Abnormal pulmonary vein drainage treatment. Partial abnormal pulmonary vein drainage

Partial abnormal pulmonary vein drainage (PAPDV)

ICD 10: Q26.3

Year of approval (frequency of revision): 2016 (revised every 3 years)

ID: KR45

Professional associations:

• Association of Cardiovascular Surgeons of Russia

Approved

Agreed

Keywords

• scimitar syndrome
• dyspnea
• heartbeat
• cyanosis
• cardiomegaly
• circulatory failure
• arrhythmias
• paradoxical embolism
• postcardiotomy syndrome
• cardiac tamponade

List of abbreviations

CHD - congenital heart defects

ASD - atrial septal defect

Ischemic heart disease

CT - computed tomography

MRI - Magnetic Resonance Imaging

OLS - general pulmonary vascular resistance

ICU - Department of Reanimation and Intensive Care

RV - right ventricle

PP - right atrium

ECG - electrocardiography

Echocardiography - echocardiography

Terms and Definitions

Cardiomegaly - an increase in the heart caused by hypertrophy of the volume of the heart muscle or dilatation of the heart chambers.

Cardiac catheterization - an invasive procedure performed for therapeutic or diagnostic purposes in the pathology of the cardiovascular system.

Postcardiotomy syndrome - complication of the early postoperative period in surgery of congenital heart defects, manifested by the presence of effusion in the pericardial cavity.

Scimitar Syndrome - infracardial form of partial abnormal flow of the right pulmonary veins into the inferior vena cava.

Echocardiography - a method of ultrasound research aimed at studying the morphological and functional changes of the heart and its valve apparatus.

1. Brief information

1.1. Definition

Partial abnormal pulmonary vein drainage (PAPDV) - an anomaly in the development of the heart, characterized by the absence of connection of one or several (but not all) pulmonary veins with the left atrium. At the same time, they can drain into the right atrium, coronary sinus, basins of the superior or inferior vena cava.

1.2 Etiology and pathogenesis

Genetic, physical, environmental and infectious factors lead to disruption of organogenesis and the formation of congenital heart disease. The risk of developing a defect in an unborn child is significantly higher in those families where there are relatives with CHD. In addition to hereditary conditions, viral diseases of a pregnant woman (rubella, chicken pox, etc.), endocrinopathies, taking certain medications and alcohol during pregnancy, industrial hazards, gestational complications (toxicosis, threat of miscarriage, etc.)

1.3 Epidemiology

The frequency of partial abnormal drainage of the pulmonary veins ranges from 0.3% of all CHD according to clinical data to 0.6% according to autopsy data. Scimitar syndrome accounts for 3-6% of all PADP cases. Half of patients with isolated PADLV are able to live up to 30-40 years. The median survival rate for patients with scimitar syndrome ranges from 20 to 30 years.

1.4 ICD 10 coding

Congenital malformations of large veins (Q26):

Q26.3 Partial abnormal drainage of pulmonary veins.

1.5. Classification

CHADLV classification:

• supracardial;
• intracardiac;
• infracardiac;
• mixed.

2. Diagnostics

2.1. Complaints and anamnesis

• When collecting anamnesis, it is recommended to ask about the presence of CHD in the family and relatives, about the course of pregnancy of the child's mother (how it proceeded, whether there were infectious diseases of the pregnant woman, complications during gestation, etc.).

• When collecting complaints, it is recommended to ask about shortness of breath, palpitations, fatigue that occurs after physical exertion; poor weight gain, hemoptysis (manifested with isolated PADLV), frequent infectious lung diseases.

Comments:As a rule, this CHD has a poor clinical picture and is often asymptomatic. The phenomena of circulatory insufficiency are insignificant and can be limited to moderate weakness, sweating of the child, cyanosis of the nasolabial triangle. Children often have an asthenic physique with a noticeable pallor of the skin.

• It is recommended to carry out differential diagnosis with ASD and other defects occurring with increased pulmonary blood flow.

2.2 Physical examination

• Auscultation of the heart is recommended.

Comments:Auscultation reveals a systolic murmur with its maximum intensity in the second or third intercostal space to the left of the sternum. With large discharges of blood in some patients, a gentle diastolic murmur is heard above the tricuspid valve; II tone over the pulmonary artery is widely split regardless of the phases of respiration. In the presence of pulmonary hypertension, the pulmonary component of the II tone is enhanced.

2.3 Laboratory diagnostics

• If a patient with ASD is admitted to a specialized hospital for surgical treatment of a defect, it is recommended to determine its group affiliation, then select blood.

• A complete blood count is recommended to determine the baseline hemoglobin level before surgery.

2.4. Instrumental diagnostics.

• The diagnosis of PADLV is recommended using imaging research methods.

• Transthoracic echocardiography (echocardiography) using color Doppler is recommended.

Comments:It is possible to suspect PADLV in the absence of visualization during transthoracic examination in the projection of 4 cameras in the mode of color Doppler mapping of the flow in the left atrium from the right or left pulmonary veins. With PAPV, its expansion and accelerated flow in it (in the continuous-wave Doppler mode) are noted in the superior vena cava. In the high right parasternal position in the projection along the long axis or with subcostal access, in the projection along the short axis, the place where the PADLV flows into the superior or inferior vena cava is visualized. Difficulties may arise in the echocardiographic diagnosis of PADV of the left pulmonary veins into the coronary sinus in the presence of an upper left vena cava, mixed form of PADV. When assessing the flow in the pulmonary vein in the Doppler mode, it is possible to determine the stenosis of the vein / collector at the confluence with the right atrium, the vena cava.

• MRI is recommended if echocardiographic findings are inconclusive.

• Patients with an unclear RV volume overload are recommended to be admitted to a specialized institution in order to conduct further diagnostic studies and identify CHD.

• Cardiac catheterization with angiography is recommended for the detection of concomitant cardiac abnormalities, as well as in patients with suspected pulmonary hypertension.

Comments:An unconditional sign of abnormal drainage of the pulmonary veins, detected during catheterization of the right heart, is the passage of the probe into the pulmonary veins directly from the vena cava or the right atrium.

• A chest x-ray is recommended.

Comments:With a conventional X-ray examination, it is possible to identify signs of an increase in the arterial vascular pattern in the lungs and an increase in the chambers of the right heart and pulmonary artery of varying severity. In Scimitar Syndrome, an abnormally draining pulmonary vein is visualized in frontal projection.

• Electrocardiography is recommended.

Comments:The electrocardiographic picture is non-specific and reflects hypertrophy of the right atrium and ventricle, as well as overload of the latter. The degree of these changes depends on the amount of blood discharge and the duration of the defect.

2.5. Other diagnostics

3. Treatment

3.1 Conservative treatment

• The appointment of diuretics is recommended.

• In the adult category of patients, it is recommended to treat supraventricular arrhythmias with antiarrhythmic drugs.

• In the case of atrial fibrillation, both antiarrhythmic and anticoagulant therapy is recommended.

• Drug therapy for pulmonary hypertension is recommended only for patients with irreversible pulmonary hypertension.

3.2 Surgical treatment

• Surgical treatment is recommended for patients with PADLV with a Qp: Qs ratio of more than 1.5.

Comments:The optimal age for performing the intervention is 1-2 years of age.

• With isolated PADLV (without ASD) of a part of one lung, surgical treatment is recommended when Qp: Qs is more than 1.8.

Comments:Isolated abnormal drainage of all pulmonary veins of one lung requires correction to prevent possible hypoxemia in the event of damage to a healthy lung. The optimal age for performing the intervention is 1-2 years of age. Correction of the scimitar syndrome with lung hypoplasia is advisable to prevent sequestration of the lung in the volume of lung resection, regardless of the magnitude of the discharge.

3.3. Other treatment

4. Rehabilitation

• Within 3 months after the operation, the patient is recommended to undergo rehabilitation treatment with limited physical activity for a year.

Comments: Most cardiac rehabilitation programs last three to six months.

5. Prevention and dispensary observation

• Regular observation by a cardiologist is recommended.

Comments: Children of the first year of life are seen by a cardiologist in the first half of the year 2 times a month, in the second - monthly. In the second year of life, 2 times a year, then the child is observed at least 1 time in 2 years. Once a quarter, it is necessary to take ECG readings, an ultrasound examination of the heart is performed twice a year, and a chest x-ray (in frontal and lateral projections) is performed once a year.

6. Additional information affecting the course and outcome of the disease

• Early postoperative symptoms such as fever, fatigue, vomiting, chest pain, or abdominal pain may indicate postcardiotomy syndrome with cardiac tamponade, in which case an immediate echocardiographic examination is recommended to rule out this complication.

Comments: Postcardiotomy syndrome and cardiac tamponade may occur several weeks after surgical correction of PADV and should be assessed clinically and by echocardiography before discharge and within a month after patient discharge. Patients, parents and primary care providers should be instructed to report fever or unusual symptoms (chest or abdominal pain, vomiting, unusual fatigue) in the first weeks after surgery, as these symptoms may represent early signs of cardiac tamponade.

Criteria for assessing the quality of medical care

	Quality criteria	Confidence level of evidence
The stage of diagnosis
	Performed auscultation of the heart
	EchoCG was performed using the color Doppler mapping mode
	Performed cardiac catheterization with angiography
Stage of conservative and surgical treatment
	Diuretics prescribed
	Completed blood selection for the recipient for surgery
	An operation was performed to eliminate CHADLV
Postoperative control stage
	EchoCG performed before discharge from the hospital
	The patient is referred for rehabilitation aftercare

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Appendix A1. Composition of the working group

d.m.s. I.V. Arnautova, Ph.D. S.S. Volkov, prof. S.V. Gorbachevsky, V.P. Didyk, MD Ermolenko M.L., prof. M.M. Zelenikin, prof. A.I. Kim, prof. I.V. Kokshenev, MD A.A. Kupryashov, junior researcher A.B. Nikiforov, Academician V.P. Podzolkov, MD B.N. Sabirov, prof. M.R. Tumanyan, prof. K.V. Shatalov, MD A.A. Schmalz, Ph.D. I.A. Yurlov.

The head of the working group is Academician of the RAS L.A. Boqueria

No conflict of interest

Target audience of the developed clinical guidelines:

1. pediatricians;
2. cardiologists;
3. cardiovascular surgeons.

Table P1 - Levels of evidence

Appendix A3. Related documents

1. On the basics of protecting the health of citizens in the Russian Federation (Federal Law of November 21, 2011 N 323-FZ)
2. The procedure for providing medical care to patients with cardiovascular diseases (Order of the Ministry of Health of Russia dated November 15, 2012 N 918n)
3. Order of the Ministry of Health and Social Development of the Russian Federation of December 17, 2015 No. 1024n "On the classification and criteria used in the implementation of medical and social expertise of citizens by federal state institutions of medical and social expertise."

Appendix B. Patient management algorithms

Appendix B. Information for Patients

Clinical manifestations and symptoms of the defect develop in the later stages of the disease. Therefore, regular follow-up by a cardiologist / pediatric cardiologist is necessary. In the first year of life (in the absence of indications for surgery) - once every 3 months, then - once every 6 months. When symptoms such as shortness of breath, cyanosis of the nasolabial triangle, palpitations, decreased resistance to physical activity, poor weight gain appear or worsen, a cardiovascular surgeon's consultation is indicated with a solution to the issue of further tactics for treating CHD.

website - 2007

When partial abnormal drainage of the pulmonary veins one or more pulmonary veins abnormally flow into the right atrium or main veins, but the normal flow of part of the pulmonary veins into the left atrium is also preserved. The most common occurrence is abnormal flow of the pulmonary veins from the right lung up to 90% of all cases. Only a partial abnormal 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.

Violation of blood circulation with abnormal drainage of the pulmonary veins is caused by abnormal discharge of arterial blood, which must enter the left 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 flowing veins, the level of their confluence, the presence and size of the atrial septal defect.

In newborns and young children, the manifestations of the defect are hardly noticeable. Older children may complain of shortness of breath, increased fatigue during exercise. Lagging in physical development is noticeable. Patients of older age groups have palpitations, cardiac arrhythmias. Often there are inflammatory diseases of the lungs - pneumonia. The course of the defect depends on the volume of blood discharge, on the duration of the defect, on the compensatory capabilities of the vascular system of the lungs. The average life expectancy of such patients without surgery is about 40 years.

Diagnosis of partial abnormal pulmonary vein drainage

When listening, a systolic murmur is detected, a splitting of the second tone above the pulmonary artery. On the electrocardiogram, you can see the load on the right heart. X-ray examination reveals an increase in the right heart, sometimes signs of abnormal drainage of the pulmonary veins are found. The diagnosis is clarified using cardiac catheterization, angiocardiography. Particularly reliably the location and place of confluence of the pulmonary veins are revealed with selective angiocardiography from the pulmonary artery.

Treatment of partial abnormal pulmonary vein drainage

Treatment of partial abnormal drainage of pulmonary veins is operative. The operation may not be recommended for patients with severe pulmonary hypertension. Sometimes, with a very mild course of the defect, surgical treatment may not be prescribed. In most cases, the operation takes place in conditions of connection to a heart-lung machine. The heart is accessed through a midline chest incision and a sternum incision. The task of surgical treatment is to move the mouths of all abnormally flowing veins into the left atrium. This is done by partially displacing the atrial septum. The easiest way to perform surgery is for a cardiac type of defect, when the abnormally located pulmonary veins flow into the right atrium. The right atrium is opened and the inflow points of the pulmonary veins are 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 abnormal veins, while the mouths of these veins end up in the formed left atrium. A very large atrial septal defect may require a patch. The patch is applied in such a way that the defect in the septum and the mouth of the abnormal veins are in the left atrium. When the pulmonary vein flows into the coronary sinus, it is also moved to the left atrium. Surgical treatment of abnormal flow of the pulmonary veins into the superior vena cava can be performed in two ways. The first of them begins with the isolation of the superior vena cava along its entire length. The vein is then sutured with a continuous suture lengthwise to form two separate, isolated canals. The antero-median 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 abnormal pulmonary veins flowing into it. Thus, arterial blood from the lungs appears in the posterolateral canal. After that, a heart-lung machine is connected, the right atrium is cut. The mouth of the formed posterolateral canal is moved to 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) above the confluence of the abnormal 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 flowing pulmonary veins are easily accessible. Then, from the upper edge of the mouth of the pulmonary veins, a patch is sutured from its own pericardium or synthetic material. A canal is formed between the patch and the posterior 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 atrial septum into the left atrium, and the communication between the left and right atrium is closed. Incisions in the right atrium and superior vena cava are sutured. Treatment of the subcardiac defect, in which the abnormal pulmonary veins drain into the inferior vena cava, is most difficult. The thorax is dissected along the anterolateral surface in the fourth intercostal space. The inferior vena cava and abnormal pulmonary veins are accessed. The vena cava is clamped with a special forceps. The mouth of the pulmonary veins with the adjacent portion of the inferior vena cava is cut off, and the resulting opening is sutured with a continuous suture. The pulmonary veins lead to the left atrium and connect to it. Surgery results are usually good. The mortality rate during surgery is about 3%. Long-term results of the operation are good. A significant improvement in the condition of patients occurs already in the first year after treatment. The worst results of treatment are usually associated with a violation of the technical side of the operation or the patient's initial serious condition.

The word "abnormal" 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. don't connect to it. There are a lot of options for their incorrect confluence.

There is a "partial" abnormal drainage - this is when one or two of the four veins flow into the right atrium (the most common variant), 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 abnormal pulmonary vein drainage (TADLV) - This is completely different. With this defect, all four pulmonary veins from both lungs are connected to one wide collector vessel. This collector of arterial blood oxidized in the lungs does not grow together with the left atrium, as it should, but connects to the venous system of the body, usually through a large vein. Arterial blood, thus, bypassing the heart, enters the large veins and into the right atrium. Only here, after passing through the atrial septal defect, will it be where it should be initially - in the left atrium, and then it makes its usual path along the large circle of blood circulation. It is difficult to imagine what such a thing can be. But children with this defect are born full-term, and the heart cope with such a situation for some time. However, this time can be very short.

First, the child's life depends on the size of the interatrial communication - the smaller it is, the more difficult it is for arterial blood to get to the left side of the heart as intended.

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 pumped into a large circle. Thus, in the child, partially venous blood begins to circulate in the arteries, and he becomes "blue", ie. the color of the skin, especially the tips of the fingers and mucous membranes (lips, mouth), is bluish. This is cyanosis, and we will talk about its causes, manifestations and consequences later.

With complete abnormal drainage, cyanosis may not be very pronounced, but it is, and it is usually noticeable soon after birth.

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

Surgical treatments exist, and the results are quite encouraging today. The operation is quite complicated, it is performed on an open heart and consists in the fact that the common collector of the pulmonary veins is sutured to the left atrium, and the opening in the median atrial septum is closed with a patch. Thus after the operation, normal blood circulation is restored in two divided circles.

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

We will not touch here on many details related to various types of defects and methods of correcting it. But we only want to emphasize that children with this defect need immediate specialized help that is very real today.

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

The frequency of total ADLV according to clinical data is 1.6-7.7% [Pariyskaya T. V., Veniaminova G. N., 1986J, according to pathological anatomical data - about 2%.
In case of total ADPV into the right atrium (cardiac form), the operation is performed using artificial circulation and hypothermia and consists in enlarging the atrial defect and applying a patch so as to direct blood flow from the orifices 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 posterior superior wall of the coronary sinus is incised, the patch displaces the wall and the coronary sinus into the left atrium. In supracardial and infracardiac types of defect, an anastomosis is applied between the pulmonary vein collector and the left atrium [Burakovsky VI et al., 1989].
With severe hypoplasia of the left ventricle, ASD correction with a perforated patch or two-stage surgical treatment is used, including: Stage 1 - anastomosis between the collector and the left atrium (allows the development of the left heart), Stage II - vertical ligation
veins through which the pulmonary veins drain into the right superior vena cava.
Mortality after surgery in children under 6 years old, according to

1. Galloway et al. (1985) was 10%. It is higher in the infracardiac form, drainage of the pulmonary veins into the superior vena cava, especially in 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 in the vessels of the pulmonary circulation and in the myocardium of the ventricles of the heart do not have time to develop. If the anastomosis shows signs of functional obstruction, reoperation is indicated.

Abnormal pulmonary vein drainage (ADPV) is a congenital heart disease characterized by pathological inflow of the pulmonary veins into the right atrium or into the vena cava of the systemic circulation (CCV). ADVLV can be partial (when one or more pulmonary veins, most often right), or total (when all pulmonary veins flow into the venous system of the CCB).

Depending on the level of inflow of the pulmonary veins into the venous system of the CCB (see figures below, a, b), there are:

1) supracardiac type - the confluence of the pulmonary veins into the superior vena cava, its branches or the left unnamed vein;

2) cardiac type - the confluence of the pulmonary veins into the right atrium or coronary sinus;

3) subcardiac type - the pulmonary veins flow into the lower hepatic or portal vein;

4) mixed type - a combined confluence of the pulmonary veins at various levels.

a - partial abnormal drainage of the pulmonary veins;
b - complete abnormal drainage of the leukemia veins into the coronary sinus and atrial septal defect.

The most common is the supracardial inflow of the right pulmonary veins into the superior vena cava and, somewhat less frequently, the cardiac type of inflow of the right pulmonary veins into the right atrium.

For a pediatrician, it is most important to distinguish two types of ADLV - partial or total, in which there are various hemodynamic and clinical manifestations of the defect. Partial ADLV is often combined with an atrial septal defect (ASD) or an open foramen ovale, and with total ADLV, the existing ASD significantly affects patient survival.

Partial abnormal drainage of pulmonary veins

In case of partial ADPV, one or several, but not all, pulmonary veins flow into the venous system of the CCB at different levels. It is the most common variant of abnormal pulmonary venous connections, accounting for about two-thirds of all cases of ADVD. It is observed in 1.5% of all patients operated on for congenital heart defects; 0.6% of all congenital heart defects are reported from autopsies and 0.3-0.69% of all clinically present congenital heart defects.

Most often, the right pulmonary veins are drained (up to 97.2%) into the superior vena cava and its branches, both separately and previously merged into a single collector. In the majority of patients (90-94.3%), partial ADLV is combined with a secondary ASD, which is usually located under the orifice of the superior vena cava, less often (41.3% of cases) the right pulmonary veins flow into the right atrium directly or through the venous sinus ... The subcardial type of ADLV is very rare, and the right pulmonary veins flow into the inferior vena cava above the diaphragm or in the form of an abnormal venous trunk ("scimitar") below the diaphragm and above the attachment of the inferior hepatic veins.

ADLV can also be combined with other complex congenital heart defects (common atrium, ventricular septal defect, transposition of great vessels, tetrad of Fallot), which is observed in about 20% of patients.

Hemodynamics. The defect is characterized by volume overload of the right atrium, right ventricle and hypervolemia of the pulmonary circulation (ICC), since a permanent abnormal arteriovenous discharge of blood at the level of pulmonary veins - right atrium leads to recirculation of "ballast" blood volume through the right heart and pulmonary vessels. The left parts of the heart are "underused" to one degree or another.

The degree of overload of the right heart and ICC depends on:

1) the number of abnormally flowing veins;

2) the level of their confluence;

3) the presence, size, and localization of concomitant ASD, as well as the presence of other cardiac and extracardiac abnormalities.

When one pulmonary vein is drained and there is no ASD or an open foramen ovale, the defect may not appear hemodynamically. With a large number of abnormally flowing veins and the presence of ASD, the hemodynamics and course of the disease are very similar to those in uncomplicated secondary ASD. Arteriovenous discharge results in volume overload, dilatation, and moderate hypertrophy of the right atrium and right ventricle. Pulmonary hypertension does not occur for a long period, and during development it does not reach high degrees (just as it happens with a defect of the interventricular septum or patent ductus arteriosus). This is due not only to a moderate amount of arteriovenous discharge, but also to the absence of a "pumping phenomenon" of direct pressure transmission from the left ventricle, which is characteristic of an interventricular septal defect.

The clinical picture. As with hemodynamic disorders, the clinical symptoms of the defect are very similar to uncomplicated variants of secondary ASD. In young children, the symptoms are scanty, there is a tendency to recurrent bronchopulmonary diseases. Later, shortness of breath, rapid fatigue during physical exertion, moderate lag in body weight and physical development appear.

In older children, the expansion of the borders of the heart to the right, an increase in the heart impulse are determined, less often there is a moderate central hump. On auscultation, as in ASD, bifurcation and accent of the II tone are determined, systolic murmur of medium intensity in the second or third intercostal space to the left of the sternum, without pronounced irradiation, the latter is due to the relative functional stenosis of the pulmonary valve opening. If the value of the arteriovenous discharge to the right is more than half of the pulmonary venous return of blood, then in patients pulmonary hypertension gradually increases and decompensation of the right ventricle develops.

X-ray enrichment and enhancement of the pulmonary pattern along the arterial bed, expansion, and later pulsation of the roots of the lungs with bulging of the pulmonary artery trunk are revealed. The heart is moderately expanded, due to the right ventricle and right atrium, the right atriovasal angle is displaced upward (see Figures, a, b).

a - straight position; b - left side position

Diagnosis: congenital heart disease, partial extracardiac drainage of pulmonary veins, secondary atrial septal defect, moderate hypervolemia of the pulmonary circulation. Moderate enlargement of the heart in diameter (cardiothoracic index \u003d 63%), due to the right sections (Goodwin's index \u003d 55%), moderate bulging of the contour of the pulmonary artery (Moore's index \u003d 37%).

When the pulmonary veins flow into the inferior vena cava in the sagittal projection, against the background of the lower lobe of the right lung along the right contour of the heart, one can find a characteristic oval shadow (the "scimitar" symptom).

Electrocardiographic a deviation of the EOS to the right is recorded, less often - a normogram. In older age groups, in the right leads (III, aVR, V 1-2) there is often a "blockade" form of right ventricular hypertrophy, with an increase in the amplitude of the late tooth R (like rSR or rsR) and hypertrophy of the right atrium in the form of a pointed P wave, especially in leads II and V 2.

Echocardiography. The results of ultrasound examination of the attached CHD are less indicative, due to the difficulty in differentiating the pulmonary veins. It is necessary to use a high resolution instrument and Doppler mapping. In the 20-mode, one can indirectly suspect ADPV by the discrepancy between the size of the MPP defect and a relatively large arteriovenous discharge (see figure).

Diagnosis: congenital heart disease, abnormal drainage of the right pulmonary veins in the right atrium, venous sinus defect. Epigastric (subcostal) access. A high atrial septal defect (ASD) in the venous sinus area, 10 mm in diameter, is determined. Abnormal inflow of the right pulmonary veins (Aposh PV) into the upper part of the right atrium (RA) (2 veins as a single collector).

In the M-mode, partial ADPV is indirectly judged by the presence of signs of volume overload and increased blood flow through the right heart (dilatation of the right atrium, increased excursions of the tricuspid valve, dilatation of the right ventricle and paradoxical movement of the interventricular septum) and underloading of the left sections (small size or hypoplasia of the left chambers of the heart).

Catheterization and angiocardiography. Analysis of blood gases taken at the level of the vena cava orifices and the right atrial cavity helps to draw a conclusion about the source of the arteriovenous discharge, and an unconditional sign of ADVD is the passage of the probe into the pulmonary veins directly from the vena cava or from the right atrium. Selective angiocardiography through the pulmonary artery allows in the levogram phase to reliably visualize the location of the confluence of the pulmonary veins.

Surgical correction of the defect. The indications for surgery and the timing of its implementation are similar to those for isolated ASD. The operation for all types of ADLV consists in relocation of the mouths of the abnormally flowing pulmonary veins into the left atrium, by partial displacement of the interatrial septum. When the pulmonary veins fall into the vena cava, complex plastic surgery is performed (using an autopericardium or an artificial patch) with the formation of two channels (tunnels) for separate systemic and pulmonary blood flow, respectively, into the left and right atria. It is possible to correct the defect with cutting off the pulmonary veins in the places of their abnormal confluence and implanting them into the left atrial appendage.

Operational mortality is 2-4%, but in children of the first year of life - 14%, and in patients under 6 months of age - 25%. Long-term results in the follow-up for more than 15 years show that in almost 85% of patients, hemodynamics are restored within a year and in the future they lead a full-fledged lifestyle. The unsatisfactory results of the defect correction, except for the technical defects of the operation, are mainly due to the late terms of referral to the operation, when there is already a sclerotic phase of pulmonary hypertension and the degenerated myocardium is worn out. Complications can occur due to postoperative deterioration of the drainage of the pulmonary and vena cava, damage to the cardiac conduction system.

Total abnormal pulmonary vein drainage

Total ADLV is characterized by the absence of connection of the pulmonary veins with the left atrium and the confluence of all pulmonary veins into the venous system of the CCB, at the level of the right atrium and coronary sinus, the superior and inferior vena cava or their branches. This is a rare defect, it occurs in 0.4-4% of all CHD cases. The defect is more often observed in men (the ratio of men and women is 3.6: 1), when it flows into the portal veins, it can be combined with asplenia, polysplenia, cat's eye syndrome (50%), with Fallot's tetrad, ventricular septal defect (VSD), tricuspid atresia.

The supracardial form of drainage of all pulmonary veins (common collector) into the superior vena cava occurs in 40-45% of cases; intracardial form of the flow of pulmonary veins into the right atrium or coronary sinus - in 20-25% of cases; subcardial form of drainage into the inferior vena cava or into the portal vein system - in 20% of cases; in 5% of cases, a mixed form of separate inflow of all pulmonary veins into the various above-mentioned sections of the venous system of the CCB is detected.

The patient is viable only in the presence of ASD or open foramen ovale, less often - VSD or patent ductus arteriosus. However, compensation of hemodynamic disturbances in VSD and PDA is insignificant, since in VSD the left atrium is excluded from the circulation, and in the case of an open arterial duct, both left chambers of the heart. Death can occur due to obliteration of an open foramen ovale or patent ductus arteriosus. T.V. Pariiskaya and G.N. Veniaminova (1986) ASD in total ADPV was detected in 26.7% of cases, in other cases (73.4%) an open foramen ovale was detected.

In 1/3 of cases, total ADVD is combined with other CHD, such as transposition of great vessels, in which ADVV creates a functional correction of the transposition of great vessels, with a single (common) atrium, with hypoplasia of the left heart, etc. In addition, in 30% cases with this defect, there are other, non-cardiac anomalies of development - agenesis of the spleen or its additional lobes, intestinal diverticula, umbilical hernia, horseshoe kidney, kidney cysts, hydronephrosis, pathology of the skeletal system, pathology of the thyroid gland and adrenal glands.

With total PADV, obstruction in the drainage of the pulmonary veins is not excluded, which aggravates hemodynamic disturbances. Drainage problems can be caused by:

1) small interatrial communication, as a result of which adequate blood flow to the left atrium is disturbed;

2) narrowing of the orifices of the pulmonary veins at the site of drainage;

3) external compression of abnormally draining pulmonary veins, which is most often associated with their passage between the left main bronchus and the left branch of the pulmonary artery or compression at the level of passage through the diaphragm in the infradiaphragmatic type of confluence.

Obstruction of venous pulmonary drainage is detected in 40-100% of patients, but more often with subcardial type of pulmonary vein drainage. Obstruction of the pulmonary veins always leads to an increase in resistance to the outflow of blood from the lungs, an increase in pressure in the venous pulmonary bed, which is inevitably accompanied by an increase in pressure in the arterial bed and the occurrence of pulmonary hypertension.

Hemodynamics. Hemodynamic disturbances are caused by the fact that all blood from the venous vessels of the large and pulmonary circulation enters the right atrium. This causes significant dilatation and an increase in intra-atrial pressure. Most of the blood from the right atrial "collector" enters the right ventricle and pulmonary artery, which leads to volume overload of the right ventricle and hypervolemia of the ICC. Part of the arteriovenous blood mixed in the right atrium along the altered pressure gradient is discharged through the ASD or the open foramen ovale into the left atrium, and then into the left ventricle and CCB. The amount of blood shunt from right to left is determined by the interatrial pressure gradient and the size of the accompanying ASD, total pulmonary resistance, and the degree of hypertrophy of the right heart, but pulmonary blood flow always exceeds the systemic one by the amount of “ballast” blood volume recirculated through the ICC.

Prolonged volume overload causes right ventricular hypertrophy, and pulmonary hypertension gradually forms due to ICC hypervolemia. The left heart as a result of hemodynamic "underload" can be moderately hypoplastic.

If the ASD is large enough, then the right-to-left shunt provides almost normal functional development of the left heart, and pulmonary hypertension does not reach pronounced degrees. If the discharge from right to left, through the ASD, is sufficient, then the early development of severe pulmonary hypertension may be due to concomitant obstruction of the outflow of the draining pulmonary veins. Hypoxemia does not reach critical values, since the mixed venoarterial blood is sufficiently well oxygenated.

Venous pulmonary obstruction, along with severe arterial hypervolemia, creates a threat of pulmonary edema, which causes compensatory reflex spasm of pulmonary arterioles, which ultimately is the main and powerful factor in rapidly increasing pulmonary hypertension. As pulmonary hypertension increases, the discharge into the ICC decreases, which leads to a decrease in blood oxygenation and an increase in hypoxemia. Later, systolic overload of the right ventricle and its decompensation occurs.

The clinical picture. Antenatally, the defect does not affect hemodynamics, since with a non-functioning ICC, the message at the atrial level and the ductus arteriosus provide normal intrauterine blood circulation, and the child is born with normal length and weight. Although T.V. Pariyskaya and G.N. Veniaminova (1986) found moderate prenatal hypotrophy in "/ 3 patients with total ADP.

The main symptom of a defect in the first weeks of life is shortness of breath, aggravated by feeding, crying, and anxiety. There is a tendency to recurrent respiratory diseases with prolonged cough. Moderate cyanosis can appear in the first weeks of life with holding the breath (crying, sucking), its intensity subsequently decreases and may increase again in the second half of life. Persistent cyanosis from the first days of life is characteristic of venous obstruction. Children lag behind in weight gain and physical development.

Physically pulse of weak filling and tension. Blood pressure is moderately reduced due to systolic pressure. 6 months after birth, an increased cardiac impulse is detected in the third intercostal space to the left of the sternum, expansion of the borders of the heart to the right. The I tone is moderately enhanced, probably due to the tricuspid component. II tone is strengthened by the pulmonary component, it is split; the III tone can be heard, connected, apparently, with fast filling of the right ventricle. Systolic murmur over the pulmonary artery of functional origin, mild, of medium intensity and duration, is caused by increased turbulent blood flow through the opening of the pulmonary valve into the dilated trunk of the pulmonary artery. However, in some children, the noise can be intense, occupy almost the entire systole and be heard along the left edge of the sternum. With a rapid increase in pulmonary hypertension, symptoms of right ventricular heart failure occur (an increase in the size of a dense painful liver, an increase in cyanosis, small peripheral edema).

Electrocardiogram.A significant deviation of the electrical axis of the heart to the right is revealed. Right ventricular hypertrophy is recorded as a blockade form of hypertrophy (type rSR) or, less often, R-forms (such as qR) in leads III, aVR, V 1 _ 2 and deep teeth 5 in leads I, V 5 _ 6. Prong height R there may be a concordant degree of pulmonary hypertension, with which signs of systolic overload of the right ventricle are also associated in the form of a displacement below the isoline of the segment STand deep teeth Tin chest leads from V to V Almost all patients have signs of hypertrophy and overload of the right atrium in the form of a high pointed tooth Rin leads II, III, V 1 _ 2. Signs of conduction disturbances often appear.

Echocardiography. There are no direct echocardiographic signs of total ADPV. Indirect signs include dilatation and hypertrophy of the right atrium and ventricle, paradoxical movement of the interventricular septum with a simultaneous relative decrease in the left heart. ADLV can only be recognized using color Doppler imaging, which detects the flow of warm tones from the right pulmonary veins into the right atrium.

Radiography. Pulmonary drawing is significantly enhanced due to arterial hypervolemia, and sometimes venous. The sizes of the right ventricle and atrium are increased, with intact or slightly reduced sizes of the left heart. With the most common supracardial type of defect, when the pulmonary veins are drained through a common collector into the superior vena cava, it is possible to detect a characteristic shadow in the frontal projection in the form of the number "8", or "snow woman". In this case, the upper circumference of the figure eight is formed by the shadow of the collector and the aneurysmically expanded superior vena cava.

Cardiac catheterization. This is the most informative and decisive method for diagnosing this defect. Mean right atrial pressure is moderately elevated, but a large atrial gradient usually indicates a small atrial septal defect. The pressure in the right ventricle and pulmonary artery is increased, but if the pressure in the pulmonary artery is 50% higher than the pressure in the aorta, then this is regarded as pulmonary hypertension. When the pressure in the pulmonary veins exceeds normal (10 mm Hg. Art.) 2 times, it is usually associated with obstruction of venous return.

The oxygen saturation in the right heart is higher than normal, and depending on the anatomical type of defect, the saturation of blood samples with oxygen is higher in the zones of abnormal drainage of the pulmonary veins, which makes it possible to differentiate the place of their confluence.

The saturation of arterial blood with oxygen is always reduced, to the greatest extent - with pulmonary hypertension, when pulmonary blood flow is significantly reduced. This, along with heart failure, is one of the reasons for the increase in hypoxemia and cyanosis with an increase in pulmonary hypertension.

Selective angiography allows the final visualization of the defect topic.

Differential diagnosis is carried out with vices occurring with the enrichment of MCC and cyanosis. These are, first of all, atrioventricular communication, transposition of great vessels, ventricular septal defect, common arterial trunk, and left-handedness syndrome.

Natural course and prognosis defects depend on the size of the defect, the presence of pulmonary hypertension and venous obstruction. Most patients have a severe course with rapid progression of pulmonary hypertension, the development of heart and respiratory failure during the first year of life. The average life expectancy of children is from 2 months to 2 years, most of the patients die during the first year of life, and only in a few cases do children live up to 3-4 years. Typically, the cause of death is heart failure and severe cardiopulmonary failure associated with recurrent pneumonia. At an early age, the cause of death can be the closure of the foramen ovale, which is observed in most children who die at the age of 1-4 months.

Only a small number of patients without pulmonary hypertension may have a relatively favorable course of the defect, and pulmonary hypertension in them develops only in the second decade of life.

Surgical correction of the defect. The indications for surgery are absolute, and, unlike other congenital heart diseases, occurring with hypervolemia of the ICC, pulmonary hypertension is not a contraindication, since with this defect it is often caused by obstruction of the pulmonary veins, the elimination of which restores blood circulation in the ICC.

If children under 2-3 months of age are in critical condition, then they may undergo palliative balloon atrioseptotomy to increase the interatrial communication.

Radical plastic surgery is reduced to creating a wide anastomosis of the pulmonary veins with the left atrium, closing the ASD and ligating the site of pathological communication of the pulmonary vein collector with the vessels of the venous system.

Surgical mortality depends on the anatomical type of the defect, the age of children, the level of pulmonary hypertension and remains high so far. With supracardial and intracardial types of defect without pulmonary hypertension, the lethality does not exceed 5-10%. Among newborns, mortality in most hospitals remains at the level of 30%, but in some clinics it has been reduced to 13.6% and even 10%. Long-term results of surgical correction of the defect in most patients are good - in 79% of young children there is a significant decrease in hypervolemia and cardiomegaly, however, in 10.5% of cases, heart rhythm disturbances associated with surgery are possible.