Mortalité maternelle chez les patientes atteintes de syndromes de Marfan ou d’Ehlers-Danlos vasculaire en France, 2001–2012

Abstract

ObjectiveTo describe maternal deaths in France associated with Marfan's syndrome or vascular Ehlers-Danlos syndrome. Study designA retrospective descriptive study based on data from the national confidential enquiry into maternal deaths, in France, during 2001–2012. Characteristics of the patients, their pregnancies and details of their deaths were analysed. The specific maternal mortality ratio by Marfan's syndrome or vascular Ehlers-Danlos syndrome was estimated. ResultsAmong 973 maternal deaths that occurred during the study period, five (0.4%) had a Marfan's syndrome (n=3) or a vascular Ehlers-Danlos syndrome (n=2), confirmed or suspected. The maternal mortality ratio due to Marfan's syndrome or vascular Ehlers-Danlos syndrome between 2001 and 2012 was 0.04/100,000 live births (IC 95% [0.011–0.2]). Three maternal deaths were caused by aortic dissections and two by other arterial ruptures. The deaths have occurred after 37 weeks of pregnancy for 4 patients, and at fifteen days of post-partum for one patient. The median age of death was 30 years. Three patients were nulliparous. Marfan's syndrome and vascular Ehlers-Danlos syndrome were not identified before the death of these five patients. ConclusionFive patients with, or suspected to have, Marfan's syndrome or vascular Ehlers-Danlos syndrome were identified. Early diagnosis of these syndromes in pregnant women before life threatening events is very important, especially to refer them to appropriate care.