Fontan palliation in women

Pregnancy and delivery in women after Fontan palliation

W Drenthen1, P G Pieper1, J W Roos-Hesselink2, W A van Lottum1, A A Voors1, B J M Mulder3, A P J van Dijk4, H W Vliegen5, K M Sollie6, P Moons7, T Ebels6, D J van Veldhuisen1, for the ZAHARA Investigators
1) Department of Cardiology, University Medical Center Groningen, Groningen, The Netherlands; 2) Department of Cardiology, Erasmus Medical Centre, Rotterdam, The Netherlands; 3) Department of Cardiology, Academic Medical Centre, Amsterdam, The Netherlands; 4) Department of Cardiology, Radboud University Medical Centre Nijmegen, Nijmegen, The Netherlands; 5) Department of Obstetrics and Gynaecology, University Medical Center Groningen, Groningen, The Netherlands; 6) Department of Thoracic Surgery, University Medical Center Groningen, Groningen, The Netherlands; 7) Department of Cardiology, University Hospitals Leuven, Leuven, Belgium

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.

Heart 2006;92:1290-1294
© 2006 by BMJ Publishing Group Ltd & British Cardiovascular Society