Vascular Type of Ehlers-Danlos Syndrome: A Case Report of an Aortic Dissection During Pregnancy

The American Journal of Case Reports
Georgios PapadopoulosVerena Veulemans

Abstract

BACKGROUND Vascular type of Ehlers-Danlos syndrome (vEDS) is a rare connective tissue disorder associated with a high prevalence rate of aortic dissection (AD). The coexistence of a pregnancy raises these rates and the diagnostic complexity of the situation. In this article, we present a different initial diagnostic approach to an acute aortic syndrome. CASE REPORT A young pregnant woman (29th week gestation) with vEDS was admitted to our clinic due to sudden tearing back pain radiating to the left arm. Four years ago, the same patient underwent a surgical aortic valve reconstruction and replace of the ascending and proximal arch of the aorta because of an acute Standford A AD. The clinical, laboratory as well as transthoracic echocardiographic findings did not reveal any objective signs of an acute aortic syndrome. Due to the relative contraindications against computed tomography imaging due to pregnancy, we conducted a transesophageal echocardiography which revealed acute progress of pre-existing AD. A follow-up computed tomography could verify our findings,showing a Standford B dissection, which was treated conservatively. After 2 weeks, due to a distal progression of dissection, our patient underwent a cesarean section. In ...Continue Reading

Citations

Oct 1, 2020·Journal of Cardiac Surgery·Lara RimmerMohamad Bashir
Feb 4, 2021·Journal of Cardiac Surgery·Lara RimmerMohamad Bashir
May 1, 2021·Journal of Cardiac Surgery·Xiangli MengQingqing Wu
Sep 17, 2021·Angiologii︠a︡ i sosudistai︠a︡ khirurgii︠a︡ = Angiology and vascular surgery·S A Cherniad'evIa M Krokhaleva
Nov 14, 2021·American Journal of Medical Genetics. Part C, Seminars in Medical Genetics·Natalie Blagowidow

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dissection
cesarean section
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