Aortic arch syndrome

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

Definition

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

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 which artery or other structure that has been affected. Symptoms 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
  • Swallowing problems
  • Transient ischemic attacks (TIA)

Treatment

Surgery is often needed to treat the underlying cause of aortic arch syndrome.

References

Braverman AC, Schermerhorn M. Diseases of the aorta. In: Libby P, Bonow RO, Mann DL, Tomaselli GF, Bhatt DL, Solomon SD, eds. Braunwald's Heart Disease: A Textbook of Cardiovascular Medicine. 12th ed. Philadelphia, PA: Elsevier; 2022:chap 42.

James WD, Elston DM, Treat JR, Rosenbach MA, Neuhaus IM. Cutaneous vascular diseases. In: James WD, Elston DM, Treat JR, Rosenbach MA, Neuhaus IM, eds. Andrews' Diseases of the Skin: Clinical Dermatology. 13th ed. Philadelphia, PA: Elsevier; 2020:chap 35.

Langford CA. Takayasu arteritis. In: Hochberg MC, Gravallese EM, Smolen JS, van der Heijde D, Weinblatt ME, Weisman MH, eds. Rheumatology. 8th ed. Philadelphia, PA: Elsevier; 2023:chap 165.

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Review Date: 5/8/2024

Reviewed By: Thomas S. Metkus, MD, Assistant Professor of Medicine and Surgery, Johns Hopkins University School of Medicine, Baltimore, MD. Also reviewed by David C. Dugdale, MD, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.


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