An Overview of Aortic Arch Syndrome

Aortic Arch Syndrome

Aortic arch syndrome was previously known as Takayasu’s arteritis in memory of the Japanese doctor who first reported the condition’s signs and symptoms. However, it was later discovered that Takayasu’s arteritis was only one cause of this disease, and it is also cause by the STD syphilis, trauma, birth defects, and atherosclerois (hardening of the arteries).

It is also commonly called the “pulseless disease.” It is an inflammatory condition that affects the main vessel of the body called the aorta. The role of the aorta is to transport oxygenated blood to the head and body. The aorta is divided into the ascending aorta, aortic arch, and the descending aorta. The descending aorta splits into various arterial branches to provide blood to the major organs.

Takayasu’s arteritis affects the aortic arch causing inflammation of the vessel wall. This condition is more prevalent in females of Asian descent. The main signs and symptoms of this disease are a result of changes taking place within the vessel walls of the aorta. When studying this condition Dr. Takayasu first noticed that his patient had engorged blood vessels at the back of the eyes. Later it was discovered that people with this condition also have absent pulses in their wrists. These are a result of inflamed aortic walls and arteries. The vessel lumen is narrowed, leading to less blood being supplied to vital areas.

Symptoms of Aortic Arch Syndrome

The person with this condition might also have thrombosis (blood clotting) or aneurysms (ballooning of arterial walls) due to vessel wall weakening. There are an increased number of white blood cells in the vessel walls as a response to inflammation. Usually the person with this disease will state that he feels weak and tired with dizziness and fainting. There may or may not be a fever present and as the blood supply is reduced, memory loss, blood pressure changes, strokes, problems breathing, numbness in arms and hands, anemia, hemiplegia, aphasia, and muscle athralgia arise. If left untreated for a long period of time, inflammation worsens, and it is during the chronic phase that an aneurysm can develop.

The reduced blood supply to the arms and hands makes them prone to Raynaud’s phenomenon, and there will be painful swelling of the fingers. The exact cause for this condition is unknown. Treatment of aortic arch syndrome is the use of corticosteroid drugs such as prednisone.


Print Source: Davidson, Stanley & C. Haslett. 2002. "Davidson’s Principles and Practice of Medicine.” Churchill Livingstone, Edinburgh.

Web Source: Karger Medical and Scientific Publishers. "Aortic Arch Syndrome .” 2009. Available: