22 February 2019 : Case report
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 Papadopoulos1ABCDEF*, Oliver Maier1DF, Florian Bönner1CDF, Verena Veulemans1ABCDEFDOI: 10.12659/AJCR.911688
Am J Case Rep 2019; 20:233-237
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 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, Aneurysm, Dissecting, aortic aneurysm, Cesarean Section, computed tomography angiography, Echocardiography, Transesophageal, Pregnancy Complications, Cardiovascular
In Press
12 Feb 2024 : Case report
Erdheim-Chester Disease Occult on Radiographs and CT but Visible on MRI and PETAm J Case Rep In Press; DOI: 10.12659/AJCR.941169
12 Feb 2024 : Case report
Surgical Treatment of Spontaneous Superficial Temporal Artery Arteriovenous Malformation: A Case ReportAm J Case Rep In Press; DOI: 10.12659/AJCR.942839
13 Feb 2024 : Case report
Warfarin Woes: A Rare Case of Hemoperitoneum with Intramural Small Bowel HematomaAm J Case Rep In Press; DOI: 10.12659/AJCR.943519
13 Feb 2024 : Case report
Hemorrhagic Presentation in Primary Central Nervous System Lymphoma: A Case StudyAm J Case Rep In Press; DOI: 10.12659/AJCR.942951
Most Viewed Current Articles
07 Mar 2024 : Case report
Neurocysticercosis Presenting as Migraine in the United StatesDOI :10.12659/AJCR.943133
Am J Case Rep 2024; 25:e943133
10 Jan 2022 : Case report
A Report on the First 7 Sequential Patients Treated Within the C-Reactive Protein Apheresis in COVID (CACOV...DOI :10.12659/AJCR.935263
Am J Case Rep 2022; 23:e935263
19 Jul 2022 : Case report
Atlantoaxial Subluxation Secondary to SARS-CoV-2 Infection: A Rare Orthopedic Complication from COVID-19DOI :10.12659/AJCR.936128
Am J Case Rep 2022; 23:e936128
23 Feb 2022 : Case report
Penile Necrosis Associated with Local Intravenous Injection of CocaineDOI :10.12659/AJCR.935250
Am J Case Rep 2022; 23:e935250