Advertisement
Canadian Journal of Cardiology

Reproductive Issues in Patients With the Fontan Operation

  • Barbara Wichert-Schmitt
    Correspondence
    Corresponding author: Dr Barbara Wichert-Schmitt, Kepler University Hospital Linz, Klinik für Innere Medizin 1, Med Campus III, Krankenhausstrasse 9, 4021 Linz, Austria. Tel.: +43 (0)5 7680-83-6220; fax: +43 (0)5 7680-83-6205.
    Affiliations
    Department of Cardiology and Medical Intensive Care, Kepler University Hospital, Medical Faculty, Johannes Kepler University, Linz, Austria
    Search for articles by this author
  • Rohan D‘Souza
    Affiliations
    Departments of Obstetrics & Gynaecology and Health Research Methods, Evidence and Impact, McMaster University, Hamilton, Ontario, Canada
    Search for articles by this author
  • Candice K. Silversides
    Affiliations
    Division of Cardiology, University of Toronto, Pregnancy and Heart Disease Program, Mount Sinai and Toronto General Hospitals, Toronto, Ontario, Canada
    Search for articles by this author
Published:April 28, 2022DOI:https://doi.org/10.1016/j.cjca.2022.04.020

      Abstract

      Patients with the Fontan operation have a unique circulation, with a limited ability to increase cardiac output, and high central venous pressure. They may have diastolic and/or systolic ventricular dysfunction, arrhythmias, thromboembolic complications, or multiorgan dysfunction. All of these factors contribute to reproductive issues, including menstrual irregularities, infertility, recurrent miscarriage, and complications during pregnancy. Although atrial arrhythmias are the most common cardiac complications during pregnancy, patients can develop heart failure and thromboembolic events. Obstetric bleeding, including postpartum hemorrhage, is common. In addition to maternal complications, adverse fetal and neonatal events, such as prematurity and low birthweight, are very common. Counselling about these reproductive issues should begin early. For those who become pregnant, care should be provided by a multidisciplinary cardio-obstetric team familiar with the specific issues and needs of the Fontan population. In this review, we discuss infertility, contraception, and pregnancy in patients with the Fontan operation.

      Résumé

      L’intervention de Fontan crée un schéma circulatoire unique se traduisant par une capacité limitée d’augmentation du débit cardiaque et une pression veineuse centrale élevée. Les patients qui ont subi cette intervention peuvent présenter une dysfonction ventriculaire diastolique et/ou systolique, des arythmies, des complications thromboemboliques ou une dysfonction multiviscérale. Tous ces facteurs contribuent à des problèmes de reproduction, notamment des irrégularités menstruelles, l’infertilité, des fausses couches répétées et des complications obstétricales. Les arythmies atriales sont les complications cardiaques les plus courantes pendant la grossesse, mais une insuffisance cardiaque et des événements thromboemboliques peuvent aussi survenir. Les saignements obstétricaux, dont l’hémorragie post-partum, sont fréquents. Outre les complications chez la mère, on note une fréquence élevée d’événements indésirables touchant le fœtus et le nouveau-né, tels que la prématurité et l’insuffisance pondérale à la naissance. Le counseling sur ces questions touchant la reproduction devrait commencer tôt. Les femmes enceintes devraient être prises en charge par une équipe cardio-obstétricale bien au fait des difficultés et des besoins spécifiques des patientes qui ont subi l’intervention de Fontan. Dans cet article de synthèse, nous abordons l’infertilité, la contraception et la grossesse chez les patientes ayant subi l’intervention de Fontan.
      To read this article in full you will need to make a payment

      Purchase one-time access:

      Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online access
      One-time access price info
      • For academic or personal research use, select 'Academic and Personal'
      • For corporate R&D use, select 'Corporate R&D Professionals'

      Subscribe:

      Subscribe to Canadian Journal of Cardiology
      Already a print subscriber? Claim online access
      Already an online subscriber? Sign in
      Institutional Access: Sign in to ScienceDirect

      References

      1. Rychik J, Atz AM, Celermajer DS, et al. Evaluation and management of the child and adult with Fontan circulation: a scientific statement from the American Heart Association [e-pub ahead of print]. Circulation:https://doi.org/10.1161/CIR0000000000000696.

        • Pundi K.N.
        • Johnson J.N.
        • Dearani J.A.
        • et al.
        40-year follow-up after the Fontan operation: long-term outcomes of 1,052 patients.
        J Am Coll Cardiol. 2015; 66: 1700-1710
        • Gewillig M.
        • Brown S.C.
        • van de Bruaene A.
        • Rychik J.
        Providing a framework of principles for conceptualising the Fontan circulation.
        Acta Paediatr. 2020; 109: 651-658
        • Diller G.P.
        • Kempny A.
        • Alonso-Gonzalez R.
        • et al.
        Survival prospects and circumstances of death in contemporary adult congenital heart disease patients under follow-up at a large tertiary centre.
        Circulation. 2015; 132: 2118-2125
        • Rychik J.
        The relentless effects of the Fontan paradox.
        Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 2016; 19: 37-43
        • Wilson W.M.
        • Valente A.M.
        • Hickey E.J.
        • et al.
        Outcomes of patients with hypoplastic left heart syndrome reaching adulthood after Fontan palliation: multicenter study.
        Circulation. 2018; 137: 978-981
        • Said S.M.
        • Dearani J.A.
        • Silversides C.K.
        • Martinez R.M.
        • Drajpuch D.B.
        Longer-term issues for young adults with hypoplastic left heart syndrome: contraception, pregnancy, transition, transfer, counselling, and re-operation.
        Cardiol Young. 2011; 21: 93-100
        • Garcia Ropero A.
        • Baskar S.
        • Roos Hesselink J.W.
        • et al.
        Pregnancy in women with a Fontan circulation: a systematic review of the literature.
        Circ Cardiovasc Qual Outcomes. 2018; 11e004575
        • du Plessis K.
        • Peters R.
        • King I.
        • et al.
        "How long will I continue to be normal?" Adults with a Fontan circulation's greatest concerns.
        Int J Cardiol. 2018; 260: 54-59
        • 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: 1454-1485
        • Haberer K.
        • Silversides C.K.
        Congenital heart disease and women's health across the life span: focus on reproductive issues.
        Can J Cardiol. 2019; 35: 1652-1663
      2. Nao Konagai HO, Noritake K, Shiraishi I, Yoshimatsu J, Kurosaki K. Menstrual problems are associated with elevated central venous pressure and predict adverse clinical events in women with congenital heart disease. Int J Cardiol Congenit Heart Dis 2021;4:100198.

        • Drenthen W.
        • Hoendermis E.S.
        • Moons P.
        • et al.
        Menstrual cycle and its disorders in women with congenital heart disease.
        Congenit Heart Dis. 2008; 3: 277-283
        • American College of Obstetricians and Gynecologists’ Committee on Adolescent Health Care
        Gynecologic considerations for adolescents and young women with cardiac conditions: ACOG committee opinion, number 813.
        Obstet Gynecol. 2020; 136: e90-e99
        • Leroy-Melamed M.
        • Katz A.
        • Shew M.L.
        Menstrual dysfunction and treatment among adolescents with congenital heart disease.
        J Pediatr Adolesc Gynecol. 2020; 33: 686-690
        • Canobbio M.M.
        • Perloff J.K.
        • Rapkin A.J.
        Gynecological health of females with congenital heart disease.
        Int J Cardiol. 2005; 98: 379-387
        • Khajali Z.
        • Ziaei S.
        • Maleki M.
        Menstrual disturbances in women with congenital heart diseases.
        Res Cardiovasc Med. 2016; 5e32512
        • McCrindle B.W.
        • Manlhiot C.
        • Cochrane A.
        • et al.
        Factors associated with thrombotic complications after the Fontan procedure: a secondary analysis of a multicenter, randomized trial of primary thromboprophylaxis for 2 years after the Fontan procedure.
        J Am Coll Cardiol. 2013; 61: 346-353
        • Peake L.J.
        • Grover S.R.
        • Monagle P.T.
        • Kennedy A.D.
        Effect of warfarin on menstruation and menstrual management of the adolescent on warfarin.
        J Paediatr Child Health. 2011; 47: 893-897
        • Tomkiewicz-Pajak L.
        • Plazak W.
        • Kolcz J.
        • et al.
        Iron deficiency and hematological changes in adult patients after Fontan operation.
        J Cardiol. 2014; 64: 384-389
        • Canobbio M.M.
        • Warnes C.A.
        • 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: e50-87
        • Lindley K.J.
        • Bairey Merz C.N.
        • Davis M.B.
        • et al.
        Contraception and reproductive planning for women with cardiovascular disease: JACC Focus Seminar 5/5.
        J Am Coll Cardiol. 2021; 77: 1823-1834
        • Roos-Hesselink J.W.
        • Cornette J.
        • Sliwa K.
        • et al.
        Contraception and cardiovascular disease.
        Eur Heart J. 2015; 36 (1734a-b): 1728-1734
        • Thorne S.
        • Nelson-Piercy C.
        • MacGregor A.
        • et al.
        Pregnancy and contraception in heart disease and pulmonary arterial hypertension.
        J Fam Plann Reprod Health Care. 2006; 32: 75-81
        • Cleland K.
        • Zhu H.
        • Goldstuck N.
        • Cheng L.
        • Trussell J.
        The efficacy of intrauterine devices for emergency contraception: a systematic review of 35 years of experience.
        Hum Reprod. 2012; 27: 1994-2000
        • Mehta L.S.
        • Warnes C.A.
        • Bradley E.
        • et al.
        Cardiovascular considerations in caring for pregnant patients: a scientific statement from the American Heart Association.
        Circulation. 2020; 141: e884-903
        • Sanghavi M.
        • Rutherford J.D.
        Cardiovascular physiology of pregnancy.
        Circulation. 2014; 130: 1003-1008
        • Ouzounian J.G.
        • Elkayam U.
        Physiologic changes during normal pregnancy and delivery.
        Cardiol Clin. 2012; 30: 317-329
        • Arif S.
        • Chaudhary A.
        • Clift P.F.
        • et al.
        Pregnancy outcomes in patients with a Fontan circulation and proposal for a risk-scoring system: single centre experience.
        J Congenit Cardiol. 2017; 1: 10
        • Gouton M.
        • Nizard J.
        • Patel M.
        • et al.
        Maternal and fetal outcomes of pregnancy with Fontan circulation: a multicentric observational study.
        Int J Cardiol. 2015; 187: 84-89
        • Zentner D.
        • Kotevski A.
        • King I.
        • Grigg L.
        • d'Udekem Y.
        Fertility and pregnancy in the Fontan population.
        Int J Cardiol. 2016; 208: 97-101
        • Cauldwell M.
        • Ghonim S.
        • Uebing A.
        • et al.
        Preconception counseling, predicting risk and outcomes in women with mWHO 3 and 4 heart disease.
        Int J Cardiol. 2017; 234: 76-80
        • Drenthen W.
        • Pieper P.G.
        • Roos-Hesselink J.W.
        • et al.
        Pregnancy and delivery in women after Fontan palliation.
        Heart. 2006; 92: 1290-1294
        • Regitz-Zagrosek V.
        • Roos-Hesselink J.W.
        • Bauersachs J.
        • et al.
        2018 ESC guidelines for the management of cardiovascular diseases during pregnancy.
        Eur Heart J. 2018; 39: 3165-3241
        • Elkayam U.
        • Goland S.
        • Pieper P.G.
        • Silverside C.K.
        High-risk cardiac disease in pregnancy: part I.
        J Am Coll Cardiol. 2016; 68: 396-410
        • Cauldwell M.
        • Von Klemperer K.
        • Uebing A.
        • et al.
        A cohort study of women with a Fontan circulation undergoing preconception counselling.
        Heart. 2016; 102: 534-540
        • Pundi K.N.
        • Pundi K.
        • Johnson J.N.
        • et al.
        Contraception practices and pregnancy outcome in patients after Fontan operation.
        Congenit Heart Dis. 2016; 11: 63-70
        • Quenby S.
        • Gallos I.D.
        • Dhillon-Smith R.K.
        • et al.
        Miscarriage matters: the epidemiological, physical, psychological, and economic costs of early pregnancy loss.
        Lancet. 2021; 397: 1658-1667
        • Pfaller B.
        • Siu S.C.
        • D'Souza R.
        • et al.
        Impact of obesity on outcomes of pregnancy in women with heart disease.
        J Am Coll Cardiol. 2021; 77: 1317-1326
      3. American College of Obstetricians and Gynecologists' Presidential Task Force on Pregnancy and Heart Disease and Committee on Practice Bulletins—Obstetrics. ACOG Practice Bulletin No. 212: pregnancy and heart disease.
        Obstet Gynecol. 2019; 133: e320-e356
        • Wolfe N.K.
        • Sabol B.A.
        • Kelly J.C.
        • et al.
        Management of Fontan circulation in pregnancy: a multidisciplinary approach to care.
        Am J Obstet Gynecol MFM. 2021; 3: 100257
        • Pieper P.G.
        Use of medication for cardiovascular disease during pregnancy.
        Nat Rev Cardiol. 2015; 12: 718-729
        • Cauldwell M.
        • Patel R.R.
        • Steer P.J.
        • et al.
        Managing subfertility in patients with heart disease: What are the choices?.
        Am Heart J. 2017; 187: 29-36
        • Rossberg N.
        • Stangl K.
        • Stangl V.
        Pregnancy and cardiovascular risk: a review focused on women with heart disease undergoing fertility treatment.
        Eur J Prev Cardiol. 2016; 23: 1953-1961
        • Silversides C.K.
        • Harris L.
        • Haberer K.
        • et al.
        Recurrence rates of arrhythmias during pregnancy in women with previous tachyarrhythmia and impact on fetal and neonatal outcomes.
        Am J Cardiol. 2006; 97: 1206-1212
        • Enriquez A.D.
        • Economy K.E.
        • Tedrow U.B.
        Contemporary management of arrhythmias during pregnancy.
        Circ Arrhythm Electrophysiol. 2014; 7: 961-967
        • Khairy P.
        • Van Hare G.F.
        • Balaji S.
        • et al.
        PACES/HRS expert consensus statement on the recognition and management of arrhythmias in adult congenital heart disease: developed in partnership between the Pediatric and Congenital Electrophysiology Society (PACES) and the Heart Rhythm Society (HRS). Endorsed by the governing bodies of PACES, HRS, the American College of Cardiology (ACC), the American Heart Association (AHA), the European Heart Rhythm Association (EHRA), the Canadian Heart Rhythm Society (CHRS), and the International Society for Adult Congenital Heart Disease (ISACHD).
        Heart Rhythm. 2014; 11: e102-e165
        • Khairy P.
        • Aboulhosn J.
        • Broberg C.S.
        • et al.
        Thromboprophylaxis for atrial arrhythmias in congenital heart disease: a multicenter study.
        Int J Cardiol. 2016; 223: 729-735
        • Pillutla P.
        • Nguyen T.
        • Markovic D.
        • et al.
        Cardiovascular and neonatal outcomes in pregnant women with high-risk congenital heart disease.
        Am J Cardiol. 2016; 117: 1672-1677
        • Budts W.
        • Roos-Hesselink J.
        • Radle-Hurst T.
        • et al.
        Treatment of heart failure in adult congenital heart disease: a position paper of the Working Group of Grown-Up Congenital Heart Disease and the Heart Failure Association of the European Society of Cardiology.
        Eur Heart J. 2016; 37: 1419-1427
        • Stout K.K.
        • Broberg C.S.
        • Book W.M.
        • et al.
        Chronic heart failure in congenital heart disease: a scientific statement from the American Heart Association.
        Circulation. 2016; 133: 770-801
        • Attard C.
        • Huang J.
        • Monagle P.
        • Ignjatovic V.
        Pathophysiology of thrombosis and anticoagulation post Fontan surgery.
        Thromb Res. 2018; 172: 204-213
        • Deshaies C.
        • Hamilton R.M.
        • Shohoudi A.
        • et al.
        Thromboembolic risk after atriopulmonary, lateral tunnel, and extracardiac conduit Fontan surgery.
        J Am Coll Cardiol. 2019; 74: 1071-1081
        • Baumgartner H.
        • De Backer J.
        The ESC clinical practice guidelines for the management of adult congenital heart disease 2020.
        Eur Heart J. 2020; 41: 4153-4154
        • Stout K.K.
        • Daniels C.J.
        • Aboulhosn J.A.
        • et al.
        2018 AHA/ACC guideline for the management of adults with congenital heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines.
        J Am Coll Cardiol. 2019; 73: e81-192
        • Girnius A.
        • Zentner D.
        • Valente A.M.
        • et al.
        Bleeding and thrombotic risk in pregnant women with Fontan physiology.
        Heart. 2021; 107: 1390-1397
        • Alsaied T.
        • Alsidawi S.
        • Allen C.C.
        • et al.
        Strategies for thromboprophylaxis in Fontan circulation: a meta-analysis.
        Heart. 2015; 101: 1731-1737
        • Chong H.P.
        • Hodson J.
        • Selman T.J.
        • et al.
        Estimated blood loss in pregnant women with cardiac disease compared with low risk women: a restrospective cohort study.
        BMC Pregnancy Childbirth. 2019; 19: 325
        • Cauldwell M.
        • Von Klemperer K.
        • Uebing A.
        • et al.
        Why is post-partum haemorrhage more common in women with congenital heart disease?.
        Int J Cardiol. 2016; 218: 285-290
        • Moroney E.
        • Zannino D.
        • Cordina R.
        • et al.
        Does pregnancy impact subsequent health outcomes in the maternal Fontan circulation?.
        Int J Cardiol. 2020; 301: 67-73
        • Pierpont M.E.
        • Brueckner M.
        • Chung W.K.
        • et al.
        Genetic basis for congenital heart disease: revisited: a scientific statement from the American Heart Association.
        Circulation. 2018; 138: e653-711
        • Easter S.R.
        • Rouse C.E.
        • Duarte V.
        • et al.
        Planned vaginal delivery and cardiovascular morbidity in pregnant women with heart disease.
        Am J Obstet Gynecol. 2020; 222 (77. e71-77.e11)
        • Ruys T.P.
        • Roos-Hesselink J.W.
        • Pijuan-Domenech A.
        • et al.
        Is a planned caesarean section in women with cardiac disease beneficial?.
        Heart. 2015; 101: 530-536
        • Monteiro R.S.
        • Dob D.P.
        • Cauldwell M.R.
        • Gatzoulis M.A.
        Anaesthetic management of parturients with univentricular congenital heart disease and the Fontan operation.
        Int J Obstet Anesth. 2016; 28: 83-91
        • Arendt K.W.
        • Lindley K.J.
        Obstetric anesthesia management of the patient with cardiac disease.
        Int J Obstet Anesth. 2019; 37: 73-85
        • Wolff D.
        • van de Wiel H.B.M.
        • de Muinck Keizer M.E.
        • et al.
        Quality of life and sexual well-being in patients with a Fontan circulation: an explorative pilot study with a mixed method design.
        Congenit Heart Dis. 2018; 13: 319-326
        • Porrett P.M.
        Biologic mechanisms and clinical consequences of pregnancy alloimmunization.
        Am J Transplant. 2018; 18: 1059-1067
        • Ross H.J.
        • Law Y.
        • Book W.M.
        • et al.
        Transplantation and mechanical circulatory support in congenital heart disease: a scientific statement from the American Heart Association.
        Circulation. 2016; 133: 802-820