Aortic dissection in pregnancy: A case of Marfan's syndrome

Abstract

1. 1. A review of the literature on aortic dissection is presented, emphasizing the different etiologic and pathogenetic factors involved and their specific histologic features. Aortic dissection in the young individual is characterized by an early degeneration of the elastic tissue components of the aortic medial layer, as is aortic dissection in pregnancy and Marfan's syndrome. Following the degeneration of the elastic laminae, secondary changes are observed in the smooth muscle tissue, with ingrowth of numerous thin-walled vessels and development of cystic mucin-filled spaces, whereby the aortic wall becomes markedly weakened and susceptible to dissection. 2. 2. A case of Marfan's syndrome with aortic dissection during pregnancy is presented exhibiting less elastic tissue bulk in the aorta than normally seen. The question is raised as to whether an absolute decrease in elastic tissue mass is a constant feature in Marfan's syndrome in addition to the fragmentation of the elastic laminae previously described. 3. 3. Repeated bouts of aortic dissection were observed from the twenty-second throughout the thirty-first week of pregnancy in the present case of Marfan's syndrome, finally leading to the development of a triple-barreled aorta. Cesarean section was done electively 2 weeks before term, with delivery of a normal male infant. After delivery no further episodes of dissection were recognized. The patient expired in congestive failure 13 months after onset of dissection, which was 8 months post partum. 4. 4. We believe that gestation enhanced the aortic dissection and shortened the patient's life. It is prudent to individualize termination of pregnancy in each case, depending on the stage of gestation at which the dissection occurs. Interruption of the early pregnancy is recommended as soon as the cardiac condition is stabilized. If possible, whenever the gestation is in the third trimester, the patient should be carried to viability of the fetus and delivery by cesarean section. The authors feel that one should deviate from the usual management of obstetric care in aortic dissection and that cesarean section is the method of choice for termination of the pregnancy after viability of the fetus is assured.