Type a aortic dissection in a postpartum woman with undiagnosed Marfan syndrome

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.