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

Challenging differential diagnosis, Management of emergency care, Rare disease

Georgios Papadopoulos, Oliver Maier, Florian Bönner, Verena Veulemans

(Clinic for Cardiology, Pneumology and Angiology, University Hospital of Düsseldorf, Düsseldorf, Germany)

Am J Case Rep 2019; 20:233-237

DOI: 10.12659/AJCR.911688

Published: 2019-02-22


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 absence of new clinical findings, the young patient was discharged the following week.
CONCLUSIONS: Patients with vEDS are at high risk of an AD and other life-threatening complications, especially during pregnancy. According to the guidelines of European Society of Cardiology (ESC), vEDS-patients should be thoroughly screened. In the case of pregnancy, physicians should consider frequent follow-up examinations and be prepared for diagnosis and treatment of the potential complications.

Keywords: aortic rupture, Ehlers-Danlos Syndrome, Pregnancy



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