Marfan syndrome - vascular manifestations
Known Marfan syndrome. Now presenting with acute onset chest pain. Suspected dissection, has a history of aortic stent, investigate possible endoleak.
CT Angiogram Chest and Abdomen
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An intimal flap is identified in the aortic arch arising at the level of the origin of the left subclavian artery and extending into the mid-descending thoracic aorta at the level of T6. The intimal flap extends into the left subclavian artery for a short distance.
Left subclavian artery branches opacify normally and arise from the true lumen. The left vertebral artery appears to arise from the the edge of the dissection flap posteriorly from the false lumen.
it appears dominant and opacifies normally.
Endovascular repair of thoracoabdominal aortic aneurysm. No extravasation of contrast to suggest leak.
Imaged abdominal viscera are unremarkable. The lungs are clear. No mediastinal, hilar or axillary lymphadenopathy. Sternotomy wires. No suspicious bony lesion.
Type B aortic dissection extending from the origin of the left subclavian artery to the mid-descending thoracic aorta at the level of T6. The intimal flap extends into the left subclavian artery.
Marfan syndrome is a multisystem connective tissue disease with autosomal dominant inheritance which primarily involving skeleton, eyes, and cardiovascular systems. It results from a defect in fibrillin 1 (FBN1) gene located on chromosome 15q21.1 which is responsible for cross-linking collagen.