Abnormal drainage of the pulmonary veins in newborns. What you need to know about abnormal pulmonary venous drainage? Complications of abnormal drainage of the pulmonary veins

Anomalous pulmonary venous drainage (ADPV) is a defect in which all (total) or individual (partial) pulmonary veins drain into the right atrium or the vena cava leading to it. The first description of the vice belongs to J. W. Winslow (1739) and J. G. Wilson (1798). The frequency of ADLV is 0.5 - 2% of all congenital heart defects [Bankl H., 1980].

Partial anomalous drainage of the pulmonary veins

Partial ADLV accounts for one third of all cases of this defect, its frequency among other congenital heart defects is 0.7 - 9.4%, at autopsy it occurs in 0.5% (I in 200 cases of all congenital heart defects) in combination with ASD in 10 - 15 % [Bankl H., 1980|. It is likely that a significant number of cases of partial ADLV remain undiagnosed due to the lack of clinical manifestations.

the pulmonary veins flow into the left innominate, superior vena cava or one of their branches; 2) cardiac level - drainage of all or part of the pulmonary veins into the cavity of the right atrium or coronary sinus; 3) the infracardial part of the pulmonary veins flows into a venous vessel located below the heart; 4) mixed,

Most patients have a concomitant secondary ASD (often a venous sinus defect) and a patent foramen ovale. Autopsy shows enlarged cavities of the right atrium and ventricle, dilated pulmonary arteries and vena cava; the left chambers of the heart are unchanged. Usually at the confluence there are several mouths of the pulmonary veins; sometimes they gather into one trunk and open at a common mouth. In 20% of cases, other congenital heart defects occur (VSD, transposition of the aorta and pulmonary artery, tetralogy of Fallot, single ventricle, pulmonary stenosis, dextrocardia).

Hemodynamics. With partial ADLV, hemodynamic disturbances are similar to those with ASD and are determined by the number of abnormally draining veins, the size of the left-to-right shunt and the size of the ASD accompanying congenital heart disease. If one pulmonary vein is drained and there is no ASD, the defect remains asymptomatic. The pressure in the pulmonary artery with partial ADPV remains normal for a long time, since blood discharge occurs at the level of the atria and there is no transfer pressure factor (from the left ventricle and aorta to the right ventricle and pulmonary artery, as with VSD and PDA).

Clinic, diagnostics. With partial ADLV, clinical manifestations may be absent for a long time or resemble those of a secondary ASD. The main symptoms of the defect are not typical: fatigue, pain in the heart, shortness of breath on exertion, possible repeated pneumonia, lag in physical development. With age, a cardiac hump appears, the boundaries of cardiac dullness expand more to the right. The auscultatory picture is similar to that of secondary ASD: soft systolic murmur at the site of the pulmonary artery projection, splitting of the second tone. Heart failure in children rarely develops, usually in cases where more than 50% of the pulmonary veins are abnormally drained; in adults, deterioration of general condition appears earlier than in cases of isolated ASD.

On the ECG with partial ADPV there are no characteristic symptoms, the electrical axis of the heart is located normally or deviated to the right, there are signs of overload of the right parts (sometimes more pronounced than with isolated ASD), incomplete blockade of the right bundle branch (rSR in lead Vi), the interval may be prolonged PR

At X-ray examination of the chest organs, the state of the pulmonary pattern is determined by the size of the arteriovenous discharge. With partial ADPV, it is more enhanced along the arterial bed if the discharge is more than 50 % minute volume of the pulmonary circulation. In these same cases, bulging of the pulmonary artery trunk and increased pulsation of the roots of the lungs are possible. The heart shadow is enlarged due to the right parts; the right atrium is dilated to a greater extent than in isolated ASD. With ADLV into the superior vena cava, its expansion is visible. An oval shadow from the right pulmonary veins, going to the inferior vena cava along the right border of the heart (saber syndrome or Turkish saber pattern), is a reliable sign of a defect in cases of abnormal flow of the right pulmonary veins into the inferior vena cava.

To date, there are no convincing echocardiographic criteria to diagnose partial anomalous drainage of the pulmonary veins. Its indirect (hemodynamic) manifestations are: dilatation of the right ventricle, paradoxical movement of the interventricular septum, small sizes of the left atrium and ventricle, increased excursion of the tricuspid valve. With two-dimensional echocardiography, partial ADPV should be suspected if the anatomical septal defect does not correspond to the hemodynamic manifestation of arteriovenous shunting,

When catheterizing the cavities of the heart, the probe from the right atrium enters one of the vena cava and from there into the free pulmonary field. It is necessary to carry out a step-by-step examination of the vena cava and the right atrium, which makes it possible to establish the level of confluence of the pulmonary veins and their number. When the probe from the right atrium enters the trunk of the pulmonary vein, it has a gentle course in the interval between the mouths of the cava and pulmonary veins, deviating to the right from the midline, or passes directly along the edge of the shadow of the right atrium. In some cases, the probe can pass into the trunk of the pulmonary vein through the ASD and the cavity of the left atrium. The connection of the pulmonary veins with the vena cava is indicated by an increase in blood oxygen saturation at the levels of the left innominate, superior or inferior vena cava.

With abnormal drainage into the right atrium, arterialization of blood in this cavity is observed, which is also typical for an isolated ASD. The pressure in the chambers of the heart may be normal.

The most valuable diagnostic technique should be considered selective angiocardiography with the introduction of a contrast agent into the trunk of the pulmonary artery or selectively into its right or left branches. After tight and rapid filling of the pulmonary artery with contrast agent, the capillary phase is visible, then the course and place of confluence of all pulmonary veins are revealed! complete veins, right atrium and ventricle, left chambers of the heart.

Treatment. When correcting partial ADLV, various operations are used depending on the type of defect (drainage level, size and location of the ASD). In case of abnormal drainage of part of the pulmonary veins into the superior vena cava or its mouth, the following tactics are used: a) creating, using a patch, a tunnel connecting the mouths of the abnormally draining pulmonary veins with the left atrium through a septal defect; b) creation of two tunnels from the trunk of the superior vena cava to form separate - systemic and pulmonary - blood flows and drain blood from the pulmonary veins. directly to left atrium through ASD; c) suturing the lower edge of the defect to the posterior wall of the vena cava above the mouths of the abnormally draining pulmonary veins. If there is abnormal drainage of the pulmonary veins into the cavity of the right atrium, patch surgery is performed. With a small ASD, it is dilated in order to create an adequate tunnel that does not impede the flow of blood from the pulmonary veins into the left atrium. In case of isolated anomalous drainage of the pulmonary veins into the superior vena cava or right atrium, it is recommended to create an artificial atrial septal defect with subsequent correction of the defect. In case of abnormal drainage of the pulmonary veins into the inferior vena cava, one of the above methods is used, or the pulmonary vein collector is cut off and implanted into the left atrium. In case of abnormal drainage of the left pulmonary veins into the left vertical vein, this vein is ligated, the left pulmonary veins are cut off and they are implanted into the left atrial appendage.

The results of the operation are good. Surgical mortality does not exceed 2 - 3%. Complications include partial narrowing of the superior vena cava, impaired drainage of the pulmonary veins, and damage to the sinus or atrioventricular nodes.

Total anomalous pulmonary venous drainage

The frequency of total ADLV according to clinical data is 1.6 - 7.7% [Pariyskaya T.V., Veniamnova G.N., 1986]. according to pathological anatomical data - about 2% [Bankl H., 1980).

Anatomy, classification. With total ADLV, there is no communication between the pulmonary veins and the left atrium at all, all the blood from the pulmonary circulation enters the right atrium or the vessels leading to it, and mixing of arterial and venous blood occurs in them. The patient's life is possible only if there is a concomitant ASD. If there is no ASD, but only a patent ductus arteriosus, patients die at an early age, since the left parts of the heart do not participate in the blood circulation; death also occurs in cases of premature (before or shortly after birth) closure of the oval window. There are many varieties of this complex congenital heart disorder, and therefore various classifications have been proposed. The simplest classification is R. Darling et al. (1957), they distinguish four types of defect: type I - supracardial - drainage of all pulmonary veins by a common trunk (collector) through the anomalous vein into the superior vena cava, through the vertical vein - into the left innominate, through the anomalous vein - into the azygos (47 %); type 11 - cardiac - the flow of all pulmonary veins into the right atrium or coronary sinus (30%); III type - infracardial - the flow of all pulmonary veins into the portal or inferior vena cava (18%); Type IV - mixed - various combinations of these three types (5%) (Fig. 13). Before flowing into the heart or the vessel leading to it, the pulmonary veins are collected into a single chamber - a collector, which is then connected to the systemic circulation at different levels. With this type of LV BP, dilatation of the right chambers and the pulmonary artery trunk are noted, while the left parts of the heart are usually normal.

With total ADPV, there may be anatomical causes of obstruction in the pulmonary venous drainage: most often this is associated with a narrowing of the mouths of abnormally draining pulmonary veins or the opening of the collector, less often there is external pressure on the anomalous vein (at the point of passage through the diaphragm, between the left pulmonary artery and the left main bronchus). At 80 % cases, obstruction of the pulmonary veins accompanies the infra-diaphragmatic variant of ADPV.

The most common concomitant congenital heart disease with total ADPV are the common atrium, a single ventricle, hypoplasia of the left, transposition of the great vessels (this combination creates a functional correction of the defect). In 25 - 30% of cases, extracardiac malformations of the gastrointestinal tract (abnormal rotation of the intestine, diverticula, umbilical hernia), spleen (agenesia, multiple accessory spleens), genitourinary

(congenital kidney cysts, hydronephrosis) and musculoskeletal systems.

Hemodynamics. With total ADLV, blood enters the right atrium from both circulations; the magnitude and direction of discharge are determined by the ratio of the resistances of the systemic and pulmonary circulations and the diastolic relaxation of the ventricles. A large amount of arterial blood enters the right atrium from the collector into which the pulmonary veins flow. In the atrium, arterial blood mixes with venous blood, from here, through the ASD, part of the blood (smaller) enters the left atrium and then into the systemic circulation; more blood is directed to the pulmonary circle, contributing to a decrease in the left chambers of the heart and the development of pulmonary hypertension. If the ASD reaches a significant size and the discharge of blood into the systemic circulation is sufficient, then the left ventricle does not become hypoplastic, and pulmonary hypertension does not exceed grade I - II. and patients can live to adulthood. In cases of pulmonary vein obstruction, venous pulmonary hypertension develops; in the absence of physiological involution in the structure of the pulmonary vessels, congenital pulmonary hypertension is also possible. Thus, total ADLV is characterized by the circulation of mixed blood with a slight decrease in the oxygen content in the systemic circulation, overload of the right parts of the heart, and pulmonary hypertension (arterial, venous, congenital).

Clinic, diagnostics. The clinical course of total ADPV is determined by the anatomical and hemodynamic features of the defect, in particular the level of pulmonary resistance, the degree of pulmonary venous obstruction, the size of the interatrial communication, and the condition of the right ventricular myocardium. The first signs of this type of defect often appear from the first days and months of life;

heart manifestations

insufficiency, repeated pneumonia and acute respiratory viral infections, cough, retardation in physical development. Cyanosis can appear at any age, often towards the end of the first year of life. As a rule, it is insignificant and noticeable only when screaming; pronounced cyanosis from the first days of life is characteristic of pulmonary venous obstruction. The cardiac hump (mostly right-sided) appears earlier than with isolated ASD. Systolic tremor is usually absent. On auscultation, 1 tone in the area of ​​the tricuspid valve is increased (a sign of increased blood flow through it). II tone above the pulmonary artery is accentuated, split, often present III tone; in the second intercostal space on the left, a medium-intensity systolic murmur is heard (as with an ASD); a mesodiastolic murmur is possible at the lower edge of the sternum on the left. If the collector drains into the superior vena cava and there is pulmonary venous obstruction, a prolonged systolic murmur may be heard above the clavicle on the right or left.

In adult patients with total ADPV and pulmonary hypertension, a systolic ejection tone is heard in the second intercostal space on the left (see Chapter 2). Right ventricular failure in combination with clinical symptoms ASD and moderate cyanosis helps to suspect total ADLV in children. In adult patients with this defect, the clinical picture is indistinguishable from ASD.

form R in Vi). Syndrome may occur W.P.W. type B. With increasing load on the right ventricle, a shift in the interval appears ST below the isoline with deep negative teeth T in leads 11, III, aVF, V (- V ^.

Phonocardiography confirms the auscultation data and has all the signs of ASD.

On the radiograph with total ADLV, the pulmonary pattern is significantly enhanced both in the arterial and venous beds, moderate or significant cardiomegaly is noted, caused by an enlargement of the right chambers, the left sections are of normal size, and sometimes an enlarged shadow of the superior vena cava is visible. For the supracardial form of the defect, a cardiac shadow in the form of a figure eight or a snow woman is typical, where the lower part is the heart itself, and additional education at the top right is a collector that collects blood from all pulmonary veins and opens into the left or right vena cava or innominate vein (Fig. 15). The latter are dilated, as they accommodate a large volume of pulmonary blood flow. Sometimes the shape of the heart imitates 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.

M - echocardiography does not reveal the defect; indirect signs are the relatively small size of the left atrium and left ventricle, dilatation of the right atrium and ventricle, paradoxical movement of the interventricular septum. When the pulmonary veins flow into the coronary sinus, two-dimensional echocardiography makes it possible to determine the space formed by the venous collector behind the shadow of the heart, which is confirmed by intravenous administration of ultrasound contrast.

Probing of the heart cavities with total ADPV reveals high blood saturation with oxygen in all parts of the heart (90 - 96%); in the pulmonary artery the saturation is sometimes the same as in the aorta. The highest saturation is observed in the right atrium or in the vein into which the pulmonary vein collector drains. Pressure in the pulmonary artery can be increased due to changes (fetal, sclerotic) in the pulmonary arterioles, increased pulmonary blood flow, obstruction of the pulmonary veins (pulmonary capillary pressure also increases at the same time). The pressure in the atria is the same or higher in the right than in the left. In such cases there are small sizes ASD and severe course of the disease from the first days. Urgent atrioseptostomy (Rush-kind procedure) during catheterization is often indicated.

The introduction of a contrast agent into the pulmonary artery allows you to immediately make the correct diagnosis (Fig. 16). The contrast agent is detected in the pulmonary vein collector (PVC), through the vertical vein (VV) enters the left innominate vein (SV), then into the superior vena cava and the right atrium.

If the collector flows into the coronary sinus, the latter appears as an ovoid formation in the lower part of the right atrium (better visible in the lateral projection). When the pulmonary veins flow into the inferior vena cava, the contrast agent, after the capillary phase, fills the collector that drains into this vessel above or below the diaphragm.

Differential diagnosis. In newborns and children in the first months of life, this defect is differentiated from mitral or aortic atresia, mitral stenosis, triatrial heart, pulmonary vein stenosis, transposition of the great vessels, lymphangiectasia; at an older age - with MGD. P.

Course, treatment. About 80 % children with total ADPV die in the first years of life; This is especially true for infracardial (infradiaphragmatic) and other variants of the defect with pulmonary venous obstruction. The main causes of death of patients: heart failure, pneumonia, premature closure of the oval window. The later the first symptoms of the disease appear, the better the prognosis. Observations are described when patients lived up to 30 years; in such cases, there is good mixing of blood at the level of the atria due to the sufficient size of the ASD, there is no venous obstruction, the level of pressure and pulmonary resistance is low, the condition of the right ventricular myocardium is satisfactory. In the presence of heart failure, the administration of cardiac glycosides and diuretics (see Chapter 22) is indicated. Effective drug therapy makes it possible to postpone surgical treatment until an older age, since correction of the defect at an early age is accompanied by a mortality rate of 35 to 50% [Falkovsky G. E. et al., 1986; Clarke D. et al., 1977|

In case of total ADLV in the right atrium (cardiac form), the operation is performed using artificial circulation and hypothermia and consists of enlarging the inter-atrial defect and applying a patch in such a way as to direct blood flow from the mouths of the pulmonary veins through the defect into the left atrium. In cases where the collector is drained through the coronary sinus, to create adequate drainage, part of the septum located between the coronary sinus and the oval window is excised, the posterosuperior wall of the coronary sinus is incised, and the patch displaces the wall and the coronary sinus into the left atrium. For supracardial and infracardial types of defect, an anastomosis is performed between the pulmonary vein collector and the left atrium [Burakovsky V.I. et al., 1989].

In case of severe hypoplasia of the left ventricle, correction of the ASD with a perforated patch or two-stage surgical treatment is used, including: stage 1 anastomosis between the collector and the left atrium (allows development of the left chambers of the heart), stage 11 - ligation of the vertical

vein through which the pulmonary veins drain into the right superior vena cava.

Mortality after surgery in children under 6 years of age, according to A. Galloway et al. (1985) was 10%. It is higher in the infracardiac form, drainage of the pulmonary veins into the superior vena cava, especially into the right (than when they flow into the coronary sinus or directly into the right atrium), with pulmonary hypertension, hypoplastic left ventricle and left atrium.

When performing an operation in childhood Long-term results are usually good, since sclerotic changes do not have time to develop in the vessels of the pulmonary circulation and in the myocardium of the ventricles of the heart. If the anastomosis shows signs of functional obstruction, repeat surgery is indicated.

On echocardiography, anomalous pulmonary venous drainage (APDV) is identified when some or all of the pulmonary veins do not connect directly to the left atrium. Moreover, if only some veins connect abnormally, then the condition on ultrasound of the heart is described as partial ADLV, and if all the veins abnormally do not flow into the left atrium, then a diagnosis of complete (total) ADLV is established. There are many possible abnormal connections from the pulmonary veins to the cardiac chambers or extracardiac vessels.

Complete anomalous pulmonary venous drainage on cardiac ultrasound

IPALV is usually present in early childhood. This pathology usually manifests itself immediately, since the pathological connection of all veins manifests itself as a critical condition in sick newborns with respiratory failure and pulmonary edema. The physiological features in these cases are similar to those of very severe mitral valve stenosis. Unobstructed connections behave like a large shunt at the level of the atria, and patients experience severe murmurs or heart failure, most often within the first year of life. PALVs are usually classified based on the position of the abnormal connections in relation to the heart. A supracardial connection occurs when the common pulmonary vein connects to the higher systemic venous circulation (superior vena cava or azygos vein). The connection between the pulmonary venous vessels and the systemic venous circulation is usually described as a vertical vein or vertical draining vein. This connection may be difficult to detect on echocardiography when the left vertical vein moves between the left pulmonary artery and the left main bronchus and is compressed by these structures, creating a so-called vascular loop.
PAPV with a cardiac connection occurs when the common pulmonary vein connects directly to the cardiac chamber or coronary sinus. These connections are almost never stenotic. On the contrary, infracardiac connections are almost always obstructive. The common pulmonary vein usually connects with the inferior vena cava, which crosses the diaphragm and connects to the hepatic circulation through the ductus venosus. These connections are always obstructive at or below the level of the diaphragm.
Mixed compounds do not have a common venous confluence. In these cases, individual pulmonary veins connect to different sites in the systemic venous chain. Connections occur in various combinations of the three types listed above. Due to mixed connections, it is necessary during an echocardiographic examination to individually identify each of the veins that drain the five main segments of the lungs. These five segments are the right upper, middle and lower lobes, and the left upper and lower lobes.

Partial anomalous pulmonary venous drainage on echocardiography

Partial anomalous pulmonary venous drainage may coexist with an ASD or it may occur in isolation. In any case, the size of the shunt from left to right is smaller than in PADLV. As a result, these patients present with symptoms and findings similar to those present with isolated ASD later in life. Obstructive connections are rare in PAD. Most patients have an abnormal connection involving only one of the two lungs. Common patterns of partial anomalous pulmonary venous drainage on echocardiography include the following:

1. The right superior and middle veins drain into the SVC or both into the right atrium - this is often associated with ASD of the sinus venosus type.
2. The right inferior pulmonary vein drains into the inferior vena cava (IVC), as in Scimitar syndrome. These patients usually have a hypoplastic right lung with an intact interventricular septum. Some patients also have incomplete connection of the lower lobe of the bronchus to the central airway and abnormal arterial supply to this area (bronchopulmonary sequestration).
3. The isolated left pulmonary vein drains into the left vertical vein (a remnant of the embryonic left anterior cardinal vein). The vertical vein connects to the SVC and the shunt flow reaches the heart. Alternatively, one or both of the left pulmonary veins may connect directly to the coronary sinus in this case.

10 U d/m2 initially and > 7 U d/m2 after the use of vasodilators); - the presence of absolute contraindications due to concomitant somatic pathology. Surgical tactics In the absence of symptoms, correction should be carried out before the age of 3 - 4 years. Timely correction allows you to avoid long-term complications of PAD: pulmonary hypertension and right ventricular failure, atrial flutter and fibrillation. In patients with Scimitar syndrom e, symptoms of heart failure develop in infancy. Surgical treatment is staged - first of all, cardiac catheterization is performed to detect and embolize aortopulmonary collaterals to the right lung. Next, depending on the symptoms of heart failure, surgical correction of PAD is performed. Surgical technique If the ASD is small or absent, it is expanded or formed. The PV orifice is moved into the RA cavity using a patch; it is sewn so as not to narrow the PV orifice. If stenosis of the vena cava occurs, additional plastic surgery of its mouth is performed with an autoxenopericardial patch. Techniques for correcting PAD: - isolation and transplantation of abnormally flowing PVs into the left atrium or into normally flowing PVs; - division of the common trunk of the superior or inferior vena cava into two channels (collector of the pulmonary veins and collector of the vena cava) with the subsequent direction of their flows into the atria; - anastomosis of the distal end of the SVC (the PV is drained through the proximal segment of the SVC); - intracardiac switching through the ASD of the venous flows of the systemic and pulmonary circulation; As a material for plastic surgery, I use tautopericardium treated with glutaraldehyde or xenopericardium. Specific complications surgical treatment: - residual shunt between the atria; - stenosis of the mouths of the vena cava or pulmonary veins; - sick sinus syndrome during plastic surgery of the orifice of the VIV; NRS, tachyarrhythmias. Postoperative observation 1. The duration of observation of patients with corrected PALV in the absence of hemodynamic disorders is no more than 2 years. Before deregistration, an ECG and ultrasound examination of the heart are performed. 2. In case of registration of NRS (bradyarrhythmias, atrial tachycardia) in the postoperative period, in addition to the examination, it is recommended that SMECG be performed every 6 months or more often. If indicated, antiarrhythmic therapy, RFA, or pacemaker implantation are performed. 3. Prevention of bacterial endocarditis is carried out according to indications in the first 6 months after surgical correction of the defect. 4. Admissibility of physical education and sports after correction of the defect." width="448" height="348" srcset="//i3.wp..jpg 448w, //i3.wp.-300x233.jpg 300w" sizes=" (max-width: 448px) 100vw, 448px"> PAD is characterized by the fact that one or more, but not all, pulmonary veins drain into the right atrium and its tributaries. The pulmonary veins can drain into the innominate vein, coronary sinus, SVC (superior vena cava vein), RA (right atrium), IVC (inferior vena cava), portal veins, hepatic veins.

Unlike TADPV (total anomalous drainage of the pulmonary veins), this defect can occur without combination with ASD (atrial septal defect) (5.7%).

The most clinically significant variants of NADLV:

— the veins of the upper and/or middle lobe of the right lung flow into the SVC below the mouth of the innominate vein (combined with ASD “sinus venosus”);

- the veins of the lower lobe of the right lung enter the IVC above or below the level of the diaphragm (part of the “Scimitar syndrome”

- abnormal arterial blood supply and hypoplasia of the lower lobe of the right lung, dextroposition of the heart);

- drainage of the left PVs (pulmonary vein(s)) into the innominate vein through the left vertical vein.

The hemodynamics of PAD is in many ways similar to ASD, with the only difference being that hypervolemia of the ICC (pulmonary circulation) is associated not with left-right shunting, but with its partial recirculation through the lungs. The volume of recirculation depends on the number of draining pulmonary veins, the presence of DMMP and the value of TPR (total pulmonary resistance). The rate of development of PH (pulmonary hypertension) depends on the volume of blood recirculating through the lungs. PH reaches a significant degree in the 3rd-4th decade of life.

CLINIC

A. Clinical manifestations diseases:

- children with PAD are usually asymptomatic;

— in the presence of drainage of the right PVs into the IVC, children may have frequent bronchopulmonary infections.

b. Physical examination: constant splitting of the II tone in the II m.r. to the left of the sternum, regardless of the phases of breathing (with a combination of PAD and ASD);

weak or moderate intensity (no more than 3/6) systolic murmur of relative stenosis of the pulmonary valve in the second stage of birth. to the left of the sternum;

Gentle mesodiastolic murmur of relative stenosis of the TC (tricuspid valve);

Along the left edge of the sternum in the lower third.

DIAGNOSTICS

1. Electrocardiography

— RV hypertrophy (right ventricle);

— complete or incomplete blockade of the right bundle branch;

- a normal variant of the ECG cannot be excluded.

1. Echocardiography

Diagnosis of PAPV is usually achieved by detecting the absence of the typical entry of one or more pulmonary veins into the left atrium in typical locations.

High ASDs of the “sinus venosus” type are usually combined with PAD.

Diagnostic criteria:

— dilatation of the right departments;

- expansion of the pulmonary artery;

- relative decrease in LA and LV;

- dilated superior vena cava.

TREATMENT AND OBSERVATION

1. Monitoring and treatment of patients with uncorrected PALV

A. Prevention of bacterial endocarditis is not indicated.

b. Correction of symptoms of heart failure (in rare cases).

V. Admissibility of physical education and sports until the defect is corrected.

1. Surgery

Indications for surgical treatment

A confirmed diagnosis of PAD is an absolute indication for surgical treatment.

Contraindications to surgical treatment:

- high pulmonary hypertension (TLC > 10 U d/m2 initially and > 7 U d/m2 after the use of vasodilators);

— the presence of absolute contraindications due to concomitant somatic pathology.

Surgical tactics

In the absence of symptoms, correction should be carried out before the age of 3-4 years. Timely correction allows you to avoid long-term complications of PAD: pulmonary hypertension and right ventricular failure, atrial flutter and fibrillation.

In patients with Scimitar syndrome Symptoms of heart failure develop in infancy. Surgical treatment is staged - first of all, cardiac catheterization is performed to detect and embolize aortopulmonary collaterals to the right lung. Next, depending on the symptoms of heart failure, surgical correction of PAD is performed.

Surgical technique

If the ASD is small or absent, it is expanded or formed. The PV orifice is moved into the RA cavity using a patch; it is sewn so as not to narrow the PV orifice. If stenosis of the vena cava occurs, additional plastic surgery of its mouth is performed with an autoxenopericardial patch.

Techniques for correcting PAD:

— isolation and transplantation of abnormally flowing PVs into the left atrium or into normally flowing PVs;

- division of the common trunk of the superior or inferior vena cava into two channels (collector of the pulmonary veins and collector of the vena cava) with the subsequent direction of their flows into the atria;

— anastomosis of the distal end of the SVC (the PV is drained through the proximal segment of the SVC);

— intracardiac switches through the ASD of the venous flows of the systemic and pulmonary circulation;

As a material for plastic surgery, I use tautopericardium treated with glutaraldehyde or xenopericardium.

Specific complications of surgical treatment:

- residual shunt between the atria;

- stenosis of the mouths of the vena cava or pulmonary veins;

— sick sinus syndrome during plastic surgery of the orifice of the VIV; NRS, tachyarrhythmias.

Postoperative follow-up

1. The duration of observation of patients with corrected PALV in the absence of hemodynamic disturbances is no more than 2 years. Before deregistration, an ultrasound examination of the heart is performed.
2. In case of registration of NRS (bradyarrhythmias, atrial tachycardia) in the postoperative period, in addition to the examination, it is recommended that SMECG be performed every 6 months or more often. If indicated, antiarrhythmic therapy, RFA, or pacemaker implantation are performed.
3. Prevention of bacterial endocarditis is carried out according to indications in the first 6 months after surgical correction of the defect.
4. Admissibility of physical education and sports after correction of the defect.

website - 2007

At partial anomalous drainage of the pulmonary veins one or more pulmonary veins drain abnormally into the right atrium or great veins, but the normal flow of some pulmonary veins into the left atrium is preserved. The most common occurrence is anomalous drainage of the pulmonary veins from the right lung in 90% of all cases. Only partial anomalous drainage of the pulmonary veins is found in 2-10% of patients; in the rest, this defect is combined with an atrial septal defect, less often with other defects.

Circulatory disorders with abnormal drainage of the pulmonary veins are caused by pathological discharge of arterial blood, which should enter the left side of the heart, from the pulmonary veins, back into the pulmonary artery system, accompanied by an increase in pressure in the vessels of the lungs. The degree of development of circulatory disorders is influenced by the number of abnormally draining veins, the level of their confluence, the presence and size of the atrial septal defect.

In newborns and children early age manifestations of the defect are subtle. Older children may complain of shortness of breath, increased fatigue when physical activity. There is a noticeable delay in physical development. In older patients age groups palpitations and disturbances occur heart rate. Inflammatory lung diseases – pneumonia – often occur. The course of the defect depends on the volume of blood discharge, on the duration of the defect’s existence, and on the compensatory capabilities of the pulmonary vascular system. The average life expectancy of such patients without surgical treatment is about 40 years.

Diagnosis of partial anomalous pulmonary venous drainage

When listening, a systolic murmur and splitting of the second tone above the pulmonary artery are detected. The electrocardiogram shows the load on the right side of the heart. X-ray examination reveals an enlargement of the right chambers of the heart, and sometimes signs of abnormal drainage of the pulmonary veins are found. The diagnosis is clarified using cardiac catheterization and angiocardiography. The location and confluence of the pulmonary veins is especially reliably revealed by selective angiocardiography from the pulmonary artery.

Treatment of partial anomalous pulmonary venous drainage

Treatment of partial anomalous drainage of the pulmonary veins is surgical. Surgery may not be recommended for patients with severe pulmonary hypertension. Sometimes, if the defect is very mild, surgical treatment may not be prescribed. The operation in most cases takes place while connected to a heart-lung machine. Access to the heart is carried out through a midline incision in the chest and dissection of the sternum. The goal of surgical treatment is to move the mouths of all abnormally flowing veins into the left atrium. This is accomplished by partially moving the interatrial septum. The easiest way to perform surgery is for the cardiac type of defect, when abnormally located pulmonary veins flow into the right atrium. The right atrium is opened and the confluence of the pulmonary veins is examined. Often, with a concomitant atrial septal defect, it is possible to suture the edge of the defect to the wall of the right atrium to the right of the mouth of the anomalous veins, with the mouths of these veins ending up in the formed left atrium. A very large atrial septal defect may require a patch. The patch is applied so that the defect in the septum and the mouth of the anomalous veins are in the left atrium. When the pulmonary vein flows into the coronary sinus, it is also moved into the left atrium. Surgical treatment of anomalous drainage of the pulmonary veins into the superior vena cava can be performed in two ways. The first of them begins with isolating the superior vena cava along its entire length. The vein is then sutured with a continuous suture along its length to create two separate, isolated channels. The anteromedial canal plays the role of the superior vena cava in which venous blood flows from the upper half of the body, and the posterolateral canal becomes a continuation of the anomalous pulmonary veins flowing into it. Arterial blood from the lungs thus ends up in the posterolateral canal. After this, a heart-lung machine is connected and the right atrium is cut. The mouth of the formed posterolateral canal is moved into the left atrium and the existing atrial septal defect is closed. The second method is currently used more often. It is called intra-atrial defect correction surgery. The superior vena cava is cannulated (closed off) above the confluence of the anomalous pulmonary veins. The right atrium and the anterior wall of the superior vena cava are opened. The vein incision is extended until the mouths of all abnormally draining pulmonary veins become well accessible. Then, from the upper edge of the mouth of the pulmonary veins, a patch is sutured from one’s own pericardium or synthetic material. A channel is formed between the patch and the back wall of the vein, into which all abnormal veins open. The lower end of the patch is connected to the lower edge of the atrial septal defect. As a result, arterial blood from the pulmonary veins flows through the canal and defect in the interatrial septum into the left atrium, and the communication between the left and right atrium is closed. The incisions in the right atrium and superior vena cava are sutured. Treatment of the subcardiac type of defect, in which abnormal pulmonary veins flow into the inferior vena cava, causes the greatest difficulties. The chest is dissected along the anterolateral surface in the fourth intercostal space. The inferior vena cava and anomalous pulmonary veins are accessed. The vena cava is clamped with a special clamp. The mouths of the pulmonary veins with the adjacent portion of the inferior vena cava are cut off, and the resulting hole is sutured with a continuous suture. The pulmonary veins lead to the left atrium and connect to it. The results of surgical treatment are usually good. The mortality rate during surgery is about 3%. The long-term results of the operation are good. Significant improvement in the patients' condition occurs within the first year after treatment. The worst treatment results are usually associated with a violation of the technical side of the operation or the initially serious condition of the patient.