A Tall, Thin 20-Year-Old With Aortic Root Dilation: Osmosis USMLE Study Question

February 19, 2021

Clinical signs and symptoms of Marfan syndrome include tall stature with long arms, fingers, and toes (often referred to as Marfanoid body habitus), scoliosis, pectus excavatum (chest that sinks in), flat feet, ectopia lentis, and a dilated aorta leading to dangerous aortic aneurysms. Due to the defect in fibrillin, these patients have cystic medial degeneration leading to weakening of the walls of blood vessels. This allows for dilation of the aortic root, as seen in this patient.

Regarding treatment, β-blockade has been shown to slow the rate of aortic dilatation. The presumption has been that β-blocker therapy reduces the exposure of weakened, histologically abnormal tissues to destructive hemodynamic stressors, both inotropic and chronotropic, and thereby slows the progression of aortic dilatation.

Major Takeaway: In a patient with Marfan syndrome, treat with β-blockers such as propranolol to slow the rate of aortic dilatation.

Read more about Marfan syndrome.

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