Stenosis of the right branch of the pulmonary artery. Pulmonary artery stenosis in children: causes, symptoms, stages, treatment

What is pulmonary stenosis?

- this is a narrowing between the right ventricle and the pulmonary artery, which carries blood to the lungs, or a narrowing of the pulmonary artery itself in its different parts.

This narrowing can occur at different levels, and depending on the location of the narrowing, the following types of pulmonary artery stenosis are distinguished: valvular (narrowing of the pulmonary artery valve), supravalvular (narrowing of the pulmonary artery trunk above the level of the valve), subvalvular (narrowing due to excessive growth of the muscles of the right ventricle, preventing blood from flowing into the pulmonary artery), and peripheral pulmonary artery stenoses (these are stenoses of various branches of the pulmonary artery itself, carrying blood to the right or left lung). Pulmonary artery stenosis is often combined with (tetralogy of Fallot, transposition of the great vessels and others).

The most common type is valvular pulmonary artery stenosis. Normally, the pulmonary valve allows venous blood to flow freely from the right ventricle (pump) into the pulmonary artery, where the blood is oxygenated. Normally, the pressure in the ventricle and pulmonary artery is the same. The pulmonary valve consists of three leaflets. When the right ventricle contracts, the valve leaflets open completely and blood flows freely into the pulmonary artery; When the right ventricle relaxes and fills with venous blood flowing from our organs and tissues, the valve leaflets close completely and prevent the blood from flowing back from the pulmonary artery into the right ventricle. With valvular pulmonary artery stenosis, the valve leaflets are partially fused to each other, and their complete opening becomes impossible (Fig. 2). In this case, the pressure in the right ventricle is very high, and the pulmonary artery is low (pressure gradient).

Natural course of vice. Or what does pulmonary artery stenosis lead to?

Pulmonary stenosis causes the right ventricle to work extra hard to push blood through the narrowing and into the lungs. Gradually, the right ventricle gets tired of working in this mode, which leads to stretching of its wall, enlargement of the cavity, the development of heart failure and heart rhythm disturbances. Due to narrowing, insufficient blood flows into the lungs, which leads to frequent bronchopulmonary diseases.

Clinical manifestations of pulmonary artery stenosis depend on the severity of the narrowing. Very severe (critical) pulmonary artery stenosis in newborns can manifest as severe heart failure and a bluish tint to the skin. Such children require immediate endovascular or surgical treatment. Not pronounced stenoses with a pressure gradient of less than 25 mm Hg. Art. may not manifest itself in any way, and such patients do not require treatment. Most often, they are recommended scheduled monitoring by a cardiologist and periodic ultrasound of the heart, which will help track the progression of the narrowing.

Treatment of pulmonary artery stenosis.

The choice of surgery depends on the location of the narrowing. Only valvular and peripheral (branch stenoses) pulmonary artery stenoses are subject to endovascular treatment; the rest of the defects remain the prerogative of surgery.

Treatment of any valvular pulmonary stenosis begins in the cath lab. This operation is called balloon pulmonary valvuloplasty. A thin tube (catheter) is inserted through the femoral vein into the pulmonary artery under X-ray control, through which a contrast agent is injected (Video 1). This manipulation allows you to determine the location and degree of narrowing. Then a catheter is inserted into the pulmonary artery, at the end of which there is a folded balloon (Fig. 3).

When the balloon reaches the point of narrowing, it is inflated, separating the valve flaps fused together (Video 2). The balloon is deflated and the catheter is removed from the patient's body. Using another catheter, pressure in the right ventricle and pulmonary artery is measured and the effectiveness of the procedure is assessed. This procedure lasts on average about one hour. After such an operation, only a trace of a puncture of the femoral vein remains on the patient’s body.

Endovascular treatment of stenoses of the branches of the pulmonary artery that carry blood to the right or left lung is also possible. Such stenoses are extremely rare as an isolated defect. More often, branch stenoses are combined with other congenital heart defects or are consequences of surgical correction. In this case, the narrowing of the branches is eliminated in the X-ray operating room. This procedure is called balloon angioplasty of the branches of the pulmonary artery. In the same way as for valvular stenosis, under X-ray control, a catheter with a balloon at the end is inserted into the site of narrowing. The balloon is inflated, stretching the narrowing area. The effectiveness of the procedure is assessed after injecting a contrast agent into the pulmonary artery and measuring pressure at different points in the vessel. Often, due to the great elasticity of blood vessels (when the walls of the vessel, after expansion, return to their previous shape), balloon angioplasty alone is not enough.

In this case, stenting of the branches of the pulmonary artery is performed. The stent is a metal weave in the form of a small tube (Fig. 4). When folded, the stent is placed over the balloon catheter (Figure 5). When the balloon catheter is inflated, the stent expands, forming a reliable metal frame for the vessel wall, and preventing the vessel from narrowing again (Video 3, 4, 5).

Rehabilitation after the procedure

Typically, patients are discharged the day after the procedure. A sterile dressing should remain at the site where the catheter is inserted into the vessel for some time. For 6 months after surgery, in case of colds, it is necessary to carry out antibiotic prophylaxis for infective endocarditis. For six months after the procedure, you must refrain from routine vaccinations.

Video 1 – Video from the operating room. The contrast agent is injected through a diagnostic catheter into the right ventricle.

Video 2 – Video from the operating room. The catheter balloon inflates the narrowed valve.

Video 3 – Video from the operating room. Sharp narrowing between the right and left branches of the pulmonary artery. Only the left branch is visualized.

Video 4 – Video from the operating room. Sharp narrowing between the right and left branches of the pulmonary artery. Only the right branch is visualized.

Video 5 – Video from the operating room. The stent is installed in the narrowing. Blood flow between the branches has been restored.

Myths and reality about endovascular surgery
congenital heart defects

Currently, X-ray endovascular surgery is attracting more and more attention from almost all media, including print media, the Internet and television. Every day we are confronted with a massive flow of information on various aspects of this modern field of medicine. Every day they write and talk about it, but, unfortunately, not everything and not always objectively. There are many erroneous statements, rumors or even myths that need to be corrected with factual information.

Myth 1. This is a very new, almost experimental field of cardiovascular surgery.

This is wrong! Endovascular surgery has a rich history and has long been widely used in medical practice. Cardiac catheterization was first performed in 1929 by R. Forsmann (Germany), for which he received the Nobel Prize in 1956. In 1964, the first balloon angioplasty was performed and since then endovascular surgery has ceased to be a purely diagnostic area of ​​medicine. Further discoveries and inventions of devices followed one after another: 1975 - spirals, 1976 - occluders, 1979 - emboli, 1986 - coronary stents, 1994 - stents for large vessels, 2005 - endovascular heart valves! To date, all of the above devices have evolved to more advanced analogues. The most common occluder in the world has become the Amplatzer occluder - more than half a million implantations since 1995. At the Amosov Institute, Amplatzer occluders have been installing their analogues since 2003. The trend in the world is that diagnostics have moved from the cath lab to echocardiography and computed tomography rooms, and the treatment of heart defects has moved from the operating room to the cath lab. In developed countries of the world (USA, Canada, Australia, Europe), ductus botellus, septal defects, and coarctation of the aorta are practically not operated on surgically. Our institute takes into account all modern global trends when treating patients.

Myth 2. Devices used to treat defects (occluders, coils, stents) are foreign bodies and can be rejected.

All these devices are made of modern high-tech biocompatible materials that do not cause rejection reactions. Six months after the operation, these devices are completely covered with endothelium (they grow with their own cells) and do not differ from the inner surface of the heart. All devices are non-magnetic; after their implantation, the patient can undergo an MRI. They do not beep at metal detectors at airports, shopping malls, etc.

Myth 3. Occluders move (fly away).

Indeed, in our and world practice such cases occur, but their frequency is about 1%. The complication is unpleasant, but not critical. There has not been a single case in the world where a displaced occluder would have led to death. As a rule, such an occluder is removed endovascularly and reinstalled or replaced with a larger one. The greatest number of displacements occurs in the first hours or days after surgery, when the patient is still in the clinic. Further, the probability of this decreases sharply; distant displacements are casuistic.

Myth 4: Atrial septal defects with missing or thinned edges are not amenable to endovascular closure.

The absence of the aortic edge of the septum is not a contraindication to occluder placement. The same applies to a thinned or aneurysmal septum. Remember that conventional (transthoracic) echocardiography does not provide a complete picture of the defect. Even if a diagnosis of absence of a margin is made, this does not mean that it is not there. The clear anatomy of the defect can only be judged after transesophageal echocardiography, which is the gold standard for selecting patients for endovascular treatment.

Myth 5. Occluders require replacement over time.

The device does not need to be replaced either as the patient grows or over time. The occluder grows into the septum within 6 months and creates the basis for its further growth. In the case of vascular stenting, it is possible to endovascularly increase the lumen of the stent with vessel growth without replacing the implant.

Myth 6. It's expensive...

Endovascular surgery is high technology, which really costs more than conventional operations. In some cases, the patient buys the device for implantation, but there is a waiting list for free implants, which are purchased by the institute. In addition, we cooperate with numerous relief funds that, in a relatively short time, raise funds to purchase devices for children. In most cases, there is no urgency in the operation, and patients have enough time to raise funds for the implant, wait their turn, or find a sponsor. Therefore, if a patient wishes to undergo endovascular surgery, there are currently no barriers to this.

FAQ

The average hospital stay is 3-4 days. As a rule, on the day of admission in the morning you undergo an examination, including a clinical and biochemical blood test (you need to arrive on an empty stomach), an X-ray, an ECG, an ultrasound examination of the heart and a consultation with a cardiologist and cardiac surgeon. If all indicators are normal, the next day an operation is performed to eliminate the defect. On the third day, we conduct control tests and discharge you.

To be admitted to our hospital, you will need a passport or a child’s birth certificate.

If the patient is a child, you need a certificate of sanitary and epidemiological conditions (stating that the child has not recently had contact with infected patients), which you will receive at the clinic at your place of residence.

It is advisable to have previous advisory reports, an ECG and a chest x-ray with you.

A referral from a local cardiologist is NOT REQUIRED. You can come for a consultation and subsequent treatment by self-referral. If you are over 30 years old or have experienced interruptions in your heart function, it is advisable to conduct Holter monitoring at your place of residence. Such a study can be carried out here, but this will increase your time in hospital by 1-2 days.

If you suffer from chronic gastritis, gastric or duodenal ulcers, you need to undergo fibrogastroduodenoscopy. If the disease is confirmed, you need to undergo treatment at your place of residence. Such a study can be carried out here, but this will increase your time in hospital by 1-2 days in the absence of ulcers and erosions.

Pulmonary artery stenosis in children is a congenital abnormality of the development of a large arterial vessel that delivers venous blood from the right ventricle of the heart to the lungs. It is one of the congenital defects of the cardiovascular system. The incidence is about 12% of the total number of identified cases of congenital heart disease. Most newborns with severe stenosis die within the first year of life if the defect is not corrected surgically.

What is stenosis and why is it dangerous?

Pulmonary artery stenosis is a narrowing of the vessel, which can have varying degrees of severity and localization. Based on the degree of severity, they distinguish between mild, moderate and severe arterial damage.

Based on location, stenosis is divided into:

1. Valvular - narrowing occurs in the area of ​​the arterial valve. In this case, the valve itself can be single-leaf, tricuspid or bi-leaf. Behind the stenotic area there is usually a zone of vessel dilatation. Valvular pulmonary artery stenosis accounts for about 90% of the total number of cases of the disease.
2. Subvalvular - narrowing of the blood drainage line under the arterial valve and the formation of a muscle bundle that prevents the ejection of blood from the heart.
3. Supravalvular - isolated pulmonary artery stenosis, occurring in the supravalvular type, can have several morphological forms.
4. Pulmonary artery stenosis is a narrowing of the passage directly between the ventricle and the trunk of the pulmonary artery.

The presence of narrowings reduces the capacity of the artery, which leads to its incomplete performance of its function. At the same time, the right ventricle of the heart works under high load, stretches, hypertrophies, and its insufficiency develops. Increased pressure inside this chamber of the heart causes the oval window to open and dump excess blood into the left half of the organ. In this case, the patient develops clinical signs of heart disease.

Note: a mild degree of valve stenosis practically does not lead to the appearance of symptoms of the disease, so the disease may not be detected in a timely manner. The development of such newborns proceeds without deviations. They grow normally, mature and lead full lives. The life expectancy of patients is practically no different from people who do not suffer from cardiac pathology.

Causes of stenosis

Stenosis can develop under the influence of mutagenic environmental factors or genetic defects of one of the parents transmitted to the fetus. It is curious that the disease often occurs in children whose parents did not suffer from a similar pathology. The reason for this lies in the peculiarities of the interaction of dominant and recessive genes in the body of the father and mother (the combination of a recessive gene for a disease with a dominant gene for a normal trait does not lead to the development of disorders).

Environmental mutagenic factors include radiation, chemical poisons (ethanol, phenols, antibiotics), and pathogens of infectious diseases. The greatest danger is the chronic entry of small doses of mutagen into the mother's body or acute poisoning by one of the pathogenic substances in the first trimester of pregnancy.

Narrowing of the mouth of the pulmonary artery can also occur in adulthood, which does not allow the pathology to be classified as a congenital defect. The cause of this may be infective endocarditis, as well as myxomas, carcinoids and other tumor processes of the heart.

Video

Video - pulmonary stenosis

Symptoms and diagnosis

Pulmonary stenosis in newborns manifests itself in the form of respiratory failure. The child has acrocyanosis, which can subsequently develop into generalized cyanosis, severe shortness of breath, and periodic loss of consciousness. A similar picture occurs if the narrowing is large enough. Small degrees of stenosis do not lead to the appearance of clinical signs.

In adult patients, pulmonary valve stenosis manifests itself in the form of the following symptoms:

• Swelling of the veins of the neck;
• Dyspnea;
• Dizziness;
• Fatigue;
• Chest pain during exercise;
• Fainting;
• Systolic tremor.

Signs of the disease can appear in the patient in full from childhood or progress as they grow older.

Diagnostics

Diagnosis of a congenital disorder associated with narrowing of the pulmonary artery is carried out using examination methods such as auscultation, ECG, echocardiography, radiography, catheterization of the cardiac cavities.

The main sign of stenosis detected by auscultation is a rough noise during systole, heard in the second intercostal space, as well as in the area of ​​the left clavicle and on the back. The moment the noise appears directly depends on the degree of narrowing. The higher it is, the later it occurs.

When conducting electrocardiography, the patient reveals signs of hypertrophy of the right ventricle, and sometimes of the atrium on the same side. Strong contractions are the cause of supraventricular tachycardia, weak ones may not lead to ECG symptoms.

Echocardiography and catheterization of the cardiac cavities makes it possible to determine the degree of narrowing of the artery by the difference in blood pressure in the vessel and the right ventricle.

The values ​​of the peak gradient and the degree of contraction corresponding to them are reflected in the table below:

X-ray can detect dilation of the artery that occurs with congenital valvular stenosis. An indirect sign of the disease is a depleted pattern of the lungs in the image.

Note: cardiac catheterization is a research method involving the insertion of a polyvinyl catheter into the heart. Access is through a peripheral artery or vein. It is used to diagnose heart defects, as well as in the treatment of rhythm disturbances. Today, SIJ is used only when other examination methods are ineffective or in preparation for surgery. Insertion of a catheter during initial diagnosis is accompanied by an unreasonable risk.

Treatment

Treatment of pulmonary artery pathology in a newborn is only surgical. In severe forms of the disease, surgery can be performed in the first days, and sometimes even hours, of the child’s life. This only happens when delay can lead to the death of the baby. Planned interventions are postponed to a later age. To correct the condition of the pulmonary artery, the child is hospitalized at the age of 3-4 years.

The surgical procedure may vary depending on the capabilities of the clinic and the qualifications of the surgeon. 15-20 years ago, the most common method was the open method of manipulation, in which the cardiac surgeon had to work on an open heart. This method was dangerous and had high mortality rates.

Today, the preferred surgical technique is balloon valvuloplasty. When performing it, a wide incision of the chest is not performed. An inflatable balloon is passed through the main blood vessel to the affected area, which is fixed in the area of ​​narrowing and inflated. The balloon expands the stenotic area, ensuring normal blood flow in the pulmonary artery and the lungs themselves.

Predictions and prevention

The prognosis for mild stenosis is favorable. As a rule, the patient has no symptoms of the disease and treatment is not required. The patient needs dynamic monitoring of his condition, but in general he lives a full life. The average life expectancy of patients with moderate disease is 20-30 years in the absence of the necessary artery correction. Patients with severe stenosis without surgery die in the first years of life. Timely surgical intervention in most cases makes the prognosis favorable, allowing the patient to lead a full life.

Preventive measures to prevent pulmonary artery stenosis have not been developed. Women of childbearing age are advised to avoid exposure to chemicals, radiation, and drugs.

Parents' regular sports activities, healthy lifestyle, and regular preventive medical examinations somewhat reduce the likelihood of a child developing pathology.

The pulmonary artery circulates venous blood from the right ventricle of the heart to the lungs. There are three separate valves along the path of blood flow. Impaired circulation leads to problems in the functioning of the heart, lungs and brain.

Valvular pulmonary stenosis occurs in 10% of cases of congenital heart failure and heart defects. A fairly high percentage of patients suffer from an acquired form of the disease.

What is pulmonary artery stenosis?

Pulmonary valve stenosis is the most common condition in newborns. The disease has the following clinical picture. Vessel narrowing increases pressure in the right ventricle. Pulmonary stenosis in children causes the heart muscle to work harder to maintain normal blood flow. As a result, the so-called “heart hump” is formed. The cause of the development of the disease in a newborn is a genetic factor.

It is almost impossible to diagnose minor stenosis during childbirth. The newborn baby has no cyanosis and a normal heart rhythm can be heard.

If there is no tendency to reduce the lumen, no additional therapy is required. The average life expectancy is identical to that of an ordinary healthy person.

Severe congenital stenosis manifests itself with a clear clinical picture and manifestations. The prognosis of the disease is extremely unfavorable. If surgical treatment is not performed, the child will die within a year.

Stenosis in adults is somewhat different in terms of clinical picture from what is diagnosed in children. The development of changes in the structure is indicated by characteristic symptoms and signs:

• Complaints of pain in the chest area.
• The appearance of blueness of the lips, discoloration of the fingertips.
• Pulsation of the veins of the cervical region.
• Development of chronic fatigue.
• Exacerbation of symptoms when lifting weights and performing strenuous physical work.

When conducting a diagnostic study, noise during stenosis can be clearly heard in the interscapular space. Another characteristic feature that helps in differential diagnosis is the absence of blood pressure surges.

How dangerous is the disease?

The prognosis of stenosis depends on the stage of development of the disease, the localization of the narrowing of the lumen and timely detected pathology.

It is customary to classify four stages of disease development:

1. Moderate stenosis - at this stage there are completely no complaints about poor health, the ECG shows initial signs of overload of the right ventricle. Moderate stenosis can go away on its own; the prognosis for therapy is positive.
2. Severe stenosis - the stage is characterized by significant narrowing of blood vessels, as well as an increase in systolic pressure in the right ventricle to 100 mmHg.
3. Severe or acute stenosis - valve insufficiency, circulatory disorders, high pressure in the right ventricle more than 100 mm Hg are diagnosed.
4. Decompensation - myocardial dystrophy develops, circulatory disorders become irreversible. If surgery is not performed, pulmonary edema and cardiac arrest occur. The prognosis is unfavorable. Surgery does not guarantee a return to normal activities.

In addition to the stages of development, the localization of stenosis also affects the prognosis of therapy. Based on this feature, it is customary to distinguish the following types of disease:

• Supravalvular stenosis - in most cases, pathological disorders of the valve structure are observed. Stenosis forms at the top of the artery. Accompanied by rubella and Williams syndrome (the patient acquires elongated facial features).
• Subvalvular stenosis - characterized by a funnel-shaped narrowing, in combination with a muscle bundle, preventing the ejection of blood from the right ventricle.
• Infundibular stenosis - occurs as an additional symptom of right ventricular valve disorders. It can also exist independently of the first disease. Combined pulmonary artery stenosis complicates the treatment of the disease and reduces the chances of a favorable treatment outcome.
• Peripheral stenosis - the pathology is characterized by multiple vascular lesions. The disease is not amenable to traditional surgical treatment.
• Isolated stenosis - refers to congenital heart defects. With moderate development, surgical and drug treatment is not required. In acute cases, surgery is performed.
• Residual stenosis - during contraction of the ventricles, a certain amount of blood remains in them. This leads to impaired blood circulation. The pathology is congenital.

Mild stenosis usually does not manifest itself and does not require therapy. The patient must undergo regular examination for the development of disorders. If clinical manifestations occur, surgery is required.

How to cure this pathology

Surgical elimination of valve stenosis is the only possible method of therapy. The absolute indication for surgery is a congenital defect, the consequence of which is significant circulatory disorders.

Thus, transposition of the great vessels (two main arteries are changed places) can be eliminated exclusively by radical surgery. The same applies to other congenital pathologies.

In adults, surgical intervention is recommended if antenatal diagnosis shows the presence of vascular decompensation. As a recommended measure, surgery is performed for severe or acute stenosis. Medications are prescribed only during the preoperative preparation period.

Prevention and prevention

Prevention of stenosis has no characteristic features and is carried out in the same way as for any other pathology of the cardiovascular system. The patient is recommended to change his lifestyle and give up bad habits, including smoking and alcohol.

A therapeutic diet and physical education classes are also prescribed. These measures will help reduce excess weight and get rid of bad cholesterol in the blood.

Treatment with folk remedies is effective in preventing atherosclerosis and maintaining the tone of the vascular system.

If you eat just half a glass of raw seeds a day, you can quickly get rid of bad cholesterol in the blood. Taking a handful of chokeberry berries daily can normalize blood pressure without taking medications.

Congenital or acquired pulmonary artery disease is treated exclusively by surgery. Since surgery carries a lot of risk, you should not hastily agree to the operation.

VALVE PULMONARY ARTERY STENOSIS honey.
Isolated stenosis of the pulmonary artery valve (PA) - CHD, characterized by an obstruction to the flow of blood at the level of the pulmonary valve. Frequency - 10-12% of patients with congenital heart disease.

Classification

Stage I - moderate stenosis. No complaints. ECG - initial signs of right ventricular (RV) overload. Systolic pressure in it is up to 60 mm Hg.
Stage II - severe stenosis with characteristic clinical manifestations. Systolic pressure in the RV -60-100 mm Hg.
Stage III - severe stenosis with right ventricular pressure more than 100 mm Hg. Severe course, signs of circulatory disorders.
Stage IV - decompensation. Characterized by myocardial dystrophy and severe circulatory disorders. Systolic pressure in the RV may not be very high, because contractile insufficiency develops.

Etiology

Hereditary disease
Embryopathy caused by rubella acquired in utero. Pathological anatomy
Initially, valvular stenosis of the pulmonary artery is formed as a result of fusion of the valve leaflets without any disturbances in the development of the outflow tract of the pancreas. However, when the pressure in the pancreas exceeds 200 mm Hg, its outflow tract eventually undergoes severe fibrosis and becomes the second level of stenosis
Typically, the valve leaflets fuse at the edges to form a membranous diaphragm.
In mild clinical cases, the lumen diameter is more than 1 cm
In severe cases - less than 3-4 mm
As a result of the impact of the blood flow passing through the narrowed opening of the valve, post-stenotic dilatation of the pulmonary artery occurs.
In extremely rare cases, valve calcification may occur in adult patients.
Pathological physiology. Hemodynamic disorders are caused by an obstruction in the path of blood flow from the pancreas to the pulmonary artery
Compensatory increase in pressure in the pancreas with an increase in its work
Pressure in the pancreas begins to increase if the area of ​​the pancreatic outlet is reduced by 40-69% of normal
With severe stenosis, the pressure reaches 200 mm Hg. or more, and the work of the ventricle increases 5-8 times
The critical area of ​​the valve bore is 0.15 cm2
PA pressure remains normal
Proper RV output is maintained by prolonging the ejection period
As diastolic pressure in the RV increases, systolic pressure in the right atrium (RA) increases. As a result, the oval window may open, and then blood from the RA enters the left one and cyanosis develops.

Clinical picture

Complaints
Shortness of breath, which initially occurs during physical activity, and in severe cases at rest
Heart pain (occurs in teenagers).
Appearance of patients
The color of the skin usually does not change. In some patients, if the oval window remains open, moderate cyanosis (bluishness of the lips) is possible.
Heart hump
Swelling and pulsation of neck veins
Systolic tremor in the LA projection (II intercostal space to the right of the sternum)
Pulsation in the epigastric region (increased pancreatic impulse).
Percussion: expansion of the borders of the heart to the left and right.
Auscultation: heart murmur detected from birth
Rough systolic murmur in the II-III intercostal spaces. The noise is directed towards the left clavicle, well auscultated in the interscapular space
The 1st tone is sharply enhanced. Strengthening of the first tone is not noted with right ventricular failure or with pronounced stenosis
II tone above the LA is weakened or absent
Sometimes a vague diastolic murmur is heard, indicating concomitant insufficiency of the pulmonary valves.
Pulse and blood pressure do not change.

Special studies

X-ray of the heart in 3 projections
Anteroposterior projection: enlargement of the heart to the right and left, expansion of the pulmonary trunk in combination with a normal or depleted pulmonary pattern
First oblique projection: the shadow of the heart is expanded posteriorly, the contour of the contrasted esophagus is not displaced (a sign of an increase in the RA)
Second oblique projection: the shadow of the heart is expanded anteriorly (due to the enlargement of the right ventricle).
The ECG reflects the degree of overload and hypertrophy of the right heart
With slight stenosis, the EOS maintains its normal position
Detect sinus rhythm and periodic supraventricular arrhythmias
Right heart overload increases as RV pressure increases
In this case, the EOS deviates to the right and angle a changes from +70° to +150°
R wave amplitude may exceed 20 mm
A downward shift of the S-T interval and a negative T wave in the right precordial leads indicate an extreme degree of overload.
Echocardiography
Significant expansion of the pancreas cavity
Poststenotic dilatation of the pulmonary trunk
Color Doppler scanning allows you to evaluate the pressure difference between the RV and PA.
Right heart catheterization
The pressure in the pancreas and the pressure difference between it and the pulmonary trunk are determined. Based on the data obtained, it is possible to diagnose a combination of valvular and subvalvular stenoses
In case of valvular stenosis, at the moment of removal of the probe from the PA into the ventricle, a sharp rise in systolic pressure is recorded
When valvular stenosis is combined with subvalvular stenosis, an intermediate zone with higher systolic pressure than in the PA, but zero diastolic pressure is determined on the pressure curve
Blood oxygen saturation in the chambers of the heart is within normal limits
Selective angiocardiography in the lateral projection - direct signs of valvular stenosis (a clearing strip between the contrasted RV and PA).

Differential diagnosis

Atrial septal defect
Riveted PA stenosis (RV conus arteriosus stenosis)
Supravalvular stenosis of the pulmonary artery (stenosis of the pulmonary trunk throughout)
Tetralogy of Fallot and other complex defects, incl. PA stenosis.
Treatment:

Surgery

Elimination of valve stenosis is the only effective method of treating the defect.
Indications for surgery are stages II and III of the disease.
Relative contraindication - stage IV. However, if drug therapy can reduce the severity of circulatory failure, it is possible to perform lighter intervention options.
Types of operational benefits
Closed surgery - percutaneous balloon valvotomy
Performed transvenously during cardiac catheterization with a special probe equipped with a balloon
The method is not effective enough for valve stenosis complicated by fibrosis of the pancreatic outflow tract
Open valvotomy, performed both under conditions of artificial circulation and moderate hypothermia.
Postoperative mortality is 0.5-1.5%.

Drug therapy

Used for preoperative preparation or instead of surgery in stage IV
PM - see.
Patient management
Regular medical supervision of patients who have not undergone surgical correction
Prevention of infective endocarditis in all patients
Identification of carriers of streptococcal infection with sanitation of chronic foci
Prophylactic antibiotic therapy during any surgical procedures
After surgery, annual repeat echocardiography is recommended.

Synonyms

Valvular pulmonary stenosis See also, Tetralogy of Fallot, Infectious endocarditis

Abbreviations

PA - pulmonary artery
RV - right ventricle
RA - right atrium

ICD

137.0 Pulmonary valve stenosis

Directory of diseases. 2012 .

See what "PULMONARY ARTERY VALVE STENOSIS" is in other dictionaries:

Honey. Mitral stenosis (MS) is a pathological narrowing of the left atrioventricular orifice caused by fusion of the mitral valve (MV) leaflets and narrowing of its fibrous ring. Frequency 0.05 0.08% of the population. The predominant age is 40–60 years… Directory of diseases

Honey. Aortic stenosis is a heart defect in the form of narrowing of the aortic opening due to pathology of the aortic valve and perivalvular structures. Frequency Isolated pure aortic stenoses are observed in 1.5 2% of cases of acquired valvular heart defects... ... Directory of diseases

The disease is a specific disease that is accompanied by a narrowing in the area of ​​the outflow tract of the right ventricle from the side of the valve located in the pulmonary artery. Such a change creates a significant obstacle, and it is through it that the ventricle has to pump blood with considerable effort, which can, in turn, affect a person’s quality of life.

Types of disease

Among all known congenital heart defects, isolated stenosis is very common, which is about 12%. Valvular stenosis is the most common, although sometimes combined stenosis can occur, which occurs along with supravalvular or subvalvular stenosis, as well as with other known congenital heart diseases.

In almost 90% of cases of this disease, stenosis is diagnosed as valvular. The remaining 10% consists of the subvalvular and supravalvular types.

Valvular stenosis is characterized by the absence of division of the valve itself into certain leaflets and its acquisition of the shape of a diaphragm in the form of a dome with an opening of up to 10 mm. Subvalvular stenosis is accompanied by an abnormal proliferation of both fibrous tissue and muscle with a funnel shape that narrows towards the outflow tract in the right ventricle. Supravalvular stenosis is characterized by local narrowing, the presence of several peripheral stenoses, etc.

The disease is classified according to the level of blood pressure and the pressure gradient (difference) occurring in the ventricle and pulmonary artery:

• Stage 1 - at the first stage, moderate stenosis of the pulmonary artery is determined, blood pressure is up to the limit of 60 mm Hg. with gradient indicators with an extreme point of up to 30 mm Hg;
• Stage 2 - at the second stage, the diagnosis becomes stenosis of the pulmonary artery orifice of a pronounced form with a pressure limit of up to 100 mm Hg. and with a gradient - up to 80 mm Hg. Art.;
• Stage 3 - at this stage the disease is defined as stenosis of the pulmonary artery with severe severity, the pressure indicator is more than 100 mm Hg. Art. with a gradient over 80 mm Hg;
• Stage 4 is the most serious stage of the disease, in which myocardial dystrophy often begins to develop and a disturbance in general circulation occurs, but the increased pressure in the ventricle decreases due to the appearance of insufficient contractions in it.

Symptoms of the disease

Pulmonary stenosis manifests itself differently depending on the level of development of the disease. It is influenced by indicators such as pressure in the right ventricle and the gradient indicated above. With low and unexpressed indicators indicated above, symptoms or complaints may be completely absent.

At an advanced stage of the disease, the following manifestations may be observed:

• fatigue, which appears very quickly even with minor exertion;
• general weakness;
• drowsiness and dizziness;
• shortness of breath and palpitations;
• cases of fainting;
• attacks and manifestations of angina pectoris in a more severe form of the disease.

When examining the patient in this case, the doctor attracts attention to the pulsation and discharge of the neck veins, pale skin, systolic chest trembling, and the presence of the so-called cardiac hump.

A decrease in cardiac output leads to cyanosis of the lips, phalanges and cheeks.

Pulmonary valve stenosis is a disease that can also occur in children. Often its manifestation is a lag in physical development, which manifests itself in low body weight and short stature.

Children suffering from this disease are characterized by frequent colds, as well as the development of pneumonia. In some cases, the disease also occurs in newborns, which may be caused by the presence of this disease in the mother.

Its presence can be noticed already during pregnancy by noise or as a result of x-rays, which indicate an enlargement of the heart from the right ventricle. In newborns, the disease may not acquire complicated forms, so they are quite capable of not negatively affecting their usual way of life.

If the form of the disease is moderate or severe, cyanosis appears from the first days, that is, a blue color in the nasolabial area, on the nails and lips. Unfortunately, if treatment is not applied, such children can die in the first year of their life.

Diagnosis of the disease

To determine pulmonary artery stenosis, it is necessary to conduct a series of examinations, which include analysis, as well as comparison of the data obtained. Not the last thing in making a diagnosis are the results of the so-called instrumental diagnostics.

With this disease, there is a displacement of the borders of the heart to the right side, and upon palpation, systolic pulsation in the ventricle on the right is noticeable. According to the data obtained after auscultation and, a rough and strong noise is heard, the second sound in the pulmonary artery weakens and then completely splits.

X-ray indicates stenosis of the pulmonary artery mouth if there is an expansion of the cardiac boundaries against the background of depletion of the pulmonary image.

An ECG can help determine the load on the ventricle. The use of echocardiography in the presence of disease often shows ventricular dilatation along with pulmonary artery dilatation.

Dopplerography is used to determine the difference in pressure between the ventricle and pulmonary trunk.

The pressure indicator can be easily checked by probing in the right side of the heart. This method is also used to determine the gradient. In the presence of chest pain in patients over 40 years of age, selective coronary angiography is indicated.

If there are symptoms of the disease in children, for the purpose of accurate diagnosis, the following is used:

• an x-ray that shows changes in the lungs;
• ECG - in order to determine overload in the right side of the heart;
• echocardiogram - for a final conclusion indicating the extent of the disease.

The most important thing in diagnosing a disease is to distinguish it from such similar diseases as defects in the area of ​​the septa of the heart chambers, Fallot's triad, Eisenmenger complex, etc.

Treatment

The most common and most effective method of treating this disease remains surgery, which involves eliminating the stenosis. It is worth considering that this operation is indicated only if pulmonary artery stenosis has reached stage 2 or 3.

Valvular stenosis is treated with open valvuloplasty, in which the fused commissures are cut. Balloon valvuloplasty (endovascular) is also widely used. This is a method of intravascular elimination of stenosis, which is carried out using an inflatable balloon and a catheter.

In case of supravalvular stenosis, reconstruction of the area of ​​narrowing is necessary using a prosthesis (xenopericardial) or a patch. Subvalvular stenosis requires infundibulectomy. This process is the removal of hypertrophied tissue (muscle) that has appeared in the area of ​​the outflow tract of the right ventricle.

Any form of stenosis is quite capable of causing serious complications that affect life expectancy. Therefore, diagnosis and treatment must be carried out in a timely manner. Despite this, even surgical intervention can be dangerous due to consequences such as the development of pulmonary valve insufficiency.

Treatment for children and the basis for its choice are based on the level of narrowing of the pulmonary artery. Surgical intervention may not be prescribed only if the disease is mild or in the absence of any complaints. If they are, treatment should be applied urgently. Usually the operation is performed at the age of 5-10 years.

In severe cases of the disease, surgery can be performed immediately. For children, a procedure such as balloon valvuloplasty or surgical reconstruction is used. Overall, this treatment produces good results and a low mortality rate.. To a small extent, it is reflected in the ordinary life of the child, who after 3 months may well return to school.

Any physical activity is limited for a period of up to two years.

Prevention of the disease and prognosis for the future

Pulmonary artery stenosis, regardless of whether it occurs in adults or children, with a minor form has virtually no effect on the quality or length of life. If we talk about a significant form of the disease from a hemodynamic point of view, then it leads to the early development of failure of the right ventricle. This, in turn, can result in sudden death.

Statistics show that as a result of the operation, 91% of patients have an average life expectancy of at least another 5 years. In the absence of severe symptoms in adult patients, surgery can be postponed for a certain period.

To prevent the occurrence of this disease, especially in children, it is necessary to provide all the necessary conditions for the normal course of pregnancy of the expectant mother. In addition, all measures must be taken to ensure timely recognition of the disease, as a result of which appropriate treatment can be prescribed.

It can also be aimed at eliminating those diseases that caused the changes acquired by the child. Each patient must be observed by specialists such as a cardiologist and cardiac surgeon, and also take all measures to prevent the development of infective endocarditis.