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Outcome of pregnancy in women with congenital heart disease

Published online by Cambridge University Press:  19 August 2008

A. A. Schmaltz ,
U. Neudorf  and
U. H. Winkler
A. A. Schmaltz*
Affiliation:
Department ofPaediatric Cardiology, Children's Hospital, and Department of Obstetrics and Gynaecology, University Hospital Essen, Germany
U. Neudorf
Affiliation:
Department ofPaediatric Cardiology, Children's Hospital, and Department of Obstetrics and Gynaecology, University Hospital Essen, Germany
U. H. Winkler
Affiliation:
Department ofPaediatric Cardiology, Children's Hospital, and Department of Obstetrics and Gynaecology, University Hospital Essen, Germany
*
Prof. Dr. A. A. Schmaltz, Dept. of Paediatric Cardiology, University Children's Hospital, Hufelandstr. 55, D-45122 ESSE, Germany. Tel: 201–723–2452; Fax: 201–723–5743
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Abstract

Improvements in diagnosis and surgical technique for correction have led to an increasing number of women with congenital heart disease reaching the child-bearing age. Pregnancy places considerable strain on the heart and circulation and necessitates marked cardiorespiratory adaptation. Today, with the exception of the Eisenmenger syndrome, there is no increased mortality associated with pregnancy in congenital heart disease. In contrast, there is still considerable morbidity, due to congestive heart failure, thromboembolic complications and disturbances of rhythm.

Fetal outcome is complicated by a high rate of spontaneous abortions (20–25%), retardation of fetal growth, and premature delivery (almost 100% in cyanotic mothers). Based on an extensive review of the literature, we discuss the specific risks in pregnancy depending on the hemodynamic situations produced by different heart defects. We also discuss the risks and advantages of different regimens for anticoagulation.

Counselling concerning contraception is frequently inadequate. The most important problems are thromboembolic complications with the use of hormonal contraception, and hyper- and dysmenorrhea in those using intrauterine devices. Finally, the genetic risks must be considered, differentiating between single gene defects and the sex of the parents suffering from congenital heart diseases.

Keywords

Pregnancycongenital heart diseasematernal riskfetal riskanticoagulationgenetic risk
Type
Continuing Medical Education
Information
Cardiology in the Young , Volume 9 , Issue 1 , January 1999 , pp. 88 - 96
Copyright
Copyright © Cambridge University Press 1999

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References

1.McFaul, PB, Dornan, JC, Lamki, H, Boyle, D. Pregnancy complicated by maternal heart disease. A review of 519 women. Br J Obst Gyn 1988; 95: 861–867.CrossRefGoogle ScholarPubMed
2.Wada, H, Chiba, Y, Murakami, M, Kawaguchi, H, Kobayashi, H, Kanzaki, T. Analysis of maternal and fetal risk in 594 pregnancies with heart disease. Nippon Sanka Fujinka Gakkai Zasshi 1996; 48: 5–262.Google ScholarPubMed
3.Mühler, EG, Franke, A, Lepper, W, Grabitz, RG, Hermann, G, Klues, HG, Messmer, BJ, Hanrath, P, v Bernuth, G. Die Betreuung von Adoleszenten und Erwachsenen mit ange-borenem Herzfehler: 3 Jahre Erfahrungen mit einer interdiszi-plinären Sprechstunde. Z Kardiol 1995; 84: 532–541.Google Scholar
4.Hennessy, TG, MacDonald, D, Hennessy, MS, Maguire, M, Blake, S, McCann, HA, Sugrue, DD. Serial changes in cardiac output during normal pregnancy: a Doppler ultrasound study. Eur J Obstet Gynecol Reprod Biol 1996; 70: 117–122.CrossRefGoogle ScholarPubMed
5.Hunter, S, Robson, SC. Adaptation of the maternal heart in pregnancy. Br Heart J 1992; 68: 540–543.CrossRefGoogle ScholarPubMed
6.Robson, SC, Dunlop, W, Moore, M, Hunter, S. Combined Doppler and echocardiographic measurement of cardiac output: theory and application in pregnancy. Br J Obstet Gynaecol 1987; 94: 1014–1027.CrossRefGoogle ScholarPubMed
7.Robson, SC, Hunter, S, Boys, RJ, Dunlop, WHemodynamic changes during twin pregnancy. A Doppler and M-mode echocardiographic study. Am J Obstet Gynecol 1985; 161: 1273–1278.Google Scholar
8.Perloff, JK: Pregnancy in congenital heart disease. The mother and the fetus. In Perloff, JK and Child JS: Congenital Heart Disease in Adults. WB Saunders, Philadelphia, 1991, pp 124–140.Google Scholar
9.Szekely, P, Snaith, L. Heart disease and pregnancy. Churchill Livingstone, Edinburgh, 1974.Google Scholar
10.Chia, YT, Yeoh, SC, Viegas, OA, Lim, M, Ratnam, SS. Maternal congenital heart disease and pregnancy outcome. J Obstet Gynaecol Res 1996; 22: 185–191.Google Scholar
11.Presbitero, P, Somerville, J, Stone, S, Aruta, E, Spiegelhalter, D, Rabajoli, F. Pregnancy in cyanoctic congenital heart disease. Outcome of mother and fetus. Circulation 1994; 89: 2673–2676.CrossRefGoogle ScholarPubMed
12.Presbitero, P, Rabajoli, F, Somerville, J. Pregnancy in patients with congenital heart disease. Schweiz Med Wochenschr 1995; 1254: 311–315.Google Scholar
13.Elkayam, U, Gleicher, N. Cardiac problems in pregnancy: diagnosis and management of maternal and fetal disease.: Alan R Liss, Inc, New York 1982.Google Scholar
14.Pajszczyk-Kieszkiwicz, T, Krzeminska-Pakula, M, Kowalska-Koprek, U, Zaslonka, J, Zaslonka, A. Pregnancy in women with valvular prostheses- follow-up observations. Z Kardiol 1986; 75, Suppl.2: 308–311.Google Scholar
15.Kaemmerer, H, Niesert, S, Daniel, WG, Lichtlen, PR, Kallfelz, HC. Schwangerschaft und angeborene Herzfehler. Z Kardiol 1994; 83: 208–214.Google Scholar
16.Perloff, JK. Congenital heart disease in adults. Circulation 1991; 84: 1881–1890.Google Scholar
17.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: 641–651.CrossRefGoogle ScholarPubMed
18.Connolly, HM, Warnes, CA. Ebstein's anomaly: Outcome of pregnancy. J Am Coll Cardiol 1994; 23: 1194–1198.Google Scholar
19.Whittemore, R, Anderson, G, Orzalesi, M, Driscoll, S. Effects of maternal hypoxia on fetal and infant development (abstr). Pediatr Res 1971; 5: 426.CrossRefGoogle Scholar
20.Presbitero, P, Prever, SB, Brusca, A. Interventional cardiology in pregnancy. Eur Heart J 1996; 17: 182–188.CrossRefGoogle ScholarPubMed
21.Loya, YS, Desai, DM, Sharma, S. Mitral and pulmonary balloon valvotomy in pregnant patients. Indian Heart J 1993; 45: 57–59.Google ScholarPubMed
22.Perloff, JK. Congenital heart disease and pregnancy. Clin Cardiol 1994; 17: 579–587.CrossRefGoogle ScholarPubMed
23.Lao, TT, Sermer, M, MaGee, L, Farine, D, Colman, JM. Congenital aortic stenosis and pregnancy – A reappraisal. Am J Obstet Gynecol 1993; 169: 540–545.CrossRefGoogle ScholarPubMed
24.Angel, JL, Chapman, C, Knuppel, RA, Morales, WJ, Sims, CJ. Percutaneous ballon aortic valvulopasty in pregnancy. Obstet Gynecol 1988; 72: 438–440.Google Scholar
25.Mclvor, RA. Percutaneous balloon aortic valvuloplasty during pregnancy. Int J Cardiol 1991; 32: 1–4.CrossRefGoogle Scholar
26.Bernal, JM, Miralles, PJ. Cardiac surgery with cardiopulmonary bypass during pregnancy. Obstet Gynecol Surv 1986; 41: 1–6.CrossRefGoogle ScholarPubMed
27.Oakley, CM. Valvular disease in pregnancy. Curr Op Cardiol 1996; 11: 155–159.Google Scholar
28.Deal, K, Wooley, CF. Coarctation of the aorta und pregnancy. Ann Intern Med 1993; 78: 706–710.Google Scholar
29.Lipscomb, KJ, Smith, JC, Clarke, B, Donnai, P, Harris, R. Outcome of pregnancy in women with Marfan's syndrome. Br J Obstet Gynaecol 1997; 104: 201–206.Google Scholar
30.Rossiter, JP, Repke, JT, Morales, AJ, Murphy, EA, Pyeritz, RE. A prospective longitudinal evaluation of pregnancy in the Marfan syndrome. Am J Obstet Gynaecol 1995; 173: 1599–1606.Google Scholar
31.Presbitero, P, D'Antonio, P, Brusca, A, Morea, M. Prognosis of Pallet's Tetralogy after palliative operations: 10–25 year follow-up. Fed Cardiol 1983; 4: 175–182.Google Scholar
32.Neumayer, U, Somerville, J. Outcome of pregnancies in patients with complex pulmonary attesia. Heart 1997; 78: 16–21.Google Scholar
33.Avila, WS, Grinberg, M, Snitcowsky, R, Faccioli, R, Da Luz, PL, Belotti, G, Pileggi, F. Maternal and fetal outcome in pregnant women with Eisenmenger's syndrome. Eur Heart J 1995; 16: 460–464.CrossRefGoogle ScholarPubMed
34.Brach-Prever, S, Sheppard, M, Somerville, J. Fatal outcome of pregnancy in the Eisenmenger syndrome. Cardiol Young 1997; 7: 238–240.Google Scholar
35.Spinnato, JA, Kraynack, BJ, Cooper, MWEisenmenger's syndrome in pregnancy: Epidural anesthesia for elective Cesarean section. N Engl J Med 1981; 304: 1215–1217.CrossRefGoogle ScholarPubMed
36.Young, D, Mark, H. Fate of the patient with the Eisenmenger syndrome. Am J Cardiol 1971; 28: 658–669.CrossRefGoogle ScholarPubMed
37.Nolan, FA, Engle, MA, Diaz, S. The pregnant adolescent with heart disease. In: Julian, DG, Wenger, NK (eds.): Cardiac problems of the adolescent and young adult. Butterworths, London, 1985, pp 213–231.Google Scholar
38.Ralstin, JH, Dunn, M. Pregnancies after surgical correction of tetralogy of Fallot. J Am Med Ass 1976; 235: 2627.CrossRefGoogle ScholarPubMed
39.Clarkson, PM, Wilson, NJ, Neutze, JM, North, RA, Calder, AL, Boyes-Barratt, BG. Outcome of pregnancy after the Mustard operation for transposition of the great arteries with intact ventricular septum. J Am Coll Cardiol 1994; 24: 190–193.CrossRefGoogle ScholarPubMed
40.Jahangiri, M, Shore, D, Kakkar, V, Lincoln, Ch, Shinebourne, E. Coagulation factor abnormalities after the Fontan procedure and its modifications. J Thorac Cardiovasc Surg 1997; 113: 989–993.CrossRefGoogle ScholarPubMed
41.Jonas, RA. Intracardiac thrombus after the fontan procedure. J Thorac Cardiovasc Surg 1995; 110: 1502–1503.CrossRefGoogle ScholarPubMed
42.Kaulitz, R, Luhmer, I, Bergmann, F, Rodeck, B, Hausdorf, G. Sequelae after modified Fontan operation: postoperative hemodynamic data and organ function. Heart 1997; 78: 154–159.CrossRefGoogle ScholarPubMed
43.Driscoll, DJ, Offord, KP, Feldt, RH, Schaff, HV, Puga, FJ, Danielson, GK. Five- to fifteen-year follow-up after Fontan operation. Circulation 1992; 85: 469–496.Google Scholar
44.Girod, DA, Fontan, F, Deville, C, Ottenkamp, J, Choussat, A. Long-term results after the Fontan operation for tricuspid atresia. Circulation 1987; 75: 605–610.Google Scholar
45.Canobbio, MM, Mair, DD, van der Velde, M, Koos, BJ. Pregnancy outcomes after the Fontan repair. J Am Coll Cardiol 1996; 28: 763–767.Google Scholar
46.Osmers, R, Osmers, M, Bartmus, D, Ruscheswki, W, Kuhn, WAusgetragene Schwangerschaft nach maternaler Fontan'scher Operation bei univentrikulärem Herzen. Z Geburtsh Neonatol 1995; 199: 195–198.Google Scholar
47.Elkayam, U. Anticoagulation in pregnant women with prosthetic heart valves: a double jeopardy. J Am Coll Cardiol 1996; 27: 1704–1706.CrossRefGoogle Scholar
48.Yuh-Jer Shen, A, Mansukhani, PW. Is pregnancy contraindi-cated after cardiac transplantation? A case report and literature review. Int J Cardiol 1997; 60: 151–156.Google Scholar
49.Wagoner, LE, Taylor, DO, Olsen, SL. Immunosuppressive therapy, management, and outcome of heart transplant recipients during pregnancy. J. Heart Lung Transplant 1993; 13: 993–1000.Google Scholar
50.Oakley, CM. Anticoagulation and pregnancy. Eur Heart J 1995; 16: 1317–1319.Google Scholar
51.Badduke, BR, Jamieson, WRE, Miyagishima, RT, Munro, AI, Gerein, AN, MacNab, J, Tyers, GFO. Pregnancy and childbear-ing in a population with biologic valvular prostheses. J Thorac Cardiovasc Surg 1991; 102: 179–186.Google Scholar
52.Hanania, G, Thomas, D, Michel, PL, Garbarz, E, Ages, C, Millaire Acar, J. Pregnancy and prosthetic heart valves: a French cooperative retrospective study of 155 cases. Eur Heart J 1994; 15: 1651–1658.CrossRefGoogle Scholar
53.Sbarouni, E, Oakley, CM. Outcome of pregnancy in women with valve prostheses. Br Heart J 1994; 71: 196–210.CrossRefGoogle ScholarPubMed
54.Dore, A, Somerville, J. Pregnancy in patients with pulmonalry autograft valve. Eur Heart J 1997; 18: 1659–1662.CrossRefGoogle ScholarPubMed
55.Salazar, E, Zajarias, A, Gutierrez, N, Iturbe, I. The problem of cardiac valve prostheses, anticoagulants, and pregnancy. Circulation 1984; 70 (suppl I): 1–169.Google Scholar
56.Ginsberg, JS, Hirsch, J, Turner, DC, Levine, MN, Burrows, R. Risks to the fetus of anticoagulant therapy during pregnancy. Thromb Haemost 1989; 61: 197–203.Google Scholar
57.Gohlke-Barwolf, C, Acar, J, Oakley, C, Butchard, E, Burckhardt, D, Bodnar, E, Hall, R, Delahaye, J-P, Horstkotte, D, Kremer, R, Krayenbiihl, HP, Krzeminska-Pakula, M, Samama, M. Guidelines for prevention of thromboembolic events in valvular heart disease. Eur Heart J 1995; 16: 1320–1330.Google ScholarPubMed
58.Iturbe-Alessio, I, Fonseca, M, del C Mutchinik, O, Santos, MA, Zajarias, A, Salazar, E. Risks of anticoagulant therapy in pregnant women with artificial heart valves. N Engl J Med 1986; 315: 1390–1393.Google Scholar
59.Salazar, E, Izagurine, R, Verdejo, J, Mutchinick, O. Failure of adjusted doses of subcutaneous heparin to prevent thromboembolic phenomena in pregnant patients with mechanical cardiac valve protheses. J Am Coll Cardiol 1996; 17: 1698–713.Google Scholar
60.Leonard, H, O'Sullivan, JJ, Hunter, S. Family planning requirements in the adult congenital heart disease clinic. Heart 1996; 76: 60–62.Google Scholar
61.Swan, L, Hillis, WS, Cameron, A. Family planning requirements of adults with congenital heart disease. Heart 1997; 78: 9–11.Google Scholar
62.Winkler, UH: Thromboembolierisiko, hormonelle Kontrazeption und Östrogensubstitution. Gynäkologe 1997; 30: 521–526.Google Scholar
63.Nora, JJ. From generational studies to a multilevel genetic-environmental interaction. J Am Coll Cardiol 1994; 23: 1468–1471.CrossRefGoogle ScholarPubMed
64.Driscoll, DJ, Michel, VV, Gersony, WM, Hayes, CJ, Keane, JF, Kidd, L, Pieroni, DR, Rings, LJ, Wolfe, RR, Weidmann, WH. Occurrence risk for congenital heart defects in relatives of patients with aortic stenosis, pulmonary stenosis, or ventricular septal defect. Circulation 1993; 87 {suppl I}: 1–114–1–120.Google Scholar
65.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: 1459–67.CrossRefGoogle ScholarPubMed
66.Nora, JJ, Nora, AH. Maternal transmission of congenital heart diseases: new reccurrence risk figures and the questions of cytoplasmatic inheritance and vulnerability to teratogens. Am J Cardiol 1987; 59: 459–463.Google Scholar
67.Clark, SL, Cotton, DB, Lee, W, Bishop, C, Hill, T, South-wick, J, Pivarnik, J, Spillman, T, DeVore, DG, Phelan, J, Hankins, GDV, Benedetti, TJ, Tolley, D. Central hemodynamic assessement of normal human pregnancy. Am J Obstet Gynecol 1989; 161: 1439–1442.Google Scholar
68.Longo, LD. Maternal blood volume and cardiac output during pregnancy: a hypothesis of endocrinologic control. Am J Physiol 1983; 245: 720–729.Google Scholar