4A44.1

Aortic arch syndrome

Síndrome do arco aórtico

Category

Definition

Arteritis, often granulomatous, predominantly affecting the aorta and/or its major branches. Onset usually in patients younger than 50.

Diagnostic Criteria

Angiographic abnormalities (conventional CT or MRI) of the aorta or its main branches and pulmonary arteries showing aneurysm/dilatation, narrowing, occlusion or thickened arterial wall not due to fibromuscular dysplasia, or similar causes (mandatory criterion) plus one out of five of the following criteria:

1. pulse deficit or claudication

2. 4 limbs blood pressure discrepancy

3. bruits

4. hypertension (blood pressure greater than 95th % for height

5. acute phase reactant

Index Terms

Aortic arch syndromeobliterative aortitisaortic arch arteritissubclavian-carotid obstruction syndromeidiopathic medial aortopathy and arteriopathyTakayasu syndromeobliterative brachiocephalic arteritis syndromepulseless diseaseRaeder-Harbitz syndromechronic subclavian-carotid obstruction syndromeTakayasu aortitisTakayasu arteritisidiopathic aortitisnonspecific aortoarteritissclerosing aortitis and arteritisMartorell 2 syndromeTakayasu diseaseyoung female aortic arch arteritis syndrome