Risk of Aortic Dissection in Pregnant Patients With the Marfan Syndrome

Patients with Marfan syndrome (MS) face a high risk of aortic dissection during pregnancy. A dilated aortic root (>40 to 45 mm) is considered a relative contraindication for pregnancy. We investigated the risk for aortic dissection and pregnancy outcome in patients with MS. Women with MS who attended our cardiology high-risk pregnancy clinic from 2006 to 2015 were followed clinically and with serial echocardiograms by a multidisciplinary team. Beta blockers were offered and titrated by blood pressure and heart rate. Patients with aortic root dilation ≥40 mm were considered high-risk patients with MS. A consistent increase in aortic root diameter of >1 mm during pregnancy was classified as dilation during pregnancy; 31 pregnancies in 19 patients with MS were followed. Four pregnancies were terminated early because of prenatal diagnosis of fetal MS and 4 additional babies born with MS. Eight pregnancies were in patients with a dilated aortic root (40 to 46 mm); 21 patients (68%) were treated with β blockers. There were 2 cases of postpartum aortic dissection (6.5%): 1 type A dissection in a woman with a dilated aortic root who declined β blockers (1 of 8, 12.5%) and 1 type B dissection. Increasing aortic root diameter (>1 mm) in pregnancy was significantly associated with later aortic dissection (2 of 6 vs 0 of 21, p = 0.04). No maternal deaths occurred. All high-risk women with MS gave birth by cesarean section, whereas in the non–high-risk group mode of delivery was by obstetric indication. Preterm delivery rate was 41% (11 of 27). One antenatal fetal death and no major neonatal morbidity or mortality were observed. In conclusion, pregnant patients with MS, especially those with a dilating aortic root, are at high risk of aortic dissection, even with tight control of blood pressure and heart rate.