Abstract

Abstract Background Cardiovascular disease is the leading cause of death during pregnancy with thoracic aortic dissection being one of the main causes. Thoracic aortic disease (TAD) is most commonly related to hereditary disorders and congenital heart malformations such as bicuspid aortic valve (BAV). Pregnancy is considered as a trigger for aortic complications in women with underlying aortic disease. With this analysis, we aim to study the maternal and fetal outcome of pregnancy in women with TAD. Methods The Registry Of Pregnancy And Cardiac disease (ROPAC) is a large, prospective and global registry and enrolled 4418 women from January 2011 onwards with known cardiac disease (mainly congenital and valvular disease) before pregnancy. Results TAD was present in 217 woman prior to pregnancy. Almost half of them were Marfan patients (MFS), 20% had a BAV, 7.4% Turner syndrome and 23% of patients had no underlying genetic defect or associated congenital heart defect. 35.5% of patients had aortic dilatation of which 6% had an aortic diameter above 45mm. Half of patients had previous pregnancies with no significant difference in parity between woman with and without aortic dilatation. Four patients of which 3 MFS patients had an acute aortic dissection (three type A and one type B aortic dissection) but no lethal (maternal of fetal) events occurred. Two patients presented with ventricular arrhythmia of whom one MFS patient. Caesarian section was not performed significantly more frequent in patients with aortic dilatation. Birth weight was lower in the group of woman with aortic dilatation, related to the use of beta-blocking agents. However, intra-uterine growth retardation and prematurity did not occur more frequently. Conclusion This ancillary analysis of the ROPAC data provides the first large prospective data on pregnancy risk of patients with a wide range of TAD. MFS patients are highly represented in the registry and are a vulnerable group to develop severe complications during pregnancy or peripartum with occurrence of aortic dissection in 3% of patients and ventricular arrhythmia in one. Serial follow-up by a specialized multidisciplinary team throughout pregnancy and postpartum period and patient tailored management of delivery is advised.

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