Other

Break of the aortic arch type A. Atresia of the aortic arch - interruption of the main artery

MORPHOLOGY
Interruption of the aortic arch occurs with equal frequency both distal to the left subclavian artery (type A) and distal to the left common carotid artery (type B). Less common is a break distal to the innominate artery (type C). In almost all cases there is an associated anomaly, most commonly a malignant posterior VSD leading to subaortic obstruction and associated patent ductus arteriosus. Other forms of VSD are less common. Pathology of ventriculoarterial connections may be observed, in

including discordance, as well as double exit from the pancreas (Taussig-Bing anomaly). The presence of a 22q11 deletion should be sought in all cases of aortic arch interruption.
PATHOPHYSIOLOGY
Most often, when there is a combination of a broken arch and a patent ductus arteriosus, the child feels well until the narrowing of the ductus causes a critical decrease in perfusion in the lower body. In most cases, children are admitted to specialized departments during the first 2 weeks of life with an acute onset of heart failure, often complicated by shock and acidosis. In rare cases, the ductus arteriosus remains patent and excess pulmonary blood flow develops as pulmonary vascular resistance decreases.
DIAGNOSTICS
CLINICAL COURSE
The most specific finding is a difference in pulse rate in the upper half of the body with weakening of the pulse in one or both arms or in one of the carotid arteries (this pattern may change in response to pharmacological effects on the patent ductus arteriosus). Auscultation is usually nonspecific due to the presence of murmurs associated with concomitant cardiac pathology.
RADIOGRAPHY
The heart is usually on the left side with signs of cardiomegaly. As a rule, the pulmonary pattern intensifies. The absence of a thymic shadow may suggest the presence of a 22q11 deletion.
ELECTROCARDIOGRAPHY There are no specific ECG signs.
ECHOCARDIOGRAPHY
With echocardiography, it is necessary to obtain a complete description of the aorta, the site of the break, and also describe the origin of the vessels of the head and neck. Careful assessment of the intracardiac anatomy for associated pathology is essential for planning the surgical strategy.
CATHETERIZATION OF CARDIAC CAVITIES
Carrying out for diagnostic purposes is usually not required. It is widely replaced by echocardiography, sometimes in addition to MRI or CT.
TREATMENT
During the neonatal period, complete restoration of the interrupted aortic arch is usually performed along with closure of the VSD. The results of the operation depend on the nature and severity of the aortic arch obstruction and the clinical condition of the child. Long-term monitoring of the aortic arch is required, as there is still the possibility of residual or recurrent arch obstruction, as in patients after aortic coarctation repair.
VENTRICULAR OUTLET TRACT OBSTRUCTION
LV outflow obstruction accounts for 4% of all congenital heart defects and can occur at the subvalvular, valvular, and supravalvular levels, with the exception of bicuspid AV pathology, which usually does not cause problems in childhood. AC stenosis can occur as an isolated lesion or be associated with other obstructive lesions of the left heart at various levels (Shawn's complex). In this case, MV stenosis is usually combined with subaortic and/or aortic stenosis, as well as hypoplasia of the aortic arch and coarctation of its section.
MORPHOLOGY
Valvular aortic stenosis is the most common form of LV outlet obstruction (75%). Valve morphology and disease severity are extremely variable. In more severe cases, detected in utero or detected in the first hours and days after birth, a small LV may be observed, excluding the possibility of two-ventricle hemorrhage.

appeals. In addition, endocardial fibroelastosis may occur, affecting LV function.
In the case of subvalvular obstruction, three different morphological types are distinguished. In the most common form, there is the presence of a fibromuscular area, usually circularly located, which can be attached to the AC leaflets and to the anterior MV leaflet. In the "tunnel" type of subaortic stenosis, there is usually narrowing of the aortic valve in addition to small flow from the LV, which is often filled with fibrous tissue. The muscular type of blood flow obstruction is formed as an integral part in the structure of signs of hypertrophic obstructive cardiomyopathy. The incidence of supravalvular aortic stenosis is only 1-2% of all LV outflow obstructions in childhood. It can occur alone or as part of Williams-Beuren syndrome. Various morphological types have been described for this syndrome, including focal or diffuse narrowing, as well as a combination with anomalies of the aortic arch, including coarctation of the aorta. In Williams-Beuren syndrome, multiple stenoses of the arteries of both the systemic and pulmonary circulation are often encountered, which is associated with deletion of the elastin gene localized on chromosome 7.
PATHOPHYSIOLOGY
Severe LV outflow tract obstruction in the neonatal period requires emergency medical attention and in most cases it is either critical stenosis of the aortic valve or outflow obstruction at various levels. The obstruction is so severe that the LV is unable to maintain systemic circulation, which is dependent on right-to-left shunting through the patent ductus arteriosus. When a critical level of aortic stenosis is diagnosed in a fetus, the prognosis is poor. A heart chamber that does not receive enough blood during the antenatal period is unable to grow normally. In the case of very severe aortic stenosis, almost all cardiac output will be from the RV, with the LA and LV unlikely to develop. Worsening left heart hypoplasia in these patients can be documented with serial antepartum echocardiograms. This has led to the use of prenatal cardiac interventional catheterization to improve ventricular development. After birth, the manifestations of AV stenosis depend on the severity of the obstruction, LV size and function. The degree of obstruction may be underestimated due to weakened LV function, in such cases an assessment of both the peak systolic pressure gradient (gt; 75 mm Hg corresponds to severe) and the area of the AV (< 0.5 cm2 / m2 - corresponds to severe).
The most common form of aortic valve pathology is a bicuspid aortic valve. It can lead to severe aortic stenosis, but most often has more favorable manifestations. This rarely results in significant stenosis in childhood and generally results in a very small, but steady, increase in the gradient across the valve throughout life. From a young age onwards, patients with bicuspid aortic valves may be characterized by sufficiently severe stenosis for which surgical intervention is indicated. It should be remembered that in some cases the tissue of the ascending aorta has a pathological histological structure similar to that of Marfan syndrome, with cystic medial degeneration. This can lead to pronounced dilation of the ascending aorta and an increased risk of dissection.
Supra-aortic stenosis often appears at birth and can progress throughout life. The coronary arteries lie in a high-pressure region, distal to the valve but proximal to the stenosis. Theoretically, this could lead to severe atherosclerosis in the coronary artery system, but there is still no data to support this.
Local subaortic stenosis, as a rule, does not manifest itself antenatally or at birth and develops throughout life. The exact causes of subaortic stenosis are unknown. The most common theory is that shear stress in the LV outflow tract, resulting from sometimes minimal anatomical abnormalities of the LV, can stimulate the development and progression of subaortic stenosis. Sometimes this can happen very quickly, within the first few months after birth, in such cases early surgical intervention is indicated. There is a risk of relapse. When subaortic stenosis develops more slowly, the recurrence rate is lower.

DIAGNOSTICS
CLINICAL COURSE
Critical aortic stenosis in newborns leads to the rapid development of HF with a sharp decrease in LV function. Patients may experience tachypnea with tachycardia, pallor, decreased or absent pulse in the periphery. The second sound is often the only one, and a gallop rhythm can be heard. A systolic ejection murmur may also be present. These signs are characteristic of manifestation during the neonatal period and differ from manifestations at later stages, which occurs in most cases and is characterized only by noise in the absence of any other signs. In more severe cases, it may be associated with exercise intolerance and sometimes chest pain. On physical examination, such patients reveal a normal or weakened peripheral pulse, a decrease in the intensity of the aortic component of the second sound with a systolic ejection click and a systolic ejection murmur with irradiation to the neck. Supravalvular aortic stenosis is usually identified when patients diagnosed with Williams-Beuren syndrome are referred for routine cardiac screening testing. The signs of subaortic stenosis are similar to those of aortic stenosis, but in the first case, patients do not have an ejection click.
RADIOGRAPHY
Critical atrial stenosis in the neonatal period is usually associated with laevocardia, cardiomegaly and pulmonary edema. In older children, chest x-rays are often normal. In the case of a bicuspid aortic valve in an adult patient, dilatation of the ascending aorta can sometimes be seen.
ELECTROCARDIOGRAPHY
There is, as a rule, a deviation of the electrical axis of the heart to the left and signs of LV hypertrophy. In more severe cases, repolarization changes may be observed in the lateral precordial leads, suggesting ischemia and strain.
ECHOCARDIOGRAPHY
Aortic stenosis can be diagnosed using transverse echocardiography. In the neonatal period, it is extremely important to assess:
LV size/volume;
MK size;
signs of mitral regurgitation;
aortic ejection size and AV size;
severity of AC stenosis using Doppler ultrasound;
presence or absence of endocardial fibroelastosis;
LV systolic function.
This will help you make proper treatment decisions. In all forms of aortic stenosis in all age groups, weakened LV function may lead to an underestimation of the severity of aortic stenosis.
In older children and adults, the most widely accepted method for assessing the severity of valve stenosis is Doppler coefficients (gradients). When deciding on the timing of intervention, it is also important to assess the degree of LV hypertrophy and its function. The ability to be treated with interventional catheterization may be affected by the degree of aortic regurgitation that may be present (see Treatment). EchoCG can usually determine the nature and assess the severity of LV outflow tract obstruction in patients with subaortic stenosis. Doppler ultrasound can measure the maximum flow velocity through the LV outflow tract. However, it is not always possible, based on this speed, to obtain reliable indicators of pressure gradients, since in case of combined obstructions that prevent flow, the simplified Bernoulli equation cannot be used. The severity of stenosis, based on pressure gradient indicators, can only be assessed using invasive measurement methods.

In case of supravalvular aortic stenosis, it is important to determine the degree and severity of the pathology of the aortic arch, as well as to assess the degree of LV hypertrophy, which may be incommensurate with the degree of supravalvular aortic stenosis. Recently, new methods of echocardiography with Doppler imaging of tissue have been used to assess diastolic function, which makes it possible to correlate it with the severity of LV outflow tract obstruction. This will help in determining the optimal timing of intervention.
CATHETERIZATION OF CARDIAC CAVITIES AND ANGIOGRAPHY
These methods are not used to make a diagnosis and are more often used as a treatment for valvular aortic stenosis in both newborns and older children (see "Treatment"). They are sometimes indicated to measure the gradient in subaortic stenosis. In case of supra-aortic stenosis, their implementation can be dangerous. MRI and CT have a role in the evaluation of all forms of LV and aortic arch obstruction.
TREATMENT
In cases of critical AV stenosis in newborns, maintaining patency of the ductus arteriosus with prostaglandin is vital before the obstruction can be corrected by balloon dilatation or surgery. Infants and children with mild aortic stenosis may remain stable for many years as it progresses slowly, in which case intervention may be postponed into adulthood. In patients with moderate and severe aortic stenosis, the disease progresses rapidly, in patients with a gradient gt; 75 mm Hg. and LV hypertrophy, there is a high risk of sudden death. Infective endocarditis is a serious complication at any age.
During the neonatal period, both balloon dilatation and surgical treatment can be performed. Although randomized studies have not been conducted, the results of both methods appear to be comparable based on published data. The outcome of both approaches is determined, on the one hand, by the severity of the valvular deformity, and on the other, by changes in the LV, which may include endocardial fibroelastosis and infarction of the papillary muscles of the MV. In cases where the LV cavity is small or where multiple obstructive lesions are present, the alternative Norwood approach may be preferable (see Hypoplastic Left Heart Syndrome). All children with detected pathology at a later age should remain under constant cardiac monitoring with regular ECG, echocardiography and stress tests. Although reliable data are lacking, the generally accepted view is that surgical intervention in childhood is indicated in cases where there are signs of progressive increase in gradient, left ventricular hypertrophy, changes in repolarization on the resting or exercise ECG, abnormal blood pressure response in response to physical activity. Valve area should be calculated as coefficients may be misleading in the setting of decreased cardiac output. Balloon dilatation is generally the procedure of choice in older children, except in cases of significant aortic regurgitation. At all ages, both anterograde and retrograde routes are possible; using a balloon one size smaller than the valve diameter significantly reduces the risk of new aortic regurgitation. Similar principles apply to pediatric surgery. The result of aortic valvotomy should be a small gradient and little or no aortic regurgitation. The risk of surgery or catheterization is high in newborns, but much lower in older patients. Both treatments are palliative and, as a rule, re-stenosis gradually develops. In childhood, repeat valvotomy can be performed unless there is significant valve calcification or significant regurgitation, but eventually AV replacement is almost always required. However, according to the US Natural History Study of Congenital Heart Disease, only 27% of children who underwent aortic valvotomy after age 2 years required reintervention within 20 years.
In young children, when replacing a valve, the method of choice is the Ross operation or implantation of an “autograft” (implantation of a PC into the LV outflow tract and a homograft into the RV outflow tract). This allows the neoaortic valve to grow and does not require anticoagulants. However, the homograft will require replacement, and the long-term durability of the neoaortic valve remains uncertain. In older children, both types of treatment can be considered: biotransplantation and the Ross procedure. For adolescents and adults, the Ross procedure is less popular, and most patients and doctors opt for mechanical or biological prostheses. The most important reason
refusal of the Ross operation in adult patients is the gradual expansion of both the neoaorta (the part of the original pulmonary trunk to which the pulmonary valve is attached and which forms the most proximal part of the aortic trunk after the Ross operation) and the natural ascending aorta with its pathological vascular wall tending to the aneurysmal dilation. This can lead to progressive aortic regurgitation. Reoperations after the first Ross procedure may be required in the event of pulmonary homograft failure, aortic homograft failure, or dilatation of the ascending aorta. These problems can occur simultaneously, in which case the whole thing is often completely redone, a new homograft is placed in the PA in combination with the Bentall procedure (prosthetic valve combined with synthetic aortic root replacement). The choice of surgical approach depends on a number of factors, including age, appropriateness and safety of anticoagulation therapy and planning for future pregnancy, as well as patient preference and local expert opinion. Sometimes, when there is multilevel LV outflow tract obstruction or when there is a very small AV, more extensive surgery such as the Konno procedure is required.
The severity of supravalvular aortic stenosis progresses over time, and patients may be at risk of sudden death. Systemic arterial stenosis in major vessels such as the carotid and renal arteries may also progress. Indications for surgery are similar to those for aortic stenosis. Interventional catheterization, however, is not suitable and surgical treatment is required. This involves the introduction of patches to enlarge the supravalvular area extending into the sinuses of Valsalva. The onset of anesthesia and cardiopulmonary bypass may compromise coronary perfusion and surgery itself may be difficult because diffuse aortopathy is present in many cases.
Due to the progressive nature of subaortic stenosis, interventional intervention is generally recommended at earlier stages than for aortic stenosis, which is controversial because in some cases the defect may be mild and stable for many years. Most doctors recommend intervention if aortic regurgitation develops, as it can progress quickly. In this case, the use of interventional catheterization is impractical and surgical intervention is required. Immediately after surgery and in the early stages, the results are excellent, but the relapse rate is high. Complete removal of the obstruction during surgery is important, and the risk of recurrence is greatly reduced if a myotomy or myectomy has also been performed.
LONG-TERM FORECAST
Lifelong surveillance is mandatory for all types of LV outflow obstruction, treated or untreated. It should include assessment of LV outflow tract structure and function (including valve stenosis and regurgitation), as well as LV size, structure and function. Ventricular arrhythmias are more common in patients with LV hypertrophy and can lead to clinical problems, including, in rare cases, sudden death. Vigorous physical activity is probably contraindicated in the presence of LV hypertrophy or residual obstruction (>30 mm Hg), but social contact can be allowed in most cases.
Counseling during pregnancy is necessary as it can have serious effects on intracardiac hemodynamics. In this case, there is also a risk of relapse.

V. Annular vasoconstriction

A. Occurrence is infrequent.

B. Etiology: preservation or malformation of the 6th embryonic aortic branchial arch; normally, arches 1 and 2 disappear; 3rd on the right - a. carotis communis dextra; on the right is the 4th - innominate artery; on the left, 4th - transverse part of the aortic arch; 5th - never fully develops; 6th on the left - ductus arteriosus.

B. Pathology: five most important anomalies.

1. Duplication of the aortic arch (DAA). Usually one branch bends around the trachea from the back and one from the front, merging into the properly located trunk of the descending thoracic aorta; occurs extremely often with severe manifestations, symptoms usually appear at a very early age.

2. The aortic arch located on the right with the ligamentum arteriosus located on the left (behind the esophagus) is the next most common.

3. The right subclavian artery located behind the esophagus. It begins distal to the left a. subclavia and passes behind the esophagus. It occurs quite often, although it is asymptomatic.

4. The abnormal origin of the common carotid artery compresses the trachea.

5. The anomalous origin of the innominate artery compresses the trachea.

D. Clinic: symptoms of compression of the trachea, rather than the esophagus, are almost always noted.

1. Symptoms:

a) in infants with UDA, symptoms of stridor (“crowing”) appear early;

b) repeated pneumonia is observed;

c) dysphagia.

2. Diagnosis:

a) passage along the esophagus with barium (will emphasize compression of the esophagus at the level of the III-IV thoracic vertebra);

b) tracheogram;

c) angiogram.

D. Treatment: only if symptoms are present:

a) UDA: separation of the smaller of the arcs;

b) others: separation, mobilization and reimplantation of vessels according to indications.

E. After surgery, monitoring for increased tracheal compression is necessary. It is secondary to the early formation of edema.

G. Surgical treatment provides excellent results: low mortality, absence of many complications.

A. It is rare: it appears 2-3 days after birth. B. Anatomy: three types of anomalies:

1. Type A: located distal to a. subclavia sinistra, 40%.

2. Type B: located between the left common carotid and left subclavian arteries, 55%.

3. Type B: located between the innominate and left carotid arteries, 5%.

4. Usually there is a combination of intracardiac anomalies, mainly VSD.

B. Pathophysiology: absolute dependence on the ductus arteriosus. If the ductus closes, severe hypoperfusion of the lower body will occur with renal failure and severe acidosis.

D. Diagnosis: In many cases, echocardiography is a good help; Urgent angiography may be required.

D. Treatment.

1. Administration of prostaglandin E 1 to maintain a functioning ductus arteriosus.

2. Correction of acidosis.

3. Operations: type A: intervention similar to that done for coarctation of the aorta by thoracotomy on the left; types B and C: one-stage correction of all anomalies through median sternotomy under conditions of deep hypothermia and circulatory arrest.

In the intrauterine period, a defect does not affect the blood circulation of the fetus, since there is a well-functioning PDA.
In the postnatal period the fate of patients is also determined by the patency of the duct. Since it is prone to early closure, without appropriate treatment, more than 60% of children die within the first month of life. No more than 26% survive to 6 months. All survivors have stable sources of blood supply to the descending aorta (PDA or collaterals). Delivery is recommended in a specialized facility that has the capacity to administer prostaglandins and subsequent surgical treatment. The defect belongs to the 2nd category of severity.

Clinical symptoms of aortic arch rupture.

Because vice is a typical ductus-dependent pathology, the first symptoms occur with the onset of PDA closure, usually in the early neonatal period. The child's condition, which initially appeared normal, begins to rapidly deteriorate. Low cardiac output syndrome develops - poor pulse filling, tachycardia, shortness of breath, cold extremities, oliguria. The clearly insufficient function of the PDA is indicated by the disappearance of the pulse in the legs. The patient becomes restless and then inhibited. Decompensated metabolic acidosis is determined in the blood. Ischemic liver damage leads to an increase in the concentration of transaminases and lactate dehydrogenase in the blood, kidney damage leads to an increase in creatinine, and intestinal damage leads to necrotizing enterocolitis.

With more favorable course of the defect symptoms are less pronounced. One can note mild cyanosis, which in 10% of patients has a differentiated character (more pronounced on the legs), feeding difficulties, and retardation in physical development. Depending on the anatomical variant of the defect, there is a different filling of the pulse in the extremities. In type A, the pulse in the arms is higher than in the legs. In type B, there is an additional decrease in pulse in the left arm, and in case of abnormal origin of the right subclavian artery, in all extremities. As hypervolemia of the pulmonary circulation or severe obstruction of the left ventricular outlet develops, symptoms of heart failure develop (tachycardia, shortness of breath, hepatomegaly). Increasing cyanosis and disappearance of heart murmur are unfavorable prognostically.

Auscultatory picture reflects the presence of concomitant heart defects - VSD and PDA. There is usually a slight systolic murmur in the second or third intercostal space on the left.
Electrocardiography for atresia of the aortic arch. In newborns, the deviation of the electrical axis of the heart to the right remains. With the long-term existence of the defect, left ventricular or biventricular hypertrophy develops.

Chest X-ray. In newborns, the x-ray picture may not change. The heart shadow is moderately dilated, the vascular bundle is often narrow due to the absence of the thymus (with DiGeorge syndrome). As the TLC decreases, the pulmonary pattern intensifies along the arterial bed; with the development of heart failure, symptoms of venous stagnation and pulmonary edema appear.

Echocardiography for atresia of the aortic arch. In most cases, echocardiography makes it possible to make a complete diagnosis necessary for treatment planning. In addition to localizing the break site, it is necessary to evaluate the diastasis between the aortic segments, the degree of left ventricular obstruction, and the diameter of the aortic valve and ascending aorta. Identification of concomitant congenital heart disease is of great importance.

Ventricular septal defect usually non-restrictive, blood discharge through it is directed from left to right or bilateral. If there is a PDA, blood flows through it from the pulmonary artery into the descending aorta.

Treatment of atresia of the aortic arch.

The main method of treatment In newborns with a rupture of the aortic arch, infusion of group E prostaglandins is recommended to maintain the patency of the PDA. At the same time, adequate intravascular blood volume should be maintained and anemia corrected.

To maintain cardiac output inotropic drugs are used, most often dopamine (4-6 mcg/kg/min). This drug has an additional positive effect on renal function, which suffers when the aortic arch is interrupted. However, high doses, which cause an increase in peripheral resistance and a shift in blood volume to the lungs, should be avoided.

In some cases it is necessary resort to artificial ventilation of the lungs, which is carried out in a mode that excludes hyperventilation and without the addition of oxygen. These measures are necessary to prevent PDA closure or excess pulmonary blood flow. You should not strive to increase systemic S02 by more than 75-85%. Constant monitoring and correction of metabolic acidosis is also necessary.

In patients with associated syndrome DiGeorge requires periodic calcium level checks; transfusing them with citrated blood can be dangerous.

Surgical intervention performed after stabilization of the patient's condition. The operation of choice is to create a direct anastomosis between the aortic segments. Interposition of a synthetic prosthesis is also possible. In case of large shunting of blood through the VSD, surgery on the aorta is supplemented with narrowing of the pulmonary artery; radical correction is performed after a few months. In clinics with extensive surgical experience, they perform immediate elimination of aortic arch interruption and accompanying congenital heart disease.

Typical coarctation of the aorta- congenital segmental narrowing or complete atresia in the area of the aortic isthmus, at the border of the distal arch and the descending aorta. The defect can be isolated or combined with other congenital heart defects (CHD), which has a significant impact on the course and prognosis of the disease. The difference between the circulatory conditions above and below the narrowing constitutes the main pathophysiological and clinical characteristic of coarctation of the aorta, regardless of the anatomy of the defect.

Depending on the degree of hemodynamic disturbances and the time of their occurrence during ontogenesis, various congenital obstructive lesions of the aortic arch may occur: interruption, atresia, tubular hypoplasia, juxtaductal coarctation.

Coarctation of the aorta is a critical congenital heart defect in the neonatal period. According to the classification of A.V. Pokrovsky, there are 4 types:

Type I isolated coarctation of the aorta;

Type II combination of coarctation of the aorta with a patent ductus arteriosus;

Type III combination of coarctation of the aorta with other congenital heart defects;

Type IV atypically located coarctation of the aorta.

The incidence of this defect among critical congenital heart defects is 10%. Modern diagnostic techniques make it possible to detect congenital heart disease not only in the first hours of a child’s life, but also prenatally. The annual growth of newborns with obstructive lesions of the aortic arch and isthmus explains the relevance of this issue from the point of view of diagnosis, treatment and prevention. At the same time, the presence of severe coarctation of the aorta, especially in combination with other complex congenital heart disease (LV hypoplasia, TMS and others) is the reason for the rapid development of a critical condition, and, in this regard, the urgency of surgical treatment becomes an objective necessity.

Critical coarctation of the aorta is a ductus-dependent pathology. In the prenatal period, this defect does not affect the development of the fetus. This is due to the fact that, due to the peculiarities of intrauterine hemodynamics, the PDA functions, and the area of the aortic isthmus is normally narrowed. The blood supply to the coronary arteries, vessels of the brain and upper extremities occurs due to the ejection of blood from the left ventricle. No more than 15% of the total ejection of the left ventricle flows through the aortic isthmus. The right ventricle plays the main role in ensuring systemic blood flow, and the blood supply to the lower half of the body is provided by the patent ductus arteriosus. After birth, closure of the patent ductus arteriosus occurs and, in the presence of true coarctation of the aorta, this leads to decreased blood flow in the descending aorta with hypoperfusion of the lower half of the trunk and internal organs and increased pressure in the brachiocephalic vessels and left ventricle.

The severity of the condition of newborns with critical obstructive lesions of the aortic arch and isthmus is caused by progressive heart failure, hypoperfusion of the lower half of the body, and the development of metabolic acidosis. The survival of children with these defects is determined by the functioning of the patent ductus arteriosus, which provides blood flow in the lower half of the body. The prognosis for rupture of the aortic arch and critical coarctation of the aortic aorta and the mortality rate depend on the degree of associated anomalies, the time of diagnosis and the tactics of preoperative management of the patient.

In most cases, manifestations of the defect begin with respiratory failure - tachypnea, shortness of breath, the filling of the pulse in the arms and legs is different: in the lower extremities the pulsation may not be detected or may be weakened. As already noted, in newborns the physiological narrowness of the aortic isthmus creates a pressure gradient between the pressure in the arms and the pressure in the legs, which in 16% of children exceeds 20, and can reach up to 30 mm Hg. Art. However, as a rule, in healthy children it decreases quickly within a few days, and in the presence of true coarctation of the aorta, on the contrary, is increasing. When the aortic arch is interrupted, depending on the anatomical variant, there is a different filling of the pulse in the extremities. With type A, the pulse in the arms is higher than in the legs, and with types B and C, there is a decrease in the pulse in the legs and left arm. With coarctation of the aorta and a break in the aortic arch (type A) in combination with an anomalous origin of the right subclavian artery, a decrease in pulse is observed in the right arm, with a break in the aortic arch (type B) in combination with an anomalous origin of the right subclavian artery - in all extremities. A poor prognostic sign is the disappearance of the pulse in the legs, which indicates closure of the ductus arteriosus. Subsequently, the symptoms of circulatory failure progress, with shortness of breath, tachycardia, fluid retention, peripheral edema, hepatomegaly, and crepitant rales in the lungs. In newborns with ductus-dependent pathology, when the ductus arteriosus is closed, a sharp deterioration in condition is possible due to the development of low cardiac output syndrome. Decompensated metabolic acidosis develops. Ischemic damage to the liver leads to increased concentrations of transaminases and lactate dehydrogenase in the blood, intestines to necrotizing enterocolitis, kidneys to oliguria, and increased levels of creatinine and urea in the blood. An unfavorable prognostic sign is a blood urea level of more than 12 mmol/l.

1. The electrocardiogram shows a deviation of the electrical axis of the heart to the right, signs of overload of the right parts of the heart.

2. During X-ray examination, an increase in the pulmonary pattern and cardiomegaly is observed.

3. Accurate topical diagnosis of aortic coarctation and aortic arch interruption can be obtained using echocardiography.

4.Angiocardiography, namely aortography, allows you to determine the location of coarctation of the aorta or interruption of the aortic arch, assess the degree of hypoplasia, the location of the vessels, the distance between interrupted segments of the aortic arch.

5.Computed tomography - allows not only accurate topical diagnostics, but also the use of 3D and 4D reconstructions to obtain a spatial image of various structures, their relationships and functional characteristics.

The neonatal period is the most critical age in which there is a high natural mortality rate for this defect. To confirm a critical obstructive lesion of the aortic arch in a newborn and provide immediate assistance, he should be urgently transported to a cardiac surgery hospital.

In order to stabilize the condition and create a favorable background for surgical treatment, intensive therapy is necessary, which is based on two principles: maintaining blood flow through the PDA by infusion of group E prostaglandins and ensuring an adequate ratio between systemic and pulmonary blood flow.

Infusion of PGE1 allows not only to maintain blood flow into the descending aorta through the patent ductus arteriosus, but also leads to relaxation of ductal tissue in the aortic wall in the area of aortic coarctation. At the same time, pulmonary vascular and general peripheral resistance decreases. Before the introduction of group E prostaglandins into practice, it was very difficult to deal with the critical condition of the patient caused by the closure of the ductus arteriosus immediately after birth. Newborns were operated on in a decompensated state of congenital heart disease - in the stage of anuria and metabolic acidosis, often the outcome was fatal. With the start of prostaglandin infusion, the patients' condition can be relatively stabilized: metabolic acidosis disappears, urination improves, and afterload on the left ventricle decreases. This simple solution allows you to postpone the operation for some time to stabilize the patient's condition.

In some cases, infusion of cardiotonic drugs is indicated to maintain adequate cardiac output. The most commonly used drugs are dopamine, dobutamine, and adrenaline. In some cases, especially in the presence of additional congenital heart defects, despite treatment, heart failure progresses with the development of pulmonary edema or cardiogenic shock. With progressive heart failure, artificial ventilation is indicated. Ventilatory parameters should be adjusted to maintain pulmonary vascular resistance high, avoiding pulmonary hypervolemia, and avoid closure of the ductus arteriosus. This goal can be achieved by limiting the use of hyperoxic gas mixtures (air ventilation), with a high PEEP value (up to 10 mbr) and maintaining pCO2 at 40-45 mm Hg. Art., since high p02 reduces pulmonary vascular resistance and increases systemic vascular resistance, distributing blood flow so that a large amount of blood remains in the vessels of the pulmonary circulation.

Our studies have shown that, if necessary, pathogenetic therapy should be started during emergency transportation of a newborn to a cardiac surgery hospital:

Preservation of blood flow through the PDA (1st principle) S Ensuring an adequate ratio of systemic and pulmonary blood flow (2nd principle) PGE infusion Fluid restriction Diuretics Cardiotonics ALV

Correction of metabolic acidosis by infusion therapy with fluid restriction to 12 ml/kg/h, administration of diuretics, infusion of cardiotonics (dopamine, dobutamine, adrenaline), artificial ventilation (air ventilation with a high PEEP value (up to 10 mbr) and maintaining pCO2 at the level 4045 mm Hg to maintain pulmonary vascular resistance at a high level).

After stabilization of the condition, surgical treatment should be performed.

Propranolol, trade name Propranololum (genus Propranololi), improves the functioning of the heart muscle, strengthening it. When taken regularly, it helps prevent the risk of heart attack. It is also used to mitigate the effects of menopause in women, is useful in labor, and helps improve muscle contraction. Despite the fact that the abstract does not contain such information, Propranolol is used in the treatment of splenic hemangioma. The treatment result is positive. The drug is created on the basis of an active substance identical to its name. Currently available in two forms - Propranolol and its namesake with the addition of Nycomed. All that distinguishes them is the manufacturer. In all other respects they are completely identical.

Compound

Propranolol is sold in pharmacies in a single format - tablets. The basis for this drug was propranolol, a non-selective beta-blocker that blocks the activity of beta-adrenergic receptors.

The pharmaceutical industry produces three dosages of Propranolol. The smallest is 10 mg. The next one contains four times more active ingredient - 40 mg. And the highest concentration is contained in the third format, which contains 80 mg of the main component.

By blocking the same beta-adrenergic receptors, the strength and frequency of heart contractions decreases. Myocardial contractility decreases and, as a consequence, cardiac output decreases. The myocardium's need for oxygen decreases.

Properties

Propranolol, by inhibiting beta-adrenergic receptors, causes the walls of blood vessels to relax, resulting in a decrease in blood pressure. Stabilization of heart rate is due to a decrease in the susceptibility of myocardial cells, and the reaction to external factors of irritation decreases. As a result of this series of actions, platelets stop attaching to the walls and forming clusters. In parallel, the drug increases the level of adrenaline in the body. For the correct rhythm of work, the body requires less oxygen; propranolol allows you to bring this consumption rate back to normal. Tachycardia is eliminated, the rhythm is restored.

As a result of long-term use, due to the stimulation to increase the lumen of blood vessels with a parallel decrease in the level of spasms, the pressure is restored and comes to a stable working state. As a result of this work, the volume of blood entering the venous return is reduced, which reduces the load on the heart and the functioning of the peripheral blood supply system comes to a stable state.

A decrease in pressure leads to a decrease in the susceptibility of receptors in the aortic arch. This allows you to maintain blood pressure and eliminate relapses.

As a result of constant use, atherosclerotic plaques become smaller, new ones do not appear. As a result, the symptoms of hypertension and coronary artery disease are not so pronounced, and the overall tone of the body increases.

It is often used in gynecology to stimulate the contractility of the uterine muscles during childbirth and after.

Once in the body, it is absorbed almost completely, but the bioavailability of propranolol is not so high, since passing through the liver it is very quickly metabolized. It is excreted from the body in urine.

Purpose

Propranolol - instructions for use

It is better to take after a meal, then the active substance is better absorbed, staying longer in the body. The dosage is prescribed by the doctor depending on the diagnosis and severity of the disease.

If you have high blood pressure, you should start with 80 mg twice a day. If after a few days the desired effect has not occurred, you can increase it to 100 mg three times a day. To make the result more noticeable and faster, diuretics can be added to the course with Propranolol.

For heart rhythm disturbances, cardiomyopathy and thyrotoxicosis, 10-40 mg is given no more than 4 times a day. The daily portion should not exceed 240 mg.

For subaortic stenosis, you should take 20-40 mg no more than 4 times a day.

For angina, tremor, migraine, portal hypertension, 40 mg is prescribed no more than three times a day.

If the initial dosage does not lead to the desired result, once a week you can increase its volume by 40 mg until the optimal course is selected.

When preparing for pheochromocytoma surgery, you should take Propranolol three days the day before.

After a myocardial infarction, you should take the drug for no longer than three weeks. 40 mg four times a day in the first three days, then you can double the single volume and take it twice a day.

To stimulate the contractility of the uterine muscles, take 20 mg every half hour (most often 6 procedures are sufficient).

After childbirth, to avoid bleeding, it is enough to take 20 mg three times a day for 5 days.

The drug is suitable for long-term therapy, lasting for years. The maximum permissible volume of the drug should not exceed 640 mg. In older patients, no additional correction is required.

Propranolol therapy should not be abruptly stopped; this should be done gradually, reducing the portions several times.

Treatment regimens for children are calculated individually in each case, depending on weight and diagnosis. The calculation of the initial dose is based on the formula 0.5-1 mg per kg of body weight per day. Subsequently, this volume is increased to the required level. The maximum allowable is 2-4 mg/kg.

Overdose

Convulsions;
Decrease in heart rate to 50 per minute;
Too much pressure drop;
Bronchospasm;
Collapse;
Difficulty breathing;
Acrocyanosis.

In case of overdose, the first step is to eliminate traces of the drug from the body by performing gastric lavage. Activated carbon can be used as a sorbent; it would be a good idea to take a laxative, which will help remove excess. Further symptomatic treatment is required. Most often, an overdose of Propranolol requires emergency intervention, including resuscitation.

Side effects

Weakness of the body, lethargy, convulsions, unstable mood, sweating, confusion in reasoning and even short-term memory loss are often noted.

The perception of taste may change, a feeling of thirst, dry eyes, runny nose, and pharyngitis may appear.

Deterioration in heart failure in the chronic phase may occur, the rhythm may decrease, pain may appear in the chest region, a decrease in blood pressure when changing position, a decrease in leukocytes and platelets.

Rarely. But there may be pain in the stomach, diarrhea, vomiting, liver problems, jaundiced skin, dark urine.

There have been isolated cases of decreased libido.

If you have diabetes, you may experience rashes, redness, and even psoriasis.

Dry cough, abnormal heart rhythm, chest pain.

During pregnancy, fetal development may be delayed.

After discontinuation of the drug, all adverse reactions go away without additional treatment, on their own.

Contraindications

Damage to arteries with ischemic syndrome;

Prinzmetal's angina;

Rare heartbeat, not exceeding 55 beats/min.;

Cardiogenic shock;

Bronchial asthma in acute form;

Upper blood pressure readings are too low;

Hypersensitivity to the main component or to any additional component;

Sinus node dysfunction syndrome;

Blockade of the sinoatrial and atrioventricular.

Interaction

Drugs that lower blood pressure and act as diuretics can cause the blood pressure to drop too much. A break of at least two weeks should be taken between MAO inhibitors and this hypoglycemic drug; taking them together may also lead to too strong a decrease in blood pressure.

A combined course of Nifedipine and Propranolol may lead to an excessive decrease in blood pressure. Combination with sleeping pills and drugs that have a sedative effect can provoke obvious depression of the central nervous system.

With diabetes, there is a possibility that the effectiveness of insulin and other drugs responsible for reducing sugar will be insufficient. Propranolol can smooth out the signs of developing hypertension and tachycardia, as a result the disease will not be detected in time. It is also able to increase the content of xanthones in the body.

NSAIDs reduce the effectiveness of taking hypoglycemic drugs, which means that the blood pressure may gradually go beyond the stable range.

While taking Propranolol, skin tests with allergens should not be performed; this combination can cause a severe allergic reaction, anaphylactic shock. Iodine-containing radiocontrast drugs can also provoke anaphylactic shock. There is still a possibility that blood pressure may drop too much. There is also a high risk of a huge “jump” down when combined with Phenytoin and inhaled anesthetic drugs.

Antihistamines may lose some of their therapeutic abilities in this combination, but the effectiveness of uterotonics, on the contrary, may increase.

Analogues

Propranolol has many analogues, both in terms of the main ingredient and the effect it has on the body. This is very convenient, since the original is quite expensive, its price reaches 1800 rubles, and it is not available in all pharmacies.

Anaprilin. The range of indications is approximately the same as that of our original, but has slightly fewer contraindications, which makes it more loyal. Allowed for use in pregnant women, but with a reservation. Quite cheap, even in the highest dosage the price range does not go beyond 66 rubles.

Inderal. It is also widely used for high blood pressure and other heart diseases. The drug is of German origin and does not have a democratic price tag.

Obzidan. Retains all the basic properties of propranolol. Icelandic manufacturer. It costs an order of magnitude cheaper than many analogues - on average 90 rubles.

Alotendin. A Hungarian drug containing amlodipine and bisoprolol. It has a hypoglycomic effect, the mechanism of action is similar to Propranolol. The cost is quite expensive, on average above 2200 rubles.

Aritel. Contains bisoprolol. Refers to beta blockers. Indicated for hypoglycemia and coronary heart disease. The cost is quite affordable, around 130 rubles.

Bidop. Like all the previous ones, it has antianginal, hypotensive properties. The main ingredient of bisoprolol is hemifumarate. The cost is also quite affordable, on average 240 rubles.

Concor. The main ingredient of bisoprolol is fumarate. The mechanism of its work is also similar to Propranolol, but it has a slightly wider list of indications, including coronary artery disease. The cost is also much cheaper than its German counterpart, about 350 rubles.

Egilok. It works with metoprolol tartrate. The effect on the body is the same as most analogues, but the cost is much lower than many of them, within 135 rubles.

The list of Propranolol analogues is quite large. Due to the fact that the original is quite expensive, you can easily select a more suitable drug from this list, both in terms of pricing policy and the number of adverse reactions and contraindications. It should be understood that you should never change medications yourself. Such prescriptions can only be made by a doctor, based on the condition of the patient’s body and his possible reaction to certain drugs.

Recipe in Latin

Rp: Tabulettam Propranololi 0.01 No. 40

Da.Signa: 1 tablet 2 times a day

Precautions

Due to the fact that treatment with Propranolol can last for months and years, do not forget that medical supervision is necessary at all times. It is quite possible that in a year or two you may need to take additional medications that have a hypoglycemic effect.

The compensatory cardiovascular response decreases when anesthesia is used, so you should stop taking the drug a few days before the intended use of anesthetics. When choosing a drug, it is better to lean toward those that have minimal negative inotropic effects. The anesthesiologist must be informed that treatment with this medication was carried out.

It is better not to use the drug for the treatment of children. In older patients, there is a high risk of third-party reactions in the central nervous system.

In case of instability in liver function in the first days, you should start taking it with small doses; medical supervision at this time is necessary.

May hide symptoms of low blood pressure when receiving insulin treatment in patients with diabetes mellitus. Therefore, constant monitoring of such patients is required.

Exacerbations of psoriasis are possible.

If possible, do not use in newborns and infants, only if the expected benefit outweighs the risk.

Requires caution in patients with bronchial asthma.

After long-term use of the drug, it should be discontinued by gradually reducing the dosage; medical supervision is required.

During treatment, alcohol intake should be avoided.

Drivers and patients whose activities involve increased concentration should use this drug with caution. In case of an increased adverse reaction to the psychomotor function of the body, it is better to avoid driving and engaging in hazardous activities.

Pregnancy and breastfeeding

During pregnancy in any trimester, treatment with this drug is possible only if the expected benefit outweighs the risk to the unborn baby. The drug should be discontinued a maximum of three days before the expected birth. Possible side effects of such treatment may include delayed fetal development, low blood pressure, and bradycardia.

The active substance passes into breast milk, so at this time it is better to stop breastfeeding completely, or to closely monitor the baby.

Reviews

What is aortic sclerosis and its treatment

Causes of the disease
Clinical picture
Damage to the thoracic artery
Damage to the abdominal part of the artery
Treatment tactics
Preventive measures
Drug therapy
Folk recipes

The largest arterial vessel in the human body is the aorta. It originates from the left ventricle of the heart, runs along the spine and supplies oxygenated blood to all organs and tissues.

Damage to the aorta occurs as a result of a violation of cholesterol metabolism in the body. At the same time, low and very low density cholesterol accumulates in the endothelium of the vessel, which leads to the proliferation of connective tissue in its wall and a decrease in elasticity. Further development of the pathology causes the formation of an atherosclerotic plaque, which constantly increases in size and reduces the patency of the artery. Violation of the integrity of the plaque leads to its instability, accumulation of platelets on the surface of the formation, which significantly reduces the lumen of the vessel and slows down blood circulation.

Aortic sclerosis can develop anywhere and occurs as a result of the formation of an atherosclerotic plaque consisting of cholesterol, fats and calcium in the vessel wall. The disease develops after the age of 40, and the male population is affected more often than the female population.

Causes of the disease

Factors contributing to aortic sclerosis include:

increased levels of low and very low density cholesterol and triglycerides in the blood;
decreased glucose tolerance;
diabetes mellitus;
hypertension, symptomatic hypertension;
metabolic disorders (gout);
physical inactivity (sedentary lifestyle);
chronic stress;
obesity;
nicotine and alcohol addiction;
hereditary predisposition.

An important role in the development of sclerotic changes in the arteries is played by poor nutrition. The predominance in food of fatty foods high in cholesterol, easily digestible carbohydrates and a lack of vitamins leads to the appearance of pathology.

Clinical picture

Symptoms of the disease depend on the level of damage to the aorta, which is divided into the thoracic and abdominal parts. The thoracic section of a large artery supplies blood to the brain, heart, lungs, and upper limbs. Vascular branches from the abdominal part supply blood to the kidneys, abdominal and pelvic organs, and lower extremities. In severe cases of sclerosis, the entire length of the aorta may be affected, which is manifested by the diversity of the clinical course of the disease.

The disease includes several stages:

Preclinical period – changes are observed with laboratory and instrumental research methods.
Period of clinical manifestations – symptoms of the disease are revealed, depending on the level of vascular pathology.

Damage to the thoracic artery

The aortic root is located at the exit of the left ventricle in close proximity to the semilunar valve. Its atherosclerotic changes cause narrowing of the vessel, which leads to increased pressure in the left parts of the heart and pulmonary circulation. The semilunar valve changes as a result of changes in hemodynamics (blood movement), and its insufficiency develops.

When the pathological process is advanced, an insufficient amount of blood enters the systemic circulation, which stagnates in the lungs. This leads to pneumosclerosis (replacement of lung tissue with fibrous tissue), impairs the enrichment of blood with oxygen, and causes the development of organ hypoxia (insufficient oxygen supply). Increasing hypertrophy of the left ventricle compresses the coronary arteries and leads to ischemic damage to the heart, leading to myocardial infarction.

Three large arterial vessels branch from the aortic arch: the brachiocephalic trunk, the left common carotid and the subclavian artery. They supply blood to the neck, upper limbs, head, including the brain. The most severe is damage to the blood vessels of the brain, which can result in the development of hemorrhagic and ischemic strokes.

Clinical manifestations of aortic sclerosis in the thoracic part:

pressing pain behind the sternum, spreading to the neck, arms, epigastrium, interscapular region;
hoarseness of voice, difficulty swallowing (if the arch of the artery is damaged);
an increase in systolic (upper) blood pressure, while diastolic (lower) remains within normal limits;
headaches and dizziness, fainting;
convulsions with a sudden change in body position;
pulsation in the intercostal area of the right half of the chest;
formation of wen on the face and neck, change in the color of the iris (trophic disorders).

Damage to the abdominal part of the artery

Sclerosis of the abdominal aorta leads to disruption of the blood supply to the abdominal organs (intestines, liver, stomach), retroperitoneal space (kidneys), and pelvic organs (uterus, gonads, bladder). The disease can also cause the development of an abdominal form of myocardial infarction. The development of pathology at the level of the artery bifurcation leads to a decrease in blood flow in the lower extremities and the occurrence of trophic disorders in them.

Clinical manifestations of sclerotic aorta in the abdominal part:

aching pain in the abdomen of an incoming nature;
tendency to constipation, flatulence;
loss of appetite, weight loss;
coldness of the lower extremities, decreased sensitivity, numbness;
intermittent claudication syndrome (pain in the legs when moving);
swelling of the lower extremities, trophic ulcers;
decreased tone of the calf muscles;
sexual impotence in men.

Changes in the aorta can be detected by palpation (feeling) through the abdominal wall in the form of compaction and curvature of its wall. In severe cases, there is no pulsation at the level of the navel, in the groin and popliteal areas, or on the vessels of the feet. Thrombosis of the mesenteric arteries is considered a dangerous complication of aortic atherosclerosis, which is accompanied by intense abdominal pain; the attack is not controlled by analgesics and leads to the development of sepsis.

Treatment tactics

These methods can be attributed to the prevention of vascular atherosclerosis. Lifestyle changes are of great importance for recovery and increase the effectiveness of therapeutic interventions with the use of medications. To reduce the development of a pathological process in the body, folk remedies therapy is widely used.

Preventive measures

To get rid of the disease, a diet is used that can restore metabolism, normalize blood pressure, and improve the body's tolerance to glucose. The basic principles of proper nutrition include:

reducing the calorie content of food to 2000 kcal/day to combat excess weight;
reducing the content of animal fats in the diet (pork, butter, lard) to normalize high-density lipoproteins in the blood;
eliminating the intake of “wrong” carbohydrates (baked goods, sweets, potatoes, rice) to normalize the process of glucose tolerance and the conversion of these products into fats;
reducing salt intake to restore normal blood pressure levels;
eating fiber (cabbage, vegetables, fruits) and foods with a high concentration of polyunsaturated fats (vegetable oil, seafood) to normalize blood cholesterol.

Physical exercise, walking, and giving up bad habits are useful, which significantly improves your health. Breathing exercises are indicated for the appearance of pneumosclerosis.

Drug therapy

If cholesterol levels do not decrease with preventive measures, treatment of atherosclerosis with medications is prescribed.

Bile acid sequesters (colestipol) interfere with the absorption of bile acids in the digestive tract and cholesterol from food.
Statins (simvastatin) normalize cholesterol metabolism in the body, stabilize atherosclerotic plaque, and prevent complications associated with its disintegration.
Vitamin PP improves fat metabolism, reduces the concentration of low and very low density cholesterol in the blood.
Fibrates (gemfibrozil) participate in enzymatic reactions of fat breakdown, thereby regulating cholesterol metabolism.

In case of irreversible changes in the aorta of the lungs and aorta of the heart, which causes a critical change in hemodynamics and the development of severe complications, surgical removal of a section of the vessel is performed, followed by prosthetics.

Folk recipes

Traditional medicine offers recipes that have no absolute contraindications, have a gentle effect on the body and can effectively complement drug therapy.

Sophora japonica tincture is prepared from a glass of crushed pods and 500 ml of medical alcohol. The medicine is infused in a cold place for 20 days. Drink a tablespoon of tincture three times a day before meals, the treatment course is 3 months.
Lemon juice, liquid honey and vegetable oil are mixed in equal proportions. Take a dessert spoon of the product in the morning on an empty stomach for three weeks, repeat the treatment if necessary.
Finely chopped garlic and grated lemon along with zest are mixed in equal quantities. The resulting slurry is poured with half a liter of water and left for 5 days. Take 50 ml in the morning on an empty stomach for a month.

The use of medicines and traditional medicine recipes must be agreed with a doctor and carried out under his watchful supervision.

Atherosclerosis of the aorta can occur without clinical symptoms for a long time and cause the development of severe complications. Preventive measures, annual medical examinations, and treatment of concomitant diseases such as diabetes mellitus, hypertension, and obesity are of great importance for preventing the disease.

Treatment of atherosclerotic plaque in the carotid artery

Abdominal aortic aneurysm and its treatment