Pulmonary atresia with ventricular septal defect. Pulmonary atresia with intact ventricular septum

Pulmonary atresia is one of the most severe congenital pathologies of the structure of the heart. This condition in a newborn requires urgent action, since he can live without intervention for no more than a few days.

A child born with this pathology suffers from a dysfunction of the pulmonary circulation caused by a lack of connection between the right heart ventricle and the pulmonary artery. This condition may be accompanied following deviations:

• complete closure of the pulmonary valve;
• infection of the pulmonary trunk;
• reduction in the size of the right ventricle;
• underdevelopment of the tricuspid valve;
• complete absence pulmonary trunk.

Availability similar pathologies can be partially compensated by the preservation of features characteristic of the embryonic period of fetal development: an open oval window and an arterial duct. Their presence ensures the flow of blood and the maintenance of the life of a small patient. If the newborn has an intact septum, he may die immediately after birth. With moderate hypoplasia of the right ventricle and valve, especially if the arch of the pulmonary artery is in normal condition, the violation can be corrected in stages.

Atresia may be part of Fallot's tetrad, or "blue heart disease":

• stenosis of the upper part of the right ventricle;
• subaortic septal defect between the ventricles;
• dextroposition, that is, the arrangement with right side, aorta;
• right ventricular hypertrophy.

There are other critical malformations, such as univentricular heart, connection of the right atrium with the pulmonary artery, heterotaxy syndrome, and so on.

Reasons for development

Pulmonary artery atresia in newborns accounts for 1 to 3% of all anomalies of the cardiac structure. The disease is congenital pathology development, which is formed at the stage prenatal development.

It is believed that the main reason for it is the impact of a number of factors on the body of a pregnant woman, for example, radiation, toxic pollution environment, work with hazardous materials (phenols, lithium), antibiotic treatment and other drugs with a teratogenic effect, drug use, alcohol, smoking, the presence of such diseases in a pregnant woman as diabetes or infections like rubella and measles and so on.

It is believed that a certain percentage of defects arise under the influence of heredity or spontaneous mutation.

How are they classified

There are several types of classifications, for example, according to Sommerville, where 4 types of pulmonary atresia are distinguished:

1. I - Valve atresia. There are formed and passable trunk and both pulmonary arteries.
2. II - Atresia of the valve and the trunk of the artery. Both pulmonary arteries work and may have a common or separate origin.
3. III - Atresia of the valve, trunk and one of the pulmonary arteries. The second pulmonary artery is normal and permeable.
4. IV - Atresia of the aortic valve, trunk, both pulmonary arteries. The blood flow in the lungs is carried out through auxiliary vessels.

A more modern version of the classification is also used:

1. Type A - there are real pulmonary arteries, no accessory arteries, pulmonary blood flow is through the PDA.
2. Type B - the presence of real pulmonary and accessory arteries.
3. Type B - true pulmonary arteries are absent, pulmonary blood flow is carried out through collaterals.

Sometimes during the diagnosis, mixed forms are detected.

Manifestations

Atresia of the tricuspid heart valve and its other forms have characteristic external signs:

The life span of a child with such a pathology depends on the size of the arterial (botall) duct, which supplies blood with oxygen to the pulmonary artery. In the presence of severe defects, the life span is several days, with less complex pathology- six months - a year.

Diagnostics

If atresia of the heart valve of the pulmonary artery is suspected, the following types of diagnostics are performed:

1. Outpatient. It includes a survey, examination, auscultation. The doctor examines the patient, detects cyanosis, severe weakness, shortness of breath, a characteristic cylindrical shape of the chest, rough sounds are heard during auscultation. systolic murmurs, I tone is strong, II tone is weak.
2. In the hospital. The patient needs to undergo echocardiography, electrocardiography, chest x-ray, phonocardiography, as well as angiocardiography and, if necessary, catheterization of the heart cavities.

Pulmonary atresia is characterized by the absence of direct communication between the right ventricle and the pulmonary arterial bed as a result of the complete congenital absence of an orifice at the level of the right ventricular outlet, pulmonary valve, right and left pulmonary arteries. When combined with VSD, this pathology is often covered in the literature as an "extreme form" of Fallot's tetrad or a "false" common arterial trunk [Litman I., Fono R., 1954; Yonash V., 1963].

History reference.

Due to the fact that one main vessel departs from the heart, long time pulmonary artery atresia with VSD was considered as one of the forms of OSA. However, the works of A. V. Ivanitsky (1977), J. Somerville (1970), performed at a high methodological level, convincingly showed that type IV of the common arterial trunk, according to the classification of R. Collett and J. Edwards (1949), is nothing more than , as atresia of the pulmonary trunk, combined with defects such as tetralogy of Fallot, as well as with one of the forms of TMS. Consequently, this group defects is an independent nosological unit.

Frequency.

Pulmonary artery atresia in combination with VSD is detected in 1-3% of all CHD cases.

The first successful radical operation for this defect was performed by J. Kirklin in 1965. To connect the right ventricle to the pulmonary arteries, the surgeon used an autopericardial tube made on the operating table. In our country, with atresia of the pulmonary trunk with hypoplasia of the pulmonary arteries, the first successful operation to connect the right ventricle with the pulmonary arteries using a tube from the autopericardium without closing the VSD, it was performed by V.P. Podzolkov in 1984.

Etiology and pathogenesis.

Pulmonary atresia in combination with VSD refers to conotruncus malformations. The pulmonary arterial bed consists of 3 main segments of different embryonic origin: 1) the pulmonary trunk is formed as a result of division of the common arterial trunk; 2) the right and left pulmonary arteries are formed from the 6th pair of aortic arches and 3) intrapulmonary arterial vessels from the rudiments of the lungs. The absence or disruption of the development of one or more segments explains the variety of anatomical variants of the defect. In connection with the violation of the development of the arterial cone, there is no fusion of the arterial and interventricular septa, which leads to the formation of VSD.

Classification.

Two classifications are most widely used in clinical practice. According to the classification of J. Somerville (1970), based on the degree of preservation of the pulmonary arteries, there are 4 types of defect: 1) atresia of the pulmonary valve (the pulmonary trunk, right and left pulmonary arteries are preserved); 2) atresia of the pulmonary valve and pulmonary trunk (the right and left pulmonary arteries are preserved, which can be connected and disconnected from each other); 3) atresia of the pulmonary valve, trunk and one pulmonary artery (the other pulmonary artery was preserved); 4) atresia of the pulmonary valve, trunk and both pulmonary arteries (the lungs are supplied with blood by the collateral systemic arteries).

C. Olin et al. (1976) distinguish 5 types pulmonary atresia: 1) atresia of the outflow tract of the right ventricle; 2) atresia of the pulmonary valve; 3) atresia of the proximal part of the pulmonary trunk; 4) diffuse atresia of the pulmonary trunk; 5) atresia of the proximal parts of the pulmonary arteries with the absence of their connection.

Pathological anatomy.

The anatomical criteria of the defect include the following 5 components: 1) atresia of the pulmonary trunk, leading to impaired communication of the right ventricle with the pulmonary arterial bed; 2) occluded output section of the right ventricle; 3) large VSD; 4) the presence of any source of collateral blood supply to the lungs; 5) dextroposition of the aortic root.

Atresia may be at the level of the right ventricular outlet, pulmonary valve, proximal pulmonary trunk, or the entire pulmonary trunk

(which in such cases looks like a narrow band) and the proximal sections of the right and left pulmonary arteries, while disrupting the communication between them. In some cases, hypoplasia of the pulmonary arteries or their local narrowing can be noted. A specific component of the defect, which distinguishes it from the common arterial trunk, is the blindly ending output section of the right ventricle and a single valve only for the aortic orifice.

The VSD is usually large, located below the supraventricular crest, in the subaortic region, and is similar to the defect seen in tetralogy of Fallot. More rarely, the VSD is located above the supraventricular crest, corresponding to the topography of the defect in the common truncus arteriosus.

In all cases, the dextroposition of the aorta can be noted. The aorta is always greatly dilated. The aortic valve usually contains three leaflets, rarely two or four. The coronary arteries are normally distributed in most cases. Rare observations include communication of the left coronary artery with the pulmonary trunk, which, in case of pulmonary atresia, is regarded as a variant of collateral pulmonary blood supply. In all cases of pulmonary atresia, right ventricular hypertrophy is noted, and in approximately 40% of cases, moderate left ventricular hypoplasia.

Among the sources of blood supply to the lungs in patients with pulmonary artery atresia and VSD, the following are noted: 1) large aortopulmonary collateral arteries; 2) open ductus arteriosus; 3) bronchial collateral arteries; 4) large mediastinal collateral arteries; 5) fistulas between the coronary and pulmonary arteries; 6) mixed molds.

The greatest difficulties are caused by the identification of systemic collateral arteries and the identification of patterns of their connection with the pulmonary arterial bed. M. Rabinovicht et al. (1981) in the pathoanatomical study established the three most common variants of systemic collateral arteries and three types of their connection with the pulmonary arterial system. The authors showed that bronchial collateral vessels most often form intrapulmonary anastomoses, when large collateral vessels, which often depart from the descending aorta, connect with the pulmonary arteries at the root of the lung (“straight” aortic arteries). Collateral vessels originating from any artery that is a branch of the aorta (for example, the subclavian artery) form extrapulmonary anastomoses ("indirect" aortic arteries).

The most common source of pulmonary blood flow is the large systemic collateral arteries, which originate from the thoracic aorta or aortic arch. In 68% of cases, the collateral arteries contain local narrowings, which are determined at the site of their departure from the aorta, along the vessel, or when they are connected to the pulmonary arteries.

With severe hypoplasia of the pulmonary arteries, it is often possible to find both true pulmonary arteries and large aortopulmonary collateral arteries in various pulmonary segments of one or both lungs - the so-called multifocal type of blood supply to the lungs.

Hemodynamics.

Hemodynamic disturbances are determined by the presence of a single main artery, the aorta, which receives blood from the right and left ventricles through the VSD. The conditions under which both ventricles function are approximately equal, therefore the same systolic pressure equal to the aortic pressure is recorded in them.

Since arterial and venous blood, then the same blood enters the systemic and pulmonary circulation gas composition and in almost all patients, severe arterial hypoxemia is determined, the degree of which depends on the magnitude of pulmonary blood flow through collaterals.

Most patients have a small PDA. The blood flow in the lungs is sharply depleted. Relatively small volume arterial blood through the lungs returns to the left ventricle and again enters the aorta. With a large aortopulmonary communication, such as through the PDA or large systemic collateral arteries, blood flow through the lungs may be quite satisfactory or even increased. In such patients, the level of arterial hypoxemia may be moderate or minimal.

AT rare cases Patients with concomitant large patent ductus arteriosus may experience pulmonary hypertension with sclerotic changes in the pulmonary vessels.

The literature describes cases with asymmetric pulmonary blood flow, when one lung has a collateral type of blood supply, and the source of blood supply to the second lung is an open ductus arteriosus. Consequently, in the second lung, the blood flow is increased [Bukharin V.I. et al., 1979].

Clinic, diagnostics.

The clinical picture of pulmonary at-resia with VSD is usually quite characteristic. Predominant signs of chronic oxygen starvation. However, there are no shortness of breath and cyanotic attacks, which distinguishes this defect from most forms of Fallot's tetralogy. Along with the general cyanosis that has existed since the birth of the child, the symptoms of " drumsticks” and “watch glasses”. To the left of the sternum, a deformity of the chest in the form of a "heart hump" can be seen. Auscultation determines the accent of the II tone above the base of the heart and, with satisfactorily developed collaterals, a systolic-diastolic murmur in the second or third intercostal space to the right or left of the sternum. Noise is well conducted to the back.

Changes to ECG uncharacteristic. Electric axle heart is deviated to the right, there are signs of hypertrophy of the right ventricle and right atrium.

At X-ray the study shows the depletion of the lung pattern, the roots of the lungs are poorly outlined. In the presence of developed branches of the pulmonary artery, an experienced researcher sees them on a survey radiograph.

Strengthening of the pulmonary pattern is usually associated with the presence of atypical shadows of collateral vessels. In some patients, asymmetry of the pulmonary pattern (Janus symptom) may be observed, when it is strengthened on the one hand, and depleted on the other. The shadow of the heart is moderately enlarged in diameter, often of normal size. Due to the fact that the arch of the pulmonary artery sinks, and the apex of the heart is raised by an enlarged right ventricle, the waist of the heart is emphasized and the shape of the heart is similar to that found in Fallot's tetrad, i.e., in the form of a wooden shoe. In oblique projections, an increase in the right sections of the heart and a decrease in the size of the left ventricle are determined. The shadow of the ascending aorta is enlarged, the amplitude of its pulsation is increased. Thus, already on the basis of non-invasive methods of examining a patient, it is possible to make a diagnosis of pulmonary artery atresia with VSD with great accuracy.

On the ECHOCG a dilated ascending aorta and a large VSD are visible. With type I defect, a sharply hypoplastic pulmonary trunk can be seen.

Cardiac catheterization performed according to the same program as in patients with Fallot's tetrad. It is usually possible to pass a catheter from the right ventricle through the VSD into the aorta. Systolic pressure the same in both ventricles and aorta. Arterial oxygen saturation is usually reduced. It is possible to pass a catheter into the pulmonary trunk only if a PDA is present. With this heart disease, as a rule, there is a need for catheterization (from the lumen of the aorta) of collateral vessels supplying blood to the lungs, to perform selective arteriography. To do this, the catheter is passed into the aorta antegrade from the right ventricle or retrograde through the femoral artery.

Angiocardiographic the study should begin with the introduction of a contrast agent into the right ventricle. At the same time, the occluded outlet section of this ventricle is contrasted, which confirms the absence of a direct communication between the right ventricle and the pulmonary trunk, and the contrast agent enters the ascending aorta through the VSD.

Thus, with the help of right ventriculography, the diagnosis of pulmonary artery atresia with VSD is established and the type of ventricular-arterial junction is determined.

An angiocardiographic study is the only way, which allows you to establish the sources of blood supply to the lungs. For these purposes, aortography is performed with the introduction of a contrast agent into the ascending aorta, which allows not only to differentiate this defect from OSA, but also to establish or exclude: a) large collateral arteries extending from the brachiocephalic arteries, and b) an open arterial duct. When large collateral arteries depart from the descending aorta, a contrast agent is injected through a catheter, the tip of which is placed at the border of the arch and the descending part of the aorta. At this stage of the study, the location of the origin of the large collateral artery is determined in order to perform selective arteriography at the next stage. This research is given great importance, since it allows us to more accurately judge the paths of collateral circulation in the left and right lung, as well as to resolve the issue of the existence of true pulmonary arteries and, therefore, indications for surgery [Ivanitsky AV, 1977; Podzolkov V.P. et al., 1981].

Contrasted large collateral arteries arising from the brachiocephalic arteries or the descending aorta may be of various diameters and lengths. According to M. de Leval (1983), in 50% of cases they have local narrowings, which are especially often observed at their junction with the lobar or segmental pulmonary artery. This causes a fairly frequent hypoplasia of the pulmonary arteries.

In the presence of an open arterial duct, the size of the pulmonary arteries is directly dependent on the diameter of the duct.

In those cases when using the above research methods it is not possible to obtain contrasting of the true pulmonary arteries, it is possible to introduce a contrast agent against blood flow into the pulmonary vein in order to obtain retrograde contrasting of the pulmonary arteries and their branches [Ivanitsky A. V., 1977; Nihill M. et al., 1978].

Natural course and forecast.

In the first days and weeks of life in patients with pulmonary atresia and VSD, mortality is associated with the closure of the PDA or progressive narrowing of the "large" aortopulmonary collateral arteries. Death usually occurs from increasing hypoxemia due to a decrease or almost complete cessation of pulmonary circulation. Deterioration in childhood often associated with the fact that there is no increase in the size of the collateral arteries, according to the growth of the patient.

However, patients with a large patent ductus arteriosus and "large" aortopulmonary collateral arteries may be in a state of compensation for a long time.

indications for surgery.

Treatment is surgical only. With this type of defect, 2 types of operations are performed: palliative and radical. The need for palliative surgery in early childhood is due to progressive cyanosis due to the closing of the open ductus arteriosus and the small size of the pulmonary arteries. Therefore, the main goal of palliative surgery is to increase blood flow by performing an aortopulmonary anastomosis, which creates the prerequisites for the growth of true pulmonary arteries, and, if possible, to ligate the aortopulmonary collateral vessels. Among various types for aortopulmonary anastomoses, preference is given to the Blalock-Taussig subclavian-pulmonary anastomosis or the connection of these vessels using a Gore-Tex prosthesis.

Aortopulmonary anastomoses often create a preferential flow on the side of the connection with the pulmonary artery, and also lead to deformation, narrowing or kinking of the latter. Therefore, at present, two types of palliative operations are used to create a uniform blood flow in both pulmonary arteries in patients with type I defect (according to the classification developed by J. Somerville, 1970). The first type is the central anastomosis, which is created by connecting the hypoplastic pulmonary trunk to the left side wall of the ascending aorta. The second type of operation performed under EC conditions is the reconstruction of the outflow tract from the right ventricle by suturing the transanular patch without closing the ventricular septal defect [Podzolkov V.P. et al., 1987]. Since hypoplasia of the pulmonary arteries is quite often observed in early childhood, such patients are shown to perform palliative operations even with moderate cyanosis.

Radical surgery is possible only if normal sizes pulmonary arteries. If with type I defect during correction it is possible to use a transanular patch (preferably with a single leaflet) to expand the narrowed sections, then with types II and III of defect, a radical operation can only be performed using an artificial trunk and valves of the pulmonary artery.

Surgery.

Due to the frequent detection of large aortopulmonary collateral arteries surgical tactics in pulmonary atresia with VSD has its own characteristics. On the one hand, if the collaterals are not ligated, then the pulmonary blood flow increases. On the other hand, ligation of a collateral artery connected to a lobar pulmonary artery whose interlobar arteries have no connection with the central pulmonary arteries can lead to pulmonary infarction. Rational tactics of surgical treatment of patients with this defect was developed by J. Kirklin et al. (1981).

Before the operation, a complete angiocardiographic examination is mandatory at the first admission of the patient to the clinic. Since hypoplasia of the pulmonary arteries is usually observed in early childhood, these patients, even with moderate cyanosis, are shown to perform the Blalock-Taussig anastomosis and ligate, if necessary, large aortopulmonary collaterals, except for those that go to the bronchopulmonary segments. favorable conditions for the operation are created when the anastomosis is performed on the side of the location of the descending part of the aorta.

Immediately after the operation, the patient is transferred to the angiocardiography room, where subclavian artery a contrast agent is injected to make sure that the blood enters all parts of the lung. If this does not happen, the patient is returned to the operating room and the ligature is removed from the collateral artery.

In the long term after the operation (at the age of 5-10 years), a repeated full angiocardiographic examination is performed. If, after anastomosis, the pulmonary arteries develop normally, then a radical operation is performed. Moreover, the expected ratio of pressure values ​​in the right and left ventricles, calculated before correction, should not exceed 0.7. If after the anastomosis there was no sufficient development of the pulmonary arteries and the expected value after correction will be more than 0.7, then one more palliative operation should be performed already under the conditions of IR creation between the right ventricle and the pulmonary arteries of an artificial trunk using a valveless or valve-containing prosthesis without closure VSD. If the repeated palliative operation contributes to the development and expansion of the pulmonary arteries, then another operation follows - the closure of the VSD.

In patients with well-developed pulmonary arteries, primary radical surgery is possible. Before performing a radical operation, it is necessary to have a complete understanding of the size of the pulmonary arteries, the left ventricle, and the nature of the collateral blood supply to the lungs. It is necessary to accurately imagine to what extent and in what areas of the lung the blood supply is carried out by true pulmonary vessels and what role belongs to the aortopulmonary collateral arteries. This is due to the fact that ligation of the collateral arteries is possible only when the pulmonary arteries have a dual blood supply: through the aortopulmonary collateral arteries and through the true pulmonary arteries. Therefore, after ligation of the collateral artery, an adequate natural way for blood supply to the pulmonary arteries.

The most difficult group for surgical treatment is represented by patients with hypoplastic pulmonary arteries. The complexity of the pathology determines the need for a multi-stage surgical approach, which most often consists of at least 3 stages. During the first stage, an operation is performed aimed at increasing pulmonary blood flow and, consequently, dilating the hypoplastic pulmonary arteries. The second stage consists in ligation of the large aortopulmonary collateral arteries or their connections with the true pulmonary arteries, which is called "unifocalization". The third stage is a radical correction: closure of the ventricular septal defect and sometimes implantation of an artificial pulmonary artery trunk. So, in one of the patients we observed with type I pulmonary atresia, multi-stage treatment consisted of 5 operations.

radical operation. The operation is performed through a longitudinal sternotomy under conditions of hypothermic IR and cardioplegia. The presence of multiple aortopulmonary collateral arteries forces a reduction in perfusion volume rates and even resort to circulatory arrest in some patients.

One of the technically difficult stages of the operation is the isolation of large aortopulmonary collaterals and bronchial arteries. This stage can be performed from the median approach by opening the anterior mediastinal pleura or the posterior pericardium between the superior vena cava and the ascending aorta. With the left-sided location of the descending part of the aorta, additional lateral thoracotomy in the fourth intercostal space is often required to isolate and bring ligatures under the collateral vessels and bronchial arteries. Median sternotomy is performed only after suturing this wound and leaving drainage. The anterior leaf of the mediastinal pleura is opened and ligatures are found under the collateral arteries.

At the beginning of EC, a decompression cannula is inserted into the left ventricle, and if necessary, the aortopulmonary collateral arteries are ligated. If there are previously imposed anastomoses of Blalock-Taussig, Waterstone-Couley and Potts, then they are eliminated according to the usual technique during CPB. In cases where there is a large return of blood through the pulmonary arteries, it is possible to isolate and clamp them for the duration of the operation. distal departments or after opening them, insert a Fogarty catheter and obturate the lumen of the arteries.

At the next stage, the right ventricle is opened with a longitudinal incision and the VSD is closed with a patch. Further, with type I defect, the incision of the right ventricle is continued through the valve ring to the pulmonary trunk and the left pulmonary artery. As with the tetralogy of Fallot, patching expands the narrowed sections and creates a message between the right ventricle and the pulmonary arteries. However, in 50–60% of cases of pulmonary atresia (especially with type II and in some cases type 1 defects), a radical operation can be done only by creating an artificial pulmonary artery trunk using a valveless or valve-containing prosthesis. For this purpose, a transverse incision is made to open the junction of the right and left pulmonary arteries and perform a distal anastomosis, and then a proximal one with an opening on the right ventricle.

Palliative surgery-reconstruction of the outflow tract from the right ventricle without closure of the VSD is performed in patients with hypoplasia of the pulmonary arteries. The difference from the radical operation described above is that the VSD is not closed, and in young children, an autopericardial tube created during the operation is used to connect the pulmonary arteries to the right ventricle, F. Alvarez-Diaz et al., F. Puga, G. Uretzky (1982) developed a method for connecting the right ventricle to the pulmonary trunk without the use of EC. The operation consists in suturing to outer surface right ventricle and pulmonary artery patch, under which they are opened.

Postoperative complications.

Most frequent complication after radical surgery is acute heart failure, which is most often caused by high pressure in the right ventricle due to hypoplasia of the pulmonary arteries. J. Kirklin et al. (1981), after analyzing the results of surgical treatment of patients with this defect, came to the conclusion that even with a successful corrective operation, the ratio of pressure values ​​between the right and left ventricles in patients without large collateral arteries was 0.58, and in their presence, 0, 87. The authors explain this difference by anomalies of the pulmonary arteries and hypoplasia of the terminal interlobar branches in patients of the latter group.

Another complication is associated with errors in assessing the blood supply to the lungs during ligation of the large aortopulmonary collateral artery leading to the lobar or segmental artery, which do not have communication with the central pulmonary arteries. This leads to a heart attack of the corresponding section of the lung with the development respiratory failure.

immediate and long-term results.

S. Olin et al. seem to have the greatest experience in surgical treatment. (1976), who presented the results of a radical operation performed in 103 patients; postoperative mortality was 9.7%. O. Alfiery et al. (1978) noted 16% of adverse outcomes after 80 radical operations, and among 48 patients who required suturing a valve-containing prosthesis, postoperative mortality was 23%, while among 32 operated patients without suturing the prosthesis, only 6.2%.

Patients who have undergone surgery, as a rule, feel well and belong to the I or II functional class, according to the classification of the New York Heart Association. AT remote period most common causes of death were chronic heart failure due to remaining high pressure in the right ventricle, or reoperation, which was required in connection with stenosis of a valve-containing prosthesis.

All parents want their children to be healthy. And most adequate mothers and fathers begin to take care of this even before the active planning of the child. They visit the right doctors undergo many studies, accept vitamin preparations, balanced nutrition and lead completely healthy lifestyle life. But sometimes all these measures are not enough. Even during the most prosperous pregnancy, things can go wrong and the fetus can develop various violations. Just one of these is pulmonary atresia, types given state consider, discuss its features in newborns and clarify what kind of prognosis doctors give to infants with such a diagnosis.

Pulmonary atresia is a congenital malformation of the heart. In this condition, the child does not have a normal communication between the right ventricle and the pulmonary artery that exits it. Such a violation can develop if there is a complete fusion of the cusps of the pulmonary valve or fusion of the pulmonary trunk. As a result of pulmonary artery atresia, blood from the right ventricle is not able to enter the pulmonary circulation, which in turn significantly disrupts hemodynamics, makes normal gas exchange and the activity of the body as a whole impossible.

In some cases, pulmonary atresia is accompanied by other disorders. At little patient not only the complete absence of an opening in the pulmonary trunk can be observed, but also a significant decrease in the volume of the right ventricle, and a significant underdevelopment of the tricuspid valve. In some cases, the pulmonary trunk is completely absent.

At the same time, the child often retains such embryonic structural features, as an open ductus arteriosus, as well as an oval window. They support respiratory function, since blood through them can penetrate into the pulmonary artery.
Types of pulmonary atresia

There are four main varieties of this defect:

Atresia of the pulmonary valve - the patient has both the pulmonary trunk and both arteries;
- atresia of the valve of the pulmonary artery, as well as its trunk - the patient has only arteries;
- atresia of the pulmonary valve, trunk, as well as one pulmonary artery - only one pulmonary artery remains intact;
- atresia of the pulmonary valve, trunk and both arteries - blood circulation in the lungs can be carried out only thanks to the collateral systemic arteries.

Danger of pulmonary atresia

Pulmonary atresia is quite rare and dangerous pathology. With such a defect, blood with a low oxygen content circulates through the body of the newborn. BUT oxygen deficiency over time causes the death of brain cells.
Pulmonary atresia poses a threat to the life of the child in the first weeks and days of his life.

Symptoms of pulmonary atresia

The disease is diagnosed already in the first hours of a newborn's life. But sometimes its symptoms appear only a few days after birth. Pulmonary atresia results in a bluish discoloration of the skin. Doctors classify this condition as cyanosis; oxygen starvation of the brain leads to it. In addition, the child often and difficult to breathe, he has shortness of breath. When feeding, a newborn gets tired very quickly.

Can children with pulmonary atresia be helped?

To date, doctors with such a diagnosis can offer surgical intervention: radical or palliative.

A radical operation allows you to eliminate the causes of the disease, it is carried out with the first type of disease - when both pulmonary arteries are present in a small patient.
In the second and third types, radical surgery is also possible, but doctors have to use an artificial trunk and pulmonary artery valves.

Palliative surgery is not aimed at eliminating the cause of the defect, but at increasing the blood supply to the lungs. It is performed with progressive cyanosis. Palliative surgery involves the imposition of an aortopulmonary anastomosis, which creates the prerequisites for active growth pulmonary arteries. Immediately after the formation of the connection, doctors perform a secondary angiocardiographic examination, which should show that all parts of the lung are bathed in blood. If this does not happen, the patient is operated on again.

Five to ten years after the palliative surgery, doctors perform another angiographic examination. In the event that during this time the pulmonary arteries have developed normally, radical surgery is performed.

Pulmonary artery atresia in newborns - what is the prognosis?

With timely therapy, the prognosis of pulmonary atresia in newborns is quite favorable. With the first type of this defect, postoperative mortality is 6.2%, however, with the second and third types of the disease, it increases to 23% (if it is necessary to sew in a prosthesis of the arterial trunk and valve).

In most cases, patients after surgery feel well, and mortality occurs due to heart failure due to high blood pressure blood in the right ventricle.

Lack of therapy is fraught with the death of the patient from oxygen starvation in the first weeks after birth.

At an older age, children with operated pulmonary atresia should be constantly monitored by a cardiologist. They are more likely to develop endocarditis.

Additional Information

Many children who have undergone major surgery in childhood face time with frequent colds. They need to strengthen their immunity, and funds will come to the rescue in this matter. traditional medicine.

So an excellent effect is given by pine needles, the beneficial properties of which strengthen the immune system. Rinse a couple of tablespoons of pine needles and brew with a glass of boiling water. Heat the medicine to a boil and boil over low heat for twenty minutes. After that, cool the broth for half an hour and strain it. Sweeten the drink with juice and give it to your child a couple of tablespoons at a time, three times a day.

Large aortopulmonary collateral vessels are usually associated with such cyanotic birth defects heart, as pulmonary atresia and tetralogy of Fallot (usually extreme form). In these patients, the systemic-pulmonary collateral network develops as compensatory mechanism replenishment of pulmonary blood flow, collateral vessels have different sizes and numbers, and they depart, as a rule, from the descending aorta, anastomosing with the branches of the pulmonary artery or in the area of ​​the hilus or at the level of the segmental bronchi.

Traditional surgical treatment of this pathology is problematic due to the difficulty of localizing collaterals, especially when performing median thoracotomy. In the case of the functioning of large aortopulmonary collaterals during the correction of the underlying heart disease, there is a high probability of complications due to a significant shunt of blood from the systemic to the pulmonary circulation and the difficulty of adequate perfusion. As a result, intraoperative hypoxia of all organs and systems may occur, and in the early and late postoperative period - pulmonary hypercirculation leading to respiratory distress syndrome or heart failure.

Preliminary endovascular occlusion of collaterals makes it possible to prepare such patients for radical correction of the defect. Embolization of collateral vessels is indicated in the presence of following conditions: a) blood supply to a certain zone of the lung, the collateral blood flow in which is supposed to be occluded, is provided by two sources - aortopulmonary collaterals and branches of the true pulmonary artery, b) aortopulmonary collaterals and branches of the pulmonary artery are well differentiated. When determining the presence of these signs during embolization of collaterals, the development of such severe complications as pulmonary infarction due to interruption of the blood supply to a certain lung lobe or occlusion of branches of the true pulmonary artery.

One of the first successful embolization of aortopulmonary collaterals was reported in 1985 by S. Mitchell et al. and S. Kaufman et al. In 1989, S. Perry et al. published the results of embolization closure of 58 collaterals with Gianturco coils, achieving total occlusion in 42 and subtotal occlusion in 14 cases.

In the NCSSH them. From 1987 to 1997, embolization of 33 pronounced aortopulmonary collaterals was successfully performed in 14 patients, 12 of whom underwent reconstruction of the outflow tract from the right ventricle due to pulmonary atresia, one suffered from Fallot's tetralogy and in one patient there was a combined pathology - a two-inflow left ventricle with hypoplasia of the right ventricle, aortic discharge from the right ventricle, pulmonary atresia, an open arterial duct and a systemic pulmonary anastomosis. The age of the patients ranged from 5 to 15 years.

All patients underwent a complete clinical examination, including ECG, FCG, echocardiography, radionuclide lung scanning and computed tomography of the lungs. To determine the exact localization of collaterals and their size, ascending and descending aortography and selective arteriography of collaterals were performed. At the same time, a well-developed collateral network is visible with contrasting of the pulmonary artery in the form of a "gull". All patients underwent right heart catheterization and angiography from the pulmonary arteries was performed in order to determine in detail the sources of blood supply to the lungs.

The study of the right parts of the heart was performed using a puncture transfemoral venous access. Aortography was performed retrograde, in all cases a 5-7F introducer was placed in the femoral artery. Most patients were intraoperatively injected with heparin (100 U/kg). After performing aortography and selective arteriography from the collateral arteries, the possibilities of collateral occlusion were assessed and the diameter of the vessels to be occluded was calculated.

Gianturco spirals with a diameter of 3 to 10 mm were used. The diameter of the spirals exceeded the diameter of the aortopulmonary collateral by at least 50%. From one to three collateral vessels were simultaneously embolized, up to 10 emboli were injected. In 3 patients, embolization was performed in two stages: the first stage was occlusion of collaterals leading to right lung, the second stage - collaterals going to the left lung. In 1 patient, prior to collateral embolization, balloon angioplasty of narrowed pulmonary arteries was performed after infundibulectomy.

Due to the high blood flow velocity and different pressure in different parts collateral vessel, migration of embolic material into the pulmonary artery system is a potential complication. To prevent this, the coils were placed in the narrowest part of the collateral vessel. In our practice, there were no cases of spiral migration. Control angiography was performed 5-10 minutes after coils were installed. Adequate occlusion was achieved in all cases. All patients received antibiotics intraoperatively and for the next two days. Complications were not noted.

A - before embolization, an extensive network of large aortopulmonary collaterals to both lungs is visible; b - after embolization, all collateral vessels are occluded with Gianturco coils

Thus, the success of the multi-stage treatment of patients with complex cyanotic heart defects was determined: the first stage was the reconstruction of the outflow tract from the right ventricle, the second stage was embolization of the aortopulmonary collaterals, and the third stage was the closure of the septal defect and the final correction of the defect.

Small bleeding collateral vessels in the pulmonary circulation

Bronchial arteries and some non-bronchial systemic arteries enlarge as a result of increased blood flow and fragility inflammatory tissue there is erosion, crack or rupture of the wall of bronchial collaterals developing over many years. Massive hemorrhage - the outflow of more than 600 ml of blood within 48 hours - leads to 50-85% mortality in conservative management of such patients. Along with the, most of patients are not subject surgical treatment due to severe lung disease. Therefore, the endovascular method is often the method of choice in the treatment of this group of patients.

Embolization of hypertrophied systemic arteries is carried out. Stopping bleeding is achieved by reducing the pressure in the bleeding vessel. Immediate elimination of hemostasis through proximal collateral embolization, according to S. Kaufman et al., can be achieved in 90-100% of cases. The site of hemorrhage is determined by performing a chest x-ray, aortography, and selective arteriography of the vessels leading to the suspected site of bleeding.

As embolizing agents, helium foam particles or, more preferably, Ivalon can be used - for small-caliber vessels, convoluted, which allows embolization of the vessel without the risk of embolus migration into the pulmonary artery, as well as ultra-modern solutions of specific particles. The use of liquid agents such as absolute ethanol is undesirable as they may cause bronchial necrosis.

B.G. Alekyan, V.P. Podzolkov, N.E. cardenas

Pulmonary atresia(ALA) is characterized by the absence of normal communication between the ventricles of the heart and the pulmonary artery.

true frequency pathology is unclear, since the studies include different statistical material (surgical, pathoanatomical, demographic) and use a different hierarchy of heart defects. Literature data indicate a significant variability of the defect among newborns - from 0.0065 to 0.02%. Among all CHD, the proportion of pulmonary atresia ranges from 1.1 to 3.3%, rising among the "critical" CHD to 6.3%.

This pathology exists in two main variants: 1) pulmonary artery atresia with VSD (ALA+VSD); 2) pulmonary atresia with an intact interventricular septum (ALA+IMZHP). In addition, pulmonary atresia may be one of the components of other complex congenital heart diseases (single ventricle, Ebstein anomaly, long form VKA, tricuspid atresia, corrected TMA, etc.).

It is most often found in combination with VSD, other combinations are twice as rare. In this section, there are two main types of defect.

Pulmonary atresia in combination with VSD

Frequency pulmonary atresia with VSD is about 0.07 per 1000 newborns, 1% among all CHD and about 3.5% among critical CHD. However, this anomaly may be integral part other complex defects, so its true occurrence is unknown.

Pathology characterized by the absence of connection of the right ventricle with the pulmonary artery; the output section of the right ventricle ends blindly. Vice also has a large VSD, the only aortic valve, dextroposition of the aorta varying degrees. When examining the vessels that bring blood to the lungs, the “true”, or native, pulmonary arteries and large aortopulmonary collateral arteries (BALKA) are isolated. Blood enters the vessels of the lungs from the aorta through a functioning PDA or BALKA. In addition, anastomoses of the pulmonary arterial bed with bronchial, mediastinal or coronary vessels. The most famous classification of defect according to J. Somerville (1970) is based on the level of pulmonary atresia: 1) pulmonary valve atresia; 2) atresia of the pulmonary valve and pulmonary trunk; 3) atresia of the pulmonary valve, pulmonary trunk and one of the pulmonary arteries; 4) atresia of the pulmonary valve, trunk and both pulmonary arteries (the lungs are supplied with blood only through the collateral arteries).

AT last years proposed classification, based on the type of blood supply to the lungs: type A - there are true pulmonary arteries, BALKA are not detected; type B - both true pulmonary arteries and BALKA are presented; type C - there are no true pulmonary arteries, only BALKA is present. Pulmonary arteries can be connected to each other (confluent arteries) or be non-confluent.

Hemodynamics in pulmonary atresia. With this pathology, all blood from the right (through the VSD) and left ventricles enters the ascending aorta. As a result, arterial hypoxemia occurs, the degree of which is inversely proportional to the amount of pulmonary blood flow. In turn, pulmonary blood flow is determined by the diameter of the PDA or systemic collateral arteries. With their stenosis or hypoplasia, the return of oxygenated blood to the left atrium is small, and hypoxemia can reach a critical degree. With adequate pulmonary circulation the level of hypoxemia can be minimal, and in rare cases even pulmonary hypertension develops.

Fetal echocardiography in pulmonary atresia. The detectability of the defect is low (from 7 to 15%). When studying the excretory tracts of the ventricles, a sharply hypoplastic pulmonary artery is revealed; there is no blood flow through the pulmonary valve. Diagnosis is also based on the detection of VSD and retrograde blood flow in the PDA. From both ventricles, blood is sent to the dilated aorta.