Aortic dissection (AoD) during pregnancy is a rare but lethal condition and highlights the need for extensive elucidation. The aim of this study is to reveal the risk factors for AoD during pregnancy and to compare the 2 main risk factors, Marfan syndrome and pregnancy itself in the previously healthy woman. The pregnant patients developed AoD at 31.7 ± 7.6 weeks of gestation. It occurred much earlier in the Marfan patients than in the previously healthy women (30.7 ± 8.6 weeks of gestation vs 34.4 ± 4.4 weeks of gestation, P = 0.0263). In the Marfan patients, AoD developed in 3 (3.2%), 15 (15.8%), and 43 (46.3%) patients in the 3 trimesters, respectively, compared with 31 of the previously healthy women, and only in the third trimester. The neonates of the Marfan patients had better Apgar scores at 1 and 5 minutes, lower intubation rates, and fewer stays in the neonatal intensive care unit than those of the previously healthy women. Marfan syndrome and pregnancy itself in the previously healthy woman were the 2 main risk factors responsible for the occurrence of AoD during pregnancy. Marfan patients may develop AoD at an early age and an early stage of pregnancy, probably due to the preexisting weakened aortic wall. Better outcomes for the surviving neonates of Marfan patients compared with neonates of the previously healthy women might be the result of the poor condition of Marfan patients causing a higher death rate for those fetuses.
Aortic dissection (AoD) during pregnancy is rare and life-threatening for both mother and fetus. In Sweden it was estimated that the incidence of AoD was 14.5/1 000 000 in pregnant women vs 1.24/1 000 000 among nonpregnant women. However, according to the International Registry of Acute Aortic Dissections, being in the peripartum period of pregnancy confers a risk for AoD of 0.2%. Aortic dissection typically occurs in the third trimester of pregnancy or during the early postpartum period. Aortic dissections often develop among individuals with connective-tissue disorders associated with abnormalities of the aortic wall, such as those present in familial thoracic aortic aneurysm/dissection, Marfan syndrome, Loeys-Dietz syndrome, vascular Ehlers-Danlos syndrome, and bicuspid aortic valve disease or Turner syndrome. The majority of patients with Turner syndrome have aortic dilation with associated cardiovascular anomalies such as a bicuspid aortic valve, coarctation of the aorta, or systemic hypertension, which place the patients at higher risk of AoD. This is the case in particular when women with Turner syndrome, who are generally infertile because the condition is associated with premature ovarian failure, become pregnant through assisted reproductive technology. Histologic findings are similar to the classic cystic medial necrosis among young patients with sporadic AoD and suggest an underlying genetic defect of connective tissue, resulting in dissection at an early age in such patients. The risk of dissection is 5× to 18× higher in the presence of a bicuspid aortic valve compared with the tricuspid aortic valve, and pathology of the dilated aorta in the presence of a bicuspid aortic valve was similar to that of Marfan patients. Parai et al demonstrated significantly less elastic tissue in the aorta of bicuspid aortic valve patients. Women with aortic-root diameter >40 mm, rapid dilation of aortic dimensions, and/or previous dissection of the ascending aorta are at an increased risk of AoD during pregnancy. In addition, the risks of complicated AoD may be increased in parturients in the presence of familial thoracic aortic aneurysm, bicuspid aortic valve, coarctation of the aorta, hypertension, and drug abuse. However, the clinical characteristics of AoD during pregnancy have not been widely elaborated and the relationship between pregnancy and the risk factors for AoD remains insufficiently unexplained so far. This study aims to assess risk factors responsible for the development of acute AoD during pregnancy, comparing the 2 main underlying risk factors and discussing management controversies based on literature analyses.