Etiology and pathogenesis of nonspecific aortoarteritis

Etiology and pathogenesis of nonspecific aortoarteritis

The etiology is unknown. The combination with diseases such as rheumatoid arthritis, ankylosing spondylitis, ulcerative colitis, suggests the possibility of an autoimmune nature. It is assumed that the disease is associated with tuberculosis, streptococcal infection.
A partial or generalized lesion of the ascending and (or) descending aorta and its branches, sometimes the pulmonary artery, is observed . The middle and outer shell of the vessel or all of the membranes ( panarteritis ) are affected . Infiltration by lymphocytes, plasma cells, histiocytes, sometimes neutrophils and multinuclear giant cells is characteristic. In the terminal stages, the infiltrates disappear, fibrosis develops. Thickening of the inner membrane and fibrosis of the middle and outer membranes lead to a narrowing or obliteration of the lumen of the vessel. There may be aneurysms, stratification of the vessel wall, sometimes with its rupture. Sometimes the aortic valve and coronary vessels are affected, the aortic opening becomes dilated, which leads to aortic insufficiency.

Clinic of nonspecific aortoarteritis

At an early stage, systemic signs are noted – fatigue, weight loss, low fever, arthralgia, or moderate synovitis . Later, ischemic symptoms appear – a decrease in heart rate and (or) noise. The upper limbs become cold, weakness in the arms develops, rapid fatigue, intermittent claudication, pain along the vessels. Dizziness and fainting are often observed, especially when moving from horizontal to vertical, as a result of occlusion of the carotid arteries. Nausea, headache, blurred vision, or ischemic ulcers; narrowing of the aorta or renal larter can lead to arterial hypertension. II far-reaching cases of arterial insufficiency can cause atrophy and ulceration of the skin on the face, scalp, hair and (or) forehead loss, dementia, abdominal pain, myocardial ischemia. Ruptured aneurysms or stratified arteries can lead to sudden death.
During the examination, the determination of the pulse on the radial, brachial, carotid arteries, aorta, auscultation of large vessels, blood pressure should be measured on both arms and legs is of primary importance. Characteristic asymmetric weakening or disappearance of the pulse on the hands, asymmetry of blood pressure. Over stenotic vessels, a rough systolic murmur can be heard. More than half of patients with increased blood pressure.
Collateral circulation can be detected on the shoulders or elsewhere. Aortic insufficiency may be observed. In the active phase of the disease, ESR is increased, moderate anemia may be noted. The white blood cell count is usually normal or slightly elevated. Serum gamma globulins are often elevated.

Diagnosis and differential diagnosis of nonspecific aortoarteritis

At an early stage of the disease, when there are only general nonspecific symptoms (weight loss, fever, arthralgia), it is difficult to make a diagnosis, but the possibility of aortoarteritis should always be considered when developing such symptoms in a young woman. Recognition of aortoarteritis in such cases is based on the detection of noise or decreased heart rate in large arteries. With an expanded picture of the disease, the diagnosis is based on the detection of cerebral ischemic syndrome, ischemic lesion of the extremities with a decrease in heart rate in large vessels, and vasorenal hypertension.
The diagnosis is confirmed by angiography or dopplerography, rheovasography . On arteriogram reveals narrowing – or patchy throughout the valley – vascular segments, expanding areas. These changes are more often observed in the proximal aorta and its branches. The affected arteries are usually too large to biopsy, but they must be examined with surgical intervention.
Differential diagnosis is carried out with arthritis observed with rheumatism, ankylosing spondylitis, Reiter’s disease, syphilis, vasorenal hypertension with atherosclerosis and fibromuscular dysplasia of the renal vessels.

Etiology of embolism

In 92–95% of patients, the causes of arterial embolism are heart diseases and, first of all, myocardial infarction (especially in the first 2-3 weeks of illness), complicated by severe cardiac arrhythmias, acute or chronic left ventricular aneurysm. Cause emboli may be combined rheumatic mitral heart disease with a prevalence of stenosis complicated intraatrial thrombosis due to atrial arrhythmia. Subacute bacterial endocarditis and congenital heart defects lead less often to arterial embolism. Sources of emboli can be aneurysms of the abdominal part of the aorta and large main arteries (in 3-4% of patients with embolism), ulcerative atheromatosis of the thoracic and abdominal parts of the aorta.

Embolism pathogenesis

As a rule, emboli are localized in the area of ​​branching or narrowing of the arteries. Embolism is accompanied by a pronounced reflex arterial spasm, responsible for the formation of an extended thrombus, which blocks the collateral network. With thrombosis and embolism of the main arteries of the limbs in the corresponding vascular pools, acute tissue hypoxia occurs, the main cause of which is a violation of blood flow in the vessels of the microvasculature. In the affected tissues, an excess of under-oxidized metabolic products is formed, which leads to the development of metabolic acidosis. The latter promotes platelet adhesion and the formation of platelet aggregates in the capillary lumen , exacerbating the severity of ischemia.
The increase in hypoxia adversely affects the course of redox processes in the tissues. They increase the content of membrane – toxins – histamine, serotonin, kinins , prostaglandins , which increase the permeability of cell membranes and intracellular membranes. As a result of impaired cellular permeability, subfascial edema of the muscles appears , in which the blood flow deteriorates even more due to compression by the bone-fascial cases. Changes in bone metabolism and cell death lead to the breakdown of lysosomes with the release of hydrolases that lyse tissues. The consequence of this is the development of soft tissue necrosis. From the ischemic tissues in the bloodstream fed suboxide – lennye metabolic products resulting in metabolic acidosis, toxic products, potassium, myoglobin. Severe disturbances in the activity of the cardiovascular system occur, manifested by a deterioration in cardiac activity, heart rhythm disturbances, and gross changes in central hemodynamics. Circulatory hypoxia increases, and renal filtration decreases.

Hypertonic conditions

An increase in blood pressure in older children and adolescents occurs, filed by various authors, in 5 – 10% of cases. It should be emphasized that the increase in blood pressure does not yet give rise to a diagnosis of hypertension, since in many cases these phenomena are transient in nature. However, the detection of arterial hypertension must necessarily lead to a comprehensive examination of the child to establish its cause.

Etiology and pathogenesis of hypertensive conditions

Arterial hypertension in childhood can be caused by various diseases: glomerulonephritis , pheochromocytoma , congenital malformations of the heart ( coarctation of the aorta), kidneys and their vessels; in young children – infectious diseases that occur with toxicoexicosis and sensitization of the body, the consequences of a birth injury to the skull. A hereditary predisposition and neurohumoral dysregulation of vascular tone play a role . There is an activation of the function of the sympathetic-adrenal system, an increase in the sensitivity of adrenoreceptors to catecholamines (which may be hereditary). This leads to an increase in systolic ejection and cardiac output with normal peripheral vascular resistance (i.e., with normal vascular tone). In some patients, vasoconstriction occurs with an increase in peripheral vascular resistance.
In children of high school age and adolescents, neuropsychic overstrain and mental fatigue with limited physical activity (irrational daily routine) may be important, especially with prolonged exposure to these factors. These points may be the cause of primary arterial hypertension.

Classification of hypertonic conditions

Three forms of arterial hypertension in children are distinguished (M. Ya. Studenikin ): vascular vegetative dystonia of the hypertensive type, hypertension, and symptomatic (secondary) hypertension.

Clinic of hypertonic conditions

In most cases, children do not complain, increased pressure is detected by chance during mass preventive examinations. However, in some cases, headache, increased irritability and fatigue, dizziness are noted. From the side of the heart, a resistant apical impulse is detected, functional noise is heard, sometimes an II tone accent above the aorta. Systolic blood pressure exceeds the age norm, and during exercise rises by 2.7 – 5.4 kPa.
In the development of hypertension, there are three stages: transient, labile and stable. Changes in blood pressure with an increase in systolic and then diastolic in parallel with changes in organs (heart, kidneys, on the fundus) are gradually increasing.

Diagnosis and differential diagnosis of hypertensive conditions

Primary arterial hypertension must be differentiated with diseases in which an increase in pressure is the leading symptom ( glomerulonephritis , pituitary and adrenal tumors, etc.). A typical clinic of these diseases helps to establish the correct diagnosis.

Hypertensive prognosis

Arterial hypertension is more often reversible. In some cases, increased blood pressure is persistent and is the initial phase of hypertension.

Hypertensive treatment

With secondary arterial hypertension, therapy of the underlying disease is necessary. Primary arterial hypertension in some cases with the vegetodystonic genesis of vascular disorders and a slight increase in blood pressure requires only the organization of a rational regime of the day, the expansion of motor activity with limited mental work. It is necessary to slightly reduce the training load, to ensure prolonged sleep and stay in the fresh air. Useful sports and exercise, but without participating in competitions. The purpose of sedatives (valerian, bromides, seduxen, elenium, trioxazine ), antihistamines and others, physiotherapy (electrophoresis according to the method of G. Kassil with solutions of calcium, magnesium salts) are shown .
In the absence of a therapeutic effect and high blood pressure, the patient is sent to a hospital and p- adrenoblockers ( anaprilin , inderal , obzidan , trazikor , wisken ) are included in the treatment regimen . Treatment begins with minimal doses, and then after 10 days the dose is increased ( anaprilin , trazikor 0.01 – 0.02 g 2 times a day, then – 0.03 g 2 times a day). Continue treatment for 2 to 3 months or more. Rauwolfia preparations in such patients are ineffective. B- Adrenergic blockers can not be used for bronchial asthma, signs of myocardial damage (according to ECG). In the absence of effect, especially in adolescents, as well as in stage II and III arterial hypertension, rauwolfia – raunatin preparations are used (0.002 – 0.003 g 1 – 2 times per day), reserpine (0.1 – 0.15 mg 3 times a day). Parallel used dihlotiazid – hydrochlorothiazide (1 every 2 – 3 days).
Treatment with rauwolfia preparations is carried out until a clear effect is obtained (lowering blood pressure), after which they are transferred to maintenance doses (2 to 3 months). If this therapy does not work, patients with high blood pressure are prescribed ganglion blockers – pentamine , isoprine and others with mandatory hemodynamic control and compliance with the appropriate regimen.

Prevention of hypertensive conditions

The correct regime of the day, physical education, sports, a fairly long sleep.

Etiology, pathogenesis of the open ductus arteriosus

The arterial ( botall ) duct (OAP) in the prenatal period connects the aorta to the pulmonary artery. This is the necessary anatomical structure of the embryonic circulation. After the birth of the baby, the duct function stops almost immediately. The process of anatomical closure of it lasts no more than 2-8 weeks . In some cases, the closure of the duct does not occur. Such a defect accounts for 10-25% of all congenital heart abnormalities. The arterial duct moves away from the aortic arch opposite the left subclavian artery, goes obliquely anteriorly and downward, falling into the bifurcation of the pulmonary trunk or into the left pulmonary artery. The duct has the shape of a cylinder or a truncated cone 10-25 mm long and up to 20 mm wide.

The primary violation of hemodynamics is associated with a difference in pressure in the aorta and the pulmonary artery, which leads to persistent systolic- diastolic aortopulmonary discharge of blood (from left to right). At the same time, there is a volume increase in pulmonary blood flow, which depends on the ratio of the diameters of the aorta and duct. With a significant diameter of the latter, most of the blood pumped into the left ventricle enters the pulmonary artery from the aorta. As a result, pulmonary blood flow may exceed that in a large circle of blood circulation. Due to chronic oxygen starvation of the body, the child lags behind in physical development. Significant volume overload of the vessels of the pulmonary circulation leads to frequent bronchitis and pneumonia. Hemodynamic compensation is initially supported by shock and minute volumes of the left ventricle due to its diastolic overload. In the future, there comes a spasm of pulmonary arterioles with their subsequent sclerosis , which leads to a significant decrease in the vascular capacity of the pulmonary circulation. This process at certain stages causes pressure balancing in the aorta and the pulmonary artery, and sometimes even leads to an inversion of blood discharge (from right to left). From this moment, blood begins to flow into the arterial bed, cyanosis appears ( cyanotization of the defect). At the same time, systolic overload of the right ventricle increases and OAA becomes its salvage drainage, the closure of which can cause acute right ventricular failure. However, in OAP, cyanotization is rare.

Clinic of the open arterial duct

The clinic depends on the magnitude of the OAA, the pressure level in the pulmonary artery and the ratio of the resistance of the vessels of the small and large circles of blood circulation. Allocate 4 stages of OAP: I – systolic pressure in the pulmonary artery is less than 40% blood pressure; II – moderate hypertension (systolic pressure in the pulmonary artery is 40-45% of blood pressure; III – severe hypertension (systolic pressure in the pulmonary artery is more than 75% of blood pressure, blood discharge is preserved from left to right); IY – state of extreme severity (systolic pressure in the pulmonary arteries is equal to or higher than blood pressure, cyanotization of the defect).

Complaints of patients with OAP are nonspecific. The most common complaints are fatigue, shortness of breath with physical exertion , sometimes sensations of interruptions in the work of the heart, palpitations, pneumonia. The lag in physical development, pallor of the skin is characteristic. When straining, cyanosis is more pronounced in the lower half of the trunk and on the lower extremities. On palpation of the chest determines the gain of the apical impulse, systolic or systolic -diastolicheskoe jitter in the projection base of the heart. The borders of the heart are expanded, but their quantitative characteristics are different. With a large arteriovenous discharge, the pulse is fast and high. Pulse pressure – with a tendency to increase. In young children, diastolic pressure can be reduced to 0. Auscultation reveals the main diagnostic sign of OAP – a rough, “machine” systolic- diastolic murmur in the second intercostal space to the left of the sternum. Noise is carried into the interscapular space and on the vessels of the neck. The diastolic component is better heard when straining ( Valsalva test ). As pulmonary hypertension there is a tendency to disappear first Diastolic – of the noise component, and subsequently – and systolic. Patients with stage IV it is virtually wrinkle ” Afonichev ” or accompanied by the appearance Protodeacon – stolicheskogo noise relative valvular insufficiency, pulmonary arterial (noise Graham Stille ). Against this background, II tone over the pulmonary artery progressively amplifies. Sometimes it can be split and the accent of the pulmonary component of II tone is heard

Diagnosis of open ductus arteriosus

In typical cases, a rhomboid-like systolo- diastolic murmur is recorded on the FCG over the pulmonary artery . As pulmonary hypertension increases, the tendency to the disappearance of the diastolic and decrease in the systolic component of the noise correlates with the amplification and splitting of II tone over the pulmonary artery. In the later stages of the defect, the protodiastolic murmur of Graham Still is recorded . There are no specific changes on the ECG. A normogram or levogram is marked . In the future, signs of hypertrophy of both ventricles with an outcome in the rightogram and isolated hypertrophy of the right ventricle are determined . When x-ray examination in the initial stages are noted: increased vascular pattern, left ventricular hypertrophy, increased aortic pulsation. In the future, a tendency to swelling and an increase in the pulse amplitude of the pulmonary artery, depletion of the pulmonary pattern, and hypertrophy of the right ventricle is determined . Dopplerographic examination in some cases captures the aorto-pulmonary shunt. When catheterization of the right heart revealed an increase in oxygen saturation of the blood in the pulmonary artery compared with the right ventricle by at least 2 vol. % Aortography makes it possible to obtain simultaneous contrasting of the pulmonary artery, into which the contrast medium enters through the OAA.

Etiology, pathogenesis of aortic coarctation

Coarctation of the aorta accounts for 4-15% of all congenital heart defects. The origin of this defect is associated with an abnormality in the transformation of the aortic isthmus during the formation of extrauterine circulation. The disease is 2–2.5 times more common in males. Bonnet proposed to distinguish between two types of coarctation of the aorta: 1) “infantile”, in which the aorta is narrowed over a large distance above the place of discharge of the OAP; 2) “adult”, in which the narrowing is observed only on a short segment of the aorta (segment), usually in the area of ​​transition of the arch to the descending part below the site of the OAA (isthmus of the aorta). For practical purposes, it is convenient to consider 4 options for coarctation of the aorta: I) isolated, 2) coarctation of the aorta in combination with OAP; 3) coarctation of the aorta in combination with DMS; 4) coarctation of the aorta in combination with other congenital heart defects.

Circulatory disorder is manifested by severe hypertension in the arterial vascular pool above coarctation and hypoperfusion below it. In this case, hemodynamic compensation is achieved due to the development of collateral arteries connecting the upper and lower parts of the body (intercostal, internal mammary arteries, internal mammary arteries). Decompensation is manifested by malignant hypertension, deep sclerotic changes in the walls of the arteries. HELL sometimes reaches 300 mm Hg . (40 kPa). The consequence of this are cerebrovascular disorders up to the development of hemorrhagic stroke, heart and kidney failure. The expansion of the intercostal arteries leads to a compressed – NIJ roots of the spinal cord, severe spinal disorders (paralysis, disruption of the pelvic organs). In the origin of arterial hypertension, in addition to the mechanical cause, a significant role is given to renal vasopressor , a weed factor that is a direct consequence of renal ischemia.

Depending on the type of defect, various hemodynamic disorders occur. With the “adult” type of coarctation with a closed arterial duct, systolic overload of the left ventricle comes to the fore. In the “infantile” type, when there is OAP, pulmonary hypertension develops rapidly with an outcome in cyanosis of the lower half of the body. The combination of defect with defects of the septa significantly accelerates the onset of pulmonary hypertension.

Clinic for aortic coarctation

In most cases, with this defect, imbalance is detected due to the pronounced development of the muscles of the shoulder girdle in comparison with the relative underdevelopment of the muscular system of the lower extremities. Moreover, blood pressure in the upper extremities is significantly increased. The pulse on the arteries of the thigh is sharply weakened or absent. The pulsation of the abdominal aorta is also not determined. In some cases, it is possible to detect the pulsation of the dilated intercostal arteries during tissue capture in the subscapular region. When ascultation is determined: a pronounced accent of II tone above the aorta, systolic murmur under the right clavicle, in the interscapular space on the left and on the vessels of the neck. The rest of the clinical picture in adult patients repeats all the signs of defects stenosing the outlet arterial tract of the left ventricle (aortic stenosis) with its pronounced systolic overload. In general, the physical picture is changeable and meager. That is why this anomaly requires special attention of doctors, since it is associated with frequent diagnostic errors.

Diagnosis of aortic coarctation

Under normal conditions, blood pressure in the lower extremities is 29-30 mm Hg higher . (2.7-4 kPa) than on the top. When comparing blood pressure levels under coarctation of the aorta, the pressure on the lower extremities (the auscultation point in the popliteal fossa with the location of the cuff on the thigh) is not determined or significantly reduced. Coarctation should be considered pronounced if the pressure gradient between the upper and lower extremities reaches 40 mm Hg . (5.3 kPa). Depending on the level of blood pressure, 3 stages of the defect are distinguished: I – moderate (blood pressure below 150 mmHg (20 kPa); II – moderate severity (blood pressure – 150-200 mmHg (20-28.7 kPa ); III – severe (blood pressure greater than 200 mmHg (26.6 kPa). X-ray and ECG data for this anomaly are characteristic of hypertension and are signs of left ventricular hypertrophy. Dopplerography and chest tomography are of great help in the diagnosis in the sagittal plane.With a typical isolated form, the anomalies of the listed means are sufficient to establish final diagnoza.V doubtful cases should resort to aortography . Thus aorta contrasted through the arterial branch of the shoulder belt with a mandatory recording layer the pressure gradient in the coarctation . When concomitant congenital heart diseases shows angiocardiography and sensing cardiac cavities.


Etiology of peripheral arterial aneurysm

The most common causes of non-traumatic aneurysms are atherosclerosis and syphilis. Traumatic aneurysms, or false, are formed after gunshot and stab wounds, less often – due to a blunt injury. Their development is possible in the presence of a narrow wound channel, a small area of ​​damage to soft tissues, covering the wound opening with skin or tissues. Under these conditions, blood poured from the damaged vessel accumulates in the surrounding tissues, exfoliating them, and a periarterial pulsating hematoma occurs . The clots that form in it are pushed to the periphery by a stream of blood, condense, organize, as a result of which a connective tissue capsule is formed and a pulsating hematoma turns into a false aneurysm . Pathological anatomy. Three types of traumatic aneurysms are distinguished: arterial, arteriovenous and combined . An arterial aneurysm has an aneurysmal sac that communicates with the lumen of the artery. Arteriovenous aneurysm is the result of simultaneous damage artery and vein, which leads to the formation or arteriovenous – th fistula or aneurysms intermediate. With combined aneurysms, a combination of these types of aneurysms is observed. With the prolonged existence of an arteriovenous aneurysm, the wall of the leading artery changes significantly , which is manifested by its thinning, a change in the internal elastic membrane, resulting in an increase in the diameter of the artery. On the contrary, in the wall of the vein extending from the aneurysm, hypertrophy of the muscle membrane and the development of the internal elastic membrane are noted. The diameter of the vein also increases.

Pathogenesis of peripheral arterial aneurysm

Arteriovenous and combined aneurysms cause severe hemodynamic disorders. In this case, not only peripheral blood circulation is disturbed, but also central hemodynamics. A pathological discharge of arterial blood into the venous system leads to difficulty in the outflow of venous blood from the affected limb, as well as to an increase in the volume of blood flowing to the right atrium. Due to venous stasis, superficial veins expand, swelling and trophic changes in the distal extremities occur. Working myocardial hypertrophy develops, which is then replaced by myogenic dilatation with cardiac decompensation.

Clinic of peripheral arterial aneurysm

The main complaint of patients is the presence of a pulsating formation in one or another segment of the limb. When viewed at the site of an arterial aneurysm, a swelling is visible, often pulsating. On palpation, it has a densely elastic consistency, is clearly delimited, round or oval, pulsates synchronously with the contractions of the heart. With auscultation over the area of ​​the aneurysm, systolic murmur is determined, which disappears when the leading artery is compressed. Ripple to the periphery from the aneurysm is often weakened. Hand applied to the site of the aneurysm, the jitter can be felt or purling (symptom of feline purring) .Patognomonichnym symptomatic arteriovenous aneurysm is a slowing of heart rate for 15-30 min in 1, combined with an increase in blood pressure in the artery leading cross-clamping (Dobrovolskaya symptom). The slowdown of the pulse is due to an improvement in cardiac activity due to a decrease in blood flow to the right atrium.

Complications of peripheral arterial aneurysm

Complications of the aneurysm are rupture of the aneurysmal sac, accompanied by profuse, life-threatening bleeding, thromboembolism and thrombotic masses contained in the aneurysm. With a traumatic aneurysm, an outbreak of a dormant infection, the development of soft tissue phlegmon are possible. A number of patients develop severe trophic disorders and cardiac disturbances, depriving them of their ability to work.

Diagnosis of peripheral arterial aneurysm

Diagnosis is not difficult when there is a pulsating swelling and vascular noise. To clarify the true shape and size of the aneurysm, localization, the state of the distal and proximal arterial bed and the degree of development of collateral blood flow, arteriography is necessary .

Temporal Giant Cell Arteritis

Temporal giant cell arteritis , cranial arteritis, temporal arteritis , Horton’s disease is a systemic lesion of arteries, mainly of muscular-elastic and muscle types.
In temporal giant cell arteritis, the vessels of the head (primarily the temporal arteries) and the thoracic aorta are most often affected. As a rule, elderly people are older than 50 years old, most often women. Several family cases of the disease are described. Temporal giant cell arteritis belongs to the group of allergic vasculitis .
The etiology is not known, possibly viral. There is a connection with respiratory infections, the content of birds (parrots) in the house, and the detection of hepatitis B surface antigen in serum.

Pathomorphology of the temporal giant cell arteritis

In areas of vascular lesions, fixed immunoglobulin M and immunoglobulin G, components of the complement of the system, are detected. The acute phase is characterized by the phenomena of mucoid swelling, fibrinoid degeneration in the intima and middle lining of the arteries with thromboangiitis obliterans, necrotic changes and the transition to sclerosis. As part of the granulation tissue, giant multinuclear cells similar to Pirogov- Langgans cells are detected .

Clinic of the temporal giant cell arteritis

The onset of the disease is most often gradual – with the appearance of pain and stiffness in the neck, shoulder girdle, malaise, nausea, loss of appetite. An acute onset is also possible – high fever , sharp headaches in the area of ​​the affected vessels, sometimes with impaired and loss of vision. A painful and throbbing temporal artery is palpated. In temporal giant cell allergy, there may be a combined lesion of the coronary and abdominal vessels.
There is a frequent combination of this disease and rheumatic polymyalgia ( Forestier – Sertonsini syndrome ), which consists in the appearance of symmetric polymyalgia in the elderly , especially in the neck muscles. shoulder girdle, and accompanied by low-grade fever, asthenia , anorexia.
In both cases, there is a sharp increase in ESR – from 50 to 100 mm / h, hyper-A2-globulinemia, hypoalbuminemia , moderate hyperchromic anemia.

Treatment of temporal giant cell arteritis

There is a pronounced effect of glucocorticosteroid therapy (differential diagnostic sign of temporal giant cell arteritis). Symptom attenuation occurs within a few days after the administration of the glucocorticosteroid drug (initial dose of 40-50 mg, followed by a decrease to a maintenance dose of 5-10 mg). Long-term use of delagil , indomethacin is indicated , for relieving pain during an attack – novocaine chipping of near-arterial tissues.

Isolated pulmonary stenosis

Isolated pulmonary stenosis accounts for 1.6–9% of all congenital heart defects.

Etiology, pathogenesis of isolated pulmonary stenosis

Anatomical variants of the defect are reduced to narrowing the path of outflow from the right ventricle of the heart. Distinguish valve stenosis (fusion of valve commissures); infundibular ( subvalvular ) stenosis due to the presence of a muscle diaphragm with a small opening or muscle hypertrophy of the exit tract of the right ventricle; supravalvular stenosis of the pulmonary trunk and branches of the pulmonary artery ( coarctation ). Sometimes there are combinations of the above variants of the anomaly.

The hemodynamics of the disease boils down to systolic overload of the right ventricle, which increases with increasing degree of stenosis. In this case, there is a restriction of pulmonary blood flow, which determines the degree of cyanosis. Compensation of the defect is supported by hypertrophy of the right ventricle. Characteristically, dila – tation it usually does not happen. Thus, hypertrophic syndrome of the “small ventricle” develops. Due to the increase in the rate of its reductions, as a rule, due MOS is kept relatively long. Over time, ischemic contracture of the hypertrophied myocardium of the right ventricle develops and it ceases to cope with a high load. Decompensation sets in: cyanosis appears, venous pressure rises, hepatomegaly , peripheral edema develop and progress .

Clinic of isolated pulmonary stenosis

The clinic is determined by the severity of stenosis and the state of compensatory mechanisms. Clinical manifestations range from asymptomatic to severe forms (severe shortness of breath at the slightest exertion, anginal pain, cyanosis). Auscultation is characterized by gross systolic murmur with an epicenter in the second and third intercostal space on the left ( infundibular stenosis). In the late stages of the defect, cyanosis, venous hypertension (visible dilatation of the cervical veins), enlargement of the liver, and peripheral edema join.

Diagnosis of isolated pulmonary stenosis

Radiologically observed: a moderate increase in the right ventricle, narrow roots of the lungs, impoverishment of the pulmonary pattern. Valvular stenosis is characterized by suprastenotic expansion of the pulmonary artery due to hydrodynamic shock of a narrow stream of blood. On the ECG – a rightogram , signs of systolic overload of the right ventricle. FCG reveals a rhomboid systolic murmur in the pulmonary artery, a decrease in the amplitude of II tone in this zone. To determine the severity of stenosis and clarify indications, surgical treatment is resorted to sounding of the heart cavities. The main clinical criteria are the systolic pressure gradient between the right ventricle and the pulmonary artery, as well as the systolic pressure in the right ventricle. The latter criterion is more informative. It determines the clinical stages of the course of the disease: I – moderate stenosis (systolic pressure in the right ventricle below 60 mm Hg (8 kPa); II – severe stenosis (systolic pressure in the right ventricle 60 to 100 mm Hg (8 —13.3 kPa), III – severe stenosis (systolic pressure in the right ventricle is higher than 100 mmHg (13.3 kPa). With coarctation of the pulmonary trunk and its branches, angiopulmonography is most informative .


Hyaloid Artery Remains

The remains of a hyaloid artery is an anomaly of development that occurs due to a violation of the reverse development of the hyaloid artery in the embryonic period. Usually found at a young age, with age, this artery resolves. The clinical picture and diagnosis. It manifests itself in the form of a strand (moorings) of the vitreous body, which goes from the optic disc to the lens. More often, the remains of an artery are detected in the anterior or posterior parts, and not along the entire length. In this case, the artery has the form of a corkscrew or rope, the end of which floats freely in the vitreous. Very rarely, the remains of the hyaloid artery are shrouded in a layer of connective tissue and can significantly impair vision.


Thrombosis is a pathological condition characterized by the formation of a blood clot in the area of ​​the vascular bed.

Etiology of thrombosis

Indispensable conditions for the occurrence of arterial thrombosis is a violation of the integrity of the vascular wall, changes in the hemostatic system and a slowdown in blood flow. This explains the high frequency of thrombosis in people suffering from obliterating atherosclerosis, diabetes mellitus. Often the development of thrombosis contribute to damage to the walls of the arteries. soft tissue bruises, dislocations and fractures of limb bones, compression of the vascular bundle by a tumor or hematoma. Acute arterial thrombosis may be preceded by angiography – cal research, reconstructive vascular surgery, intra-arterial blood transfusion. Thrombosis also occurs against the background of certain hematologic (polycythemia) and infectious (typhus) diseases.

Pathogenesis of thrombosis

In all these cases, the response to damage to the endothelium of the vascular wall is adhesion and subsequent platelet aggregation. The resulting aggregates tend to further growth, which is associated with the action of physiologically active substances released from platelets. Released from platelets trombotsitarnge factors and biologically active substances contribute not only platelet aggregation, but also the activation of the blood coagulation system, reducing its fibrinoli – cal activity. Thus, thrombocytic aggregates become a functionally active field in which the activation and interaction of coagulation factors of the blood occurs. As a result, fibrin filaments are adsorbed on the surface of the aggregate and a network structure is formed, which, delaying the formed elements of the blood, turns into a blood clot. With significant inhibition of the lytic link of the hemostatic system, thrombosis can take on a common character.

Temporal arteritis and polymyalgia rheumatica

Temporal arteritis (giant cell arteritis, Horton’s disease ) – a disease of the elderly and senile, more common in women, characterized by damage to the aorta and arteries of the head and neck. Rheumatic polymyalgia is a disease of people of the same age and gender, characterized by pain and stiffness in the proximal parts of the muscles of the shoulder and pelvic girdle in combination with fever, weight loss. Both diseases are often found in the same patient, and therefore their common origin is not excluded, the role of a viral infection, including hepatitis B virus, is possible. Temporal arteritis is characterized by granulomatous inflammatory infiltrates with lymphocytes and giant multinucleated vessels in the vessels extending from the aortic arch; more often the temporal arteries are affected. Gradually, a thickening of the inner membrane develops with a narrowing of the lumen of the vessels and parietal thrombosis. The most common symptom is a burning throbbing headache. The temporal arteries are painful and swollen, the skin above them is hyperemic. Subsequently, the temporal arteries condense, become crimped. The most serious complications are eye damage, 20% of patients develop vision loss due to ischemia of the optic nerves. Intermittent pain in the masticatory muscles, sometimes in the muscles of the tongue and the muscles involved in swallowing, can be observed . In 10-15% of patients, large arteries are affected, ischemic pains in the upper or lower extremities, noises or a decreased pulse on the vessels of the neck or limbs, sometimes aortic rupture are noted. More rare symptoms are cough, depression, psychosis, hemiparesis, peripheral neuropathy , acute hearing loss, cerebrovascular accident, myocardial infarction. Most patients have a fever, sometimes of an unclear origin. Almost all patients note a sharp weakness, weight loss, anorexia. Half of the patients have signs of rheumatic polymyalgia – pain and stiffness in the muscles of the shoulder and pelvic girdle, neck. Often there is moderate normochromic anemia, thrombocytosis. The white blood cell count is usually normal. A significant increase in ESR is characteristic. In 1/3 of patients, moderate liver dysfunction is detected. The diagnosis of temporal arteritis should be discussed in any patient older than 50 years who has a headache, transient or sudden loss of vision, pain and stiffness in the muscles, prolonged fever, anemia, and an increase in ESR. The diagnosis is confirmed by biopsy of the temporal artery. Differentiate temporal arteritis with periarteritis nodosa, which can affect the temporal arteries, nonspecific aortoarteritis , RA, vasculitis , migraine, trigeminal neuralgia, intracranial pathological processes, including tumors. After diagnosis, glucocorticoids should be prescribed immediately (prednisone at a dose of 45-60 mg per day). After a marked improvement in clinical and laboratory signs, the dose can be slowly reduced (2.5-5 mg every 2-4 weeks ). Long-term maintenance therapy is indicated, however, in most patients, glucocorticoids can subsequently be canceled. The prognosis is usually favorable , the disease does not affect life expectancy. The main serious complication is blindness. The prognosis worsens with damage to large vessels and aorta. The main clinical sign of rheumatic polymyalgia is pain in the muscles of the shoulder and pelvic girdle. Morning stiffness, as well as stiffness after a period of immobility, restriction of movements in the cervical spine, shoulder and hip joints are characteristic . Patients hardly get out of bed, dress, comb their hair, rise and go down the stairs; the gait becomes minced. Muscle strength usually does not decrease, although atrophy from inaction (due to pain ) can develop . With a careful examination of the joints, the detection of synovitis is possible . Pain in the proximal joints, tendons, and joint bags may be noted. Unpleasant sensations may appear first on one side, but soon become bilateral, common symptoms are characteristic – weakness, moderate fever, weight loss. Some patients have signs of temporal arteritis, primarily a headache, which may be the first sign of the disease. ESR is sharply increased, sometimes up to 60-80 mm / h. Mild normochromic anemia may be detected . Increased levels of fibrinogen, C-reactive protein. Polymyalgia rheumatica should be considered when there are severe bilateral pains in the muscles of the neck, shoulder and pelvic girdle, accompanied by a significant increase in ESR in patients over the age of 50 years. The disease should be differentiated with systemic vasculitis , dermatomyositis, RA, paraneo -plastic syndrome.

Antihypertensive conditions

Lowering blood pressure in children is quite common (from 5.8 to 14.8% – V.N.; 3asukhina and E.V. Fedorova), and it is often underestimated in terms of health status. Arterial hypotension is primary and secondary: with cardiovascular, endocrine, infectious diseases, against the background of nutritional disorders and dystrophy.

Etiology and pathogenesis of hypotensive conditions

In the development of arterial hypotension, a hereditary predisposition, a violation of the function of the endocrine glands (neurohumoral dysregulation is important . A major role is played by foci of chronic infection (tonsillitis, sinusitis, cholecystocholangitis , dental caries).
The basis of the changes are corticovisceral disorders, leading to a drop in blood pressure (neurocirculatory dystonia).

Clinic of antihypertensive conditions

In most cases, children do not complain, although they periodically have a decrease in blood pressure, headache, excessive sweating, irritability, fatigue, periodic dizziness and fainting. The pulse is labile. From the side of the heart – muffled I tone, systolic murmur of a functional nature. Blood pressure is lowered, initially systolic, then diastolic can also decrease. These changes may be transient, less often they become persistent.

Differential diagnosis of hypotensive conditions

Arterial hypotension should be differentiated with various chronic intoxications and the latent course of rheumatism. Secondary hypotension must be excluded.
The prognosis is favorable in most children with a transient decrease in blood pressure. However, the disease often proceeds for a long time, periods of improvement are replaced by periods of deterioration (under the influence of various adverse factors). With secondary hypotension, the prognosis depends on the course of the underlying disease.

Treatment of antihypertensive conditions

The correct regime of the day, the maximum stay in the fresh air, physical education, water procedures. Of great importance is a nutritious diet with sufficient intake of vitamins. In severe cases, you can prescribe caffeine, tincture of ginseng, Schisandra chinensis, zamanicha, aralia, pantocrine, bromides, tincture of valerian, elenium, andaxine . Physiotherapeutic procedures are applied with a tonic effect on the body. It is necessary to treat a focal chronic infection (debridement). With secondary hypotension, rational therapy of the underlying disease is of great importance.

Prevention of antihypertensive conditions

The correct regime of the day, a combination of training with physical education and sports, hardening procedures.

Obliterating brachiocephalic arteritis

Obliterating brachiocephalic arteritis (synonyms: pulseless disease, Takayasu’s disease ) is a non-specific inflammatory lesion of the aortic wall and large major vessels with their subsequent obliteration. Obliterating brachiocephalic arteritis has been described by Japanese physician Takayasu . Most often, young women get sick. The etiology is not known. A connection with repeated infections (tonsillitis, flu, typhus), hypothermia, trauma, and pregnancy is assumed.

Pathogenesis of obliterating brachiocephalic arteritis

Obliterating brachiocephalic arteritis belongs to the group of allergic vasculitis The hyperergic nature of inflammation is confirmed by the presence of immune complexes at the site of the lesion and anti-arterial antibodies in the serum.

Pathomorphology of obliterating brachiocephalic arteritis

With obliterating brachiocephalic arteritis, panarteritis is observed with an initial lesion of adventitia and intima with their exudative infiltration, disorganization, spreading to the middle membrane, subsequent fibrinoid organization, sclerosis and the development of parietal thrombosis.

Clinic for obliterating brachiocephalic arteritis

The disease is slowly progressive in nature. Most often, obliterating brachiocephalic arteritis begins with common symptoms: malaise, fever, arthralgia, night sweat, gradual asthenia . As the limbs ischemic , a feeling of numbness, an increase in weakness in the arm, a one-sided or symmetrical absence of a pulse on the affected limbs appear. Often “eye” symptoms (vision loss, pain in the eyes) associated with the formation of arteriovenous retinal aneurysms and optic atrophy join in . In most cases, hypertension associated with damage to the abdominal aorta and renal arteries is noted. Less characteristic (up to 20% of cases) damage to the pulmonary and coronary vessels. With obliteration of the carotid arteries, neurological symptoms associated with cerebral ischemia develop Laboratory data are not characteristic. Possible anemia, ESR increased to 50 mm / h. Factor rheumatoid and antinuclear antibodies are not determined. System complement components are normal. The diagnosis is confirmed by arteriography .

Treatment of obliterating brachiocephalic arteritis

Long-term administration of glucocorticosteroid drugs, indomethacin delagil , drugs that improve peripheral blood flow ( trental compliance angiotrophip prodectin ), heparin and indirect anticoagulants is indicated . In recent years, reconstructive surgery of affected vessels has been proposed.

Etiology of transposition of the great vessels

This defect is based on the abnormal position of the ascending aorta and the trunk of the pulmonary artery. There are 2 main types of this anomaly: complete and corrected transposition of the aorta and pulmonary artery. Complete transposition of the great vessels makes up 4.5–20.8% of all congenital heart defects. Together with tetralogy of Fallot, this vice takes a leading place among the “blue” vices. Its hemodynamics boils down to the fact that the aorta departs from the anatomically right ventricle, and the pulmonary artery from the left. As a result, blood circulation occurs in two divided circles. In the absence of communication between them, the newborn dies immediately after birth due to the lack of conditions for blood oxygenation . Therefore, a prerequisite for viability is communication between these independent circles of blood circulation. Usually they are represented by defects of the septa, OAA, aortopulmonary fistula, abnormal venous drainage, or combinations thereof. In this case, cross blood circulation is formed, which determines the severity of the course of the defect. It is determined by the degree of overflow of one and “robbing” of another circle of blood circulation. The heart in these conditions works with overload. Nevertheless, the larger the shunt, the more favorable the course of the defect. So, the most favorable combination of septal defects with moderate stenosis of the pulmonary artery, which prevents excessive pulmonary blood flow. Corrected transposition of the great vessels makes up 1–1.4% of all congenital heart diseases. In this case, the aorta and pulmonary artery are transposed, but the right ventricle receives blood from the left atrium, and the left from the right. Thus, arterial blood enters the large circle of blood circulation, and venous blood enters the small circle. If there are no other concomitant anomalies, hemodynamic disturbances are absent.

Clinic of transposition of the great vessels

Shortness of breath, cyanosis, fatigue, finger changes in the form of drumsticks, peripheral circulatory disorders, lag in physical development are noted. The auscultatory picture is diverse and is determined by the shape of the shunt between the circles of blood circulation. Along with the presence of pronounced systolic murmur along the left edge of the sternum, noise is often absent.

Diagnosis of transposition of the great vessels

X-ray examination early reveals cardiomegaly due to both parts of the heart. More often the heart has a spherical shape. There is an increase in vascular pattern of the lungs, pronounced vascular pulsation on the periphery. An ECG allows you to identify only signs of overload of various parts of the heart, single-beam blockade of the conduction system. The most complete information about anatomical and hemodynamic malformations is provided by Doppler ultrasound, heart sounding, and angiocardiography .

Hereditary hemorrhagic telangiectasia

Hereditary hemorrhagic telangiectasia (synonyms of the disease: Rendu- Weber- Osler disease) is a disease caused by thinning and dilatation of the walls of arterioles and capillaries. Hereditary hemorrhagic telangiectasia is transmitted by an autosomal dominant type, manifested in adults. The alleged defect is associated with impaired collagen synthesis in the vascular wall. Patients on the skin and mucous membranes of the nose, mouth, and gastrointestinal tract have characteristic vascular “spiders” of red color that disappear with pressure, which later rise above the surface and form nodules; in the lungs, arteriolovenous aneurysm of ism develops . Rupture of a dilated vessel (with mechanical compression, spontaneous) can cause hemorrhages.