Marfan Syndrome

 

Image Library Home >> Diseases of the Aorta >>Marfan Syndrome

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Marfan syndrome can have varying cardiac and aortic manifestations as seen on echocardiography. This case shows a strikingly severe dilation of the ascending aorta to more than 10 cm. Classically there is effacement of the sinotubular junction, which is clearly seen here. Note the aortic valve almost doesn't coapt due the severe aortic dilation. In addition there is a type A aortic dissection (the flap is seen above the aortic valve leaflets.) Some individuals also have varying degrees of mitral valve prolapse. This was not seen in this individual.

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As expected based on the 2D image, there is moderate to severe aortic insufficiency noted here with color Doppler. Pressure half time was 250 ms in the 3 chamber view.

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As a result of the aortic regurgitation, the LV has dilated as shown in the parasternal long and short axis images here.

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The aortic dissection is seen better with the probe up one interspace in the parasternal position.

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It is also clearly seen in the suprasternal position here with 2D

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And here with color Doppler.