Abstract
Background Pregnant women with Marfan syndrome (MFS) are known to be at increased risk of aortic
dissection; however, cases of aortic rupture are extremely rare. There is lack of
consensus on the exact site and size of aortic diameter measurement that increases
this risk, and whether this applies to both Type A and Type B dissections.
Case A 23-year-old G2P1001 with known Marfan syndrome who underwent an uncomplicated antepartum
and intrapartum course. She experienced persistent backache 10 days postpartum that
led to the diagnosis of Stanford Type B dissection. The patient was hospitalized for
close observation. Dissection progressed to aortic rupture within 24 hours that required
emergent thoracic endovascular aortic repair. She had an uncomplicated postoperative
course.
Conclusion Our report demonstrates rupture of a known aortic dissection within a very short
time in the postpartum period. The case highlights the importance of patient education
and close surveillance especially in the postpartum period. It also brings home the
value of imaging of the whole aorta rather than focusing on the ascending alone. Multidisciplinary
care and timely diagnosis and intervention likely led to the favorable outcome in
our case.
Keywords
Marfan syndrome - pregnancy - aortic rupture - Type B aortic dissection
