Aortic Arch Disease

The aorta is the body's largest artery. It runs from the heart, through the chest, and down into the abdomen. Aortic arch conditions are abnormalities in the structure of the arteries that branch off the top of the aorta. These abnormalities can reduce blood flow to vital organs.

Aortic arch disease can result from blood pressure changes, clots, trauma, a congenital disorder (one that is present from birth), or Takayasu's arteritis, an autoimmune disorder that inflames the aorta and the pulmonary artery (the main artery of the lung). Takayasu's mostly affects Asian females between the ages of 10 and 30.

Aortic arch conditions eventually block the blood vessels that branch off the aorta, leading to decreased blood flow to areas of the body. Narrowing can reduce blood flow, and weakening can lead to the formation of an aneurysm, or abnormal bulge, in the artery wall. Aneurysms are a life-threatening condition because they can rupture. Atherosclerosis, or hardening of the arteries, is the most common cause of aortic arch disease.

Signs and Symptoms

Symptoms depend on the phase of the disease. In the first phase, the blood vessels become inflamed. Later, during the occlusive phase, blood vessels narrow.

In the first phase, about half of all patients develop one or more of the following symptoms:

• Fever
• Fatigue
• Poor appetite
• Weight loss
• Night sweats
• Joint pain
• Chest pain
• Muscle aches
• Swollen glands
• Tenderness above the affected arteries.

During the occlusive phase, symptoms include:

• Fatigue
• Muscle weakness
• Pain
• Cramping
• Nausea
• Vomiting
• Cold or white hands or feet
• High blood pressure
• Weak or absent pulse
• Vision problems
• A difference in blood pressure between the arms and the legs.

Serious conditions can occur because of the occlusive phase of the disease. They include hypertension, renal (kidney) failure, angina (chest pain), congestive heart failure, transient ischemic attack (TIA, or mini-stroke), and stroke.

Risk Factors

In the United States, the estimated incidence of the condition is 2.6 cases per 1 million Americans. In Asia the incidence is far higher. The cause of aortic arch conditions is unclear. People at increased risk include women and people of Asian descent.

Diagnosis

It is difficult to diagnose aortic arch conditions early because symptoms typically emerge only once an artery has narrowed.

To rule out any other diseases with similar symptoms, a physician will review a patient's complete medical history and then perform a thorough physical exam. During this examination, the physician will measure blood pressure and will listen through a stethoscope for abnormal whooshing sounds made by blood rushing through the blood vessel.

Other tests that a physician may order include:

• Blood tests
• Arteriography (also called angiography; involves injecting a contrast dye into the arteries and then taking x rays)
• Computed tomography (CT) scan
• Doppler ultrasound;
• Magnetic resonance imaging (MRI)
• Magnetic resonance angiography (MRA)

Treatment and Surgery

Lifestyle modifications and medications are the first line of treatment for aortic arch conditions.

People who experience blood vessel inflammation and narrowing may slow or prevent the progression of the disease by:

• Quitting smoking
• Exercising
• Eating a diet low in saturated fat and cholesterol
• Losing weight.

Medications prescribed for aortic arch conditions include:

• Blood pressure medications, such as diuretics, beta-blockers, and angiotensin converting enzyme (ACE) inhibitors
• Corticosteroids and immunosuppressant medications, which control the autoimmune response that occurs in Takayasu's arteritis.

When aortic arch conditions become so advanced that arteries become blocked, surgery to widen or repair narrowed arteries may be necessary. An endarterectomy is the most common procedure for removing plaque and damaged tissue from the inner lining of the arteries. Angioplasty, bypass, and stenting are also used to widen narrowed arteries.