Risk of Aortic Dissection in Pregnant Patients With the Marfan Syndrome

Peripartum type B aortic dissection in patients with Marfan syndrome who underwent aortic root replacement: a case series study

“…2,9,10 Since the release of the revised Ghent nosology, there have been two cohort studies on pregnancy outcomes of MFS patients collected from multiple institutions 11,12 and five case series. [13][14][15][16][17] Collectively, these reports indicate that (1) pregnant MFS patients have a higher risk of AD compared with non-pregnant MFS patients, 11,12 and (2) pregnancy increases the risk of aortic complications in the long-term. 13 However, further evaluation of pregnancy outcomes in patients with MFS diagnosed according to the revised Ghent nosology is still required to establish guidelines for management of pregnant patients with this syndrome.…”

“…Diagnostic criteria of MFS were revised in 2010 (revised Ghent nosology) to place more emphasis on causative genetic analyses, aortic root aneurysm/dissection, and ectopia lentis (EL) . Since the release of the revised Ghent nosology, there have been two cohort studies on pregnancy outcomes of MFS patients collected from multiple institutions and five case series . Collectively, these reports indicate that (1) pregnant MFS patients have a higher risk of AD compared with non‐pregnant MFS patients, and (2) pregnancy increases the risk of aortic complications in the long‐term .…”

Pregnancy and Marfan syndrome

“…Such an assumption is supported by several reports of significantly lower rate of complications. Pyeritz et al (17) Several studies focused on the potential growth of the aorta in pregnant women with MFS and have reported contradictory results (19,22,51,53,55). Rossiter et al (19) prospectively evaluated 45 pregnancies in 21 women with MFS and found AoD in only 2 while the remaining women with an aortic diameter <40 mm tolerated pregnancy well without an increase in aortic diameter.…”

“…Maternal and fetal mortality were reported in two patients, and fetal loss in two cases despite of maternal survival. Reviewing the English literature between 1995 and May 2017 (23-35), we found 38 additional MFS cases with pregnancy-related complications (36)(37)(38)(39)(40)(41)(42)(43)(44)(45)(46)(47)(48)(49)(50)(51)(52). Sixty-three women developed an acute AoD: 59% of the ascending aorta, 32% of the descending aorta and both in 9%.…”

“…Pregnancy in women with MFS has been shown to be associated with a high rate (up to 40%) of obstetric complications, such as premature delivery, mainly due to premature rupture of membranes, intrauterine growth restriction, small for gestational age and neonatal mortality (8,9,22,41,51,56,65,66).…”