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

Publication/Presentation Date

8-1-2008

Abstract

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.

Volume

112

Issue

2 Pt 2

First Page

472

Last Page

475

ISSN

0029-7844

Disciplines

Business Administration, Management, and Operations | Health and Medical Administration | Management Sciences and Quantitative Methods

PubMedID

18669769

Department(s)

Administration and Leadership

Document Type

Article

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