Fatal aortic dissection in a patient with a family history of Marfan syndrome

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.