Pregnancy-Associated Aortic Dilatation or Dissection in Japanese Women With Marfan Syndrome

Abstract

Background: Aortic dilatation and dissection are severe complications of pregnancy that may cause maternal death. The purpose of the present study was to investigate risk factors for aortic dilatation or dissection in pregnant Japanese women with Marfan syndrome. Methods and Results:A total of 28 patients with Marfan syndrome were investigated retrospectively during pregnancy and after delivery at 1 institution. These patients were divided into 2 groups: those who experienced aortic dilatation or dissection (group D, n=11) and those who did not (group ND, n=17). In group D, aortic dilatation or dissection occurred in 7 cases during pregnancy (2 in the 2 nd trimester, 5 in the 3 rd trimester) and 4 cases after birth. The 2 cases in the 2 nd trimester involved aortic dilatation >60 mm and those patients underwent hemiarch replacement and a David operation, respectively. Delivery by cesarean section (64% vs. 18%, P<0.05), sinus of Valsalva ≥40 mm (86% vs. 21%, P<0.05), aortic size index (size of sinus of Valsalva/body surface area) ≥25 mm/m 2 (7/7, 100% vs. 0/14, 0%, P<0.

Pregnancy-Related Acute Myocardial Infarction in Japan

“…The vessel wall can become weakened during pregnancy and remain weakened for up to 3 months postpartum. 25 A decrease in collagen synthesis, eosinophilic infiltration, excess progesterone, and fluctuating estrogen levels might contribute to the development of dissection. 23 An association between ritodrine and AMI suggests that increased stroke volume and shear forces are involved.…”

Pregnancy and Marfan syndrome

“…In summary, the rates of aortic complications reported in prospective studies appear to reach on average 4.0% (3,19,41,53) and is 2-6% in large retrospective reports including 1,142 pregnancies (20,22,(53)(54)(55)(56)(57)(58). The expected rate of AoD is ranging from 1% in women with aortic diameter <40 mm to as much as 10% in high-risk patients (those with aortic root diameter >40 mm, rapid dilatation or previous dissection of the ascending aorta) (3,9,53,59).…”

Analysis of the Risk Associated With Pregnancy in Women With Marfan Syndrome

“…Katsuragi et al clearly demonstrate that the aortic size index (diameter of the Valsalva/body surface area) is a better indicator than the absolute size. 7 They demonstrate that an aortic size index >25 mm/m 2 is a risk for an aortic event in pregnant Japanese women with MFS.…”

“…Katsuragi et al report that FBN1 mutation was found more commonly in patients with aortic complications than in those without them, though the difference did not reach statistical significance. 7 Although more than 100 FBN1 mutations have been described in the literature, the identification of the type of mutation is still insufficient to predict the phenotype of MFS. 5 Moreover, the current screening techniques identify FBN1 mutations in at least 70% of patients with classic MFS.…”