Aortic arch syndrome

  • Definition
    • Aortic arch syndrome refers to a group of signs and symptoms associated with structural problems in the arteries that branch off the aortic arch. The aortic arch is the top part of the main artery carrying blood away from the heart.

  • Alternative Names
    • Subclavian artery occlusive syndrome; Carotid artery occlusion syndrome; Subclavian steal syndrome; Vertebral-basilar artery occlusive syndrome; Takayasu disease; Pulseless disease

  • Causes
    • Aortic arch syndrome problems can be due to trauma, blood clots, or malformations that develop before birth. These defects result in abnormal blood flow to the head, neck, or arms.

      In children, there are many types of aortic arch syndromes, including:

      • Congenital absence of a branch of the aorta
      • Isolation of the subclavian arteries
      • Vascular rings

      An inflammatory disease called Takayasu syndrome may result in narrowing (stenosis) of the vessels of the aortic arch. This typically occurs in women and girls. This disease is seen more often in people of Asian descent.

      Vascular ring
  • Symptoms
    • Symptoms vary according to the affected artery, but may include:

      • Blood pressure changes
      • Breathing problems
      • Dizziness, blurred vision, weakness, and other brain and nervous system (neurological) changes
      • Numbness of an arm
      • Reduced pulse
      • Transient ischemic attacks
  • Treatment
    • Surgery is most often needed to treat the underlying cause of aortic arch syndrome.

  • References
    • Braverman AC. Diseases of the aorta. In: Bonow RO, Mann DL, Zipes DP, Libby P, Braunwald E, eds. Braunwald's Heart Disease: A Textbook of Cardiovascular Medicine. 10th ed. Philadelphia, PA: Elsevier Saunders; 2015:chap 57.

      Lederle FA. Diseases of the aorta. In: Goldman L, Schafer AI, eds. Goldman's Cecil Medicine. 25th ed. Philadelphia, PA: Elsevier Saunders; 2016:chap 78.