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European Heart Journal Advance Access originally published online on August 17, 2005
European Heart Journal 2005 26(23):2581-2587; doi:10.1093/eurheartj/ehi439
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© The European Society of Cardiology 2005. All rights reserved. For Permissions, please e-mail: journals.permissions@oupjournals.org

Cardiac complications relating to pregnancy and recurrence of disease in the offspring of women with atrioventricular septal defects

Willem Drenthen1,*, Petronella G. Pieper1, Karin van der Tuuk1, Jolien W. Roos-Hesselink2, Adriaan A. Voors1, Bianca Mostert2, Barbara J.M. Mulder3, Philip Moons4, Tjark Ebels5, Dirk J. van Veldhuisen1 on behalf of the ZAHARA Investigators

1Department of Cardiology, University Medical Centre Groningen, PO Box 30.001, 9700 RB Groningen, The Netherlands
2Department of Cardiology, Erasmus Medical Center, Rotterdam, The Netherlands
3Department of Cardiology, Academic Medical Center, Amsterdam, The Netherlands
4Department of Cardiology, University Hospitals of Leuven, Leuven, Belgium
5Department of Thoracic Surgery, University Hospital Groningen, Groningen, The Netherlands

Received 16 March 2005; revised 30 June 2005; accepted 7 July 2005; online publish-ahead-of-print 17 August 2005.

* Corresponding author. Tel: +31 50 361 0248; fax: +31 50 361 4391. E-mail address: w.drenthen{at}thorax.umcg.nl

This paper was guest edited by Prof. M.A. Gatzoulis, Royal Brompton Hospital, London, UK

Aims In most pregnancy reports, atrioventricular septal defects (AVSD) are not differentiated from more simple septal defects, thus underestimating the risks of pregnancy. To investigate the magnitude and determinants of risk during pregnancy in female patients with balanced AVSD.

Methods and results Using a nation-wide registry (CONCOR), 79 female patients with balanced/isolated AVSD were identified. A total of 29 patients had 62 pregnancies, including 12 miscarriages (19%) and two elective abortions. Detailed recordings of each completed (>20 weeks gestation) pregnancy (n=48, 26 women) were obtained. Cardiovascular events complicated almost 40% of the completed pregnancies. In particular, post-partum persistence of pregnancy-related New York Heart Association (NYHA) class deterioration [23% mainly patients with residual atrial septal defects (ASD)] and deterioration of pre-existing left AV-valvular regurgitation (17%) were frequently recorded. Additional cardiac complications were arrhythmias (19%) and symptomatic heart failure (2%). Congenital heart disease (CHD) recurred in six children (12%): AVSD (n=4, three with left-sided hypoplasia), patent ductus arteriosus (n=1), and ASD (n=1). Three children died including two children with left-sided hypoplasia.

Conclusion Pregnancy is not always well tolerated in women with AVSD, predominantly due to NYHA class deterioration and worsening of pre-existing AV-valvular regurgitation. Offspring mortality is high (6.3%), primarily due to recurrence of complex CHD.

Key Words: Congenital heart disease • Pregnancy • Atrioventricular septal defect


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