Aortic arch syndrome is stenosis of one or more arteries of the aortic arch. Causes include congenital malformations of the blood vessels, autoimmune diseases, and vascular diseases such as atherosclerosis. Treatment depends on the cause and usually involves vascular surgery.
What is aortic arch syndrome?
In aortic arch syndrome, one or more arteries branching off the aortic arch are narrowed. The condition is also called Mangold-Roth disease, pulseless disease, or obliteration syndrome. The aortic arch is located in the immediate vicinity of the heart and is a section of the aorta that has multiple branches. Therefore, in aortic arch syndrome, there may also be involvement of the aorta. In some cases, all branches of the aortic arch, including the aorta itself, are affected by stenosis, or narrowing. The narrowing may correspond to an existing occlusion or a partial occlusion. This phenomenon affects blood pressure and the cardiovascular system, as well as perceptual structures and the brain. Aortic arch syndrome may be congenital. However, acquired forms also occur and usually result from vascular disease.
Causes
The congenital forms of aortic arch syndrome are malformations of the vessels, as they occur in the context of various hereditary diseases. Congenital aortic arch syndromes occur less frequently than the acquired form. Various vascular diseases are possible causes of the obliteration syndrome. One of the most frequent causes is Takayasu’s arteritis or arteriosclerosis. Takayasu’s arteritis is an autoimmune disease in which the aorta and its main branches become inflamed. In arteriosclerosis, on the other hand, blood fats, thrombi, connective tissue, and calcium deposits into the arteries, narrowing the blood vessels. An equally conceivable cause may be endangiitis obliterans. This disease corresponds to systematic vasculitis of small and medium arteries and veins. The above are merely the most common conditions associated with aortic arch syndrome.
Symptoms, complaints, and signs
Patients of obliteration syndrome show different symptoms depending on which branches are affected by occlusion and in the context of which disease the occurrence occurs. For example, in inflammation-related causes, affected individuals suffer from fever. They are weak and lose weight. If the subclavian artery is affected by the occlusion, paresthesias and pallor are the main symptoms in addition to pain. A sensation of cold and a reduced pulse rate also occur as part of this. Arterial hypotension is present on the affected side. If, on the other hand, the internal carotid artery is narrowed, neurological symptoms develop. In addition to dizziness and ringing in the ears, visual disturbances and other disturbances of consciousness occur. Speech disorders and cognitive abnormalities may also occur. Equally conceivable are paresthesias, mainly affecting the face. If the external carotid artery, the external carotid artery, is affected, the patient complains of pain in the area of the jaw and temples.
Diagnosis andProgression
The physician usually makes the diagnosis of aortic arch syndrome based on the patient’s medical history and imaging techniques such as sonography. Palpation of the arteries may also confirm his suspicion. However, imaging is mandatory for final diagnosis and localization of the occlusion. The course of the phenomenon is determined by how many and which arteries are affected by the occlusion. The cause and severity of the occlusion also influence the course seen in individual cases.
Complications
Complications associated with aortic arch syndrome depend largely on the course of the causative factors and which of the arteries branching off the aortic arch are affected. It always involves stenosis in one or more arteries that originate from the aortic arch. In some cases, the aortic arch itself is also affected by stenosis. If aortic arch syndrome is left untreated despite progressive underlying disease, serious complications can develop. The nature of these, in turn, depends on which of the branching arteries are affected by stenosis and to what extent.The arteries responsible for supplying the head, neck and upper extremities branch off from the aortic arch. If one of the two carotid arteries is affected and parts of the head and brain are not optimally supplied with oxygen-rich blood and nutrients, sensory deficits may develop, sensations of cold, a reduced pulse rate and low blood pressure. Dizziness, tinnitus and visual disturbances often occur when the branching internal carotid artery is affected, which supplies the front part of the brain located toward the forehead. If the narrowing is caused by inflammatory processes and shows progression, serious complications with poor prognosis can occur unless treatment is given. Treatments, which may include replacement arterioplasties for the affected arterial segments, prevent such serious complications.
When should you see a doctor?
Aortic arch syndrome encompasses a broad and differentiated clinical picture. Depending on which of the arteries branching off the aortic arch have stenoses and which causative factors are in question. The prognosis of the further course of the disease also depends largely on the causative factors. For example, if arteriosclerosis is present or if the autoimmune disease Takayasu arteritis is detected, it is advisable to seek medical advice and appropriate therapy immediately. In other cases, genetic predispositions play a role, which have led to a mild to severe malformation of one or more arteries leading from the aortic arch. In these cases, only a slight progression of the disease is to be expected, so that if the parameters are otherwise normal, constant consultations with a cardiologist or a vascular physician (angiologist) are not necessary. However, if fever develops, as well as pain, sensory disturbances in certain areas of the skin and concentration problems, a visit to the family doctor or directly to the cardiologist or angiologist is urgently recommended. The visit to a specialist is also urgently recommended if dizziness, ringing in the ears, visual disturbances and cold sensations occur. The above symptoms indicate that the right shoulder artery (subclavian artery), for example, is affected by a serious stenosis because it can obviously no longer supply part of the brain with sufficient oxygen and nutrients.
Treatment and therapy
Treatment of aortic arch syndrome depends on the specific cause. For example, if arteriosclerosis is the cause, reconstructive plastic surgery of the affected arteries takes place. This surgical intervention may correspond to either angioplasty or thromboendarterectomy. In angioplasty, the physician dilates the affected blood vessels by inserting a catheter. In thromboendarterectomy, the arteries are surgically reopened. If the occlusion is not significant, treatment may also be aimed at reducing atherosclerotic risk factors. The risk of thrombosis must also be reduced. Blood clotting, for example, can be downregulated as part of this. If, on the other hand, an autoimmune disease such as Takayasu’s arteritis has been identified as the cause of aortic arch syndrome, long-term treatment with immunosuppressants is undertaken. Severe narrowing can be repaired by vascular surgical procedures. However, the surgical procedures require the patient to be in an impeccable constitution. This means, above all, that there should be no severe inflammation in the organism at the time of surgery. Appropriate medications are given to treat inflammation. Sometimes, in the context of aortic arch syndrome, bypass anastomosis is also suggested. In this procedure, the doctor creates a bypass circuit. Henceforth, the blood does not have to pass through the narrowed blood vessels, but is diverted. The ends of different vessels are united during anastomosis. This union can take place both end-to-end and laterally. In some cases, such anastomoses also involve the use of vascular prostheses, which are incorporated into the vascular circulation.
Outlook and prognosis
With today’s medical options, aortic arch syndrome can usually be treated well. Nevertheless, the prognosis depends on several factors. They include the severity of the damage, the timing of the start of treatment, and the patient’s age and preexisting conditions.The more complex the blood vessels are affected, the more difficult it is to treat them. It is often not enough to achieve a permanent cure with just one intervention. If the narrowing of the arteries is noticed very late or not in time, the vessels may burst. There is a risk of serious diseases that can lead to lifelong damage to the organism or have a fatal course. The older a patient is, the weaker his age-related health condition is. If other diseases or impairments of the heart or blood vessels are also present, the prospects of recovery decrease. In addition, the patient’s emotional state is important for a good prognosis. Factors such as persistent stress, trauma or mental illness worsen the chances of recovery. In order to achieve a lasting improvement in health after a successful surgical procedure, it is often necessary to adapt lifestyle habits and physical stress to the available possibilities. If this is possible, a patient with aortic arch syndrome can subsequently live symptom-free for a long time.
Prevention
Atherosclerotic aortic arch syndrome can be prevented by eating a deliberately balanced diet, avoiding cigarette use, and getting enough exercise to regulate blood pressure. Little to nothing can be done about autoimmune diseases such as Takayasu’s arteritis or congenital malformations of the arteries. Thus, aortic arch syndrome can be influenced by individual lifestyle only to a certain extent.
Follow-up
Aortic arch syndrome usually entails lifestyle adjustments after successful treatment. Patients must take appropriate measures on their own responsibility. These include avoiding addictive substances such as nicotine and alcohol and reducing excess weight. A balanced diet is just as important as sufficient daily exercise. There is no immunity after an illness. The typical symptoms may recur. The syndrome can be congenital or acquired. For old people, the prognosis is usually unfavorable. They require several mostly surgical interventions. Since the body does not regenerate as quickly with increasing years of life, there is a risk of permanent damage. For diagnosis, doctors primarily use physical assessment and blood tests. However, imaging techniques such as an MRI, CT scan and Doppler ultrasonography also provide clarity on the progression of aortic arch syndrome. The physician will provide information about necessary appointments. Disease can lead to death. Follow-up care aims to prevent complications in advance. This often requires the help of the patient’s environment. Relatives can do a lot to take the stress out of everyday life for those affected. Since aortic arch syndrome not infrequently occurs in combination with other diseases, an extension of treatment is necessary.
Here’s what you can do yourself
Everyday behavior and possible self-help measures depend on which of the arteries branching off from the aortic arch are affected, how severely the cross-section of the affected arteries is narrowed, and what causes have been identified for the occurrence of the disease. For example, if the right subclavian artery is affected, restrictions in the central nervous system are also expected because the right carotid artery originates from the right subclavian artery and provides part of the blood supply to the CNS. If only minor symptoms occur and the causes lie in a congenital defect, no special behavioral measures are required after clarification except measures to inhibit blood clotting. This is to prevent the formation of a blood clot at the narrowed site of the artery. One of the most important causes of acquired aortic arch syndrome is arteriosclerosis in one of the branching arteries. At the affected site, the cross-section of the artery narrows due to plaque deposits in the middle wall (media). Plaques can form, for example, from insufficiently transported cholesterol fractions. Here, too, measures to inhibit coagulation are important to prevent stroke or myocardial infarction, so that no thrombus can form which can then be carried via the vascular system to the CNS or coronary arteries.Basically, in these cases, a diet that contains as many naturally left food components as possible is considered to prevent further atherosclerosis and progression of existing atherosclerosis.
