Aortic Arch Syndrome - Causes, Risks, Diagnosis, and Treatment Strategies

What Is the Aorta?

Aorta is the most significant artery in the body, which carries the oxygenated blood in the heart to various body parts. The aorta arises from the left ventricle of the heart. The ventricles are the lower chambers of the heart. The length of the aorta is about one foot, and the diameter of the aorta varies in dimension, with one inch being the largest. After arising from the left ventricle, the aorta rises upward for a short distance before it curves down. It ends in the pelvis, and between its path to the pelvis, the aorta supplies the chest and the abdominal organs.

What Are the Branches of the Aorta?

The aorta branches off into small branches of arteries that supply blood to several organs, nerves, and muscles. Because of this, the aorta serves as an essential source of blood supply, oxygen, and nutrients to various organs of the body.

Aorta divides into the following branches:

1. Ascending Aortic Branches-

• Coronary arteries.

2. Aortic Arch Branches-

• Brachiocephalic trunk.

• Left subclavian artery.

• Left carotid artery.

3. Descending Thoracic Aortic Branches-

• Bronchial arteries.

• Mediastinal arteries.

• Esophageal arteries.

• Pericardial arteries.

• Superior phrenic arteries.

4. Descending Abdominal Aortic Branches-

• Inferior phrenic arteries.

• Celiac trunk arteries.

• Superior and inferior mesenteric arteries.

• Renal arteries.

• Gonadal arteries.

• Lumbar arteries.

• Median sacral arteries.

• Iliac arteries.

What Is Aortic Arch Syndrome?

An aortic arch syndrome is the collection of signs and symptoms caused by any abnormality that affects the aortic arch or its branches. As a result of these abnormalities, the blood flow to the organs supplied by the aorta is compromised. Reduction in blood flow is seen with narrowing of the artery while weakening causes aneurysm formation.

How Is Aortic Arch Syndrome Otherwise Called?

• Carotid artery occlusion syndrome.

• Pulseless disease.

• Subclavian syndrome.

• Takayasu disease.

• Subclavian artery occlusive syndrome.

• Vertebral-basilar artery occlusive syndrome.

What Causes Aortic Arch Syndrome?

The aortic arch syndrome may result from any of the below causes:

1. Formation of blood clots.

2. Trauma.

3. Changes in blood pressure.

4. Congenital malformations.

5. Takayasu's Arteritis: It is an autoimmune disorder characterized by an inflammation of the major arteries supplying the body. It usually involves the aorta and its branches. This disease is most commonly seen in the Asian population with a female gender prevalence. Although the cause of the disease is still not known, it has been attributed that the T lymphocytes aid this type of inflammatory reaction. Takayasu's disease causes the blood vessels to narrow, which is the main diagnostic feature of this disease.

What Are the Types of the Aortic Arch Syndrome in Children?

In the case of children, the following types of aortic arch syndromes are seen:

• Vascular Rings: Vascular rings are congenital malformations that involve the aorta or the pulmonary artery. In this disease, the aorta forms a ring around the trachea, thus pressing it and causing difficulty breathing and swallowing. The encirclement can either be partial or complete. Treatment involves surgically splitting the vascular ring to depressurize the surrounding structures.

• Absence of an aortic branch since birth.

• Isolated Subclavian Artery: An isolated subclavian artery occurs when a connection loss with the aorta causes the subclavian artery to arise from the pulmonary artery. It can occur either due to patent or closed ductus arteriosus.

What Are the Symptoms of Aortic Arch Syndrome?

Depending on the type of abnormality, the general symptoms include:

• Difficulty in breathing.

• Dizziness.

• Alteration in the blood pressure.

• Difficulty in swallowing.

• Blurry vision.

• Neurological changes.

• Fatigue.

• Numbness involving the arms.

• Transient ischemic attacks (blood flow to the part of the brain is compromised for a short period resulting in stroke-like symptoms).

In the case of arterial occlusion, symptoms depend on the type of phase, which includes the following:

1. Inflammatory Phase:

   • Weakness.

   • Fever.

   • Pain involving the joints, muscles, and chest.

   • Loss of weight.

   • Sweating at night.

   • Swollen glands.

   • The affected arteries show tenderness.

2. Occlusive Phase:

   • Cold extremities.

   • Hypertension (high blood pressure).

   • Reduced or absence of a pulse.

   • Cramps.

   • Weakness.

   • Problems with vision.

   • Nausea and vomiting.

   • Variation in blood pressure between the legs and arms.

3. In severe cases, the below symptoms are seen:

   • Chest pain.

   • Renal failure.

   • Transient ischemic attack.

   • Stroke.

   • Congestive heart failure.

What Increases the Risk of Aortic Arch Syndrome?

Below are the risk factors of the aortic arch syndrome:

• Asian descent. Although the American population is also affected, it is more prevalent among Asians.

• The aortic arch syndrome has a female gender predisposition.

How Is Aortic Arch Syndrome Diagnosed?

Since aortic arch syndrome brings about arterial occlusion, the symptoms are seen soon after an occlusion has occurred. Along with the medical history of the patient, the following diagnostic tests are performed:

1. Physical Examination: The blood pressure is measured, and the presence of abnormal sounds is determined with the help of a stethoscope by the examining doctor. For example, abnormal whooshing sounds are heard when blood is rushing through an occluded artery.

2. Blood Examination: Blood tests like sedimentation and red blood cell count help in identifying the presence of inflammation and anemia, respectively.

3. Chest X-Ray: It helps in determining any small abnormal notches in the rib cage. These notches occur due to pulsatile abnormality of the blood vessels.

4. Angiogram: The presence of abnormality or constriction in the blood vessels is identified with the help of an angiogram. Also called an arteriogram, this test involves the injection of a contrast dye into the blood vessel, which is then followed by an X-ray. The injected dye increases the visibility of the blood vessels.

5. Imaging Tests: In addition to the above, imaging tests like computed tomography (CT) scan, magnetic resonance imaging (MRI) scan, doppler ultrasound, or magnetic resonance angiography (MRA) are used in the diagnosis of aortic arch syndrome.

What Is the Treatment for Aortic Arch Syndrome?

Treatment for aortic arch syndrome involves the following conditions:

1. Medications - Elevated blood pressure is treated with the antihypertensives like beta-blockers, diuretics, and angiotensin-converting enzyme (ACE) inhibitors. In the case of Takayasu's disease, drugs that control the autoimmune response like immunosuppressants (Cyclosporine, Methotrexate, Azathioprine, and Cyclophosphamide) and corticosteroids (Prednisolone) are recommended.

2. Surgical Treatment - Surgical corrections are needed to correct the blockage in the arteries, which involves repairing or widening the arteries.

3. Endarterectomy - In patients with deposition of plaque or damaged tissues in the arterial lining, endarterectomy is done to remove it.

4. Angioplasty - It is carried out to open the constricted arteries with the help of a balloon.

5. Stenting - In cases with arterial constriction, a stent is placed to widen the arteries.

6. Coronary Bypass Surgery - When there is severe blockage or if there are too many blocks, coronary bypass surgery is recommended.

How Can the Progression of Aortic Arch Syndrome Be Prevented?

The following lifestyle modifications can help prevent or slow down the disease progression:

• Avoid smoking or alcohol.

• Maintain ideal body weight.

• Exercise regularly.

• Take an adequately nutritious diet that is lower in saturated and trans fats.

Conclusion:

An aortic arch syndrome is a group of conditions affecting the aortic arch, which is caused due to a variety of causes. Causative treatment is recommended for managing this condition. Symptoms appear early, and hence management is possible with prompt treatment. In addition, certain lifestyle modifications help prevent the progression of the disease. Do reach out to a healthcare provider at the earliest when one experience any of the above symptoms.