In Marfan syndrome, the aorta is at risk for which complication?

In Marfan syndrome, the aorta is at risk for which complication?

• A. Dilation and dissection of the aorta
• B. Aortic stenosis
• C. Pulmonary embolism
• D. Renal artery stenosis

Answer: (A)

In Marfan syndrome, the aorta is prone to dilation and dissection because of weakness in the aortic media from cystic medial degeneration caused by defective fibrillin-1. Fibrillin-1 normally helps form the elastic scaffold of the vessel wall; when it’s defective, the media degenerates, making the aortic root and ascending aorta susceptible to progressive dilation. The thinned, weakened media can tear, allowing blood to dissect through the wall, which can propagate and lead to catastrophic rupture or compromise of aortic valve function. This combination of dilation and dissection is the classic vascular risk in Marfan. Aortic stenosis is not the typical consequence here since it involves valve narrowing rather than wall weakness. Pulmonary embolism and renal artery stenosis are not primary risks driven by this connective-tissue defect, though dissection can affect other vessels if it extends.

In Marfan syndrome, the aorta is prone to dilation and dissection because of weakness in the aortic media from cystic medial degeneration caused by defective fibrillin-1. Fibrillin-1 normally helps form the elastic scaffold of the vessel wall; when it’s defective, the media degenerates, making the aortic root and ascending aorta susceptible to progressive dilation. The thinned, weakened media can tear, allowing blood to dissect through the wall, which can propagate and lead to catastrophic rupture or compromise of aortic valve function. This combination of dilation and dissection is the classic vascular risk in Marfan.

Aortic stenosis is not the typical consequence here since it involves valve narrowing rather than wall weakness. Pulmonary embolism and renal artery stenosis are not primary risks driven by this connective-tissue defect, though dissection can affect other vessels if it extends.