Advertisement
Case Conference| Volume 37, ISSUE 1, P167-176, January 2023

Anesthetic Management in Parturients With Fontan Physiology

      The Fontan procedure is performed as the final stage of palliation for patients with single-ventricle congenital heart diseases, such as tricuspid atresia, hypoplastic left-heart syndrome, or severely unbalanced atrioventricular canal defect. It creates a functional series circulation in which all pulmonary blood flow is derived from the systemic venous return that subsequently supplies ventricular preload in the absence of a subpulmonic pumping chamber. Since its inception in 1971, surgical advancement in the Fontan procedure has led to improved long-term outcomes, with transplant-free survival reaching 84% at 20 years.
      • Fontan F
      • Baudet E.
      Surgical repair of tricuspid atresia.
      ,
      • Khairy P
      • Fernandes SM
      • Mayer Jr., JE
      • et al.
      Long-term survival, modes of death, and predictors of mortality in patients with Fontan surgery.
      There are an estimated 28,000 adult patients with Fontan physiology alive worldwide, a population that is expected to double within 20 years.
      • Akintoye E
      • Miranda WR
      • Veldtman GR
      • et al.
      National trends in Fontan operation and in-hospital outcomes in the USA.
      ,
      • d'Udekem Y
      • Iyengar AJ
      • Galati JC
      • et al.
      Redefining expectations of long-term survival after the Fontan procedure: Twenty-five years of follow-up from the entire population of Australia and New Zealand.
      Many of these patients are females of childbearing age, making pregnancy and childbirth in the parturient with Fontan physiology a reality.

      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. doi: 10.1161/cir.0000000000000696. Accessed May 1, 2022.

      Key Words

      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 Journal of Cardiothoracic and Vascular Anesthesia
      Already a print subscriber? Claim online access
      Already an online subscriber? Sign in
      Institutional Access: Sign in to ScienceDirect

      References

        • Fontan F
        • Baudet E.
        Surgical repair of tricuspid atresia.
        Thorax. 1971; 26: 240-248
        • Khairy P
        • Fernandes SM
        • Mayer Jr., JE
        • et al.
        Long-term survival, modes of death, and predictors of mortality in patients with Fontan surgery.
        Circulation. 2008; 117: 85-92
        • Akintoye E
        • Miranda WR
        • Veldtman GR
        • et al.
        National trends in Fontan operation and in-hospital outcomes in the USA.
        Heart. 2019; 105: 708-714
        • d'Udekem Y
        • Iyengar AJ
        • Galati JC
        • et al.
        Redefining expectations of long-term survival after the Fontan procedure: Twenty-five years of follow-up from the entire population of Australia and New Zealand.
        Circulation. 2014; 130: S32-S38
      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. doi: 10.1161/cir.0000000000000696. Accessed May 1, 2022.

        • Kutty S
        • Jacobs ML
        • Thompson WR
        • et al.
        Fontan circulation of the next generation: Why it's necessary, what it might look like.
        J Am Heart Assoc. 2020; 9e013691
        • Garcia Ropero A
        • Baskar S
        • Roos Hesselink JW
        • et al.
        Pregnancy in women with a Fontan circulation: A systematic review of the literature.
        Circ Cardiovasc Qual Outcomes. 2018; 11e004575
        • Dimopoulos K
        • Diller GP
        • Koltsida E
        • et al.
        Prevalence, predictors, and prognostic value of renal dysfunction in adults with congenital heart disease.
        Circulation. 2008; 117: 2320-2328
        • Khairy P
        • Poirier N
        • Mercier LA.
        Univentricular heart.
        Circulation. 2007; 115: 800-812
        • 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-e87
        • Regitz-Zagrosek V
        • Roos-Hesselink JW
        • Bauersachs J
        • et al.
        2018 ESC Guidelines for the management of cardiovascular diseases during pregnancy.
        Eur Heart J. 2018; 39: 3165-3241
        • Monteiro RS
        • Dob DP
        • Cauldwell MR
        • et al.
        Anaesthetic management of parturients with univentricular congenital heart disease and the Fontan operation.
        Int J Obstet Anesth. 2016; 28: 83-91
        • Elkayam U
        • Goland S
        • Pieper PG
        • et al.
        High-risk cardiac disease in pregnancy: Part I.
        J Am Coll Cardiol. 2016; 68: 396-410
        • Davis MB
        • Walsh MN.
        Cardio-obstetrics.
        Circ Cardiovasc Qual Outcomes. 2019; 12e005417
        • Harris PA
        • Taylor R
        • Thielke R
        • et al.
        Research electronic data capture (REDCap)–a metadata-driven methodology and workflow process for providing translational research informatics support.
        J Biomed Inform. 2009; 42: 377-381
        • Book WM
        • Gerardin J
        • Saraf A
        • et al.
        Clinical phenotypes of Fontan failure: Implications for management.
        Congenit Heart Dis. 2016; 11: 296-308
        • Byrne RD
        • Weingarten AJ
        • Clark DE
        • et al.
        More than the heart: Hepatic, renal, and cardiac dysfunction in adult Fontan patients.
        Congenit Heart Dis. 2019; 14: 765-771
        • Sanghavi M
        • Rutherford JD.
        Cardiovascular physiology of pregnancy.
        Circulation. 2014; 130: 1003-1008
        • Walker F.
        Pregnancy and the various forms of the Fontan circulation.
        Heart. 2007; 93: 152-154
        • Gewillig M
        • Brown SC
        • Eyskens B
        • et al.
        The Fontan circulation: Who controls cardiac output?.
        Interact Cardiovasc Thorac Surg. 2010; 10: 428-433
        • Gewillig M
        • Brown SC.
        The Fontan circulation after 45 years: Update in physiology.
        Heart. 2016; 102: 1081-1086
        • Cohen AM
        • Mulvein J.
        Obstetric anaesthetic management in a patient with the Fontan circulation.
        Br J Anaesth. 1994; 73: 252-255
        • Rodriguez FH
        • Book WM.
        Management of the adult Fontan patient.
        Heart. 2020; 106: 105-110
        • 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
        • Yokouchi-Konishi T
        • Ohta-Ogo K
        • Kamiya CA
        • et al.
        Clinicopathologic study of placentas from women with a Fontan circulation.
        Circ J. 2021; 86: 138-146
        • McLaughlin VV
        • Shah SJ
        • Souza R
        • Humbert M.
        Management of pulmonary arterial hypertension.
        J Am Coll Cardiol. 2015; 65: 1976-1997
        • Gewillig M.
        The Fontan circulation.
        Heart. 2005; 91: 839-846
        • Nayak S
        • Booker PD.
        The Fontan circulation.
        EJA Educ. 2008; 8: 26-30
        • Horlocker TT
        • Vandermeuelen E
        • Kopp SL
        • et al.
        Regional anesthesia in the patient receiving antithrombotic or thrombolytic therapy: American Society of Regional Anesthesia and Pain Medicine Evidence-Based Guidelines (Fourth Edition).
        Reg Anesth Pain Med. 2018; 43: 263-309
        • Eagle SS
        • Daves SM.
        The adult with Fontan physiology: Systematic approach to perioperative management for noncardiac surgery.
        J Cardiothorac Vasc Anesth. 2011; 25: 320-334
        • Lee S CM
        • Wendler R
        Cardiac effects of drugs used for induction of labour and prevention and treatment of postpartum hemorrhage.
        IJC Congenital Heart Disease. 2021; 5100208
        • Bishop L
        • Lansbury A
        • English K.
        Adult congenital heart disease and pregnancy.
        BJA Educ. 2018; 18: 23-29
        • Cauldwell M
        • Swan L
        • Uebing A
        • et al.
        The management of third stage of labour in women with heart disease needs more attention.
        Int J Cardiol. 2016; 223: 23-24

      Linked Article