Midaortic Dysplastic Syndrome (Middle Aortic Syndrome)

 

Synonym: middle aortic syndrome

 

Atypical coarctation of the aorta refers to narrowing of the aorta affecting:

(1) the ascending aorta

(2) the descending thoracic aorta near the diaphragm (distal to classic coarctation)

(3) the abdominal aorta

 

The midaortic dysplastic syndrome encompasses the second and third entities.

 

Anatomic features:

(1) narrowing of the affected segment

(2) involvement of branch arteries arising along the affected segment

(2a) renal arteries

(2b) superior or inferior mesenteric arteries

(2c) iliac arteries

Segment Affected	Clinical Finding
renal arteries	hypertension
mesenteric arteries	intestinal ischemia with abdominal angina
iliac arteries	intermittent claudication, leg fatigue and cold feet

 

Clinical findings may include:

(1) blood pressure in the upper extremities greater than that in the lower extremities

(2) headache

(3) systolic murmur over the aorta

 

The condition can present at any age. depending on the cause and the severity of the narrowing.

 

Conditions that can cause the aortic narrowing include:

(1) Takayasu's arteritis

(2) giant cell arteritis

(3) Behcet's disease

(4) Cogan's syndrome

(5) atrophic polychondritis

(6) sarcoidosis

(7) ankylosing spondylitis

(8) rheumatoid arthritis

(9) neurofibromatosis

(10) Williams syndrome

(11) congenital rubella syndrome

(12) tuberous sclerosis

(13) Alagille syndrome

 

Pathologic features may include:

(1) arteritis

(2) fibromuscular dysplasia