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Pregnancy and delivery in women after Fontan palliation
  1. W Drenthen1,
  2. P G Pieper1,
  3. J W Roos-Hesselink2,
  4. W A van Lottum1,
  5. A A Voors1,
  6. B J M Mulder3,
  7. A P J van Dijk4,
  8. H W Vliegen5,
  9. K M Sollie6,
  10. P Moons7,
  11. T Ebels6,
  12. D J van Veldhuisen1,
  13. for the ZAHARA Investigators
  1. 1Department of Cardiology, University Medical Center Groningen, Groningen, The Netherlands
  2. 2Department of Cardiology, Erasmus Medical Centre, Rotterdam, The Netherlands
  3. 3Department of Cardiology, Academic Medical Centre, Amsterdam, The Netherlands
  4. 4Department of Cardiology, Radboud University Medical Centre Nijmegen, Nijmegen, The Netherlands
  5. 5Department of Obstetrics and Gynaecology, University Medical Center Groningen, Groningen, The Netherlands
  6. 6Department of Thoracic Surgery, University Medical Center Groningen, Groningen, The Netherlands
  7. 7Department of Cardiology, University Hospitals Leuven, Leuven, Belgium
  1. Correspondence to:
    Dr Willem Drenthen
    Department of Cardiology, University Medical Center Groningen, University of Groningen, PO Box 30.001, 9700 RB Groningen, The Netherlands; w.drenthen{at}


Objectives: To evaluate the outcome of pregnancy in women after Fontan palliation and to assess the occurrence of infertility and menstrual cycle disorders.

Design and patients: Two congenital heart disease registries were used to investigate 38 female patients who had undergone Fontan palliation (aged 18–45 years): atriopulmonary anastomosis (n  =  23), atrioventricular connection (n  =  5) and total cavopulmonary connection (n  =  10).

Results: Six women had 10 pregnancies, including five miscarriages (50%) and one aborted ectopic pregnancy. During the remaining four live-birth pregnancies clinically significant complications were encountered: New York Heart Association class deterioration; atrial fibrillation; gestational hypertension; premature rupture of membranes; premature delivery; fetal growth retardation and neonatal death. Four of seven women who had attempted to become pregnant reported female infertility: non-specified secondary infertility (n  =  2), uterus bicornis (n  =  1) and related to endometriosis (n  =  1). Moreover, several important menstrual cycle disorders were documented. In particular, the incidence of primary amenorrhoea was high (n  =  15, 40%), which resulted in a significant increase in age at menarche (14.6 (SD 2.1) years, p < 0.0001, compared with the general population).

Conclusion: Women can successfully complete pregnancy after adequate Fontan palliation without important long-term sequelae, although it is often complicated by clinically significant (non-)cardiac events. In addition, subfertility or infertility and menstrual disorders were common.

  • CONCOR, CONgenital CORvitia
  • CS, caesarean section
  • NYHA, New York Heart Association
  • TCPC, total cavopulmonary connection

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  • Published Online First 31 January 2006

  • Supported by Netherlands Heart Foundation grant 2002 B125 to Dr P G Pieper and the Interuniversity Cardiology Institute, the Netherlands (ICIN). Prof Dr D J van Veldhuisen is an Established Investigator of the Netherlands Heart Foundation (grant D97.017)

  • Competing interests: None declared.

  • Ethics approval: The institutional review board or ethics committee at each of the participating tertiary centres (University Medical Center Groningen, Erasmus Medical Centre, Academic Medical Centre, Radboud University Medical Centre Nijmegen, Leiden University Medical Centre, University Hospitals Leuven) have approved the protocol