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Mid aortic syndrome and Alagille syndrome
  1. C P P Hia,
  2. S H Quak,
  3. S C Quek

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This is a magnetic resonance (MR) angiogram of an abdominal coarctation in a 13 year old patient with Alagille syndrome. Although her femoral pulses were weak, she was asymptomatic with no upper limb hypertension or symptoms of intermittent claudication. At cardiac catheterisation, there was a pull back gradient of 30 mm Hg from the ascending aorta to the descending abdominal aorta. The suprarenal abdominal aorta is normal in diameter but becomes significantly narrowed for a considerable length below the level of the renal arteries. Distal to this segment, the abdominal aorta is mildly to moderately narrowed up to the bifurcation of the common iliac arteries. There is also narrowing of the proximal renal arteries bilaterally. The findings of the MR angiogram were similar to that of invasive angiography performed previously.

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Infrarenal high grade abdominal aortic stenosis (arrow) demonstrated on gadolinium magnetic resonance angiogram (maximum intensity projection, coronal source images, 1.5 T GE Horizon MR Scanner). Bilateral proximal renal artery stenosis is also demonstrated (arrowhead).