A 56-year-old woman presented to the Emergency Department with 5 days of chest and back pain. On exam she was 1.83 m tall and had exam findings of Marfan syndrome (high arched palette, dental crowding, arachnodactyly, pes planus, pectus excavatum). Vital signs were stable. Computed tomography (CT) scan showed an aortic root aneurysm with maximum diameter 6.1 cm, ascending aortic aneurysm with maximum diameter 4.9 cm, and aortic dissection beginning in the aortic root, extending throughout the thoracoabdominal aorta, and terminating at the aortic bifurcation. She underwent emergent mechanical composite root replacement and ascending aortic and hemiarch replacement under hypothermic circulatory arrest with antegrade cerebral perfusion. Post-operatively, her course was uncomplicated, she was anticoagulated for her mechanical valve, and she was discharged to home on post-operative day seven. Subsequent genetic testing confirmed heritable thoracic aortic disease (Marfan, FBN1 p.R861*).

Clinical Topics: Cardiac Surgery, Congenital Heart Disease and Pediatric Cardiology, Noninvasive Imaging, Vascular Medicine, Aortic Surgery, Cardiac Surgery and CHD and Pediatrics, Congenital Heart Disease, CHD and Pediatrics and Imaging, CHD and Pediatrics and Prevention, Computed Tomography, Nuclear Imaging

Keywords: Aftercare, Marfan Syndrome, Arachnodactyly, Blood Pressure, Patient Discharge, Aneurysm, Dissecting, Aortic Aneurysm, Aorta, Aortic Diseases, Tomography, X-Ray Computed, Patient Care Team, Emergency Service, Hospital, Cerebrovascular Circulation, Genetic Testing, Tomography, Back Pain, Surgeons

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