Maternal complication of pregnancy in Marfan syndrome

Pacini, Laure; Digne, Frank; Boumendil, Ariane; Muti, Christine; Detaint, Delphine; Boileau, Catherine; Jondeau, Guillaume

doi:10.1016/j.ijcard.2008.04.035

« Previous Next »International Journal of Cardiology
Volume 136, Issue 2 , Pages 156-161, 14 August 2009

Maternal complication of pregnancy in Marfan syndrome

Laure Pacini
- Centre de Référence National Syndrome de Marfan et apparentés⁎, Hôpital Bichat, AP-HP, Faculté Paris 7, 75018 Paris, France
Frank Digne
- Centre de Référence National Syndrome de Marfan et apparentés⁎, Hôpital Bichat, AP-HP, Faculté Paris 7, 75018 Paris, France
Ariane Boumendil
- Service d'informatique locale, Hôpital Ambroise Paré, AP-HP, Faculté Paris-Ile-de-France-Ouest, 92100 Boulogne-Billancourt, France.
Christine Muti
- Centre de Référence National Syndrome de Marfan et apparentés⁎, Hôpital Bichat, AP-HP, Faculté Paris 7, 75018 Paris, France
Delphine Detaint
- Centre de Référence National Syndrome de Marfan et apparentés⁎, Hôpital Bichat, AP-HP, Faculté Paris 7, 75018 Paris, France
- Service de Cardiologie, Hôpital Bichat, AP-HP, Faculté Paris 7, 75018 Paris, France
Catherine Boileau
- Centre de Référence National Syndrome de Marfan et apparentés⁎, Hôpital Bichat, AP-HP, Faculté Paris 7, 75018 Paris, France
- Service de Biochimie, d'Hormonologie et de Génétique Moléculaire, Hôpital Ambroise Paré, AP-HP, Faculté Paris-Ile-de-France-Ouest, 92100 Boulogne-Billancourt, France
Guillaume Jondeau
- Service de Cardiologie, Hôpital Bichat, AP-HP, Faculté Paris 7, 75018 Paris, France
- Centre de Référence National Syndrome de Marfan et apparentés⁎, Hôpital Bichat, AP-HP, Faculté Paris 7, 75018 Paris, France
- Corresponding author. Centre de Référence National Syndrome de Marfan et apparentés, Hôpital Bichat, 46 rue Henri Huchard, 75018 Paris, France. Tel.: +33 1 40 25 68 10; fax: +33 1 40 25 67 32.

Received 30 October 2007; received in revised form 14 April 2008; accepted 23 April 2008. published online 16 July 2008.

Abstract

Objectives

Evaluate the risk of aortic complications (aortic dissection or prophylactic aortic surgical replacement) associated with pregnancy in Marfan syndrome in the absence of specific care.

Background

Data are scarce on aortic complications during pregnancy in Marfan syndrome.

Methods

Retrospective study on a large population (415 patients) followed up in our multidisciplinary out-patient clinic devoted to Marfan syndrome. Women over 18 years of age were divided into 2 groups: 85 had been pregnant (MFSP+) giving birth to 136 children through 160 pregnancies; 68 had not been pregnant (MFSP−). The occurrence of aortic complication was compared between the 2 groups using a piecewise discrete-time model.

Results

In MFSP+, 7 aortic complications occurred during 160 pregnancies (4.4%) and 17 aortic complications occurred during 1870 years of follow-up out of pregnancy. In MFSP−, 14 aortic complications occurred over 940 years of follow-up. Pregnancy was associated with a 5 fold increase in risk of aortic complication, which did not translate into increased risk during life, suggesting that pregnancy may act as a revealer in women prone to aortic complication.