• Alex U Siegel, Alexandra Castro, and Jacob Sechrist.
• UPMC Presbyterian Hospital, 200 Lothrop Street, Pittsburgh, PA 15213, USA. Electronic address: siegelau@upmc.edu.
• Am J Emerg Med. 2021 Jul 1; 45: 684.e1-684.e3.

AbstractThis case highlights the unusual life-threatening findings found in a patient with Marfan syndrome (MFS) in the emergency department setting. MFS is a rare autosomal dominant disease that affects 1 in 3000-5000 individuals and has a highly variable range of clinical severity. This case is a 63-year-old male with COPD, scoliosis, aortic and mitral valve replacements on warfarin, and MFS who presented with acute onset hemoptysis, tachypnea, and oxygen saturation of 77% on 4 l nasal cannula. Emergent chest computed tomography angiography (CTA) revealed both a contained rupture of a left subclavian artery aneurysm and active extravasation from his left internal mammary artery (LIMA) into his left chest. The patient was on warfarin and reversed with IV vitamin K and prothrombin complex concentrate. Vascular surgery emergently took the patient to the operating room for embolization of his LIMA and stenting of the contained ruptured left subclavian artery aneurysm. The patient was discharged home one month after admission. This case report illustrates the potential severe sequelae of MFS and the importance of rapid recognition by emergency physicians. An expanded understanding of the pathophysiology of MFS has resulted in great advancement in medical therapies and lifestyle modification and thus has significantly prolonged life expectancy in these patients. Increased awareness and familiarity will facilitate continued high-quality management and treatment by emergency physicians.Copyright © 2020 Elsevier Inc. All rights reserved.

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