Which congenital condition is known to increase the risk for aortic dissection?

Which congenital condition is known to increase the risk for aortic dissection?

• A. Marfan syndrome
• B. Hypertrophic cardiomyopathy
• C. Tetralogy of Fallot
• D. Down syndrome

Answer: (A)

Dissection risk rises when the aortic wall is weakened, especially the media, so conditions that cause fragile connective tissue predispose to tearing under normal blood pressure. Marfan syndrome, due to a fibrillin-1 gene mutation, leads to defective connective tissue and cystic medial degeneration of the aorta. This weakens the aortic wall and often causes dilation of the aortic root; as the wall becomes thinner and more distensible, a tear can start in the intima and blood can dissect through the media, producing an acute dissection. Clinically this is a classic association you’re expected to recognize, and it guides management toward strict blood pressure and heart-rate control to reduce shear stress, with surgical considerations if the aorta is dangerously dilated. Hypertrophic cardiomyopathy mainly causes left ventricular outflow tract obstruction and arrhythmias rather than primary weakness of the aortic wall. Tetralogy of Fallot is a cyanotic congenital defect with different hemodynamics and not typically linked to aortic dissection. Down syndrome has various associated heart defects but is not characteristically known for increasing dissection risk through aortic wall weakening.

Dissection risk rises when the aortic wall is weakened, especially the media, so conditions that cause fragile connective tissue predispose to tearing under normal blood pressure. Marfan syndrome, due to a fibrillin-1 gene mutation, leads to defective connective tissue and cystic medial degeneration of the aorta. This weakens the aortic wall and often causes dilation of the aortic root; as the wall becomes thinner and more distensible, a tear can start in the intima and blood can dissect through the media, producing an acute dissection. Clinically this is a classic association you’re expected to recognize, and it guides management toward strict blood pressure and heart-rate control to reduce shear stress, with surgical considerations if the aorta is dangerously dilated.

Hypertrophic cardiomyopathy mainly causes left ventricular outflow tract obstruction and arrhythmias rather than primary weakness of the aortic wall. Tetralogy of Fallot is a cyanotic congenital defect with different hemodynamics and not typically linked to aortic dissection. Down syndrome has various associated heart defects but is not characteristically known for increasing dissection risk through aortic wall weakening.