Marfan’s Syndrome During Pregnancy

Abstract

Marfan's syndrome is characterized by progressive dilatation of the aortic root. This dilatation is accelerated by pregnancy, exposing patients to an increased risk of aortic dissection. Literature on the anesthetic management of delivery in patients with Marfan's syndrome consists only of case reports. We therefore conducted a retrospective review of medical records focusing on anesthetic management of delivery in patients with Marfan's syndrome in a national referral center. We reviewed the medical records of all pregnant women with Marfan's syndrome who were followed at their institution over a 6-year period. Sixteen pregnancies in 15 patients were analyzed. The initial aortic root diameter was larger than 40 mm in 9 patients and larger than 45 mm in 1 patient. Two patients did not receive β-blockers throughout pregnancy because of poor tolerance. One patient with an aortic root diameter of 47 mm did not receive β-blocker before 33 weeks' gestation because of late referral. This woman developed acute type 1 aortic dissection at 37 weeks, requiring emergency cesarean delivery under general anesthesia followed by aortic repair. Thirteen other patients underwent cesarean delivery, 1 under spinal anesthesia and 12 under general anesthesia. General anesthesia management included close arterial blood pressure monitoring, avoidance of high blood pressure, administration of opioids before delivery, and titrated nicardipine administration. Two patients (including one with intrauterine fetal death) underwent vaginal delivery under epidural analgesia. There were no maternal deaths. Pregnant women with Marfan's syndrome who received care in a multidisciplinary tertiary care setting that included active peripartum involvement of anesthesiologists had good clinical outcomes.