 1996; 76:60.
  57. Famuyide AO, Hopkins MR, El-Nashar SA, et al. Hysteroscopic sterilization in women with severe cardiac disease: experience at a tertiary center. Mayo Clin Proc 2008; 83:431.
  58. Sable C, Foster E, Uzark K, et al. Best practices in managing transition to adulthood for adolescents with congenital heart disease: the transition process and medical and psychosocial issues: a scientific statement from the American Heart Association. Circulation 2011; 123:1454.
  59. Dajani AS, Taubert KA, Wilson W, et al. Prevention of bacterial endocarditis. Recommendations by the American Heart Association. Circulation 1997; 96:358.