Abstract
A case of sudden death during early labour is reported from a small hospital. The patient had an incomplete Marfan syndrome and death resulted from rupture into the pericardium of a dissecting aneurysm of the aorta. No other similar cases with this combination of the MS, pregnancy and fatal aortic dissection were found among the records of 46 maternal deaths in 75 087 deliveries at two larger hospitals in Southwestern Finland. The case here reported and 12 others previously described showed a median age of 25 years at death. The dissection of the aorta, typically with a rupture into the pericardium and tamponade, usually occurred after the 8th month of gestation or in the puerperium. If the pregnancy continued to at least 8 months a healthy infant was obtained in most cases. No infants showed signs of the MS. Mostly the anatomical basis of the aortic dissection seemed to be typical cystics medionecrosis with small initial haemorrhages from the vasa vasorum.