Key result
Fatal postpartum aortic dissection in Marfan syndrome highlights the critical need for multidisciplinary screening.
Why the study?
Marfan syndrome patients have great genotypic and phenotypic variability and face increased risk of complications during and after pregnancy.
Case Report (n=1)
Patients with a family history of Marfan syndrome require thorough multidisciplinary evaluation and genetic counseling regardless of physical appearance, due to the high risk of fatal cardiovascular complications like aortic dissection during or after pregnancy.
Alerts to peripartum mortality risk in Marfan syndrome; leaves open optimal surveillance strategies.
BACKGROUND: Marfan syndrome has great genotypic and phenotypic variability. Marfan syndrome patients face an increased risk of maternal and fetal complications during and after pregnancy. CASE: A 5-ft, 4-in-tall 40-year-old gravida 4 para 2 with a family history of Marfan syndrome presented 4 days after spontaneous vaginal delivery with sudden onset of shortness of breath, air hunger, syncope, and collapse, with resultant asystole. Resuscitation attempts were unsuccessful, and postmortem findings showed dissection and cystic medial necrosis of the aorta. CONCLUSION: Regardless of physical appearance, any patient reporting a family history of Marfan syndrome should undergo and have documented a thorough multidisciplinary evaluation of the skeletal, ocular, cardiovascular, pulmonary, and skin/integumentary systems and dura, in addition to genetic counseling.
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Birsner et al. (2008) conducted a case report in Aortic dissection in Marfan syndrome (n=1). Family history of Marfan syndrome was evaluated on Fatal aortic dissection. A 40-year-old postpartum woman with a family history of Marfan syndrome suffered a fatal aortic dissection 4 days after delivery, highlighting the need for thorough multidisciplinary evaluation.
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