A patient with the Marfan syndrome died suddenly from aortic rupture and 
dissection in the early puerperium of her second pregnancy. Although the 
association of the Marfan syndrome and pregnancy is extremely rare, the case 
reported here being only the fifth on record, the concurrence of dissecting 
aneurysm or aortic dissection with pregnancy is more frequent. Furthermore it is 
accepted that aortic dissection in young women below the age of 40 is more 
common in the pregnant than those not pregnant. The cause of the enhancing 
effect of pregnancy is unknown but is thought to be endocrine since the 
stability of connective tissue can be influenced by hormones, particularly the 
sex steroids. An unusual feature of the present case is the florid inflammatory 
reaction in the adventitia of the aorta, not specifically related to pregnancy 
or to the Marfan syndrome, and it is assumed that in this patient the congenital 
defect of connective tissue assumed to be the basis of the Marfan syndrome is 
associated with an acute collagen change or necrosis, possibly illustrating a 
link between the heritable disorders of connective tissue and the diffuse 
collagen disease.