Type A Aortic Dissection in a Postpartum Woman with Undiagnosed Marfan Syndrome
Keywords:
Aortic dissection; Marfan syndrome; pregnancyAbstract
Objective: To describe perioperative management for type A aortic dissection in a postpartum woman with
undiagnosed Marfan syndrome.
Case presentation: A 30-year-old G2P1 Thai female at 39 weeks of gestation with undiagnosed Marfan syndrome
gave birth at Siriraj Hospital, Bangkok, Thailand. After delivery, she developed episodes of hypotension and right
hemiparesis. She was diagnosed with Marfan syndrome by clinical features of severe myopia, aortic root diameter
Z-score ≥ 2 and bonafide FBN1 mutation detected by genetic study. The imaging studies showed strong evidences
of type A aortic dissection and she underwent uneventful ascending aortic replacement under general anesthesia
with extracorporeal bypass. The perioperative management was described.
Conclusion: Hemodynamic instability and pregnancy-related physiologic changes are the key factors to be concerned
for perioperative management. The collaboration among cardiac surgeon, cardiac anesthesiologist and perfusionist
is of great importance.
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