Peripheral stenosis of the pulmonary artery. Causes

13.08.2020

There are several factors in the onset of pulmonary artery stenosis in children. They can be congenital and acquired. As a rule, children are most often born with this condition. It ranks second in the total number of congenital heart defects.

The causes of the appearance of a congenital form of pulmonary stenosis are pathologies and conditions that affect the fetus even during pregnancy (antenatal period):

taking antibiotics, drugs and psychotropic substances in the first three months of pregnancy;

late first childbirth, when the age of the expectant mother is more than 38 years old;
genetic predisposition on the part of the mother or father (the presence in the family of persons with diagnosed heart defects);
unsuitable working conditions for a pregnant woman, for example, working with chemicals, poisons and toxic substances. Within the framework of this factor, living in a radioactive zone is noted;
multiple pregnancy;
past infections during gestation (pregnancy): rubella, measles, mononucleosis;
x-ray machine radiation, etc.

However, pulmonary stenosis is not always congenital. It can also be acquired. In this case, the causal factors are:

inflammatory lesion of the inner wall of the vessel, observed in syphilis, tuberculosis;
atherosclerotic lesions of vessels with a large diameter, incl. and pulmonary artery;
heart damage by the type of hypertrophy (hypertrophic myocardiopathy), in which the intracardiac part of this vessel is compressed;
compression of the trunk of the pulmonary artery by formations from the outside, for example, with tumors of the mediastinum (area near the heart);
calcification (deposition of calcium salts in the vascular wall).

Symptoms

This is one of the most common malformations in children. Therefore, the symptoms are expressed from the first days of life.
Signs of pulmonary artery stenosis include the following:

shortness of breath while eating and even while the child is at rest;
poor weight gain in the first months of life;
anxiety;
lethargy;
slight developmental lag;
bluish skin tone, most often of the nasolabial triangle.

These symptoms of the disease are typical for newborn babies.

For older children, you can point out the following signs of the disease:

dizziness;
heart palpitations;
swelling of the neck veins;
drowsiness;
dyspnea;
frequent colds;
weakness after exercise;
swelling of the body.

In situations where the pressure in the right ventricle of the heart exceeds 75 mm. rt. Art., clinical signs of pulmonary stenosis may be absent. This is because the heart can easily push blood into the pulmonary trunk. But as soon as the heart pressure decreases or stenosis increases, symptoms will gradually begin to appear.

Stenosis and its effect on heart function

After pulmonary stenosis has formed, the heart muscle begins to suffer. This is as follows:

the load on the right ventricle increases, because blood is pushed out of it through a narrow lumen;
intake of less blood in the lungs than normal. Therefore, only its small volume is saturated with oxygen, which provokes hypoxia;
stagnation of blood flowing through the veins to the heart. It develops due to increasing pressure in the right ventricle against the background of the residual volume not pumped into the pulmonary artery.

All these pathological mechanisms lead to the development of heart failure. In the absence of therapy, it is a deadly complication. However, adequate supportive therapy brings a fulfilling life. In addition, the lack of treatment leads to gradual wear and tear of the myocardium. the load that he has to overcome by pushing out blood is many times higher than the norm.

Types of pulmonary stenosis

Doctors have identified several varieties of this disease:

Supravalvular stenosis.
Subvalvular (infundibular) stenosis.
Valvular stenosis is the most common form that occurs in children and adults.
Combined.

Pulmonary valve stenosis in a child is the most common form of the disease, which is diagnosed in 90% of children with heart defects. In this case, the valve has an abnormal structure associated with a narrowing of the outflow tract of the pulmonary artery.

Supravalvular stenosis is most often an integral component of Noman or Williams syndrome. In this case, the obstruction to blood flow is located below the valve. Subvalvular stenosis is a condition in which there is a narrowing above the pulmonary valve. It is often combined with a ventricular septal defect.

Depending on the severity of the disease, pulmonary artery stenosis can be mild, moderate, or severe. The mild form can persist for a long time, it can be suspected by frequent respiratory infections of the child and developmental delay. If we talk about severe forms of pulmonary stenosis, then the first symptoms may appear in the first hours of birth.

The table below will show the differences between the different degrees of this disease.

Degree / distinguishing characteristic	Systological blood pressure of the right ventricle, mm Hg	Pressure gradient between the right ventricle and LA, mm Hg
1 moderate degree	60	20-30
2 moderate	60-100	30-80
3 pronounced degree	over 100	more than 80
4 decompensatory degree	below normal	Lack of contractile function

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Features of the disease

The size of the hole in pulmonary artery stenosis can be varied and directly affect the patient's condition.

With a hole size of one millimeter, the newborn requires urgent surgical intervention, otherwise it will be fatal.
But with the normal size of the hole, the defect can be detected after a couple of years of life, and even then by accident. After all, the characteristic manifestations are not very symptomatic: the pressure in the right section is lower than in the left, and tremors over the heart cannot be an accurate sign.

With stenosis of the pulmonary artery, the right ventricle of the heart works with constant overload. This always leads to a thickening of its wall and expansion of the internal cavity. Therefore, a child with suspicion of such a defect must be constantly monitored. Best of all, the development of the disease will show the level of pressure on the heart valve. When the difference between the pulmonary artery and the right ventricle is fifty millimeters of mercury, then surgery is necessary. This mostly occurs before the age of nine.

Therefore, in adulthood, the question of replacing the heart valve with a prosthesis may arise. But this is, rather, the consequences. Now let's look at the degree of pulmonary stenosis.

View of the heart with stenosis of the pulmonary artery

Stages of pulmonary artery stenosis

There are four stages of pulmonary artery stenosis:

moderate stenosis - stage I. The patient has no complaints, the ECG shows a slight overload of the right ventricle of the heart muscle. Systolic pressure up to sixty millimeters of mercury;
severe stenosis - stage II. It is characterized by a clear manifestation of symptoms. The systolic pressure of the heart muscle in the right ventricle is from sixty to one hundred millimeters of mercury;
sharp stenosis - stage III. Severe stage of the course of the disease, there are signs of circulatory disorders, pressure on the valve of the pulmonary artery and right ventricle is above one hundred millimeters of mercury;
decompensation - stage IV. Obvious signs of myocardial dystrophy, a very strong violation of blood flow. Contractile failure of the right ventricle develops, so the systolic pressure may be low.

By the level of blood flow, it is possible to distinguish valvular (the most common), subvalvular and supravalvular stenosis of the pulmonary artery in a newborn child. The combined form of the disease is also very rare.

With valvular stenosis, the leaflets of the valve grow together, and it has a domed shape with an opening in the middle.
The subvalvular degree of the disease looks like a funnel-shaped narrowing of the outflow tract of the right ventricle as a result of abnormal proliferation of fibrous and muscle tissue.
Supravalvular stenosis can be represented by an incomplete or complete membrane, localized narrowing, multiple peripheral pulmonary artery stenosis, diffuse hypoplasia.

Causes of occurrence

Pulmonary artery stenosis can be:

congenital. Poor heredity, rubella transmitted by the expectant mother during pregnancy, chemical and drug intoxication and many other factors;
acquired. As a result of various diseases, valve vegetation can develop and, as a result, stenosis. Sometimes it occurs as a result of compression of the pulmonary artery by enlarged lymph nodes or its sclerosis.

Pulmonary stenosis symptoms

The symptoms of pulmonary artery stenosis depend on its stage. With a systolic pressure of fifty to seventy millimeters of mercury, they are absent.

The most common symptoms are:

increased fatigue during exercise;
dyspnea;
auscultation (heart murmur);
dizziness;
weakness;
drowsiness;
pain in the region of the heart;
fainting;
angina pectoris;
swelling and pulsation of the neck veins;
heart hump.

Diagnostics

Diagnosis of pulmonary artery stenosis includes a combination of various instrumental studies and physical data.

In the second intercostal space, to the left of the sternum, a rough systolic murmur is heard. It is carried out towards the clavicle and is perfectly audible in the interscapular region. The second tone in the first and second stages of the disease is heard practically unchanged, but with severe stenosis it can disappear completely.
With a slight stenosis, the electrocardiogram does not show abnormalities. At all other stages of the disease, there are signs of hypertrophy of the right ventricle of the heart muscle. Supraventricular arrhythmias may occur.
Echocardiography shows dilatation of the right ventricle of the heart muscle and post-stenotic dilatation of the pulmonary artery. Doppler ultrasound allows you to identify and determine the pressure difference between the pulmonary trunk and the right ventricle.
X-ray studies show a depletion of the pulmonary pattern and post-stenotic expansion of the pulmonary artery trunk.

Therapeutic and medication methods

It is used to prepare for surgery or to alleviate the patient's condition at an inoperable IV stage.

Patient monitoring includes:

regular echocardiography;
prevention of infective endocarditis;
prophylactic antibiotic therapy;
identification of carriers of streptococcal infection with the rehabilitation of chronic foci.

Operation

The only effective treatment for this heart disease is surgery. Cardiac surgeons have been successfully performing it since 1948 and since then the methods have progressed significantly. It is desirable to operate on the patient at the second or third stage of the development of the disease. Patients with moderate stenosis are monitored but not prepared for surgery.

Eliminate the defect using several types of operations:

closed pulmonary valvuloplasty. It was previously used to correct many defects, but now it is most often used to treat pulmonary artery stenosis. The operation is performed through a left-sided anterolateral approach to the heart in the fourth intercostal space. With the help of a special tool, the valvulot, the fused walls of the valves are dissected, with the help of special holders bleeding is reduced. After dissecting the membrane, the hole is widened with a Fogarty probe or dilator;
pulmonary valvotomy. An operation that is performed using catheterization. The probe is inserted through the vein and equipped with special knives or a balloon;
open valvotomy. It is carried out by connecting artificial blood circulation and opening the chest to gain access to the heart. An incision is made in the lumen of the pulmonary trunk and through it the structure of the fused valves is studied. Then they are cut strictly according to the position of the commissures, from the median opening to the base of the valves. The valve opening and subvalvular space are monitored visually or with the help of a heart surgeon's finger. This method of eliminating the defect is considered the most effective.

The following video will tell you how the operation is performed for pulmonary stenosis:

Disease prevention

Prevention of intrauterine development of the defect includes limiting the influence of harmful factors on the expectant mother and ensuring the normal course of pregnancy. All patients with suspected pulmonary artery stenosis are required to be regularly monitored by a cardiologist and take measures to prevent infective endocarditis.

Complications

With stenosis of the pulmonary artery, myocardial dystrophy, frequent inflammatory diseases of the respiratory tract and an increased predisposition of patients to them may develop, septic endocarditis may appear. In severe cases:

stroke;
failure of the right ventricle;
myocardial infarction.

And finally, we will tell you about the average life expectancy and prognosis for pulmonary artery stenosis.

Forecast

In modern medicine, cardiologists prevent the development of the disease by performing an operation in early childhood. But if some parents refuse surgery for religious or other reasons, the likelihood of death increases several hundred times. If untreated, the patient dies within five years.

The main thing, remember, even if the doctor diagnosed the fetus with pulmonary artery stenosis, everything is fixable, you should not despair!

Another type of surgery for pulmonary valve stenosis will be described in the video below:

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Classification of stenosis

Pulmonary stenosis can be either isolated or in combination with other defects. Isolated stenosis of the pulmonary artery is often observed in congenital and acquired defects, when complex changes (Tetralogy of Fallot) are characteristic only of genetic mutations of the fetus.

At the site of localization of pulmonary artery stenosis, 4 forms are distinguished:

supravalvular stenosis - it has several types: the formation of an incomplete and complete membrane, localized narrowing, diffuse hypoplasia, multiple peripheral strictures of the pulmonary trunk;
valvular stenosis (the most common pathology) - in this case, the valve flaps are in a fused state, which forms a domed shape with incomplete closure in the middle;
subvalvular stenosis - at the outlet, the right ventricle has a funnel-shaped narrowing due to excessive proliferation of muscle tissue and fibrous fibers;
combined stenosis (changes in the walls are in several places and at different levels in relation to the valve).

Reasons for the development of stenosis

Combined and isolated pulmonary artery stenosis in congenital heart defects can occur due to various factors that have occurred during pregnancy:

teratogenic factors in the form of taking medications that affect the development of germ layers (first trimester): psychoactive drugs, antibacterial drugs, the use of narcotic drugs;
burdened family history: genetic predisposition in a related line;
viral infectious diseases of the mother throughout pregnancy: rubella, chickenpox, herpes type 1.2, infectious mononucleosis, active stage of viral hepatitis;
unfavorable working conditions and uncoordinated rest regime - inhalation of harmful dust, chemical poisonous agents;
the use of radiation methods of treatment during pregnancy - the pathological effect of increased radioactive radiation on the differentiation and development of the tissues of the child's body;
harmful environmental factors: increased radioactive background;
excessive intake of genetically modified foods.

Acquired PA stenosis is more related to organic pathology that develops in adulthood, and is characterized by a number of reasons:

inflammatory processes of the endocardium and intima of the pulmonary artery against the background of attack of cells by foreign organisms (more often streptococcal infection - as a late complication of angina);
valve calcification;
compensatory proliferation of myocardial cells with a decrease in the lumen of the vessel at the exit from the right sections;
nonspecific autoimmune lesions of the heart valves - the consequences of the fight of immunity with treponema pallidum (syphilis), tubercle bacillus, systemic lupus erythematosus, systemic scleroderma and dermatomyositis;
compression from the outside: compression of the vessel by a tumor of the mediastinum, enlarged regional lymph nodes, aortic dissecting aneurysm.

Symptoms of the disease

The identified symptoms and well-being usually directly depend on the degree of stricture of the vessel lumen and the stage of development of the disease. Therefore, it is advisable to consider the clinical picture in stages.

Moderate stenosis - pressure during systole in the right ventricle is less than 60 mm Hg. - with such a defect, the patient does not notice any changes in the state of health and leads a normal life.
Severe stenosis - if the systolic pressure in the right ventricle is within 60-100 mm Hg. A corresponding symptomatic picture begins to appear: shortness of breath with moderate physical exertion and at rest, headaches, periodic dizziness associated with subcompensated brain hypoxia, fatigue, fainting; in children - frequent respiratory diseases and lag in mental and physical development.
A pronounced stage of stenosis - the pressure during systole in the right ventricle is more than 100 mm Hg. The initial stages of myocardial dystrophy (compensatory hypertrophy and ventricular dilatation) are observed. Dyspnea develops at rest, the above symptoms are aggravated, swelling and pulsation of the cervical veins is observed (with the addition of tricuspid valve insufficiency), fainting may occur, a heart hump appears, a rough systolic murmur, pain in the heart region. Peripheral cyanosis is also characteristic - cyanosis of the perioral triangle, phalanges of the fingers.
Decompensated stage - dystrophic changes in the myocardium and severe heart failure develop. Due to the development of pathological processes, an oval window opens, and the discharge of blood from the right heart to the left begins. As a result, the hypoxic state of the body tissues worsens - general cyanosis is added to the progression of the above symptoms.

Diagnostic steps

If stenosis of the pulmonary trunk is a congenital pathology and makes itself felt immediately after birth, then a neonatologist will draw up a plan of diagnostic measures and treatment already in the maternity hospital.

In case of a later manifestation of CHD, you should contact your local pediatrician, who will diagnose and begin treatment.

If a compensated congenital defect made itself felt only in adulthood or acquired stenosis was formed, then you should contact your family doctor, therapist or cardiologist for the correct diagnosis.

To confirm the pathological process, establish the stage and neglect of the process, in order to select the most effective methods of treatment in the future, the supervising doctor will be helped by:

chest x-ray;
Echocardiography;
catheterization of the right ventricle through the peripheral veins to determine the systolic pressure;
angiography of the vessels of the heart;
ventriculography.

Healing activities

The only effective method of dealing with pulmonary artery stenosis is surgery. Conservative therapy is symptomatic and is acceptable only with moderate stenosis, when the patient, in addition to the established valve pathology, is no longer worried about anything. It is also indicated for inoperable cases and refusal of the parents or adult patient from surgical treatment for various reasons.

Surgical treatment is carried out using various types of surgical interventions.

Closed pulmonary valvuloplasty. Surgical manipulations are performed using a left-sided anterolateral approach to the heart at the level of the fourth intercostal space. After getting to the valve, the fused valve leaflets are dissected with a special tool valvulotome, after which the bleeding is stopped on special holders. Then, as the bleeding was stopped and the fused valve was dissected, the resulting opening is further enlarged with a Fogarty probe or dilator. After the performed procedure, the operative access is sutured tightly.
Pulmonary valvotomy. The procedure is performed with a special probe, which is equipped with a dissection scalpel and a balloon for opening the opening. Such a probe is inserted through the venous subclavian access. This procedure is the least invasive.
Open valvotomy. This type of surgery is the most effective, as it is performed on an open heart. The patient is connected to a heart-lung machine, the chest is opened, the base of the pulmonary trunk is dissected. The affected areas are visualized, which are further excised. With the help of a scalpel, the surgeon, under the control of tactile sensation with a finger strictly along the commissures, dissects the valve leaflets from apex to base.
For subvalvular stenosis, open heart surgery is also performed. Only instead of commissurotomy, hypertrophied or stenotic areas of the pulmonary artery orifice are excised.
Supravalvular stricture requires surgical intervention using a patch. Find the affected areas, excise them. And for physiological recovery, instead of the affected excised vascular wall, a part of the pericardial bag is applied.

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The cause of stenosis of the pulmonary artery, in 50% of all cases of cardiac pathologies, is called congenital anomalies of the body.

Pathology can form even in the perinatal period if:

There is a genetic predisposition to diseases of the cardiovascular system;
The expectant mother, while carrying a fetus, used psychotropic substances, strong tranquilizers, drugs, antibiotic drugs. Such factors attract special attention if these funds were taken in the first trimester of the term;
Constant or frequent finding of the expectant mother under the influence of toxic substances (fumes) - this can be the production of chemical fertilizers, contact with building odor mixtures, etc.
Acute viral or infectious diseases transferred by a pregnant woman, including hepatitis and herpes;
Frequent (regular) exposure to X-ray machines, or living near powerful locating devices, places with a heavy radioactive background.

As a rule, this is a consequence of a history of diseases:

Atherosclerotic vascular lesions (aorta);
Changes in the configuration of the heart valves due to rheumatic manifestations;
Complex diseases that contribute to irreversible changes in the tissues of the pulmonary artery: tuberculosis, syphilis;
Tumor formation pressing on the vessel from the outside and significantly narrowing the lumen;
Inflammation of the lymph nodes, also compressing the artery from the outside.

Symptoms of pulmonary artery stenosis in children may appear immediately after the baby is born, or, with insufficiently expressed pathology, they may not identify themselves for a long time. Sometimes, until the child enters adulthood. However, in general, the disease makes itself felt immediately, or during the first months of life.

You can find out pulmonary artery stenosis in newborns by focusing on the following signs:

A pronounced nasolabial triangle of a changed, cyanotic color. The baby's fingertips, feet, palms will be of the same shade. Changes in the color of the entire body of the child are also possible;
While eating, the baby begins to choke, throw the chest. This symptom can also appear in his relaxed state;
The infant behaves apathetically, does not show interest in anything, or, conversely, is anxiously worried;
Up to six months of life, the baby almost does not gain weight, it significantly lags behind peers in the tables of weight and height norms;
Pulmonary artery stenosis in newborns is, first of all, the baby's shortness of breath and, on the basis of this factor, all other concerns.

In adults

Pulmonary artery stenosis in adults coincides with childhood symptoms only in one point - it can also be in a "dormant" state for a fairly long period, without tormenting the body with an excessive deficiency of nutrients. A person can live a long life and not even suspect that something in his structure differs from the general norms.

However, if the acquired defect begins to manifest itself clearly, the development of the disease does not stop until it can be stopped by cardinal methods of treatment.

Symptoms of pulmonary artery stenosis in adults are caused by:

Loss of performance, dizziness and nausea;
Shortness of breath, acute lack of air with little physical effort, but, during the development of the disease and during periods of relaxation;
Swelling of the legs at the very primary stage of heart failure.
The next step is the accumulation of fluid in the abdominal and chest cavities.

Stenosis, which does not manifest itself in any way and is detected only by the results of a preventive examination, does not require any therapeutic measures. The body has already fully adapted to the level of supply of oxygen and nutrients that can supply blood flow and has regulated normal life activity on this basis.

However, in the case of vivid symptoms, the only real measure to eliminate disturbances in the body is required - this is surgery.

Treatment of pulmonary artery stenosis involves several surgical techniques:

Supravalvular stenosis... Using a tissue section of the outer cardiac membrane, the operated, excised section of the artery is "darned";
Valvular stenosis... Most often, the method of minimal trauma is used, by correcting the pulmonary artery valve using a special balloon stretched through the vessels.

Also, the diagnosis of "heart failure", with minimal complications, lends itself well to correction with medications, but their appointment is strictly individual and should not come from independent sources. Only an experienced cardiologist can prescribe treatment and set limits.

How dangerous

Even if the baby has a stenosis of the pulmonary artery and its congenital nature is confirmed, one should not panic and look for a huge number of negative factors in the growing child that should distinguish him from his peers. Most likely, the disease will not manifest itself in the development of the child, however, it is possible and necessary to follow the development of the disease itself, because how dangerous stenosis of the pulmonary artery in each case is, is determined with age.

The only thing that distinguishes children with a congenital malformation of this nature is a weakened immune system and an almost instant reaction to various respiratory viral infections. This imposes certain prohibitions on the educational process - such children should not be overstrained with studies and it is categorically not recommended to be sent to any professional sports.

A child who is born with increased stenosis, in almost 80% of cases, immediately enters the operating table. The remaining 20%, with the appropriate decision of the doctor, are under strict medical supervision and surgical intervention in this case occurs as needed.

Forecast

The prognosis for stenosis of the pulmonary artery is regulated by a number of possible complications in the development of the disease.

If stenosis of the pulmonary artery does not manifest itself for a long time, the prognosis is usually optimistic. Treatment is not required and the supervision of doctors is enough.

It is impossible to say the same about people who have to undergo a complex operation. The survival rate among them is quite high - about 90% of those operated on, however, the quality of life itself is significantly deteriorated, due to the huge number of restrictions and imposed prohibitions.

Aortic aneurysm

Isolated peripheral pulmonary artery stenosis was first described by Mangars and Schwalbe. With the introduction of cardiac catheterization, especially angiography, the number of publications about this anomaly has increased dramatically. In two thirds of cases, it is combined with other vices. Peripheral pulmonary stenosis is associated with Noonan syndrome, Alagille syndrome, cutis laxa, and Ehlers-Danlos syndrome.

Embryology

The pulmonary artery and its branches develop from three separate vascular components. The proximal part of the pulmonary trunk just above the semilunar valve develops from the bulb of the heart. The rest of the pulmonary trunk comes from the common arterial trunk. The proximal segments of the right and left branches of the pulmonary artery develop from the VI branchial arch on each side. The distal part of the right VI arch completely disappears, while the left arch persists as a ductus arteriosus, subsequently obliterating with the formation of the arterial ligament. The peripheral parts of the branches of the pulmonary artery originate from the "postgill pulmonary vascular plexus", which is closely related to the growing rudiments of the lungs.

The pathogenesis of peripheral pulmonary stenosis is unknown. It is obvious that many factors and types of pathological changes can lead to one result - narrowing of the lumen of the branches of the pulmonary artery. The high frequency of concomitant intracardiac anomalies indicates that the pathogenesis of these lesions is associated with a violation of developmental mechanisms. Any teratogenic effect on the primary rudiments of the pulmonary arteries can lead to atresia, hypoplasia, or stenosis. On the example of congenital rubella syndrome, it can be seen that the effect of the virus on the formation of elastic tissue may be the principal mechanism for the development of this pathology.

Anatomy and classification

Various variants of narrowing of the pulmonary arteries were classified by Gay and co-authors in 1963, who divided the whole variety of stenoses into 4 types. In type I stenosis, there is a local narrowing of the pulmonary trunk, right or left branch of various lengths. Stenosis can be in the form of a diaphragm within a vessel or an elongated decrease in the outer size of an artery. Type II is represented by bifurcation hypoplasia involving the distal part of the pulmonary trunk and the orifices of both branches. The length of the constriction ranges from a short local stenosis to a long segment of obstruction. Type III is characterized by multiple narrowing of the orifices of segmental pulmonary arteries with post-stenotic dilatation. The pulmonary trunk and central branches are not changed. In type IV, the central and peripheral pulmonary arteries are narrowed. The pulmonary trunk is usually not dilated, even with a sharp narrowing of its distal part, bifurcation and branches. Only occasionally does a mild pretenotic dilatation occur, which never reaches the degree seen in pulmonary hypertension.

Occurrence

Peripheral stenoses of the pulmonary artery can be isolated, as a component of the somatic syndrome, can be an element of various CHD, or acquired. They are often the most vulnerable part of complex congenital heart anomalies. Isolated stenoses are observed in 40% of cases. In CHD, peripheral stenosis of the pulmonary arteries occurs with a frequency of 2-3%. In particular, they can accompany:

valvular stenosis of the pulmonary artery;

fallot's tetrad;

Peripheral stenosis of the pulmonary artery in congenital somatic syndromes

Peripheral stenosis of the pulmonary artery is quite common in various congenital somatic syndromes, being one of the expected signs. The most typical are Williams-Beuren and Alagille syndromes, as well as CATCH-22, Di George, Keutel, Noonan syndromes, congenital total lipodystrophy, congenital rubella, Ehlers-Danlos syndrome, cutis laxa.

Right heart obstructive anomalies are often associated with syndromes such as Di George syndrome, soft palate-heart-face syndrome, and conotruncalface anomaly syndrome. The combination of signs of dysmorphism, anomaly of the soft palate, hypoplasia of the thymus, parathyroid gland and heart disease is called Di George syndrome. Velocardiofacial syndrome and conotruncal face anomaly syndrome have similar features to Di George syndrome and are grouped as CATCH-22 syndromes. Among the defects of conotruncus, the most common is the common arterial trunk. The defects included in the general syndrome include a break in the aortic arch type B and the right-sided aortic arch, tetralogy of Fallot, the separation of both major vessels from the right ventricle, transposition of large vessels and the absence of a pulmonary valve. Obstructive changes in the pulmonary vessels can be combined with any of the listed heart defects.

Williams-Beuren syndrome was first described by Williams and Beuren as a combination of supravalvular aortic stenosis, narrowing of the pulmonary arteries, a characteristic appearance, and mental and physical retardation. Vascular defects are a consequence of a deficiency of the elastin gene and a violation of gene production of troelastin, which is involved in the formation of the architecture of vascular elastic fibers.

Narrowing of the pulmonary artery in this syndrome occurs in 39-83% of patients. They can be local or diffuse, involving central and / or peripheral branches, including multiple bilateral lobar and segmental arteries in their mouths.

Alagille syndrome is a genetic disorder in which autosomal dominant traits with variable expression are inherited. The phenotype consists of 5 main features, including intrahepatic cholestasis, diffuse hypoplasia of the pulmonary arteries, ocular pathology, congenital absence or underdevelopment of half of the vertebrae, and characteristic appearance. Pulmonary artery stenosis occurs with this syndrome in 70-85% of cases. The lesions of the pulmonary arteries in Alagille syndrome are usually bilateral and extend to the periphery to segmental branches, may be diffuse or local. Weak skin syndrome is a pronounced hereditary disease characterized by a deficiency of elastic skin fibers in combination with pulmonary emphysema, intestinal diverticula, hernias and narrowing of the peripheral pulmonary arteries.

Congenital rubella syndrome

The teratogenic effect of rubella virus in the first trimester of pregnancy was first described in 1941. The most common manifestation of congenital rubella is a combination of congenital rubella, cataract and deafness. After the successful cultivation of the rubella virus in 1962 and extensive research conducted during the rubella epidemic in the United States in 1964, information was obtained on the natural course of the infectious process, pathogenesis, epidemiology and preventive measures. It was found that in addition to the above triad of congenital anomalies, the clinical picture contains microphthalmia, retinopathy, thrombocytopenic purpura, hepatosplenomegaly, low birth weight, bone pathology and other, more rare, pathology.

It has been established that the rubella virus enters through the uterus and persists throughout pregnancy and childbirth. The virus can be isolated from fetal tissue obtained through therapeutic abortion weeks or months after the mother is infected. After birth, the virus can be isolated from urine, cerebrospinal fluid, and other tissues and fluids. The virus content gradually decreases after birth, but this process slows down with age. In infants, the rubella antibody titer is comparable to that of the mother and persists for many years. Persistence of asymptomatic congenital rubella in children who appear normal during the first months of life can lead to the development of mild psychotropic disorders. These infants are contagious and should be isolated to prevent the spread of infection to susceptible women in the early stages of pregnancy.

The use of live attenuated rubella virus vaccine effectively reduces the incidence of congenital rubella, but the problem of eradicating this infection in the general population remains unresolved.

The list of cardiovascular pathology associated with rubella syndrome is extensive. Early reports indicated that the most common defect was PDA. The involvement of maternal rubella as a possible cause of pulmonary stenosis was first reported by Arvidsson et al. In 1963, Rowe drew attention to the frequent occurrence of pulmonary stenosis in children of mothers who contracted rubella during an epidemic in New Zealand. Other authors have confirmed this observation. Stenoses of the pulmonary arteries were localized in all segments of the pulmonary arterial basin. Lesions of the pulmonary arterial vessels were isolated or combined with PDA, pulmonary valvular stenosis, ASD. Isolated peripheral pulmonary arterial stenosis in rubella occurred with the same frequency as PDA. Rubella syndrome is also associated with:

valvular or supravalvular aortic stenosis;

fallot's tetrad;

coarctation of the aorta;

Generalized lesions of systemic arteries - aorta, coronary, cerebral, mesenteric and renal - and diffuse hypoplasia of the abdominal aorta are described. Histological changes in the aorta and large vessels are manifested by focal thickening of the intima, loss of fibroelastic tissue, fragmentation of elastic fibers, and vacuolization of the media. In small vessels, the internal elastic plate is also involved in the pathological process.

The clinical picture in newborns and infants with cardiovascular pathology with rubella syndrome depends on the severity of the defects and concomitant anomalies of other organs and systems. Signs of heart failure appear early. The prognosis depends on the type of defect and its severity. Moderate peripheral narrowing does not progress and even disappears with age. Severe stenosis tends to worsen.

Pulmonary artery stenosis in newborns

In newborns, physiological narrowing of the pulmonary artery is often observed, due to the relative hypoplasia of its branches. In most cases, it decreases with the growth of the child and disappears by 6 months of age. Narrowing is clinically manifested by a transient systolic murmur. Echocardiographic studies have shown that in the presence of noise, the dimensions of the pulmonary trunk and branches are smaller than in children without noise, and on average are 60% of the expected norm. Doppler echocardiography reveals the turbulent nature of the current and a significantly higher blood flow velocity in the branches. By the age of 3 months in 70% of children, noise disappears, the size of branches increases and the blood flow rate decreases.

Left pulmonary artery stenosis and ductus arteriosus

Closure of the ductus arteriosus in a healthy child may develop narrowing of the left pulmonary artery, but especially often in patients with valvular stenosis or pulmonary atresia. The mechanisms of development of narrowing of the left pulmonary artery and coarctation of the aorta are identical and are associated with the botallus duct, therefore the term “coarctation of the pulmonary artery” would correspond to the essence of this anomaly. Postmortem histological studies of pulmonary artery stenosis revealed duct tissue in the wall of the pulmonary artery from the side of the duct. A case of local narrowing of both branches of the pulmonary artery at the confluence of the bilateral botallic duct is described. The role of the Botallic duct in the mechanism of narrowing of the left branch is shown in Doppler studies of premature infants before and after closure of the duct. Every third child developed significant transient stenosis of the left pulmonary artery. No pressure gradient was detected in the right pulmonary artery. Blood flow asymmetry and transitional changes from fetal to adulthood were also observed in the right and left pulmonary vascular basins.

Taking into account the proven fact of the role of the duct in the development of coarctation of the pulmonary artery, complete excision of the duct tissue from the pulmonary artery wall is recommended to prevent unilateral hypoplasia of the pulmonary arterial tree.

Acquired narrowing of the pulmonary arteries

Acquired narrowing of the pulmonary arteries is observed after palliative and reconstructive surgery:

systemic-pulmonary anastomoses;

narrowing of the pulmonary artery;

unifocalization with pulmonary atresia with VSD;

arterial switching operations during transposition of the great arteries and the divergence of both large vessels from the right ventricle with subpulmonary VSD;

with staged single ventricular correction.

Narrowing of the arteries occasionally occurs with fibrosing mediastinitis or with mediastinal tumors due to external compression.

Modified Blalock-Taussig anastomosis is often complicated by hypoplasia, discrete stenosis, or kinked pulmonary artery. Angiographic studies preceding the complete correction of Fallot's tetrad showed that in one third of patients the distal parts of the right pulmonary artery are less developed than in patients who did not undergo palliative intervention. Particularly pronounced deformities and hypoplasia are observed in children after anastomoses performed at neonatal age. They require additional catheter or surgical interventions.

After arterial switching operations, due to the application of the Le Compte maneuver and the resulting tension and flattening, the pulmonary trunk has an oval shape, its cross-section is less than normal, and the branches of the pulmonary artery are underdeveloped and narrowed in every fourth patient.

Hemodynamics

Hemodynamics in peripheral stenosis of the pulmonary artery is basically identical to that in isolated valve stenosis. In accordance with the severity of the obstruction, the systolic pressure in the right ventricle and areas of the pulmonary artery proximal to the stenosis is increased. The capacity of the pulmonary arterial bed is reduced proximal to the narrowing. With severe obstruction, the duration of ejection from the right ventricle is lengthened and the pulmonary trunk proximal to the narrowing behaves like a continuation of the excretory tract of the right ventricle. The pulmonary artery pressure is peripheral to the constriction of the right ventricle and the pulmonary valve remains open as long as there is a systolic pressure gradient between the right ventricle and the distal pulmonary artery. This explains the later closure of the pulmonary valve, despite the very high systolic pressure in the pulmonary vascular bed. The pressure curve proximal to the stenosis resembles its shape in the right ventricle with high systolic and low diastolic pressure. In severe multiple constrictions of the peripheral pulmonary arteries, including small branches, the pulmonary valve closes early, approximately at the same time as the aortic valve closes.

Clinic

Patients with mild to moderate bilateral or unilateral stenosis are usually asymptomatic. Shortness of breath on exertion, fatigue, and signs of congestive heart failure are noted only with severe obstruction. Subtle differences in the auscultatory picture make it possible to differentiate valvular stenosis from peripheral stenosis. The first tone is usually normal with no burst click. The second tone is usually split and the intensity of the pulmonary component is normal or slightly increased. You can catch a distinct difference between peripheral and valvular stenosis. The width of the cleft depends on the severity of the constriction, as in valvular stenosis, and varies with breathing phases, except in cases of very severe obstruction. There is a systolic ejection murmur along the upper left edge of the sternum, which is carried out under the arm and posteriorly, but not to the neck. In patients with multiple peripheral stenosis, the II tone over the pulmonary artery is so loud that pulmonary hypertension can be suspected. However, unlike pulmonary hypertension, with multiple peripheral constrictions, soft blowing systolic or continuous murmurs can be heard over both lungs and on the back - a clear difference from pulmonary hypertension, in which there is no murmur or there is a very short systolic ejection murmur along the upper left edge of the sternum. A continuous murmur is sometimes heard with stenosis of the central or distal branches, especially with increased pulmonary blood flow.

Electrocardiography

The ECG does not differ from that of valvular stenosis. A high frequency of deviation of the electrical axis to the left is observed in patients with congenital rubella syndrome. This is due to myocardial damage by the rubella virus. Left axis deviation is also seen in many patients with subvalvular pulmonary stenosis and Noonan syndrome.

X-ray examinations

In patients with mild or moderate stenosis, the size of the heart shadow and pulmonary pattern are normal. Unlike valvular stenosis, the pulmonary arch does not bulge. The pulmonary vascular pattern is normal on both sides, even in cases of unilateral pulmonary artery narrowing. Only with severe unilateral stenosis and increased pulmonary blood flow is there a difference in the severity of the vascular pattern of both pulmonary fields. With bilateral severe narrowing of the pulmonary arteries, the size of the cardiac shadow is increased due to the right ventricle and atrium.

Echocardiography

On echocardiography, hypertrophy of the anterior wall of the right ventricle can be detected and increased pressure in its cavity can be assumed. Two-dimensional echocardiography allows you to see the pulmonary trunk and proximal branches, as well as the cavity of the right ventricle and the pulmonary valve. The bifurcation and initial sections of the branches are available for examination, in contrast to the distal ones. Doppler echocardiography measures the pressure gradient at the constrictions located in the pulmonary trunk and its branches.

Heart probing

Cardiac catheterization is a critical method for confirming the clinical diagnosis of peripheral pulmonary stenosis. Removing the catheter from the distal branches allows you to measure the systolic pressure gradient across the narrowed segment of the artery. Small values \u200b\u200bof the gradient may turn out to be unreliable, since they may be the result of a discrepancy between the sizes of the vessel and the catheter. In infants, and especially premature infants, there may be a small to moderate systolic gradient between the branches of the pulmonary artery and the pulmonary trunk. This normal physiological phenomenon disappears as the child grows. It is caused by the disparity between the lumen of the pulmonary trunk and the branches of the pulmonary artery. The gradient of systolic pressure is more than 10 mm / Hg. Art. considered abnormal in the absence of increased pulmonary blood flow. With unilateral narrowing, a pressure gradient is found in the corresponding segment, and the pressure in the proximal region is within the normal range. However, during exercise or in the presence of a left-to-right shunt, a gradient can occur with a simultaneous increase in pressure in the proximal pulmonary artery.

In peripheral stenosis, pressure distal to the constriction is usually low, with a slow rise and fall and a wide wave. The pressure curve in the segment proximal to the constriction has a characteristic shape that is important for diagnosis.

The shape of the curve is identical to that in the right ventricle in height and time to the dicrotic wave. The dicrotic wave is low, followed by a low diastolic pressure equal in magnitude to the diastolic pressure distal to the obstruction. The width of the pulse wave in the proximal pulmonary artery increases according to the severity of the obstruction.

The nature of the pressure curve proximal to the stenosis is determined by the dysfunction of the pulmonary trunk, the wall of which is very thickened and fibrosed, with limited elasticity. As emphasized above, the pretenotic part of the pulmonary trunk becomes an extension of the outflow tract of the right ventricle, and since its function is determined by the degree of obstruction, pressure in the pulmonary artery reflects changes in the right ventricle rather than in the distal pulmonary circulation. Since the pressure in the distal pulmonary artery is lower than in the ventricle, the pulmonary valve remains open. Closing of the valves occurs during the early phase of isometric ventricular relaxation. As a result, there is a sharp increase in the capacity of the pulmonary trunk with a corresponding drop in pressure and the formation of a dicrotic wave. The slow decrease in diastolic pressure reflects the slow leakage of blood from the pulmonary trunk into the distal branches. The slowdown of diastolic blood outflow occurs due to obstruction and impaired elastic recoil of the thickened fibrosed wall of the pulmonary trunk.

Peripheral pulmonary stenosis is often associated with valvular narrowing. If the pressure in the pulmonary trunk is moderately elevated, this means that peripheral constriction is dominant. If valvular stenosis is predominant, right ventricular pressure is elevated with normal or slightly elevated pulmonary pressure. The severity of peripheral constriction is difficult to assess with supravalvular stenosis, since the severity of the valve narrowing is masked. With a combination of contractions at different levels, angiography is the decisive method for assessing the severity of supravalvular stenosis.

Angiocardiography

Selective angiocardiography is the most valuable method for diagnosing peripheral pulmonary artery narrowing. It allows you to see the exact location, extent and distribution of lesions. In severe unilateral obstruction, delayed filling of the corresponding pulmonary veins with contrast medium may be observed.

Differential diagnosis

Isolated stenosis of the peripheral pulmonary arteries may be suspected by a characteristic systolic murmur that is widely conducted into the armpit and back, no ejection click, a widely split II tone with normal respiratory changes, and normal or slightly increased pulmonary component intensity. ECG and Echocardiography are helpful in assessing the severity of the obstruction.

Since peripheral stenosis of the pulmonary artery is often accompanied by other intracardiac and extracardiac abnormalities, the diagnosis of the dominant pathology can be established from the clinical picture.

A history of maternal rubella, familial congenital heart disease, persistent neonatal jaundice, facial features, and other features of Williams or Noonan syndromes suggest that peripheral arterial stenosis may be the cause of systolic murmur.

Course and forecast

Routine endocarditis prophylaxis is recommended for patients with peripheral pulmonary stenosis. In the natural course, the prognosis depends on the severity of the obstruction and is basically the same as in valve stenosis. However, in multiple severe peripheral stenoses, it is comparable to primary pulmonary hypertension when dilatation and stenting are ineffective. Progression of obstruction is possible. On the other hand, in many cases, the pressure gradient recorded in infancy may disappear with age. Post-stenotic aneurysmal dilatation of small elastic arteries is complicated by arteritis, thrombosis, or pulmonary hemorrhage. Severe peripheral stenosis can lead to death, both in early infancy and later life. Severe peripheral stenosis is very rare in adults.

In Williams-Beuren syndrome, in most cases, the caliber of the vessels increases over time, while the pressure in the right ventricle decreases. Positive dynamics is observed even with severe stenosis and pressure in the right ventricle equal to the systemic one. The most dramatic improvement occurs in cases of extremely high baseline right ventricular pressures, with striking improvements in pulmonary angiography. The decrease in pressure in the right ventricle is due to an increase in systolic extensibility, and not a true increase in the lumen of the arteries.

Alagille's syndrome is characterized by multiple organ pathology. The life expectancy of these patients depends on the presence of congenital heart disease, in which the survival rate by the 20th year is 40%, in the absence of a defect - 80%. The ten-year survival rate of patients with concomitant tetralogy of Fallot or with pulmonary atresia and VSD is 66% and 25%, respectively, in the absence of Alagille syndrome - 89% and 58%.

Treatment

Moderate isolated unilateral or bilateral stenosis does not require treatment. However, pronounced peripheral stenosis may necessitate elimination using one of the known methods. Until 1981, surgical removal of obstruction was used in a limited contingent of patients with accessible narrowing of the pulmonary trunk and central branches. However, the results were not unequivocally satisfactory in the presence of multiple distal constrictions that were unavoidable at that time.

Balloon angioplasty.

In 1980, Martin et al described the first attempt at percutaneous intravascular angioplasty for peripheral pulmonary stenosis. Then Lock and co-authors in an experiment on newborn lambs used transvenous angioplasty for artificially created stenosis of the branches of the pulmonary artery. The authors were able to expand the constrictions with the improved Gruntzig dilatation balloon. Histological studies in 4 patients showed that the expansion occurred due to rupture of the intima and stretching of the medial layer of the pulmonary artery wall. Complete healing of the intima occurred by the 2nd month after dilatation. When re-examining after 4-14 months. after dilatation, the achieved lumen diameter was preserved. Histological studies showed that the rupture of the intima and media was filled with scar tissue. In one of the arteries, the dilated segment distal to the residual narrowing was with signs of pronounced intimal proliferation. Histological examinations of the vessels that failed to expand were filled with reactive fibrous tissue. These studies stimulated the widespread clinical use of balloon angioplasty of stenosis of the peripheral pulmonary vessels.

The following technique is commonly used. The balloon catheter is placed in the area of \u200b\u200bstenosis. Use a balloon 2-4 cm long and 6-20 mm in diameter. After angiography, a suitable size is selected. It should be 3-4 times thicker than the tapered segment. The balloon is inflated under low pressure with a contrast solution diluted in half. The "waist" of the balloon should be centered. Then, under constant fluoroscopic monitoring, the balloon is inflated under high pressure for 10-60 seconds, until the waist disappears, and then rapidly deflated. Follow-up angiography is performed after dilatation. The effectiveness of dilation is confirmed by an increase in the diameter of the narrowed portion of the artery and a decrease in the systolic gradient between the proximal and distal portions of the pulmonary artery. The reasons for the failure are inadequate technique and physical properties of the constriction. Vessels resistant to dilatation are more common in children over 2 years of age and in patients with discrete stenosis. As a rule, narrowing in the area of \u200b\u200bthe systemic-pulmonary anastomosis and narrowing associated with the operation of arterial switch in complete TMA are not amenable to dilatation. Cases have been described when a balloon catheter could not be passed through a stenotic portion of an artery during catheterization, but intraoperative balloon angioplasty was successful.

Significant complications of percutaneous balloon dilatation of stenosis of the peripheral pulmonary arteries are:

bleeding from a ruptured pulmonary artery;

hemoptysis;

transient pulmonary edema;

thrombosis of the iliac vein;

pulmonary aneurysm;

transient arrhythmias;

cyanosis and hypotension;

death from cardiac arrest, paradoxical embolism, and low cardiac output.

To stop fatal bleeding from the injured pulmonary artery, occlusive springs are successfully used.

Intravascular stenting.

Balloon expandable stents were developed by Palmaz et al. Mullins et al., Benson et al., Rocchini et al. Demonstrated the technical feasibility of installing stents in normal and narrowed vessels on an experimental model of pulmonary artery stenosis. Based on these experimental studies, the method of placing balloon-expandable intravascular stents has been introduced into clinical practice.

The stent placement technique is well established. First, a guidewire with a diameter of 10-12 Fr is inserted distal to the narrowing site. Dimensions of the stent in the initial unfolded state: diameter 3.7 mm, length 3 mm, thickness 0.076 mm. A stent attached to a balloon angiographic catheter is passed into the distal pulmonary artery. After angioplasty, the catheter is placed over the stenosis, and the guidewire is pulled into the pulmonary trunk or right ventricle. Then the balloon is inflated under a pressure of 6-14 atm. After placement of the stent, the catheter with which the angioplasty was performed is replaced with an angiographic one. Control angiography is performed and hemodynamic parameters are changed. Positive results of intravascular stenting in patients with dilatation-resistant narrowing of the branches of the pulmonary artery improve the prognosis for this pathology.

Narrowing of the pulmonary artery and single ventricular correction according to Fontan.

The normal size of the pulmonary arteries is essential for a physiologically successful Fontan operation. Curvature, hypoplasia, or narrowing of the pulmonary arteries cause postoperative venous hypertension and low cardiac output. The pathology of the pulmonary arteries in these patients can be of congenital origin and acquired.

Correction of these narrowings is performed by surgery or percutaneous balloon dilatation and stenting.

A quantitative assessment of the adequacy of the pulmonary arteries was proposed by Mc Goon and Nakata. The most widely used index is Nakata - the total section of both pulmonary arteries in mm 2, referred to the body surface. This indicator was studied by the author in 40 healthy people, 46 patients with tetralogy of Fallot, 26 patients after Rastelli surgery and 15 patients after Fontan surgery. The sizes of the pulmonary arteries were measured by angiograms. In healthy individuals, the Nakata index is 330 + 30 mm 2 / m2 of body surface and does not depend on the size of the body surface in all age groups, from infants to adults. After surgery, the frequency of small cardiac output syndrome was the higher, the lower the index value, especially if it was< 150 мм 2 /м 2 . Зависимость летальности от величины индекса особенно проявилась в группе пациентов после операции Fontan. Двое больных с индексом < 250 мм 2 /м 2 умерли, в то время как 12 из 13 больных с индексом > 250 survived. Thus, Fontan operation is indicated for patients with a Nakata index\u003e 250 mm 2 / m 2.

Balloon dilation results

Peripheral stenosis of the pulmonary artery remains an unresolved problem. Surgical methods of correction, as shown by the early experience of interventions, are technically difficult, ineffective and can lead to deformation of the vessels.

Balloon dilation gives an incomplete effect and can be considered only as a palliative intervention, which alleviates the condition in half of the patients. Some authors cite data on an increase in the diameter of narrowed arteries by more than 75%, a decrease in peak systemic pressure by 50% and an increase in lung perfusion by 40%.

The Registry of Congenital Anomalies Treated by Valvuloplasty and Angioplasty presented the results of 182 balloon angioplasty procedures in 156 patients from 27 institutions. The diameter of the vessel at the site of constriction increased on average from 5 to 7 mm, the peak of systolic pressure decreased on average from 49 to 26 mm Hg. Art., the pressure in the proximal segment decreased from 69 to 63 mm Hg. Art. The use of high pressure balloons increased the rate of successful dilation from 50% to 81%. Complications occurred in 13% of patients. The same conclusion was made by Gentles et al., Who performed angioplasty in 52 patients with tetralogy of Fallot, pulmonary atresia with VSD, single ventricular heart, and isolated peripheral pulmonary artery stenosis. Dilation by high pressure increased the diameter of the vessel by more than 2 times, the pressure in the right ventricle decreased by an average of 20%. Thus, balloon angioplasty of the pulmonary arteries provides a significant direct hemodynamic effect in cases when surgical treatment is unsuccessful.

Many researchers were unable to predict the determinants of success. In the long-term period, restenoses have been described, but information about their frequency, nature and timing of occurrence has been insufficiently studied. Bush et al reported restenosis of successfully dilated arteries in 35% of patients, Hosking et al in 17%.

Results of pulmonary stenting

Stenting of the pulmonary arteries provides a more effective primary dilation and the possibility of re-dilatation, the need for which arises with an increase in body weight and intimal proliferation. Stenting allows for multiple repeated dilations, thus avoiding reoperation for various defects. Palmatz expandable balloon stents are introduced not only percutaneously, but also during operations for intracardiac defects in cases where vascular access is limited. Stenting is used for various pathologies - peripheral stenosis of the pulmonary arteries, after Fontan operation, with narrowing of the pulmonary veins, conduits.

Stent implantation is safer than dilatation and is accompanied by fewer complications, in particular, as a rule, there are no vascular ruptures, bleeding and embolism.

Ing et al. Reported a low rate of restenosis at 3 years after implantation and a high efficiency of repeated dilations in 94% of cases, without complications. Long-term results based on extensive data reported by McMahon et al. 664 stents were implanted in 330 patients after correction of Fallot's tetrad, pulmonary atresia with VSD, after arterial switch operation during transposition and Fontan operation. The mean gradient of systolic pressure after stenting decreased from 41 to 9 mm Hg. Art., the average diameter of the arteries increased from 5 to 11 mm. On average, after 5 years, the average pressure gradient was 20 mm Hg. Art., the ratio of RV / LV pressure is 0.5 and the average diameter of the lumen is 9 mm. Experts attribute the improvement in results to the use of a conservative series of dilations with the exclusion of excessive expansion, the use of shorter stents, an improved balloon shape and expansion from the beginning of the central part of the stent.

In infants and young children, this method is limited due to lack of stent flexibility, vascular narrowing, and the development of fixed obstruction following small diameter stent placement in growing patients. Small stents with a maximum achievable diameter of 9-10 mm in the future expose the patient to the need for surgery in order to expand the stented area. Despite this, implantation of such stents is viable in the immediate postoperative period. Larger stents can be implanted in the operating room to shorten the time for pulmonary arterioplasty and prevent external compression. Larger stents, which can be dilated to adult sizes, simplify the correction technique in difficult cases.

Syndrome treatment results

The high likelihood of reversibility of pulmonary arteriopathy in Williams-Beuren syndrome requires a critical attitude to the determination of indications for surgical or catheter intervention. Currently, the indications for such interventions are systemic or suprasystemic pressure in the right ventricle, severe biventricular obstruction, or the presence of symptoms with less pronounced obstruction. Intervention on the pulmonary arteries in subsystemic right ventricular hypertension is advisable as a pre-surgical stage of treatment before correction of supravalvular aortic stenosis.

In severe generalized arteriopathy, including peripheral and central pulmonary arteries, pre-surgical catheter dilatation of the distal pulmonary arteries is indicated, followed by surgical intervention on the proximal pulmonary arteries and aorta. Unlike distal constrictions, which are balloon dilatable, central stenoses are resistant to dilatation due to their elasticity. Patients with severe biventricular systemic overload and generalized arteriopathy have an increased risk of myocardial ischemia during extracorporeal circulation. After elimination of supravalvular aortic stenosis, right ventricular failure often develops; therefore, a rational approach involves a combination of preoperative catheter intervention with subsequent surgery.

The results of transcatheter interventions for Williams-Beuren syndrome were published by Geggel et al. Successful balloon dilation was achieved in 51% of cases. In 25 patients, 124 dilations were performed during 39 procedures. The diameter of the constricted vessels in successful cases increased by 112 ± 65%. Dilation in the arteries of the lung parenchyma was more effective than in mediastinal and smaller vessels, provided that the size of the balloon exceeded the diameter of the narrowed area by 3 or more times. Experts recommend a series of dilations of distal pulmonary stenoses and subsequent surgical correction of the proximal sites. In high-risk patients, it is advisable to create an interatrial communication.

In Alagile syndrome, the presence of stenosis of the peripheral pulmonary arteries did not affect the outcome of liver transplantation. The death of 5 out of 17 operated patients was not associated with heart problems. In 12 patients who survived 6 years after transplantation, peripheral stenoses of the pulmonary arteries did not manifest themselves clinically. During the liver transplant operation, no hemodynamic disturbances were found even in severe stenosis. Therefore, transplantation is not contraindicated in patients with high right ventricular hypertension. Surgical dilation of the pulmonary arteries is not feasible due to diffuse distal constrictions, and balloon dilation is ineffective. Experience with stent implantation is limited.

Despite the noncardiac causes of mortality after liver transplantation, hospital mortality is still higher in Alagille syndrome than in patients without the syndrome. According to Razavi et al, if the pressure in the right ventricle is more than 50% of the systemic pressure, it is advisable to perform catheter stent implantation to reduce the risk of graft failure.

) Is a stenosis of the pulmonary artery. About eleven percent of all patients with various heart defects have this very disease. Pulmonary artery stenosis is characterized by the presence of an obstacle to blood flow in the area of \u200b\u200bthe pulmonary valve. The reason for the obstruction is the spliced \u200b\u200bvalve flaps. Most often they form a continuous membrane with a hole in the center. For the most part, such a congenital heart disease as stenosis of the pulmonary artery in a newborn child is valvular stenosis, but sometimes it occurs in combination with other heart defects.

Features of the disease

The size of the hole in pulmonary artery stenosis can be varied and directly affect the patient's condition.

With a hole size of one millimeter, the newborn requires urgent surgical intervention, otherwise it will be fatal.
But with the normal size of the hole, the defect can be detected after a couple of years of life, and even then by accident. After all, the characteristic manifestations are not very symptomatic: the pressure in the right section is lower than in the left, and tremors over the heart cannot be an accurate sign.

Adults do not suffer from stenosis of the pulmonary artery, since the defect is eliminated by cardiac surgeons in infancy or childhood. But when the defect is eliminated by simple dissection of the valves, their geometric structure is not restored. And, accordingly, the patient may develop insufficiency of the heart valves, because they let the blood pass well, but do not slam shut completely.

Therefore, in adulthood, the question of replacing the heart valve with a prosthesis may arise. But this is, rather, the consequences. Now let's look at the degree of pulmonary stenosis.

View of the heart with stenosis of the pulmonary artery

Stages of pulmonary artery stenosis

There are four stages of pulmonary artery stenosis:

moderate stenosis - stage I. The patient has no complaints, the ECG shows a slight overload of the right ventricle of the heart muscle. Systolic pressure up to sixty millimeters of mercury;
severe stenosis - stage II. It is characterized by a clear manifestation of symptoms. The systolic pressure of the heart muscle in the right ventricle is from sixty to one hundred millimeters of mercury;
sharp stenosis - stage III. Severe stage of the course of the disease, there are signs of circulatory disorders, pressure on the valve of the pulmonary artery and right ventricle is above one hundred millimeters of mercury;
decompensation - stage IV. Obvious signs of myocardial dystrophy, a very strong violation of blood flow. Contractile failure of the right ventricle develops, so the systolic pressure may be low.

With valvular stenosis, the leaflets of the valve grow together, and it has a domed shape with an opening in the middle.
The subvalvular degree of the disease looks like a funnel-shaped narrowing of the outflow tract of the right ventricle as a result of abnormal proliferation of fibrous and muscle tissue.
Supravalvular stenosis can be represented by an incomplete or complete membrane, localized narrowing, multiple peripheral pulmonary artery stenosis, diffuse hypoplasia.

Causes of occurrence

Pulmonary artery stenosis can be:

congenital. Poor heredity, rubella transmitted by the expectant mother during pregnancy, chemical and drug intoxication and many other factors;
acquired. As a result of various diseases, valve vegetation can develop and, as a result, stenosis. Sometimes it occurs as a result of compression of the pulmonary artery by enlarged lymph nodes or its sclerosis.

Symptoms of pulmonary stenosis

The symptoms of pulmonary artery stenosis depend on its stage. With a systolic pressure of fifty to seventy millimeters of mercury, they are absent.

The most common symptoms are:

increased fatigue during exercise;
dyspnea;
auscultation (heart murmur);
dizziness;
weakness;
drowsiness;
pain in the region of the heart;
fainting;
angina pectoris;
swelling and pulsation of the neck veins;
heart hump.

Diagnostics

Diagnosis of pulmonary artery stenosis includes a combination of various instrumental studies and physical data.

In the second intercostal space, to the left of the sternum, a rough systolic murmur is heard. It is carried out towards the clavicle and is perfectly audible in the interscapular region. The second tone in the first and second stages of the disease is heard practically unchanged, but with severe stenosis it can disappear completely.
With a slight stenosis, the electrocardiogram does not show abnormalities. At all other stages of the disease, there are signs of hypertrophy of the right ventricle of the heart muscle. Supraventricular arrhythmias may occur.
Echocardiography shows dilatation of the right ventricle of the heart muscle and post-stenotic dilatation of the pulmonary artery. Doppler ultrasound allows you to identify and determine the pressure difference between the pulmonary trunk and the right ventricle.
X-ray studies show a depletion of the pulmonary pattern and post-stenotic expansion of the pulmonary artery trunk.

Treatment

Therapeutic and medication methods

It is used to prepare for surgery or to alleviate the patient's condition at an inoperable IV stage.

Patient monitoring includes:

regular echocardiography;
prevention of infective endocarditis;
prophylactic antibiotic therapy;
identification of carriers of streptococcal infection with the rehabilitation of chronic foci.

Operation

Eliminate the defect using several types of operations:

closed pulmonary valvuloplasty. It was previously used to correct many defects, but now it is most often used to treat pulmonary artery stenosis. The operation is performed through a left-sided anterolateral approach to the heart in the fourth intercostal space. With the help of a special tool, the valvulot, the fused walls of the valves are dissected, with the help of special holders bleeding is reduced. After dissecting the membrane, the hole is widened with a Fogarty probe or dilator;
pulmonary valvotomy. An operation that is performed using catheterization. The probe is inserted through the vein and equipped with special knives or a balloon;
open valvotomy. It is carried out by connecting artificial blood circulation and opening the chest to gain access to the heart. An incision is made in the lumen of the pulmonary trunk and through it the structure of the fused valves is studied. Then they are cut strictly according to the position of the commissures, from the median opening to the base of the valves. The valve opening and subvalvular space are monitored visually or with the help of a heart surgeon's finger. This method of eliminating the defect is considered the most effective.

Caution! The video shows how the operation is performed for pulmonary stenosis (click to open)

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Disease prevention

Complications

failure of the right ventricle;
myocardial infarction.

And finally, we will tell you about the average life expectancy and prognosis for pulmonary artery stenosis.

Forecast

The main thing, remember, even if the doctor diagnosed the fetus with pulmonary artery stenosis, everything is fixable, you should not despair!

Caution! The video shows the operation for valve stenosis of the pulmonary artery (click to open)

Pulmonary artery stenosis occurs quite often among other heart defects. It can be compared in prevalence with defects of the interventricular, interatrial septa. According to statistics, in the total number of all kinds of malformations of the heart muscle, stenosis occupies about eleven percent. It is very important to know the signs of stenosis, diagnose the disease in time and immediately begin treatment.

A characteristic feature of the disease is the presence of an obstacle that slows down the total blood flow in the area of \u200b\u200bthe pulmonary valve. The main reason for the development of the disease is the fusion of the valve leaflets. In most cases, they form a continuous membrane, in which a hole remains in the center. Stenosis is almost always valvular. This is a congenital malformation of the heart muscle, which is diagnosed in newborn babies. However, in some cases, stenosis appears in combination with other heart diseases.

When pulmonary artery stenosis is observed, the size of the hole can be of different sizes. It is he who largely influences the well-being of a sick child. If the size is normal, it ensures the outflow of blood, such an ailment may not be detected immediately. Sometimes the diagnosis reveals the disease after a few years of life, under random circumstances, for example, during a routine examination. It is worth noting that the characteristic signs do not give a clear clinical picture: the specialist can determine that the pressure in the left section is higher when compared with the right section. In this case, even the characteristic tremor that arises over the heart cannot be considered an obvious symptom.

When the hole has a minimum diameter of less than one millimeter, urgent surgery is required for the baby. In this case, the outflow of blood is disturbed so seriously that only surgical intervention saves the child from death.

Complications in the development of pulmonary stenosis are associated with a significant load on the right ventricle of the heart muscle. Ultimately, the internal cavity of the ventricle expands, and the wall thickens noticeably. If the baby already has a suspicion of the development of this heart defect, he needs to be constantly monitored, to conduct regular examinations. A characteristic picture of the development of the disease is given by pressure, which is measured directly at the heart valve. The critical data of the examinations are as follows: fifty millimeters is the difference established during the examination between the pulmonary artery and the right ventricle. In this case, there is a need for urgent surgical intervention. In accordance with statistical data from medical practice, usually operations with established artery stenosis are performed on children under 9 years of age.

In adults, this defect is most often not observed, since it is operated on in infants, or in childhood. However, if the defect is eliminated due to the standard dissection of the valves, then the correct geometric structure is no longer restored. In the future, the patient may begin to suffer from dysfunction of the heart valves. That is, the blood is passed through normally, but the valves will no longer be able to slam shut completely. Therefore, adults who have had stenosis as a child may need a special heart prosthesis instead of a valve. This complication is becoming a common consequence of this disease.

It is important.Nowadays, prostheses are being installed in many hospitals. The operations are successful, there is no threat to life.

Stages of development of the disease

It is customary to distinguish only four stages along which pulmonary artery stenosis progresses.

Stage of the disease	Symptoms, clinical presentation, disease development
Stage 1. Moderate stenosis	The clinical picture is not pronounced, the patient has no complaints. Systolic pressure is usually kept within specific limits, without critical jumps. More often it is no more than sixty millimeters. In this case, the ECG records non-critical overloads of the right ventricle of the heart
Stage 2. Stenosis becomes pronounced	Symptoms are more pronounced. The pressure of the heart increases markedly: the systole rate is 60-100 millimeters
Stage 3. Pulmonary artery stenosis at this stage is already sharp	The disease is in a difficult stage. Symptoms of a violation of normal blood circulation are sharply manifested. The pressure observed at the valve of the artery is already more than one hundred millimeters. The pressure measured on the right ventricle is considered
Stage 4. Decompensation	There are obvious symptoms characteristic of myocardial dystrophy. Blood circulation is severely impaired. Critical right ventricular contractility can be diagnosed. Systolic blood pressure is likely to be low or normal. At this stage, this symptom is no longer significant.

Taking into account the level of blood flow, specialists have identified supravalvular, subvalvular with valvular stenosis. Most often, valve-type stenosis develops. The combined type of the disease is extremely rarely diagnosed. Let's consider the key features of stenosis of these types.

The supravalvular is characterized by localized narrowing, as well as numerous peripheral arterial stenoses, a membrane of complete and incomplete type, diffuse hypoplasia.
When the disease occurs in the subvalvular form, the outflow ventricle is characteristically narrowed. This constriction is of a funnel-shaped type. This occurs when tissue overgrowth. It is precisely the abnormal growth that is observed, and the fibrous, muscle tissues increase.
In such cases, the valve flaps acquire the shape of a dome, have a gap in the middle. This is due to the fusion of the valve leaflets.

The most common stenosis is of the valve type.

Stenosis reasons

Let's consider the key factors provoking the development of stenosis.

Acquired stenosis occurs due to valve vegetation. Sometimes the disease appears due to compression of the pulmonary artery. In this case, there is sclerosis of the artery, the lymph nodes are enlarged.
Stenosis of a congenital type is associated with diseases of the mother that occur during pregnancy. For example, common rubella sometimes provokes the development of stenosis. Medicinal and chemical poisoning also has a noticeable effect.

Hereditary stenosis also occurs.

Clinical picture

The symptomatology of the disease is far from always obvious. For example, when the systolic pressure is in the range of 50 to 70 millimeters, symptoms are not detected.

The following symptoms are characteristic, manifested with severe stenosis of the pulmonary artery:

angina pectoris;
heart murmurs;
fainting;
drowsiness;
throbbing, swelling of the veins in the neck;
heart hump;
painful sensations in the heart;
excessive fatigue associated with physical exertion, even minor;
dyspnea;
constant weakness;
dizziness.

Note!If a child has these symptoms, it is necessary to urgently consult a doctor and undergo an appropriate examination.

Diagnosis of the disease

The diagnosis of stenosis includes a number of physical indicators, the most complex instrumental studies. This is how the examination takes place.

First of all, the heart is carefully listened. A strong systolic murmur, rather rough, can be heard on the left side of the sternum, in the second intercostal space. The noise is very well detected in the section between the shoulder blades, follows in the direction of the clavicle.
When the disease passes the first, second stages, the second tone practically does not change, it is heard well. The third stage of stenosis is characterized by poor listening to this tone or even its complete disappearance.
The electrocardiogram reveals stenosis well enough. It fixes the hypertrophy of the right ventricle of the heart. Supraventricular rhythm disturbances can also be identified. However, if the stenosis is minor, no pathological changes are recorded on the ECG.
Doppler sonography is performed. It is with its help that the difference in pressure diagnosed for the right ventricle and the pulmonary trunk is revealed.
Echocardiography is mandatory. It fixes the expansion on the pulmonary artery, which becomes a consequence of the disease. Also, sometimes dilatation of the heart, diagnosed on the right ventricle, is detected.
X-ray also determines the characteristic signs of the disease: simplification of the pulmonary pattern, significant expansion of the trunk.

For accurate diagnosis, it is necessary to conduct a comprehensive examination for any symptoms characteristic of this disease.

Treatment

Patients must be fully monitored, which includes:

diagnosis of streptococcal infection, concomitant sanitation, carried out in chronic foci;
echocardiography;
preventive measures to prevent infective endocarditis;
prophylactic antibiotic therapy.

The therapy is carried out medically, therapeutically. The main method of dealing with this serious ailment is surgery. However, the patient needs to be prepared for surgical intervention, as an extreme and laborious measure. Most often, they operate when stenosis develops at the second or third stage. In addition, treatment is carried out to improve the general condition of the patient if the stage is already inoperable.

Operation

It is important!Effective treatment of pulmonary artery stenosis can only be carried out using a surgical method. When diagnosing stenosis, you cannot refuse the operation.

For almost 70 years, cardiac surgeons have been performing these operations quite successfully, while the techniques are constantly being improved. The optimal moment for surgery is the second and third stages of the development of the disease. If there is moderate stenosis, patients are constantly examined, but they are not prepared for surgery.

You can get rid of this malformation of the heart muscle by performing various types of surgical operations. Let's consider the main ones.

Pulmonary valvotomy is performed. The operation is performed using modern catheterization techniques. A special probe is promptly inserted through the veins. It is equipped with a special cylinder and knives.

Valvotomy can be open. It is performed according to the following algorithm.

In this case, the chest is opened so that direct access to the heart is obtained.
In this case, an artificial circulatory system is connected.
An incision is made directly in the lumen of the pulmonary trunk, and through it a study of the structure of the valves that have managed to grow together is already carried out.
Then these transformed valves are cut. This is done directly according to the position of the commissars.
The incision is made right to the very base of the valves, from the central lumen.
In this case, it is necessary to monitor the state of the subvalve space, behind the valve opening. This is done using the finger as well as by visual observation.

At the moment, it is this method of operation that is recognized as the most effective.

Valvuloplasty can also be closed. It is now quite often performed precisely to eliminate stenosis. The operation is performed in the fourth intercostal space, directly through the left-sided approach to the heart muscle. The walls, which had time to grow together, are dissected using a valvulot. This is a special tool. Bleeding is stopped with holders. When the membrane has already been dissected, the opening is widened using a dilator and a Fogarty probe.

Complications

A number of complications may develop. Let's list the main ones:

myocardial infarction;
failure detected on the right ventricle;
stroke;
septic endocarditis;
inflammation of the respiratory system;
myocardial dystrophy.

Prevention

Prophylaxis can be carried out to prevent intrauterine stenosis.

Important!First of all, it is necessary to provide normal conditions for the course of pregnancy. It is extremely important to limit the impact of all negative factors on the expectant mother.

If a child already has a suspicion of a disease, it is imperative to be observed by a cardiologist, constantly undergo appropriate examinations. Timely prevention of infective endocarditis is of great importance.

Forecasts

Now cardiologists are trying to take all measures to prevent the development of this disease. However, unfortunately, parents sometimes simply refuse to perform the operation. And then the risk of death increases significantly.

Note:stenosis is treated, operations are successful. Even if an ailment is detected in the fetus, there is no need to despair! All of this is curable, and modern medicine offers safe, effective methods to get rid of the disease.

Video - Endovascular treatment of pulmonary artery stenosis

Pulmonary artery stenosis (PAS) is a pathological condition that changes the size of the pulmonary trunk, as a result of which it becomes narrower, which interferes with normal blood flow. This disease is one of the varieties of heart disease.

During narrowing, there is a weak exit of blood from the right ventricle, due to the fact that the lumen of the lung artery becomes smaller. Therefore, the small circle of blood circulation fails.

The diagnosis of the disease occurs already in children from an early age. In most cases, stenosis is observed in combination with various changes.

According to various statistics, from 3 to 12 percent of the narrowing of the pulmonary trunk falls on one thousand registered heart defects.

Classification of pulmonary stenosis

The main classification of stenosis occurs according to the location of the narrowing, which can form in the following places:

Valve. The decrease in the lumen of the pulmonary artery occurs directly in the heart valve. This type of pulmonary artery stenosis is mainly recorded;
Undermined. The artery narrows below the valve;
Supravalve. There is a decrease in the passage of the artery up the trunk, above the valve;
Combined. If there is a narrowing of the artery in several places.

Forms of pulmonary stenosis

In ninety percent of cases of registration of pulmonary artery stenosis, valvular stenosis is diagnosed.

Also, the division occurs according to the degrees of overlap of the lumen:

Light degree;
Average degree;
Severe degree.

In practice, a division is successfully applied by qualified doctors, which is based on the level of detection of systolic blood pressure (blood pressure) in the right ventricle, and the ratio of pressure between the right ventricle and the pulmonary trunk.

The levels of this pressure are divided by degrees:

1st degree. Arterial pressure in systoles - 60 mm Hg, ratio - 20-30 mm Hg;
2nd degree. Arterial pressure in systoles is from 60 to 100 mm Hg, and the ratio is 30-80 mm Hg;
3rd degree. The pressure in this case is more than 100 mm Hg, and the ratio is more than 80;
4th degree. It is a decompensatory stage. Ventricular insufficiency progresses, due to its weak contractions, dystrophy of the heart muscle occurs, the pressure in the ventricle decreases.

Pulmonary artery stenosis

What provokes the ULA?

Throughout life, the decrease in the lumen of the artery of the lung progresses quite rarely. In the overwhelming majority of cases, it is marked by a congenital disease, and is in second place in terms of prevalence among all congenital heart defects.

The factors that affect the stenosis of the pulmonary trunk include those that, while carrying a child, can affect the formation of the vascular system, and lead to congenital heart defects.

These include:

Reception by women carrying a child, psychoactive substances, drugs, antibiotics, mainly in the first trimester of pregnancy;
Inappropriate working conditions during pregnancy... When carrying a child, work is contraindicated in paint and varnish, chemical, industrial enterprises, and other structures in which the expectant mother can inhale chemical and toxic fumes;
Genetic disposition... In this case, stenosis of the arteries is transmitted mainly from the mother (or from the father) to the child;
Diseases of viral origin when carrying a child... Congenital stenosis of the pulmonary trunk can be affected by: rubella, herpes, mononucleosis and other viral diseases;
Ionizing radiation, including X-rays, when carrying a child;
Environmental factors. Unfavorable environment, mostly manifested in a high concentration of radiation in certain regions of the country.

As the narrowing of the lung artery progresses throughout life, the most common causes may be:

Disease of the rheumatism group... They affect the valves of the pulmonary artery, causing stenosis;
Inflammatory processes of the walls inside the artery of the lung... They are registered in rare cases, but one should not exclude from the list the defeat of syphilis, tuberculosis, etc.;
Deposition of atherosclerotic plaques... The deposition of cholesterol plaques can also occur on the walls of the pulmonary artery, causing it to narrow;
Pressure on an artery of the lung outside... In most cases, the reasons for this are tumor formations, enlarged lymph nodes, and saccular protrusion of the aorta;
Calcification. Deposition of calcium salts on the walls and valve of the lung artery. The walls of the artery are irritated, leading to narrowing.

Pulmonary stenosis symptoms

The detection of symptoms directly depends on the magnitude of the narrowing of the patency in the pulmonary artery. In a mild stage, stenosis may not appear for a long time. Mostly in children under one year old.

Symptoms in more severe forms of stenosis make themselves felt almost from birth.

They manifest themselves in the following symptoms:

Pronounced cyanosis, which manifests itself in blue tones of the skin at the tips of the fingers and toes, the area between the nose and lip, or in cyanosis of the skin all over the body;
Hard breath;
Loss of consciousness is possible;
Weak weight gain;
Infant lethargy and obvious anxiety.

In the adult age category, the manifestation of symptoms is somewhat different. They may not give signals for many years, or throughout their life.

The obvious symptoms of stenosis of the artery of the lung of more severe stages include:

Rapid fatigue after minor physical exertion, progressing to permanent fatigue;
Dizziness and loss of consciousness;
On auscultation of the heart, tones of a dull character are heard, a rough systolic murmur in the left side of the chest and the third intercostal space;
Heavy breathing during physical exertion, or at rest, which becomes stronger when lying down;
Fingers are inherent in the form of "drumsticks" of flat formation;
There is a pulsation of the veins of the cervical spine;
Swelling of the legs, and with the progression of heart pathologies and swelling of the whole body.

How dangerous is an ULM aircraft?

The formation of stenosis of the pulmonary artery can occur as a consequence of acquired causes, and congenital.

During its formation, the following processes take place in the heart:

When the artery narrows, it becomes more difficult for the right ventricle to expel blood, and a large load is created on it;
As a result, less blood is delivered to the lungs. Hypoxia of internal organs occurs, provoking insufficient oxygen saturation of the blood;
The heart muscle wears out over time with the regular exposure to heavy loads on the right ventricle. This leads to its failure, provoked by an increase in the mass of the heart muscle;
Due to the fact that the amount of blood that is not completely thrown into the artery of the lung regularly increases, there is a reverse ejection of the incoming blood into the right atrium, this leads to a failure in the blood circulation, as well as the processes of stagnation and oxidation of blood. Stronger oxygen starvation occurs;
Obviously manifested stenosis leads to the progression of heart failure. Death is the most common outcome unless prompt surgery is performed.

Which doctor is treating?

At birth in a maternity hospital, absolutely all newborn babies are examined by a neonatologist, who determines the presence of diseases and pathological conditions in the infant. If deviations are found, he draws up a plan for further examination.

If symptoms of pulmonary artery stenosis occur in adolescence, you should consult a qualified pediatrician.

When signs of pulmonary artery stenosis appear in the older age group, you need to consult a therapist or cardiologist.

Diagnostics

At the first visit, the doctor listens to the patient's complaints, examines the anamnesis, and then makes an initial examination to identify obvious signs of the disease. Suspecting pulmonary artery stenosis, the doctor may send the patient for additional instrumental examinations for an accurate diagnosis.

Arrows on the radiograph show the dilated arch of the pulmonary artery and the left atrium.

Hardware studies prescribed for suspected pulmonary artery stenosis include:

Electrocardiogram (ECG). An ECG study is performed after exercise. Helps to detect pronounced stenosis, due to overload of the right ventricle and atrium, as well as extrasystoles;
Ultrasound examination of the heart... When conducting such a study, the doctor receives an overview of the valve ring, which helps to determine the level of pressure in the right ventricle and the ratio of pressures in the right ventricle and pulmonary trunk. The more the pressure in the ventricle, the more the vessel will be blocked;
Chest x-ray... Helps to determine the degree of increase in the dimension of the heart, which led to a pathological increase in the muscle of the heart;
Ventriculography. A contrast agent is introduced into the vessels, which penetrates into the right side of the heart, after which an ultrasound is done. According to the results, it is possible to detect to what extent the stenosis of the pulmonary artery progresses;
Right heart catheterization. It is performed to measure the pressure in the right ventricle and pulmonary trunk;
Probing.

Treatment

The most effective treatment for pulmonary artery stenosis is surgery.

With mild manifestations, drugs of the groups are prescribed:

Glycosides;
Vitamin complexes;
Preparations saturated with potassium.

Any drugs are prescribed only to maintain the patient's condition. For treatment, only surgery is needed. Surgical treatment is aimed at improving blood circulation in the pulmonary trunk.

Surgical intervention is prescribed depending on the location of the narrowing of the lumen.

Among them:

With supravalvular stenosis. Surgical intervention is used to remove part of the wall in which the narrowing occurred. A patch taken from the patient's pericardium is applied to the remote site;
With subvalvular stenosis... When carrying out this operation, at the exit site of the right ventricle, the hypertrophied portion of the heart muscle is removed;
With valve stenosis. Operation with balloon valvuloplasty is used. This implies the introduction of a balloon into a vessel, as a result of which a stent is installed, which expands it;
Combined defects. With several places of narrowing at once, suturing of the interventricular and interatrial foramen is performed.

After surgery, normal blood circulation begins through the dilated pulmonary trunk. Symptoms gradually subside, activity appears.

School-age children can return to assignments after three months.

How to prevent pulmonary artery stenosis?

Since stenosis of the pulmonary trunk is predominantly a congenital heart defect, prevention is mainly aimed at maintaining better conditions for pregnant women.

The set of actions that are necessary for preventive actions to prevent stenosis of the lung artery include:

Maintaining a healthy lifestyle;
Creation of ideal conditions during the period of bearing a child;
Diagnosing the disease in the early stages;
If the first symptoms are detected, consult a specialist;
Do not work, during the period of bearing a child, in "harmful" jobs;
Walk more in the fresh air;
Observed by an obstetrician-gynecologist;
Give up smoking and alcohol;
Do not succumb to ionic radiation.

Life expectancy and prognosis?

In the absence of the necessary surgical intervention, pulmonary artery stenosis is fatal. At any age, stenosis requires constant monitoring and prompt surgery.

With congenital pulmonary artery stenosis and untreated, patients live up to a maximum of 20 years.

Carrying out timely surgical intervention, even with severe degrees of pulmonary artery narrowing, gives a chance to live for 5 years.

And while maintaining a correct lifestyle and nutrition, avoiding stress - more than 5 years (for 90 percent of patients).

If you find any symptoms of the manifestation of the disease, contact your doctor immediately. Early detection of the disease will help to diagnose it in advance and carry out surgical intervention. There is no drug treatment.

Symptoms

Stenosis and its effect on heart function

Types of pulmonary stenosis

Features of the disease

Stages of pulmonary artery stenosis

Causes of occurrence

Pulmonary stenosis symptoms

Diagnostics

Therapeutic and medication methods

Operation

Disease prevention

Complications

Forecast

Classification of stenosis

Reasons for the development of stenosis

Symptoms of the disease

Diagnostic steps

Healing activities

In adults

How dangerous

Forecast

Embryology

Anatomy and classification

Occurrence

Hemodynamics

Clinic

Differential diagnosis

Course and forecast

Treatment

Balloon dilation results

Results of pulmonary stenting

Syndrome treatment results

Features of the disease

Stages of pulmonary artery stenosis

Causes of occurrence

Symptoms of pulmonary stenosis

Diagnostics

Treatment

Therapeutic and medication methods

Operation

Disease prevention

Complications

Forecast

Stages of development of the disease

Stenosis reasons

Clinical picture

Diagnosis of the disease

Treatment

Operation

Complications

Prevention

Forecasts

Video - Endovascular treatment of pulmonary artery stenosis

Classification of pulmonary stenosis

What provokes the ULA?

Pulmonary stenosis symptoms

How dangerous is an ULM aircraft?

Which doctor is treating?

Diagnostics

Treatment

How to prevent pulmonary artery stenosis?

Life expectancy and prognosis?

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