Surgery for acute type A aortic dissection in pregnant patients with Marfan syndrome

Objective: Acute type A aortic dissection during pregnancy can be fatal to both the mother and the fetus. The goal of the present study was to characterize the prevalence, treatment and outcomes of this dangerous condition in an effort to determine optimal management. Methods: A retrospective study was conducted using data from four Marfan patients with acute type A aortic dissection during pregnancy at our institution between 1991 and 2003. Results: The mean gestational period at the time of operative repair was 31 weeks, with a range of 26–34 weeks, and the aortic root diameter ranged from 35 to 85 mm. Two of the four patients underwent a combined operation with cesarean section followed by aortic repair. One patient underwent operative aortic repair following spontaneous delivery. The final patient underwent aortic repair with the fetus remaining in situ. Median sternotomy and cardiopulmonary bypass were established via the femoral artery with direct right atria drainage and left atrial venting in all patients. Composite graft replacement combined with re-implantation of the coronary artery and aortic valve replacement were performed in three patients, and aortic valve replacement with coronary artery bypass grafting of the right coronary artery was performed in one patient. Three of four patients underwent aortic arch repair utilizing antegrade cerebral perfusion and deep hypothermia with total circulatory arrest. The patient that underwent operative correction with the fetus remaining in situ experienced fetal demise with miscarriage just after cardiac surgery, and the patient died 4 days later secondary to disseminated intravascular coagulation and multi-organ failure. The remaining three cases recovered uneventfully, and the mothers and babies were discharged in good condition. Conclusions: Based on these data, we advocate cesarean section with concomitant aortic repair for patients with Marfan syndrome and acute type A aortic dissection during pregnancy. Minimization of deep hypothermic circulatory arrest time is also recommended for cases in which the fetus remains in situ.