TICKING TIME BOMB: CORONARY ARTERY ANEURYSM THROMBOSIS IN A PATIENT WITH MARFAN SYNDROME

Document Type

Conference Proceeding

Publication Date

4-1-2024

Publication Title

J Am Coll Cardiol

Abstract

Background Coronary artery aneurysm (CAA) in adults is associated with Marfan syndrome (MFS), connective tissue disorders, and vasculitides. CAA are at risk for thrombosis which obstructs coronary flow resulting in myocardial infarction. Case A 56-year-old man with MFS and prior aortic-root replacement presented with several hours of typical angina. Workup revealed a hsTnI elevation to 16211 ng/L and inferior lead T-wave inversions. He was medically managed for ACS. Invasive coronary angiography showed diffuse severe aneurysmal disease involving all coronary arteries; the distal-RCA was poorly visualized with contrast pooling in the aneurysmal mid-RCA, suggestive of aneurysm thrombosis causing coronary occlusion. No intervention was performed. Decision-making Our patient's extensive CAA predisposed him to RCA aneurymsla thrombosis, resulting in coronary occlusion. Coronary CTA confirmed CAA up to 2.3 cm in the distal RCA with a thrombosed fusiform aneurysm. Literature on revascularization in such cases is limited. Since our patient's symptoms resolved without intervention, he was managed for NSTEMI and maintained on therapeutic anticoagulation with a Vitamin K Antagonist to reduce the risk of coronary artery thrombosis. [Formula presented] Conclusion CAA is a lesser-known complication of MFS. It confers high morbidity and mortality due to the risk of aneurysm thrombosis. Dedicated imaging to screen for and monitor CAA in this patient population could be useful to prevent disease progression.

Volume

83

Issue

13

First Page

2720

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