Emergency cesarean section due to acute aortic dissection type A (Debakey I) without Marfan syndrome: a case report and review of the literature

Abstract

Background: Acute aortic dissection during pregnancy is an uncommon but important emergency due to its lethal risk to both mother and child. The dissection usually involves the ascending aorta or the aortic arch. Although additional affection of the descending aorta up to bifurcation is possible, further increasing the risk of organ malperfusion, full-length aortic dissection (DeBakey I) is known to be very rare. Dissection during pregnancy has been reported predominantly in combination with Marfan syndrome. Acute aortic dissection Stanford type A (AADA) DeBakey I during pregnancy without signs of Marfan syndrome as a warning signal is very uncommon in the current literature.

Objectives: The etiology, diagnosis, differential diagnosis, and management of this rare disease are discussed in relation to the current literature.

Case report: We report the case of an athletic 34-year-old woman in the third trimester of pregnancy, without history of previous diseases, who presented to our Emergency Department after collapsing. In the resuscitation department, an emergency cesarean section was performed due to the start of circulation failure in the mother. Computed tomography scan revealed a severe aortic dissection starting from 1 cm distal the aortic valve over the full length up to the iliac arteries, involving the brachiocephalic and carotid arteries up to the level of the larynx. Emergency replacement of the ascending aorta and the aortic arch was performed. Both the mother and baby survived and were doing well 1 year postoperatively.

Conclusion: This alarming result of AADA (DeBakey I) in late pregnancy without obvious warnings such as Marfan syndrome illustrates the importance of performing early imaging in similar cases.

Keywords: Emergency Department; acute aortic dissection type A (AADA); aortic replacement; cesarean section; pregnancy.