[Maternal and fetal outcomes with aortic dissection in pregnant patients with Marfan syndrome].

OBJECTIVE

To evaluate the clinical characteristics of aortic dissection in pregnant patients with Marfan syndrome and the maternal and fetal outcomes in cardiovascular surgery.

METHODS

Seven pregnant women with Marfan syndrome with aortic dissection were identified, who were treated in Beijing Anzhen Hospital Affiliated to Capital Medical University between January 2012 and September 2014. Patient charts were reviewed for cardiovascular surgery, occurrence of complications, clinical features and the maternal and fetal outcomes.

RESULTS

(1) Among 7 patients, 4 cases were diagnosed as type A aortic dissection and 3 were cases diagnosed as type B aortic dissection. The diagnosis mainly depends on CT angiography. New York Heart Association (NYHA) classify into 5 of level II, 1 of level III, 1 of leveI IV. Except for 1 patient with cardiac tamponade lead to heart failure, the remaining 6 cases had no complications. (2) Three patients underwent heart surgery with cardiopulmonary bypass in second trimester and two patients underwent heart surgery in third trimester. Two patients terminated pregnancy before heart surgery (one of whom underwent artificial abortion, one of whom underwent cesarean section in second trimester). (3) The methods of cardiovascular surgeries were as follow: 3 of Bentall+Sun', 1 of Bentall+Sun'+ right coronary artery bypass grafting, 1 of Bentall, 1 of the whole chest aorta replacement surgery, and 1 of femoral artery catheter chest aorta with membrane mesh stent implantation. The diameter of aortic roots measured during operation were 5 cm in 2 cases, 7 cm in 2 cases and 10 cm in 2 cases respectively. Among the 7 cases, 3 were conducted cesarean sections during cardiovascular surgery, 1 was terminated pregnancy due to intrauterine fetal death after cardiovascular surgery, and 1 was conducted cesarean section due to severe early-onset preeclampsia at 30 weeks of pregnancy after cardiovascular surgery. (4) Among the 7 cases, 3 were conducted cesarean sections during cardiovascular surgery, the order of which was implemented cesarean section under general anesthesia firstly and then operated cardiovascular surgery with cardiopulmonary bypass and heparinization. Two were conducted cardiovascular surgery after termination of pregnancy by early artificial abortion operation or hysterotomy. 1 was conducted Bentall surgery at 18 weeks of pregnancy, after that the patient was receiving warfarin until the fetal brain hemorrhage was examined by ultrasound at 31 weeks of pregnancy; the patient was conducted cesarean section due to intrauterine fetal death. 1 was conducted heart surgery at 24 weeks of pregnancy and continue the pregnancy to 30 weeks, was conducted cesarean section due to severe early-onset preeclampsia at 30 weeks of pregnancy. (5) Among 7 patients, three patients underwent heart surgery with cardiopulmonary bypass combined with cesarean section. Two patients terminated pregnancy after heart surgery. Two patients terminated pregnancy before heart surgery. Six patients were alive and one patient died of multiple organ failure. (6) 3 cases of newborn with birth body mass between 1 080 to 1 490 g.1 case of birth died after 14 d. 2 cases for newborns were alive without exception.

CONCLUSION

Aortic dissection poses serious risk for pregnant women with Marfan syndrome and the fetus, and the mortality rates for both the mother and the fetus are high. Early diagnosis and appropriate treatment should be based on maternal and fetal conditions (such as aortic dissection, gestational age). When fetus is mature, cardiovascular surgery should be carried out with cesarean section. A multi-disciplinary team between obstetric and cardiovascular surgery is crucial to the outcome of these critical patients.