Author:Vivian Lee, M.D., Ph.D.

New York University, United States

Authored date:11-30-2003

Patient History

30 year-old male has a diastolic murmur and a MR angiography of the thoracic aorta was requested.

Figure 1:
Gadolinium-enhanced 3D MR angiogram demonstrates widening of the ascending aorta that involves the sinotubular junction. This appearance is pathognomonic for cystic medial necrosis (sometimes associated with Marfans syndrome).

Figure 2:
Cine gradient echo imaging through the left ventricular outflow tract during systole shows normal opening of the valve leaflets and confirms widening of the sinotubular junction or annuloaortic ectasia.

Figure 3:
Cine gradient echo imaging during diastole shows a transient area of signal loss emanating from the aortic valve leaflets back into the left ventricle. This finding is consistent with aortic insufficiency, a common complication of cystic medial necrosis.

Diagnosis

Annuloaortic ectasia (cystic medial necrosis) with aortic insufficiency.

Results and Discussions

Evaluation of ascending aortic pathology should include not only black blood spin echo imaging (to exclude mural or extramural pathology such as dissection or intramural hematoma) but also cine gradient echo imaging through the left ventricular outflow tract. These acquisitions are useful for identifying coexistent aortic valvular pathology such as aortic stenosis or insufficiency, as shown in this case. If quantification of the regurgitant volume is desired, cine phase-contrast gradient echo imaging can also be performed.