Totally anomalous pulmonary venous drainage. Congenital heart disease: abnormal pulmonary venous drainage

All pulmonary veins drain into the right heart.

TADLV classification:

- sujaracardial (pulmonary veins drain into the ascending vertical vein, innominate vein);

- cardiac (pulmonary veins drain into the right atrium, coronary sinus);

- infracardial (pulmonary veins drain into intra-abdominal veins).

Despite the different anatomy of the malformation, in terms of circulatory pathophysiology, it can be classified into two types depending on the presence or absence of pulmonary vein obstruction. Important for the continuation of life in the postnatal period is the presence of communications between the PP (right atrium) and LP (left atrium) (, LLC).

The hemodynamics of the non-obstructive variant of TALV is similar to that of a major ASD. Blood from the pulmonary veins enters the RA, then the RV (right ventricle) and, in the presence of an ASD, the LA. In this case, the amount of blood that returns through the ASD to the LA is determined by the size of the defect and the degree of prostate extensibility. However, under any conditions, a much larger part of the blood enters the pancreas and circulates in the ICC (pulmonary circulation), causing volume overload of the pancreas and marked hypervolemia of the ICC. The pressure in the LA (pulmonary artery) remains low. Due to the fact that the mixing of venous and arterial blood coming from the pulmonary veins occurs at the level of PP, the saturation in the LA and the aorta is identical.

In the obstructive variant of TALV, the outflow of oxygen-enriched blood from the pulmonary veins is difficult. This leads to the development of pulmonary venous hypertension and then to an increase in pressure in the LA and pancreas. A situation resembling hemodynamics in mitral valve stenosis is created. In this case, the risk of developing pulmonary edema is high, because. hydrostatic pressure in the capillaries becomes much higher than the osmotic pressure of the blood. As long as the existing ASD provides sufficient volume of right-to-left blood shunting, the pancreatic cavity remains small. The left side of the heart, as in the case of the non-obstructive variant of TALV, remains "underloaded" and has a relatively small size. The level of oxygen saturation in the aorta and LA is equal, but its values ​​are significantly lower than in the non-obstructive variant of the defect. The degree of arterial desaturation will be inversely proportional to the volume of blood flow in the ICC.

The prognosis for patients with TALV is extremely unfavorable. Without surgical correction, two-thirds of patients with a non-obstructive form of the disease die by the end of the first year of life.

The cause of death is often pneumonia. With an obstructive variant, life expectancy is 2-3 months.

CLINIC

a. Clinical manifestations of the disease:

Obstructive variant of TADLV:

- characterized by rapidly progressive cyanosis from birth, which increases with feeding, which is associated with compression of the pulmonary veins by the esophagus;

- shortness of breath and signs of pulmonary edema in the neonatal period.

- moderate cyanosis from birth;

- lag in physical development, frequent bronchopulmonary infections;

- signs of heart failure (tachycardia, shortness of breath, hepatomegaly).

b. Physical examination:

Obstructive variant of TADLV:

- loud II tone at the base of the heart;

- in most cases, there are no murmurs in the heart area;

Non-obstructive variant of TADLV:

- II tone is split at the base of the heart (the pulmonary component of the II tone is accentuated);

- weak or medium intensity(no more than 3/6) systolic murmur of relative stenosis of the pulmonary valve in II m. to the left of the sternum;

- mesodiastolic murmur of relative stenosis of the TC (tricuspid valve) along the left edge of the sternum in the lower third (with a significant flow from the RA).

DIAGNOSTICS

1. Electrocardiography

Obstructive variant of TADLV:

hypertrophy of the pancreas (R-type);

- less often PP hypertrophy.

Non-obstructive variant of TADLV:

- hypertrophy of the pancreas according to the type of blockade of the right leg of the His bundle in lead Vj (a consequence of volume overload of the pancreas);

- less often PP hypertrophy.

1. echocardiography

Diagnosis of TALVV is based on the absence of all pulmonary veins flowing into the left atrium in typical places.

Diagnostic criteria:

- sharp dilatation of the right parts of the heart;

- expansion of the pulmonary artery;

- a significant reduction in the left chambers;

- dilated vertical vein and superior vena cava;

- the presence of an ASD with a right-left discharge.

TREATMENT AND OBSERVATION

1. 1. Observation and treatment of patients with uncorrected TALV

The obstructive variant of TALV is an emergency indication for surgery. Before the patient enters the cardiac surgery clinic, the following actions should be taken:

a. IV infusion of prostaglandin E 1 preparations, which will keep the duct open and ensure the discharge of blood from the LA into the aorta. This will reduce the risk of developing pulmonary edema and increase the volume of blood flow in the BCC.

in. Correction of metabolic acidosis.

Non-obstructive variant of TADLV:

a. Intensive care for heart failure (diuretics, digoxin).

b. If metabolic acidosis occurs, it is corrected.

1. Endovascular treatments

When establishing the diagnosis of TALV with the presence of a small / restrictive ASD, the Rashkind procedure is performed to increase the volume of blood flow in the BCC. The indication for the procedure is the presence of restrictive communication between the atria - a pressure gradient of more than 6 mm Hg.

1. Surgery

Indications for surgical treatment:

— establishment of the diagnosis of TALVV — an absolute indication for surgery.

Contraindications for surgical treatment:

- the presence of absolute contraindications for concomitant

- somatic pathology;

- high venous resistance of the vessels of the lungs.

Surgical tactics

The timing of surgery is determined by the presence or absence of pulmonary venous obstruction.

With obstructive TALV intervention is performed immediately within the first hours after diagnosis. Usually this is the first hours of a child's life.

For non-obstructive TALV surgical treatment may be delayed and performed during the first months of life.

Surgical technique

under IR conditions. Allocate a common collector LV at the place of its confluence. Usually the manifold is long enough (even in the case of an infracardiac drain) to move it into the left atrium. The collector is widely opened, eliminating areas of narrowing. Widely open the left atrium. A wide anastomosis is formed between the PV collector and the LA. Additional elements of the venous system (vertical vein, etc.) are tied up.

Specific complications of surgical treatment

- residual pulmonary hypertension;

- violation of the heart rhythm (syndrome of weakness of the sinus node), atrial tachycardia;

- reduced cardiac output due to residual obstruction of the outflow tract from the PV.

The word "anomalous" means "wrong". With this defect, the pulmonary veins (and there are four of them), which should flow into left atrium, do not fall into it, i.e. do not connect with him. There are a lot of options for their wrong confluence.

There is a "partial" anomalous drainage - this is when one or two of the four veins flow into the right atrium (the most common option), and in the vast majority of cases it is combined with atrial septal defects, and we talked about this in the chapter on ASD.

Full or total anomalous pulmonary venous drainage (TADLV)- This is completely different. With this defect, all four pulmonary veins from both lungs are connected into one wide vessel-collector. This collector of arterial blood oxidized in the lungs does not fuse with the left atrium, as it should, but connects to the body's venous system, usually through a large vein. Arterial blood, thus, bypassing the heart, enters the large veins and into the right atrium. Only here, having passed through the defect of the interatrial septum, it will be where it should be initially - in the left atrium, and then it makes the usual path through the systemic circulation. It's hard to imagine what it could be like at all. But children with this defect are born full-term, and the heart copes with such a situation for some time. However, this time can be very short.

Firstly, the life of a child depends on the size of the interatrial message - the smaller it is, the more difficult it is for arterial blood to get to its destination in the left half of the heart.

Secondly, in this left half of the heart, a significant part of the blood is simply venous, i.e. unoxidized, and it will again be forced into a large circle. In a child, thus, partially venous blood begins to circulate in the arteries, and he becomes "blue", i.e. the color of the skin, and especially the tips of the fingers and mucous membranes (lips, mouth) is cyanotic. This is cyanosis, and we will talk about its causes, manifestations and consequences later.

With complete anomalous drainage, cyanosis may not be very pronounced, but it is present and is usually noticeable shortly after birth.

In most cases, the condition of children with complete anomalous pulmonary venous drainage is "critical" from the very beginning of life. If nothing is done, they will die in a few days or months.

Surgical treatment exists, and the results today are quite encouraging. The operation is quite complicated, it is performed on the open heart and consists in the fact that the common pulmonary vein collector is sutured to the left atrium, and the hole in the atrial septum is closed with a patch. In this way after the operation, normal blood circulation is restored in two separated circles.

Sometimes an emergency option is also acceptable - the expansion of the defect during probing as the first, life-saving stage, which allows you to somewhat delay the main intervention.

We will not go into many of the details here. various types defect and methods of its correction. But we only want to emphasize that children with this defect need immediate specialized help, which today is absolutely real.

The long-term results of the operation are quite good - after all, the main defect has been eliminated. However, children should be under the supervision of cardiologists because complications are possible in the form of rhythm disturbances or narrowing of the pulmonary veins at the sites of suturing (this is due to the fact that the heart that has undergone such a major operation continues to grow). And again we want to emphasize: this child is not disabled. He must lead an absolutely normal life, and the sooner the operation is done, the sooner everything will be forgotten.

Abnormal pulmonary venous drainage occurs in approximately 3% of newborns with congenital malformations of the heart and blood vessels.
With this defect, the pulmonary veins flow into the right atrium, vena cava or coronary sinus. This vice in its purest form is incompatible with life. The life of the patient is possible only in the presence of a concomitant defect of the interatrial or interventricular septum. If there are no such defects, and there is only an open oval window, then babies die early. Death also occurs in cases of closure shortly after the birth of the foramen ovale.

There are many anatomical varieties of this defect that determine its hemodynamic features. The combination with a ventricular or atrial septal defect is accompanied by severe cyanosis and circulatory failure, predominantly of the left ventricular type with severe congestion in the lungs.

Circulation. With this anomaly, a significant part of the arterial blood from the pulmonary veins does not enter the left atrium, but into the right one, then through the right ventricle again into the pulmonary circulation. Due to this return, most of the blood circulates within the pulmonary circulation, the amount of circulating blood in the systemic circulation is reduced.

With the confluence of all pulmonary veins into the right atrium, life after birth is possible only if there are other anomalies in the structure of the heart or blood vessels that facilitate the flow of blood from the right atrium into the arterial bed - a defect in the interventricular, interatrial septum.

Usually noted anomalous location 1-2 veins of the right lung, often in combination with an atrial septal defect. Characterized by mixed blood circulation with a slight decrease in the oxygen content in the systemic circulation, overload of the right heart, pulmonary hypertension.

Clinical picture This defect is determined by anatomical and hemodynamic features: the level of general pulmonary resistance, the degree of pulmonary venous obstruction, the size of the interatrial communication, the state of the right ventricular myocardium.

The first signs of vice often appear from the first days of life in the form of heart failure, repeated pneumonia, SARS, cough, small weight gain. From birth, the child has tachypnea and dyspnea, the presence of moist rales in the lungs. During auscultation, systolic murmur on the left in the second intercostal space, accent and splitting of the II tone over the pulmonary artery can sometimes be noted.

Some children in neonatal period a pronounced obstruction of the venous return develops, which is typical for the confluence of the pulmonary veins into the system below the diaphragm. At the same time, pronounced cyanosis and a sharp tachypnea appear. Murmurs in the region of the heart in this case may not be heard.

For another group of newborns with significant left-to-right shunt characteristic early development congestive heart failure. Due to pulmonary vascular hypertension, the disease is very difficult. On auscultation, systolic murmurs, sometimes a gallop rhythm, are heard along the left edge of the sternum. Cyanosis is mild.

In newborns without impaired outflow from the pulmonary veins, with a left-to-right shunt and the absence of pulmonary hypertension, clinical symptoms in the neonatal period are mild or absent. Cyanosis practically does not occur. Decompensation develops at a later age.

Diagnostics. On the electrocardiogram, signs of deviation of the electrical axis of the heart to the right, blockade of the right leg of the His bundle, enlargement of the right atrium, right ventricle are recorded.

On the radiograph, the pulmonary pattern significantly enhanced, there is moderate or significant cardiomegaly due to an increase in the right chambers of the heart. The left sections are of normal size, sometimes an enlarged shadow of the superior vena cava is visible.

In the supracardial form of the defect, a figure-eight shadow is characteristic, where the lower part is the heart itself, and the upper part is a collector that collects blood from all the pulmonary veins and opens into the left or right vena cava or innominate veins, which are dilated due to the large volume of blood. Sometimes the shape of the heart mimics an enlarged thymus gland.
When the pulmonary veins flow into the coronary sinus or inferior vena cava, there are no characteristic radiological manifestations of the defect.

Echocardiogram reveals signs of right ventricular overload, intermediate or paradoxical oscillations of the interventricular septum. The size of the left ventricle is 50-65% of normal. The aortic root is reduced. The common venous canal, into which the pulmonary veins flow, can be located when it is located directly behind the left atrium.
Correction. Surgical treatment. In this case, transplantation of the pulmonary veins into the left atrium is performed.
Heart failure with this defect, it is difficult to treat with diuretics and cardiac glycosides.
The prognosis is usually unfavorable.

Anatomical variants of total anomalous pulmonary venous drainage:
supracardial variant, in which the pulmonary veins are drained either through a vertical vein (A),
or directly into the superior vena cava (SVC) with the location of the mouth of the collector near the mouth of the unpaired vein (B).
(B) Cardiac variant of drainage through the coronary sinus into the right atrium.
(D) An infracardial variant of drainage through a vertical vein into the portal vein or inferior vena cava (IVC).

Video EchoCG with total anomalous pulmonary venous drainage in the fetus

- congenital pathology of the heart, the morphological basis of which is the abnormal flow of the pulmonary veins into the right atrium or the vena cava entering it. Abnormal drainage of the pulmonary veins is manifested by fatigue, lag in physical development, pain in the heart, shortness of breath, repeated pneumonia. For the diagnosis of abnormal pulmonary vein drainage, radiography, ECG, ultrasound of the heart, cardiac sounding, angiopulmonography, atrio- and ventriculography, MRI are performed. Surgical options for anomalous pulmonary venous drainage vary depending on the type of defect.

General information

congenital heart disease, in which the mouths of all or individual pulmonary veins flow into the right atrium, coronary sinus or vena cava. Abnormal pulmonary venous drainage accounts for 1.5-3% of all CHD in cardiology, with a predominance in males.

Most often, abnormal pulmonary venous drainage is associated with an atrial septal defect and an open foramen ovale; in 20% of cases - with a common arterial trunk, VSD, transposition of the great vessels, tetralogy of Fallot, pulmonary artery stenosis, hypoplasia of the left heart, single ventricle of the heart, dextrocardia. Of the extracardiac pathology in patients with abnormal pulmonary vein drainage, intestinal diverticula, umbilical hernias, polycystic kidney disease, hydronephrosis, horseshoe kidney, as well as various malformations of the bone and endocrine systems occur.

Causes of Abnormal Pulmonary Vein Drainage

The common causes that form abnormal pulmonary venous drainage do not differ from those in other CHD.

Direct separation of the pulmonary veins from the left atrium may be due to two factors: the absence of their connection or early atresia of the common pulmonary vein. In the first case, under the influence of unfavorable conditions, the left atrial outgrowth does not properly communicate with the venous plexuses of the lung bud, which leads to the formation of abnormal venous drainage. In early atresia, there is an initial connection of the common pulmonary vein and the pulmonary vascular bed, but later their lumen is obliterated, so pulmonary venous return begins to occur through other available collateral pathways.

Classification of anomalous pulmonary venous drainage

When the mouths of all pulmonary veins flow into the venous system of the systemic circulation or the right atrium, they speak of complete (total) anomalous drainage of the pulmonary veins. If one or more pulmonary veins drain into the right atrium or a large circle, this form of defect is called partial. Most often (in 97% of cases), the veins draining from the right lung are abnormally drained.

Based on the level of entry of the pulmonary veins, anomalous drainage is classified into 4 anatomical types (options).

• I option- supracardial (supracardial). The pulmonary veins are drained by a common collector or separately into the superior vena cava or its branches (azygous vein, left innominate or accessory superior vena cava).
• II option- intracardiac (cardiac). Abnormal pulmonary venous drainage occurs in the right atrium or coronary sinus.
• III option- subcardial, infracardial (subcardiac). The pulmonary veins drain into the inferior vena cava or portal vein (rarely into the lymphatic duct).
• IV option- mixed. Abnormal drainage of the pulmonary veins into the venous system is carried out at various levels in various combinations.

Features of hemodynamics in anomalous drainage of the pulmonary veins

In the antenatal period, the defect usually does not have hemodynamic manifestations, since intracardiac circulation in the fetus implies the flow of blood from the right atrium to the left (through the open foramen ovale) and into the ductus arteriosus. After birth, the severity of hemodynamic disturbances is determined by the form (complete or partial), the variant of abnormal pulmonary venous drainage, and the combination of the defect with other heart defects.

From the point of view of hemodynamics, total anomalous pulmonary venous drainage is characterized by the fact that all oxygenated blood from the lungs enters the right atrium, where it mixes with venous blood. In the future, one part of the blood enters the right ventricle, the other - through interatrial communication into the left atrium and the systemic circulation. In this case, abnormal pulmonary venous drainage is compatible with life only if there is a communication between the two circulations, the role of which is played by an ASD or an open foramen ovale. Hemodynamic disturbances in total anomalous pulmonary venous drainage are accompanied by hyperkinetic overload of the right heart, pulmonary hypertension, and hypoxemia.

With a partial form of abnormal pulmonary venous drainage, hemodynamic disturbances are similar to those with interatrial defects. The decisive role among them belongs to pathological arteriovenous shunting of blood, leading to an increase in blood volume in the pulmonary circulation.

Symptoms of abnormal pulmonary venous drainage

The clinical manifestations of abnormal pulmonary venous drainage are based on anatomical and hemodynamic features: the level of general pulmonary resistance, the degree of venous return obstruction, the size of the interatrial communication, and the functioning of the right ventricular myocardium. In the absence of a defect in the interatrial septum or its extremely small size, the defect is incompatible with life - in this case, only emergency endovascular balloon atrioseptostomy according to Rashkind can save the child.

In children with abnormal pulmonary vein drainage, frequent repeated pneumonia and acute respiratory viral infections, small weight gain, shortness of breath, mild cyanosis, cough, fatigue, lag in physical development, heart pain, tachycardia are noted. With severe pulmonary hypertension, pronounced cyanosis, a heart hump and heart failure develop already in early childhood.

Diagnosis of abnormal pulmonary venous drainage

The auscultatory picture of abnormal pulmonary venous drainage resembles an ASD and is characterized by a non-rough systolic murmur in the projection of the pulmonary artery, splitting of the II tone. ECG data indicate an overload of the right departments, an incomplete blockade of the right leg of the bundle of His; EOS is deflected to the right. Phonocardiography corresponds to all signs of ASD.

When probing the cavities of the heart, a catheter is passed from the right atrium or vena cava into the pulmonary vein. At this stage of diagnosis, it is possible to establish the localization of the confluence and the number of abnormally draining pulmonary veins. Right atriography, ASD plasty. Children under 3 months of age who are in critical condition undergo a palliative operation of a closed atrial septotomy, aimed at increasing interatrial communication.

To general principles radical correction of total anomalous pulmonary vein drainage include: creation of an anastomosis between the pulmonary veins and the left atrium, closure of an ASD, ligation of the pathological communication of the pulmonary veins with venous vessels. After surgery, sick sinus syndrome, an increase in pulmonary hypertension associated with inadequate provision of outflow tracts from the pulmonary veins, may develop.

Prognosis of abnormal pulmonary venous drainage

The natural course of total anomalous pulmonary venous drainage is unfavorable: 80% of children die in the first year of life. Patients with partial pulmonary venous drainage can live up to 20-30 years. The death of patients is associated with severe heart failure or pulmonary infections.

The results of surgical correction of abnormal pulmonary venous drainage are satisfactory, but intra- and postoperative mortality among newborns remains high.

Total anomalous pulmonary venous drainage (TADLV) accounts for 1-2% of all CHD in children under 1 year of age.

Morphology
The classification of morphological variants of the defect includes four main types of TADLV.

1. Supracardial (45-55%) - all pulmonary veins are drained by a common collector into the vertical, innominate or accessory left superior vena cava. Usually, four abnormally located pulmonary veins (two from each lung) join just behind the LA and form a common venous collector called the vertical vein, which runs anteriorly from the left pulmonary artery and left main bronchus to join the innominate vein. Less common is anomalous drainage into the left brachiocephalic vein, right superior vena cava, or azygos vein.

Venous obstruction is uncommon in type I TALV, but external obstruction may occur if the vertical vein lies between the anterior left pulmonary artery and the posterior left main bronchus.

2. Cardiac (25-30%) - the connection of the pulmonary veins with the heart occurs at the level of the heart. All pulmonary veins drain into the coronary sinus or into the right atrium.

3. Infracardial (13-25%) - drainage of the pulmonary veins occurs in the portal or inferior vena cava, i.e. at the level either under the heart or under the diaphragm. The pulmonary veins join behind the LA to form a common vertical descending vein that runs anterior to the esophagus and then follows through the diaphragm through the esophageal opening. This vertical vein usually joins the portal vein, either the hepatic veins or the inferior vena cava.

4. Mixed (5%) - a combination of several of these types, when at least one of the main pulmonary lobar veins drains separately from the rest into the listed vessels.

Hemodynamic disorders
With this defect, the patient cannot survive without an accompanying atrial septal defect, since all venous blood from the lungs enters the right atrium. The leading disorder in TADLV is obstruction of the pulmonary venous blood flow, which is most pronounced in the infracardial type of defect. Because of this, serious pulmonary hypertension develops, in which venous congestion is accompanied by damage to the arterial bed of the lungs with spreading hypertrophy and the development of the muscle layer, up to the walls of small pulmonary arteries. The left atrium is small due to low volume load, and LV size is usually in the lower limit of normal. Such functional LV hypoplasia is often accompanied by a low cardiac index in the early postoperative period.

Since all venous return, both systemic and pulmonary, goes to the right side of the heart, the survival of the child depends on the presence of a right-to-left shunt, which is more often an ASD or a patent foramen ovale. Due to the fact that mixed arterial and venous blood enters the left sections, patients always have cyanosis, the degree of which depends on the volume of arterial blood from the pulmonary veins that entered the RA, and this, in turn, is determined by the presence or absence of obstruction of venous blood flow in the lungs. Obstruction of venous blood flow in the lungs almost always leads to an increase in pressure in the pancreas and pulmonary artery.

Severe obstruction of pulmonary venous flow rarely occurs when systolic pressure in the pancreas is less than 85% of the systemic level. blood pressure. If there is no severe obstruction of venous blood flow, then the volume of blood in the lungs is significantly increased, since venous blood freely returns from the lungs to the compliant pancreas. An increase in the volume of pulmonary blood flow can lead to an increase in pressure in the pancreas to the level of systemic blood pressure. However, an excess of pressure in the pancreas over systemic blood pressure is hardly possible without obstruction of the pulmonary veins. Due to the obstruction of the pulmonary venous blood flow, muscle hypertrophy of the arteries of the pulmonary circulation, up to small vessels, quickly joins, which leads to pulmonary hypertensive crises in the postoperative period.

Timing of symptoms
Depends on the degree of obstruction of the pulmonary venous blood flow. If it is expressed, then the symptomatology unfolds from the first hours of life.

Symptoms
On auscultation, the II tone is split or enhanced at the base of the heart. There is either no heart murmur at all, or a soft systolic ejection murmur over the LA is heard in the second or third intercostal space to the left of the sternum. With an increase in pulmonary hypertension and a sharp dilatation of the pulmonary artery, a diastolic murmur may appear above it. On examination, the borders of the heart were enlarged to the right and the right ventricular pulsation was increased. To characteristics This defect includes shortness of breath, tachycardia, hepatomegaly, peripheral and abdominal edema. With severe obstruction of the pulmonary venous return, cyanosis, respiratory distress syndrome, tachycardia and arterial hypotension occur from the 1st day of life. Such patients may die in the first days of life from pulmonary edema and low cardiac output syndrome.

If there is no severe obstruction of pulmonary venous blood flow, then the symptoms are determined by the degree of pulmonary hypertension. With significant pulmonary hypertension with a large volume of pulmonary blood flow, shortness of breath, severe sweating, feeding difficulties, poor weight gain, moderate cyanosis (blueness of the lips during exercise draws attention first of all). Such children without surgical treatment may die during the first year of life from congestive pneumonia or heart failure. When the pressure in the pulmonary circulation is not increased sharply, the symptoms of heart failure and cyanosis are mild and therefore the diagnosis may be delayed.

Diagnostics
On a frontal chest x-ray, changes depend on the age of the child and the type of TALV. With severe venous obstruction in newborns, the heart shadow is normal or dilated and signs of pulmonary edema are expressed. In supracardial drainage, the heart shadow appears in a figure-of-eight or snowman pattern on anterior chest x-ray and is due to dilatation of the venous collector and superior vena cava. The head of this "snowwoman" is formed by the dilated vertical vein on the left, the innominate vein above, and the superior vena cava on the left. right side, and the torso - increased PP. Typically, all four abnormally draining pulmonary veins (two from each lung) join just behind the LA to form a common vein, known as the "vertical vein," which runs anterior to the left pulmonary artery and left main bronchus to join the innominate vein.

In the cardiac type, there are no specific changes, except for a sign of compression of the esophagus by the expanded coronary sinus in the lateral projection. With the infracardial type, the shadow of the heart is not changed, but signs of venous congestion in the vessels of the lungs are expressed.

On the electrocardiogram - the deviation of the electrical axis to the right (+90...+180?), signs of hypertrophy of the pancreas and PP.

Laboratory data - decrease in SpO2 and PaO2, metabolic acidosis.

Doppler echocardiography reveals dilatation of the right and left ventricles, the LA trunk, paradoxical movement of the IVS, signs of tricuspid regurgitation, increased pressure in the right and left ventricles, the presence of PFO or ASD with right-left shunt.

The natural evolution of vice
With an obstructive form, death can occur in the first weeks of life, and with a non-obstructive form, life expectancy ranges from several years to 2-3 decades.

Observation before surgery
With signs of heart failure and pulmonary hypertension, diuretics are prescribed. Perhaps the appointment of digoxin in low doses. With obstructive TALV, intubation, mechanical ventilation in hyperventilation mode with 100% O2 and positive expiratory pressure, intravenous infusion of prostaglandin E1, dopamine, correction of metabolic acidosis are prescribed. Tachycardia should not be fought because it compensates for the cardiac index.

Terms of surgical treatment
In the obstructive form of TALVD, immediate radical surgery is required in the neonatal period, since there are no methods of palliative correction. The introduction of prostaglandin E1 can lead to an increase in cardiac output due to a right-to-left shunt through the duct, but will not save the patient from deep hypoxemia, which leads to decompensated acidosis and death. If severe obstruction of venous blood flow has not developed after birth, then vascular resistance in the ICC decreases, and the right ventricle is able to take a significant volume load from the pulmonary bed. This is accompanied by right-sided dilatation and congestive right ventricular failure. To avoid this complication, radical surgery should be performed in the first months of life to avoid serious complications of these disorders.

Types of surgical treatment
In the intracardiac form, abnormally draining pulmonary veins are sutured to the left with simultaneous closure of the ASD with one large patch. In the supracardial form, if it is anatomically possible, an anastomosis is placed between the pulmonary vein collector and the left atrium and the communication of the collector with the SVC system is closed. With a significant distance of the pulmonary vein collector from the walls of the LA, the collector is cut off from the SVC system and directly stitched into the LA. Both techniques are complemented by the closure of an ASD. In the infracardial form, a tunnel is created with a patch in the IVC with the transition to the RA for the outflow of blood from the pulmonary veins into the LA, and the interatrial defect is closed. With a mixed form, a combination of the above methods can be used.

The result of surgical treatment
Early postoperative mortality is less than 10%. The results are better with the cardiac type of TALV and worse with the infracardial type.

Postoperative follow-up
Within 1 year - 2 times a year, later - 1 time per year. For 6 months after surgery or longer, drugs are prescribed to treat congestive heart failure and pulmonary hypertension.

Sometimes, with a favorable course of the early postoperative period, obstruction of the venous pulmonary blood flow may develop from the 3rd to the 6th month after surgery. The cause of this is most often fibrointimal dysplasia of the pulmonary veins near the anastomotic area. It is not possible to manage this cause with the help of surgical methods (including balloon dilatation). Expansion of the narrowed areas with patches from the pericardium is ineffective, a patch from the atrial tissue is effective in relatively mild cases. The main factor determining this complication is the low value of the ratio of the sum of the diameter of the pulmonary veins to the body surface area; at higher values ​​of this ratio, postoperative pulmonary venous obstruction develops much less frequently.