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.


METHODS
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.


CONCLUSIONS
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.
Birsner
Meredith L
ML
Department of Obstetrics and Gynecology, Division of Maternal-Fetal Medicine, Jefferson Medical College of Thomas Jefferson University, Philadelphia, PA, USA.
Farber
John L
JL
Berghella
Vincenzo
V
D000328:Adult; D001011:Aorta / Q000473:pathology*; D001019:Aortic Rupture / Q000235:genetics / Q000473:pathology*; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D008297:Male; D008382:Marfan Syndrome / Q000235:genetics / Q000473:pathology*; D049590:Postpartum Period