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Triple‐Barrelled Aortic Dissection in Ehlers‐Danlos Syndrome
source: Heart
year: 2006
authors: Einstein AJ, Dellegrottaglie S, Rajagopalan S
summary/abstract:An 18-year-old female student with a history of asthma and Ehlers-Danlos syndrome presented with chest pain and shortness of breath. She was hospitalised, treated with antibiotics for presumed pneumonia, and discharged home. She continued to be very dyspnoeic, with minimal exercise tolerance. On repeat examination, a diastolic murmur was noted. Transoesophageal echocardiography revealed a dilated aortic root with proximal dissection and severe aortic regurgitation. Multidetector-row computed tomography (MDCT) confirmed the proximal dissection and also revealed a distinct dissection of the descending aorta. A Bentall procedure was performed to replace the aortic root and ascending aorta.
organization: Mount Sinai School of Medicine (MSSM)DOI: 10.1136/hrt.2005.075267
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