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Congenital heart disease
Original research
Bleeding and thrombotic risk in pregnant women with Fontan physiology
  1. Andrea Girnius1,
  2. Dominica Zentner2,3,
  3. Anne Marie Valente4,
  4. Petronella G Pieper5,
  5. Katherine E Economy6,
  6. Magalie Ladouceur7,8,
  7. Jolien W Roos-Hesselink9,
  8. Carri Warshak10,
  9. Sara L Partington11,12,
  10. Zhiqian Gao13,
  11. Nicholas Ollberding14,15,
  12. Michelle Faust13,
  13. Saulius Girnius16,
  14. Harald Kaemmerer17,
  15. Nicole Nagdyman17,
  16. Scott Cohen18,
  17. Mary Canobbio19,
  18. Teiji Akagi20,
  19. Jasmine Grewal21,
  20. Elisa Bradley22,
  21. Yonathan Buber23,
  22. Joseph Palumbo24,
  23. Niki Walker25,
  24. Jamil Aboulhosn26,
  25. Erwin Oechslin27,28,
  26. Helmut Baumgartner29,
  27. Wesam Kurdi30,
  28. Wendy M Book31,
  29. Barbara J M Mulder32,
  30. Gruschen R Veldtman33
  1. 1 Anesthesiology, University of Cincinnati College of Medicine, Cincinnati, Ohio, USA
  2. 2 Department of Cardiology and Department of Genomic Medicine, Royal Melbourne Hospital, Melbourne, Victoria, Australia
  3. 3 Faculty of Medicine, Dentistry and Health Sciences, University of Melbourne, Melbourne, Victoria, Australia
  4. 4 Brigham and Women's Hospital, Department of Medicine, Division of Cardiovascular Disease, Department of Cardiology Harvard Medical School, Boston, Massachusetts, USA
  5. 5 Department of Cardiology, University Medical Center Groningen, Groningen, The Netherlands
  6. 6 Brigham and Women's Hospital, Department of Obstetrics and Gynecology, Division Maternal Fetal Medicine, Brigham and Women's Hospital, Boston, Massachusetts, USA
  7. 7 Cardiology, Hopital Europeen Georges Pompidou, Paris, France
  8. 8 Pediatric Cardiology, Hopital Universitaire Necker-Enfants Malades, Paris, France
  9. 9 Department of Cardiology, Erasmus MC, Rotterdam, The Netherlands
  10. 10 Obstetrics and Gynecology, University of Cincinnati College of Medicine, Cincinnati, Ohio, USA
  11. 11 Division of Cardiovascular Medicine, Hospital of the University of Pennsylvania, Philadelphia, Pennsylvania, USA
  12. 12 Division of Cardiology, The Children’s Hospital of Philadelphia, Philadelphia, Pennsylvania, USA
  13. 13 Heart Institute, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, USA
  14. 14 Department of Pediatrics, Cincinnati Children's Hospital Medical Centre, Cincinnati, Ohio, USA
  15. 15 Division of Biostatistics and Epidemiology, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, USA
  16. 16 Department of Hematology, Trihealth Cancer Institute, Cincinnati, Ohio, USA
  17. 17 Deutsches Herzzentrum München, Germany, Munich, Germany
  18. 18 Department of Cardiology, The Medical College of Wisconsin, Milwaukee, Wisconsin, USA
  19. 19 School of Nursing, University of California Los Angeles, Los Angeles, California, USA
  20. 20 Cardiovascular Medicine, Okayama University, Okayama, Japan
  21. 21 Cardiology, The University of British Columbia Faculty of Medicine, Vancouver, British Columbia, Canada
  22. 22 Adult Congenital Heart Disease, The Ohio State University & Nationwide Children's Hospital, Nationwide Children's Hospital, Columbus, Ohio, USA
  23. 23 Department of Medicine, Division of Cardiology, University of Washington School of Medicine, Seattle, Washington, USA
  24. 24 Haematology, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, USA
  25. 25 Golden Jubilee Hospital, Glasgow, UK
  26. 26 Ahmanson/UCLA ACHD Center, David Geffen School of Medicine at UCLA, Los Angeles, California, USA
  27. 27 Toronto Congenital Cardiac Centre for Adults, Peter Munk Cardiac Centre, University Heatlh Network and University of Toronto, Toronto, Ontario, Canada
  28. 28 Bitove Family Professorship of Adult Congenital Heart Disease, Toronto General Hospital, Toronto, Ontario, Canada
  29. 29 Department of Cardiology III: Adult Congenital and Valvular Heart Disease, University Hospital Muenster, Muenster, Albert Schweitzer Campus 1, Building A1, 48149 Muenster, Germany
  30. 30 King Faisal Specialist Hospital and Research Center, Riyadh, Saudi Arabia
  31. 31 Internal Medicine, Division of Cardiology, Emory University, Atlanta, Georgia, USA
  32. 32 Cardiology, Academic Medical Center, Amsterdam, The Netherlands
  33. 33 Adult Congenital Heart Disease, King Faisal Specialist Hospital and Research Centre, Riyadh, Saudi Arabia
  1. Correspondence to Dr Gruschen R Veldtman, Adolescent and Adult Congenital Heart Disease Program, King Faisal Specialist Hospital and Research Center, Riyadh 45229, Saudi Arabia; gruschen{at}me.com

Abstract

Background/objectives Pregnancy may potentiate the inherent hypercoagulability of the Fontan circulation, thereby amplifying adverse events. This study sought to evaluate thrombosis and bleeding risk in pregnant women with a Fontan.

Methods We performed a retrospective observational cohort study across 13 international centres and recorded data on thrombotic and bleeding events, antithrombotic therapies and pre-pregnancy thrombotic risk factors.

Results We analysed 84 women with Fontan physiology undergoing 108 pregnancies, average gestation 33±5 weeks. The most common antithrombotic therapy in pregnancy was aspirin (ASA, 47 pregnancies (43.5%)). Heparin (unfractionated (UFH) or low molecular weight (LMWH)) was prescribed in 32 pregnancies (30%) and vitamin K antagonist (VKA) in 10 pregnancies (9%). Three pregnancies were complicated by thrombotic events (2.8%). Thirty-eight pregnancies (35%) were complicated by bleeding, of which 5 (13%) were severe. Most bleeds were obstetric, occurring antepartum (45%) and postpartum (42%). The use of therapeutic heparin (OR 15.6, 95% CI 1.88 to 129, p=0.006), VKA (OR 11.7, 95% CI 1.06 to 130, p=0.032) or any combination of anticoagulation medication (OR 13.0, 95% CI 1.13 to 150, p=0.032) were significantly associated with bleeding events, while ASA (OR 5.41, 95% CI 0.73 to 40.4, p=0.067) and prophylactic heparin were not (OR 4.68, 95% CI 0.488 to 44.9, p=0.096).

Conclusions Current antithrombotic strategies appear effective at attenuating thrombotic risk in pregnant women with a Fontan. However, this comes with high (>30%) bleeding risk, of which 13% are life threatening. Achieving haemostatic balance is challenging in pregnant women with a Fontan, necessitating individualised risk-adjusted counselling and therapeutic approaches that are monitored during the course of pregnancy.

  • Fontan physiology
  • pregnancy

Data availability statement

Data is available upon reasonable request and upon agreement of the coathors and individual insitutions

https://doi.org/10.1136/heartjnl-2020-317397

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    Data availability statement

    Data is available upon reasonable request and upon agreement of the coathors and individual insitutions

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    Footnotes

    • Twitter @gabapt20

    • Contributors AG and GRV are primarily responsible for the conceptualisation, planning, data analysis and writing. ZG and NO are responsible for the primary statistical analysis and contributed to the manuscript. MF is responsible for project management and coordination, including data collection. DZ, AMV, PGP, KEE, ML, JWR-H, CW, SLP, SG, HK, NN, SC, MC, TA, JG, EB, YB, JPV, NW, JA, EO, HB, WK, WMB and BJMM contributed to conceptualisation, data collection and editing of the manuscript. The corresponding author has the right to grant on behalf of all authors and does grant on behalf of all authors, an exclusive license (or non-exclusive for government employees) on a worldwide basis to the BMJ Publishing Group Ltd and its Licensees to permit this article (if accepted) to be published in HEART editions and any other BMJPGL products to exploit all subsidiary rights.

    • Funding The Heart Institute, Cincinnati Children’s Hospital Medical Center, 333 Burnett Avenue, Cincinnati.

    • Competing interests None declared.

    • Patient and public involvement Patients and/or the public were not involved in the design, or conduct, or reporting, or dissemination plans of this research.

    • Provenance and peer review Not commissioned; externally peer reviewed.