Moderate valvular stenosis of the pulmonary artery. Pulmonary stenosis life expectancy

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

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

What is pulmonary artery stenosis

The most common valvular stenosis of the pulmonary artery in newborns. The disease has the following clinical picture. The narrowing of the vessel increases the pressure in the right ventricle. Pulmonary stenosis in children causes the heart muscle to exert great effort to maintain normal blood flow. As a result, the so-called "heart hump" is formed. The causes of the development of the disease in a newborn is a genetic factor.

Minor stenosis is almost impossible to diagnose during childbirth. The newborn child has no cyanosis, a normal heart rhythm is heard.

In the absence of a tendency to reduce the lumen, no additional therapy is required. The average life expectancy is identical to an ordinary healthy person.

Congenital stenosis in severe form is manifested by a clear clinical picture and manifestations. The prognosis of the disease is extremely unfavorable. If you do not carry out surgical treatment, the child will die within a year.

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

• Complaints of pain in the chest.
• The appearance of cyanosis of the lips, discoloration of the fingertips.
• Pulsation of the veins of the cervical region.
• development of chronic fatigue.
• Exacerbation of symptoms with heavy lifting and heavy physical work.

When conducting a diagnostic study, the noise during stenosis is well audible in the interscapular space. Another characteristic feature that helps in differential diagnosis is the absence of jumps in blood pressure.

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 the timely detected pathology.

It is customary to classify four stages of the development of the disease:

1. Moderate stenosis - at this stage, there are no complaints of poor health, the ECG shows the initial signs of overload of the right ventricle. Moderate stenosis can pass on its own, the prognosis of therapy is positive.
2. Severe stenosis - the stage is characterized by a significant vasoconstriction, as well as an increase in systolic pressure in the right ventricle up to 100 mm Hg.
3. Sharp 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 you do not perform a surgical operation, there is pulmonary edema, cardiac arrest. The prognosis is unfavorable. Surgery does not guarantee a return to normal life.

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

• Supravalvular stenosis - in most cases, pathological disorders of the structure of the valve are observed. Stenosis is formed in the upper part 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 that prevents the ejection of blood from the right ventricle.
• Infundibular stenosis - occurs as an additional symptom of right ventricular valve disorders. It can also exist regardless of the first disease. Combined pulmonary artery stenosis complicates the treatment of the disease and reduces the chances of a favorable outcome of treatment.
• Peripheral stenosis - 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 medical treatment is not required. In the acute form, surgery is performed.
• Residual stenosis - during the contraction of the ventricles, a certain amount of blood remains in them. This leads to impaired blood circulation. The pathology is congenital.

Mild stenosis, as a rule, does not manifest itself and does not need therapy. The patient needs to undergo regular examination for the development of violations. When clinical manifestations occur, surgery is required.

How to cure this pathology

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

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

For adults, surgical intervention is recommended if antenatal diagnosis has shown the presence of vascular decompensation. As a recommended measure, surgery for severe or acute stenosis. Medications are prescribed only in the period of preoperative preparation.

Warning 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 a change in lifestyle, giving up bad habits, including smoking, 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 as a prevention of atherosclerosis and maintaining the tone of the vascular system.

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

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

Pulmonary stenosis in children is a congenital anomaly in the development of a large arterial vessel that delivers venous blood from the right ventricle of the heart to the lungs. Refers to the number of congenital malformations of the cardiovascular system. The frequency of occurrence is about 12% of the total number of identified cases of CHD. Most newborns with severe stenosis die within the first year of life if the defect is not corrected by surgery.

What is stenosis and why is it dangerous?

Stenosis of the pulmonary artery is a narrowing of the vessel, which can have a different degree of severity and localization. According to the degree of severity, weak, moderate and severe damage to the artery is distinguished.

According to localization, stenosis is divided into:

1. Valvular - narrowing occurs on the area of ​​​​the arterial valve. In this case, the valve itself can be single-leaf, three-leaf or double-leaf. Behind the stenotic area is usually a zone of expansion of the vessel. Valvular stenosis of the pulmonary artery accounts for about 90% of the total number of cases of the disease.
2. Subvalvular - narrowing of the blood outlet line under the arterial valve and the formation of a muscle bundle that prevents the ejection of blood from the heart.
3. Supravalvular - isolated stenosis of the pulmonary artery, proceeding according to the supravalvular type, can have several morphological forms.
4. Stenosis of the mouth of the pulmonary artery is a narrowing of the passage directly between the ventricle and the trunk of the pulmonary artery.

The presence of constrictions reduces the capacity of the artery, which leads to the incomplete fulfillment 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 leads to the opening of the oval window and the discharge of excess blood into the left half of the organ. In this case, the patient has clinical signs of heart disease.

On a note: a weak degree of severity of valvular 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 a full life. The life expectancy of patients practically does not differ 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 the recessive gene of the disease with the dominant gene of the normal trait does not lead to the development of disorders).

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

The narrowing of the mouth of the pulmonary artery can also occur in adulthood, which does not allow the pathology to be attributed to the number of congenital malformations. The reason for this may be infective endocarditis, as well as myxomas, carcinoids and other tumor processes of the heart.

Video

Video - pulmonary artery stenosis

Symptoms and Diagnosis

Stenosis of the pulmonary artery in newborns manifests itself in the form of respiratory failure. The child has acrocyanosis, which can subsequently turn into generalized cyanosis, severe shortness of breath, periodic loss of consciousness. A similar picture takes place 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 trembling.

Signs of the disease can be manifested 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 heart cavities.

The main sign of stenosis, detected during auscultation, is a rough murmur at the time of systole, heard in the second intercostal space, as well as in the region of the left clavicle and on the back. The moment of appearance of noise 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 the atrium on the same side. Strong constrictions are the cause of supraventricular tachycardias, weak ones may not lead to ECG symptoms.

Echocardiography and catheterization of the cardiac cavities allows you 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 taper corresponding to them are shown in the following table:

X-ray allows you to detect the expansion of the artery that occurs with valvular congenital stenosis. An indirect sign of the disease is the depletion of the pattern of the lungs in the picture.

Note: Catheterization of the heart cavities is a research method associated with the introduction 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, CPS is used only when other examination methods are ineffective or in preparation for surgery. The introduction of a catheter during the initial diagnosis is accompanied by an unreasonable risk.

Treatment

Treatment of pathology of the pulmonary artery in a newborn is only surgical. In severe forms of the disease, the operation can be performed in the first days, and sometimes even hours of the child's life. This happens only 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 technique of surgical intervention may vary depending on the capabilities of the clinic and the qualifications of the surgeon. 15-20 years ago, the most common was the open method of manipulation, in which the cardiac surgeon had to work on the open heart. This method was dangerous and had high mortality rates.

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

Forecasts and prevention

The prognosis for mild stenosis is favorable. As a rule, the patient has no symptoms of the disease, 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 an average degree of the disease is 20-30 years in the absence of the necessary correction of the artery. 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 stenosis of the pulmonary artery have not been developed. Women of childbearing age are advised to avoid exposure to chemicals, radiation, and drugs.

Somewhat reduce the likelihood of developing a pathology in a child, regular exercise by parents in sports, their healthy lifestyle, regular preventive medical examinations.

Stenosis (narrowing of the lumen) of the pulmonary artery is the most common type of heart disease that occurs in newborns and children. About 10% of children born with a heart defect have this particular pathology.

Stenosis of the pulmonary artery is characterized by a decrease in the lumen of the blood vessel in the region of the pulmonary valve. As a result, the outflow of blood from the right ventricle worsens. The pulmonary artery is of great importance in the circulatory system. It provides blood flow to the lungs to saturate it with oxygen. When the pulmonary artery narrows, the load on the right ventricle increases. This entails its hypertrophy. As a result, the blood supply to the pulmonary artery slows down, which causes heart failure.

Pathology is congenital and acquired. In the case of CHD (congenital heart disease), pulmonary stenosis is defined as valvular stenosis.

The reasons

The main factors affecting the development of congenital stenosis are:

• intoxication of the mother's body during pregnancy,
• transferred rubella,
• heredity.

The causes of acquired pathology can be:

• infectious endocarditis,
• myxoma of the heart
• formation of carcinoids
• aortic aneurysm,
• ventricular hypertrophy,
• enlarged lymph nodes.

By localization, pulmonary stenosis can vary in:

• valve,
• subvalvular,
• mixed.

The disease has four stages:

• moderate
• expressed,
• pronounced,
• the stage of decompensation, which is characterized by a serious violation of blood circulation.

Symptoms

Depending on the stage of the disease, clinical manifestations may be different. In the early stages of the development of pathology, symptoms are invisible and often absent altogether.

With a slight narrowing of the artery, there will be practically no manifestations. You can detect the disease after a few years by chance during a routine examination by a cardiologist or pediatrician.

In newborns with stenosis, congestive heart failure is noted, and shortness of breath is observed even at rest. With a lumen of 1 mm, an urgent surgical operation is required. Otherwise, death may occur.

The first signs of pulmonary stenosis are:

• shortness of breath even with minor physical exertion,
• fatigue,
• heart murmur,
• general weakness,
• frequent dizziness,
• fainting
• pain in the region of the heart,
• swelling and pulsation of the cervical veins.

Examination may reveal a heart hump. Symptoms tend to get worse during physical activity. Therefore, for children with such a diagnosis, sports and physical education are contraindicated.

If during a routine examination by a pediatrician, a heart murmur was detected in a baby, do not panic. An accurate diagnosis can only be made by a cardiologist based on research results.

Diagnosis of pulmonary stenosis

To diagnose the disease, you must do the following:

• FCG (phonocardiography) to study the presence and type of heart murmurs;
• ECG (echocardiography), which allows to detect right ventricular hypertrophy;
• x-ray showing enlargement of the heart.

Analyzes of the data obtained allow you to make an accurate diagnosis and determine the degree of stenosis. Only after that treatment is prescribed.

If you are in doubt about the correctness of the diagnosis, contact several specialists in the field of cardiology.

Complications

Whatever the form of stenosis, the disease can cause serious problems that affect the duration and quality of life. Timely diagnosis and operation will help to avoid these problems.

Why is pulmonary stenosis dangerous? This pathology is the cause of the development of myocardial dystrophy. Without treatment, the narrowing of the lumen of the pulmonary artery can lead to consequences such as stroke, myocardial infarction, and right ventricular failure. Without surgery, a patient with stenosis dies within five years.

If your child is diagnosed with pulmonary stenosis, do not panic. Timely and adequate treatment will allow your baby to develop harmoniously along with peers and live a full life for many years.

Treatment

What can you do?

With a slight severity of narrowing of the pulmonary artery, special therapeutic measures are not carried out. Treatment is symptomatic. If the disease progresses, the degree of stenosis increases, the child is shown a surgical operation. The most favorable age for holding is the period from 5 to 10 years.

What does a doctor do?

Depending on the stage and type of stenosis, the treatment method is selected. With valvular stenosis, an operation is performed using the method of open or balloon valvuloplasty.

Supravalvular stenosis is treated by reshaping the area of ​​narrowing with a prosthesis or patch. As a rule, the prognosis is favorable.

The rehabilitation period for children is about three months. Physical activity is allowed two years after the operation. Without treatment, significant developmental stenosis can lead to right ventricular hypertrophy and heart failure. This can lead to death.

If stenosis was detected in the fetus during ultrasound and dopplerography, do not despair. The operation can be performed after the birth of the baby, and he will be able to live a full life. Identified stenosis in the fetus will allow you to prepare in advance for the operation and take emergency measures in a timely manner.

Prevention

To prevent the development of pulmonary artery stenosis in a child, during pregnancy, the expectant mother must carefully monitor her health. Timely diagnosis of the disease will allow choosing the optimal and effective treatment without the risk of further development of pathology.

If pulmonary stenosis is suspected, the child must be observed by a cardiologist and follow his recommendations for the prevention of infective endocarditis.

Pulmonary artery stenosis is a disease that is considered one of the types of congenital heart disease (CHD) and is characterized by a violation of the outflow of blood from the right ventricle of the heart in the region of the pulmonary artery, where there is a significant narrowing. There are several types of stenosis:

• valve;
• subvalvular;
• supravalvular;
• combined.

In 90% of all patients, valvular stenosis of the orifice of the pulmonary artery is diagnosed. Depending on the severity of the disease, several stages are distinguished:

• light;
• average;
• heavy.

For practical purposes, doctors use a classification based on the level of determination of systolic blood pressure in the right ventricle of the heart and the pressure gradient between the right ventricle and the pulmonary artery:

1. I moderate degree - systolic blood pressure 60 mm Hg, gradient - 20-30 mm Hg.
2. II moderate degree - systolic blood pressure 60-100 mm Hg, gradient - 30-80 mm Hg.
3. ІІІ pronounced degree - systolic pressure is more than 100 mm Hg, and the gradient exceeds 80 mm Hg.
4. IV decompensatory stage - insufficiency of the contractile function of the ventricle of the heart develops, myocardial dystrophy occurs, the pressure in the ventricle falls below normal.

Causes and features of hemodynamics

In order to prescribe an adequate treatment for stenosis, it is necessary to know the cause and mechanism of the development of the disease. According to the mechanism of development, two types of stenosis are distinguished:

• congenital;
• acquired.

The cause of congenital stenosis of the pulmonary artery may be a genetic predisposition, fetal influence on the intrauterine development of the fetus by chemicals, medicines, and certain infections such as rubella.

Stenosis of the mouth of the pulmonary artery may be acquired. The cause of such a pathological condition is infectious (syphilitic, rheumatic) lesions, oncological neoplasms, enlarged lymph nodes, aortic aneurysm.

This disease is characterized by a violation of hemodynamics, which is associated with the presence of obstacles in the path of blood flow from the right ventricle to the pulmonary trunk. The functioning of the heart with a constant load leads to myocardial hypertrophy. The smaller the area of ​​the outlet of the ventricle, the higher the systolic blood pressure in it.

Symptoms of the disease

The clinical picture of the disease directly depends on the severity and type of stenosis. If the systolic blood pressure in the ventricle does not exceed 75 mm Hg, the symptoms of the disease may be practically absent. When the pressure builds up, the first symptoms of the disease may appear in the form of dizziness, fatigue, heart palpitations, drowsiness and shortness of breath.

With congenital stenosis, children may experience a slight lag in physical and mental development, a tendency to colds, fainting. With IV decompensated degree, frequent fainting and angina attacks are possible.

If pathological processes increase, and there is no treatment, then an oval window may open, through which veno-arterial blood is discharged.

During the examination of a patient with stenosis of the pulmonary trunk, it is objectively possible to identify: cyanosis of the extremities or the whole body, the skin is pale, the veins in the neck swell and pulsate.

In the chest, you can notice the presence of systolic trembling, the appearance of a heart hump, cyanosis of the limbs or the whole body.

Most often, in the absence of adequate treatment, the patient may develop heart failure or septic endocarditis, which can lead to death.

Diagnostics

The life of the patient depends on the timeliness of treatment and diagnosis. In order to clarify the diagnosis, the doctor will prescribe the following methods of additional research:

• general and biochemical analysis of blood and urine;
• Ultrasound of the heart;
• x-ray examination;
• phonocardiography;
• echocardiography;
• sounding.

Isolated pulmonary stenosis should be differentiated with diseases such as Fallot's tetrad, open ductus arteriosus, and atrial septal defect.

Treatment Methods

Isolated pulmonary stenosis is treated with surgery alone. At the initial stage of the disease, when there are no obvious signs, there is no need for the use of expensive drugs and operations. But when the severity of the disease prevents the patient from leading a normal active lifestyle, then surgical treatment of pulmonary artery stenosis gives a chance to prolong and maintain the patient's quality of life. This is especially true for children with congenital heart disease (CHD).

An indication for urgent surgical intervention (valvuloplasty) is an indicator - a pressure gradient between the pulmonary artery and the right ventricle, which is more than 50 mm Hg.

Depending on the severity and type of disease, valvuloplasty is performed in several ways:

Open valvuloplasty is an abdominal surgery performed under general anesthesia using a heart-lung machine. This type of surgical treatment perfectly restores hemodynamics, but is dangerous for the development of complications in the form of pulmonary valve insufficiency.

Closed valvuloplasty is a surgical intervention in which a valvulot is used, which excised excess tissue that impedes normal blood flow.

Balloon valvuloplasty is considered the safest method of surgical treatment.

Balloon valvuloplasty is the least traumatic method of treatment, in which there is no need to make abdominal incisions, but it is enough to make several small punctures in the thigh area.

Forecast and prevention

Pulmonary artery stenosis is a serious disease that, if left untreated, can be fatal. For adult patients, minor disorders in hemodynamics do not affect general well-being. Stenosis of the pulmonary trunk in children requires constant monitoring and repeated surgical intervention.

When identifying such a diagnosis as pulmonary stenosis, do not panic. Today, medicine does not stand still and with proper care and treatment, patients can lead quite an active lifestyle. As practice shows, after surgical treatment, the five-year survival rate is 91%, which is a good indicator.

Prevention of pulmonary stenosis is:

1. Leading a healthy and active lifestyle.
2. Creating ideal conditions during pregnancy.
3. Early diagnosis and treatment of diseases.
4. When the first signs of the disease appear, you should not self-medicate, but it is better to immediately seek qualified medical help.
5. After treatment, patients should be under the supervision of a cardiologist, strictly following all recommendations.

Remember! Health is the greatest value given to us by nature, which must be protected!

Isolated pulmonary stenosis occurs in 2.5-2.9% (Abbott, 1936; Galus, 1953) of cases of congenital heart anomalies. In 10% of cases, it is combined with other heart defects (J. Grinevetsky, J. Moll, T. Stasinski, 1956). Isolated stenosis of the pulmonary artery refers to congenital malformations of the "pale", aciano type.

The pulmonary artery narrows at the level of the valves (valvular stenosis) or under the valves, in the region of the right ventricular outflow tract - subvalvular, or infundibular, stenosis. In 80% of cases, valvular stenosis occurs and in 20% - subvalvular stenosis (A. A. Vishnevsky, N. K. Galankin and S. Sh. Kharnas, 1962).

Difficulty in the exit of blood from the right ventricle into the narrowed pulmonary artery leads to its hypertrophy (Fig. 14). Over time, right ventricular failure develops.

Rice. 14. Narrowing of the mouth of the pulmonary artery (scheme). 1 - narrowing of the mouth of the pulmonary artery (43% of cases - at the level of the valve of the pulmonary artery, 43% - in the area of ​​the cone of the pulmonary artery, 14% - simultaneous narrowing in the area of ​​the valve and the cone); 2 - dilatation and hypertrophy of the right ventricle.

During intrauterine life, even significant pulmonary stenosis does not create a load on the fetal circulatory system, and therefore, at birth, the heart usually has a normal size. After the birth of a small or moderate degree, stenosis often does not cause a pronounced increase in the right ventricle; with significant stenosis, as a rule, there is a gradual increase in the right ventricle.

Thus, the size of the right ventricle to a certain extent serve as an indicator of the degree of narrowing of the pulmonary artery. Often there is also an increase in the right atrium.

The value of systolic pressure in the right ventricle can reach 300 mm Hg. Art. instead of the normal 25-30 mm Hg. Art.

The clinical picture of the defect is varied and depends on the degree of stenosis. Minor pulmonary stenosis proceeds favorably, often without causing subjective disorders. Such patients live to old age, maintaining full working capacity.

With an average degree of stenosis of the pulmonary artery, usually during puberty, shortness of breath occurs, which increases even with slight physical exertion, palpitations, and pains appear in the region of the heart. The child lags behind in growth and development.

Significant stenosis of the pulmonary artery already in childhood causes a gross violation of blood circulation. Shortness of breath is pronounced even at rest and increases with the slightest exertion. It is usually not accompanied by cyanosis (“white defect”), but with prolonged circulatory failure, bluish cheeks and lower extremities can be observed.

Thickening of the fingers in the form of drumsticks in these patients also usually does not develop or is mild; no polycythemia observed. The symptom of the position of the patient on his haunches is extremely rare, with a significant narrowing. The reserve capacity of the hypertrophied right heart is small, and the increase in right ventricular failure, as a rule, leads to death at a young age.

Patients with isolated narrowing of the pulmonary artery often have a cardiac hump (a consequence of right ventricular hypertrophy).

In the region of the pulmonary artery - in the second intercostal space at the left edge of the sternum - systolic trembling ("cat's purr") is determined by palpation. The heart is enlarged to the right. Auscultation reveals a rough, scraping systolic murmur along the left edge of the sternum with a maximum sound in the second intercostal space. Noise in a number of patients is transmitted to the carotid arteries and is heard from the back to the right and left of the spine. Such irradiation of noise is caused by transition to an aorta and distribution on it (S. Sh. Kharnas, 1962). Valvular stenosis is characterized by the absence or significant weakening of the second tone above the pulmonary valve. With infundibular stenosis, the second tone is preserved. Arterial pressure and pulse have no characteristic features.

An x-ray shows enlargement of the right ventricle. The top of the heart is rounded. The arc of the pulmonary artery with valvular stenosis does not pulsate. Often there is a significant post-stenotic aneurysmal expansion of the trunk of the pulmonary artery. The lung fields are light, the vascular pattern is poor. The roots of the lungs are not expanded.

The electrocardiogram shows signs of overstrain of the right ventricle, deviation of the electrical axis of the heart to the right.

On phonocardiograms over the pulmonary artery, a spindle-shaped murmur begins with a slight pause after the first tone; reaches the second tone and ends before the beginning of its pulmonary part. The second tone is bifurcated. Systolic murmur may radiate to the aorta, Botkin's point, to a lesser extent to the apex of the heart.

When diagnosing isolated stenosis of the pulmonary artery, one should also take into account possible concomitant anomalies in the development of the heart, such as septal defects, open ductus arteriosus, etc.

The prognosis depends on the degree of stenosis and the severity of clinical symptoms. Patients are predisposed to inflammatory! respiratory diseases and bacterial endocarditis.

Pronounced stenosis early causes the development of right ventricular failure and sudden death.

The presence of severe symptoms of the defect and its progressive course serve as indications for surgical treatment.

However, given that over time, stenosis, even mild, leads to significant irreversible violations of the contractile function of the myocardium and circulatory disorders, it is recommended to operate on patients not only with severe and moderate, but also with mild stenosis and as early as possible (A. A Korotkov, 1964).

Produce transventricular closed valvotomy (according to Brock), transarterial (through the pulmonary artery) according to Sellors or intracardiac surgery using cardiopulmonary bypass. According to S. Sh. Harnas (1962), the mortality in the surgical treatment of valvular stenosis of the pulmonary artery is 2%, with infundibular stenosis - 5-10%.

Congenital stenosis of the pulmonary artery has long been considered a disease that is completely incompatible with pregnancy (Kraus et al.). This view is currently subject to revision.

We observed 5 pregnant women (all primiparous) with isolated pulmonary artery stenosis (including three after surgical correction of the defect) were aged from 21 to 30 years.

Pregnancy in one of 2 patients who did not undergo surgical treatment (T., 30 years old) ended
urgent spontaneous delivery of a live baby weighing 2800 g, 51 cm long. The second patient underwent abdominal (at the 22nd week of pregnancy) with sterilization (under endotracheal anesthesia). A year after the termination of pregnancy at the Institute of Cardiovascular Surgery of the USSR Academy of Medical Sciences, she underwent pulmonary valvotomy under hypothermia and a positive result was obtained.

In one of the 3 patients who underwent heart surgery for pulmonary artery stenosis (S., 22 years old), it occurred 3 years later, in the other (L., 27 years old) - 2 years after transventricular valvotomy. The pregnancy proceeded favorably and ended with a normal term delivery. Live children were born (weight 2700 and 3400 g, length 49 and 50 cm, in a satisfactory condition).

The third patient (K., 21 years old) came to us at the 8th week of pregnancy with complaints of shortness of breath, fatigue, general weakness, interruptions in the work of the heart. The patient's condition worsened during pregnancy. She categorically refused to terminate the pregnancy. It was decided to send her for surgical treatment. At the 12th week of pregnancy, the patient underwent a transventricular valvotomy. After the operation, the patient's condition improved. The pregnancy ended in premature birth (at 33-34 weeks). A live fetus was born weighing 1700 g, 40 cm long; the child was transferred to the Institute of Pediatrics of the USSR Academy of Medical Sciences, where he developed normally and was discharged home in a satisfactory condition, with normal weight.

Thus, only one of 5 patients with isolated pulmonary artery stenosis had a late pregnancy; (a caesarean section was performed at the 22nd week of pregnancy), the rest of the women gave birth to live children (one premature).

Our experience shows that with a slight narrowing of the pulmonary artery, which occurs without a significant load on the right heart, the patient can cope with pregnancy and spontaneous childbirth. Severe narrowing of the pulmonary artery leading to right ventricular failure requires either termination of pregnancy or surgical correction of the heart defect.