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Aortic arch syndrome

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

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.

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: Zipes DP, Libby P, Bonow RO, Mann DL, Tomaselli GF, Braunwald E, eds. Braunwald's Heart Disease: A Textbook of Cardiovascular Medicine. 11th ed. Philadelphia, PA: Elsevier; 2019:chap 63.

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, Silman AJ, Smolen JS, Weinblatt ME, Weisman MH, eds. Rheumatology. 7th ed. Philadelphia, PA: Elsevier; 2019:chap 165.

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    • Heartbeat

      Heartbeat

      Animation

    •  

      Heartbeat - Animation

      The heart has four chambers and four main blood vessels that either bring blood to the heart, or carry blood away. The four chambers are the right atrium and right ventricle and the left atrium and left ventricle. The blood vessels include the superior and inferior vena cava. These bring blood from the body to the right atrium. Next is the pulmonary artery that carries blood from the right ventricle to the lungs. The aorta is the body's largest artery. It carries oxygen-rich blood from the left ventricle to the rest of the body. Beneath the tough fibrous coating of the heart, you can see it beating. Inside the chambers are a series of one-way valves. These keep the blood flowing in one direction. Dye injected into the superior vena cava, will pass through all the heart's chambers during one cardiac cycle. Blood first enters the heart's right atrium. A muscle contraction forces the blood through the tricuspid valve into the right ventricle. When the right ventricle contracts, blood is forced through the pulmonary semilunar valve into the pulmonary artery. Then it travels to the lungs. In the lungs, the blood receives oxygen then leaves through the pulmonary veins. It returns to the heart and enters the left atrium. From there, blood is forced through the mitral valve into the left ventricle. This is the muscular pump that sends blood out to the rest of the body. When the left ventricle contracts, it forces blood through the aortic semilunar valve and into the aorta. The aorta and its branches carries the blood to all the body's tissues.

    • Heart - section through the middle

      Heart - section through the middle - illustration

      The interior of the heart is composed of valves, chambers, and associated vessels.

      Heart - section through the middle

      illustration

    • Vascular ring

      Vascular ring - illustration

      Vascular ring is a term used to describe a number of abnormal formations of the aorta, the large artery that carries blood from the heart to the rest of the body, or of the pulmonary artery. The abnormal vessel(s) forms a ring, which encircles and may press down on the windpipe (trachea) or the esophagus. The additional pressure on the windpipe (trachea) and esophagus can lead to breathing and swallowing problems.

      Vascular ring

      illustration

    • Heartbeat

      Animation

    •  

      Heartbeat - Animation

      The heart has four chambers and four main blood vessels that either bring blood to the heart, or carry blood away. The four chambers are the right atrium and right ventricle and the left atrium and left ventricle. The blood vessels include the superior and inferior vena cava. These bring blood from the body to the right atrium. Next is the pulmonary artery that carries blood from the right ventricle to the lungs. The aorta is the body's largest artery. It carries oxygen-rich blood from the left ventricle to the rest of the body. Beneath the tough fibrous coating of the heart, you can see it beating. Inside the chambers are a series of one-way valves. These keep the blood flowing in one direction. Dye injected into the superior vena cava, will pass through all the heart's chambers during one cardiac cycle. Blood first enters the heart's right atrium. A muscle contraction forces the blood through the tricuspid valve into the right ventricle. When the right ventricle contracts, blood is forced through the pulmonary semilunar valve into the pulmonary artery. Then it travels to the lungs. In the lungs, the blood receives oxygen then leaves through the pulmonary veins. It returns to the heart and enters the left atrium. From there, blood is forced through the mitral valve into the left ventricle. This is the muscular pump that sends blood out to the rest of the body. When the left ventricle contracts, it forces blood through the aortic semilunar valve and into the aorta. The aorta and its branches carries the blood to all the body's tissues.

    • Heart - section through the middle

      Heart - section through the middle - illustration

      The interior of the heart is composed of valves, chambers, and associated vessels.

      Heart - section through the middle

      illustration

    • Vascular ring

      Vascular ring - illustration

      Vascular ring is a term used to describe a number of abnormal formations of the aorta, the large artery that carries blood from the heart to the rest of the body, or of the pulmonary artery. The abnormal vessel(s) forms a ring, which encircles and may press down on the windpipe (trachea) or the esophagus. The additional pressure on the windpipe (trachea) and esophagus can lead to breathing and swallowing problems.

      Vascular ring

      illustration

    A Closer Look

     

    Tests for Aortic arch syndrome

     
     

    Review Date: 4/30/2020

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

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