Marfan Syndrome Presenting as Spontaneous Coronary Artery Dissection and Arteriopathy. Issue 1 (January 2022)

• Record Type: Journal Article
• Authors: Blech, Benzion; Dhamija, Radhika; Ingall, Timothy
• Is Part Of: Neurologist. Volume 27:Issue 1(2022)
• Publication Date: 2022-01
• Subjects: FBN1 -- Marfan -- SCAD -- carotid dissection -- fibromuscular dysplasia -- vascular neurology; Nervous System Diseases -- Periodicals; Nervous system -- Diseases -- Periodicals; Neurologie; 616.8
• DOI: 10.1097/NRL.0000000000000358
• Languages: English
• ISSNs: 1074-7931

Abstract

Abstract : Introduction: Spontaneous coronary artery dissection (SCAD) is a term used to define a spontaneous separation of the coronary artery wall not related to underlying risk factors, such as trauma or underlying atherosclerotic disease. While SCAD has a range of different etiologies, with fibromuscular dysplasia being the most common, most cases of SCAD have no concomitant arteriopathy. Case Report: Here we describe a case of a patient who presented to our institution with SCAD and evidence of an asymptomatic arteriopathy involving extracranial segments of the carotid and vertebral arteries, later found to have a pathogenic variant in the FBN1 gene and ultimately diagnosed with Marfan syndrome. This has only been rarely described in the literature as an etiology for SCAD. Conclusion: Although rare, it is important to consider underlying connective tissue disorders in patients presenting with spontaneous coronary artery dissection and arteriopathy without underlying cardiovascular risk factors.