The aortic arch syndrome is a stenosis of one or more arteries of the aortic arch. The cause may be congenital malformations of the bloodstream, autoimmune diseases and vascular diseases such as arteriosclerosis in question. The treatment is based on the cause and usually involves a vascular surgery.

What is the aortic arch syndrome?

The aortic arch syndrome is a stenosis of one or more arteries of the aortic arch. The aortic arch syndrome may also involve involvement of the main artery.
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In the aortic arch syndrome, one or more arteries are narrowed, which branch off from the aortic arch. The disease is also referred to as Mangold-Roth Disease, Pulseless Disease or Obliteration Syndrome. The aortic arch lies in the immediate vicinity of the heart and is a portion of the main artery, which has several branches. In aortic arch syndrome, there may therefore also be involvement of the main artery.

In part, all branches of the aortic arch including the aorta itself are affected by stenoses, ie narrowing. The restriction may correspond to an existing closure or a partially existing closure. This phenomenon affects both blood pressure and the cardiovascular system, as well as the structures of perception and the brain. The aortic arch syndrome can be innate. However, acquired forms also occur and usually result from a vascular disease.

causes

The congenital forms of the aortic arch syndrome are malformations of the vessels, as they occur in the context of various hereditary diseases. Congenital aortic arch syndromes are less common than the acquired form. Various vascular diseases come as a cause of obliteration syndrome in question. The most common is cystic syndrome as part of Takayasu's arteritis or arteriosclerosis.

Takayasu's arteritis is an autoimmune disease in which the aorta and its main branches are inflamed. In arteriosclerosis, on the other hand, blood fats, thrombi, connective tissue and lime enter the arteries and thus narrow the bloodstream. Another conceivable cause may be endangiitis obliterans. This disease corresponds to a systematic vasculitis of small and medium arteries and veins. The mentioned are only the most common diseases associated with the aortic arch syndrome.

Symptoms, complaints & signs

Patients of the obliteration syndrome show various symptoms, depending on which branches are affected by a closure and in what disease the appearance occurs. For inflammatory causes, for example, sufferers of fever. They are weak and lose weight. When the subclavian artery is affected by the occlusion, paresthesia and paleness appear in addition to pain.

Also, a feeling of cold and a reduced pulse rate occur in the context of this. There is arterial hypotension on the affected side. If, on the other hand, the internal carotid artery is narrowed, neurological symptoms appear. In addition to dizziness and ear noises, especially visual disturbances and other disturbances of consciousness occur.

There may also be speech disorders and cognitive abnormalities. Equally conceivable are paresthesias, which primarily affect the face. When the external carotid artery, the external carotid artery, is affected, the patient complains of pain in the area of ​​the jaw and temples.

Diagnosis & History

The doctor usually diagnoses the aortic arch syndrome based on history and imaging such as sonography. Also the palpation of the arteries can secure his suspicion. Imaging is essential for final diagnosis and localization of occlusion. The course of the appearance is determined by how many and which arteries are affected by the occlusion. The cause and the severity of the closure also influences the course form in the individual case.

complications

Complications related to the aortic arch syndrome depend strongly on the course of the causative factors and on which of the arteries branching from the aortic arch are affected. These are always stenoses in one or more arteries that arise from the aortic arch. In some cases, the aortic arch itself is also affected by a stenosis.

If the aortic arch syndrome remains untreated despite progressing underlying disease, serious complications may occur. The way, in turn, depends on which of the branching arteries are severely affected by a narrowing. From the aortic arch branch off the arteries, which are responsible for the care of the head, neck and upper extremities.

If one of the two carotid arteries is affected, and parts of the head and brain are not optimally supplied with oxygenated blood and nutrients, sensory deficits, cold sensation, decreased pulse rate, and low blood pressure can result. Dizziness, tinnitus, and visual disturbances often occur when the branching carotid interna affecting the anterior part of the brain is affected.

If the constrictions are caused by inflammatory processes and show progression, serious complications with poor prognosis may occur unless treatment is given. Treatments, which may also include replacement surgery for the affected arterial sections, prevent such serious complications.

When should you go to the doctor?

The aortic arch syndrome includes a wide and differentiated clinical picture. Depending on which of the aortic arch outgoing arteries have stenoses and which causative factors come into question. The prognosis of the further course of the disease also depends essentially on the causative factors. For example, it is advisable in the presence of atherosclerosis or finding the autoimmune disease Takayasu arteritis immediately seek medical advice and appropriate therapy.

In other cases, genetic predispositions have played a role, leading to a mild to severe malformation of one or more arteries leaving the aortic arch. In these cases, only a slight progression of the disease is to be expected, so that in otherwise normal parameters no constant visit of a cardiologist or a vascularist (angiologist) is required.

However, if fever sets in as well as pain, discomfort in certain areas of the skin and concentration problems, it is highly advisable to go to the family doctor or directly to the cardiologist or angiologist. The walk to the specialist is also urgently required if it comes to dizziness, ear noises, blurred vision and cold sensations. The above symptoms indicate that, for example, the right shoulder artery (subclavian artery) is affected by a severe stenosis because it apparently can no longer provide enough oxygen and nutrients to part of the brain.

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Treatment & Therapy

The treatment of the aortic arch syndrome depends on the particular cause. If arteriosclerosis is the cause, for example, reconstructive sculptures of the affected arteries take place. This surgical intervention may correspond to either angioplasty or thrombendarteriectomy. In angioplasty, the doctor dilates the affected bloodstream by inserting a catheter.

In the case of thrombendarteriectomy, the arteries are reopened surgically. If the occlusion is not significant, treatment may also target the reduction of atherosclerotic risk factors. The risk of thrombosis must also be reduced. The blood coagulation can be downregulated in the context of this, for example. In contrast, if autoimmune disease such as Takayasu's arteritis has been identified as the cause of the aortic arch syndrome, long-term treatment with immunosuppressive drugs will be performed.

Severe constrictions can be remedied by vascular surgery procedures. However, the operative measures require a perfect constitution of the patient. This means, above all, that at the time of the operation no severe inflammation should be present in the organism. To treat inflammation, appropriate medication is given. Sometimes a bypass anastomosis is also proposed as part of the aortic arch syndrome.

In this procedure, the doctor creates a bypass circuit. From this point on, the blood does not have to pass through the narrowed bloodstreams, but is redirected. The ends of various vessels are united at the anastomosis. This union can take place end-to-end as well as laterally. In part, such anastomoses also vascular prostheses are used, which are incorporated into the vascular cycle.

Outlook & Forecast

With today's medical options, aortic arch syndrome can usually be treated well. Nevertheless, the prognosis depends on various factors. These include the severity of the damage, the time of the start of treatment and the age of the patient and his pre-existing conditions.

The more complex the blood vessels are affected, the more difficult a treatment. It is often not enough to achieve a permanent cure with just one procedure. If the narrowing of the arteries is noticed very late or not in time, the vessels may burst. It threatens serious illnesses that can lead to lifelong damage to the organism or have a fatal course.

The older a patient is, the weaker is his age-related health condition. If there are other illnesses or impairments of the heart or blood vessels, the chances of recovery are reduced. In addition, for a good prognosis the emotional state of the patient is important. In factors such as persistent stress, trauma or mental illness, the chances of recovery worsen.

In order to achieve a lasting improvement in health after a successful surgical procedure, habits and physical stress often have to be adapted to the existing possibilities. If this is possible, a patient with an aortic arch syndrome can then live a long-term symptom-free life.

prevention

The arteriosclerotic aortic arch syndrome can be prevented by a well-balanced diet, abstinence from cigarette smoking, and sufficient exercise to regulate blood pressure. Against autoimmune diseases such as Takayasu's arteritis or congenital malformations of the arteries little to nothing can be done. Thus, the aortic arch syndrome is only to some extent influenced by the individual lifestyle.

aftercare

The aortic arch syndrome usually brings after a successful treatment with an adjustment of life habits. Patients must carry out suitable measures on their own responsibility. These include the avoidance of addictive substances such as nicotine and alcohol as well as the reduction of obesity. A balanced diet is just as real as a sufficient daily exercise. After illness there is no immunity.

The typical symptoms can recur. The syndrome may be congenital or acquired. For old people usually results in an unfavorable prognosis. You need several mostly surgical procedures. Since the body does not regenerate so quickly with the increasing years of life, there is a threat of permanent damage.

For diagnosis, physicians mainly use the physical examination and blood tests. However, imaging techniques such as MRI, CT and Doppler sonography also provide clarity on the progress of the aortic arch syndrome. The doctor informs about necessary appointments. A disease can lead to death.

The aftercare aims to eliminate complications in advance. This often requires the help of the environment. Relatives can do some things to take the stress of everyday life from those affected. Since the aortic arch syndrome is not uncommon in combination with other diseases, an extension of the treatment is necessary.

You can do that yourself

The behavior in everyday life and possible self-help measures depend on which of the arteries branching off the aortic arch are affected, how strongly the cross-section of the affected arteries is narrowed and which causes of the disease were found.

For example, if the right clavicle artery (right subclavian artery) is affected, central nervous system limitations are also likely because the right carotid artery of the right subclavian artery will emerge and take over some of the CNS blood supply. If only minor symptoms occur and the causes are due to a congenital malformation, no special behavior is required after clarification, except measures for the prevention of coagulation. This is to prevent the formation of a blood clot at the narrowed site of the artery.

One of the major causes of acquired aortic arch syndrome is arteriosclerosis on one of the branching arteries. At the affected site, the cross section of the artery narrows by plaque deposits in the middle wall (Media). Plaques can form, for example, from insufficiently transported cholesterol fractions.

Again, measures for anticoagulation are important for the prevention of stroke or myocardial infarction, so that no thrombus can form, which can then be transported via the vascular system into the CNS or into the coronary arteries.

Basically, in these cases, a diet containing as many natural food components as possible prevents prevention of further atherosclerosis and progression of the existing arteriosclerosis.

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