Pulmonary Atresia with Ventricular Septal Defect (PA with VSD): Clinical Guidelines

Applies tochildren2024 editionQ25.5

Clinical Guidelines of the Russian Ministry of Health, Pediatric. 2024 Edition, ICD-10 codes: Q25.5.

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Brief for Practice

WhiteCoat Pro editorial summary based on the 2024 guideline text. Each item links to the original section — verify against it before prescribing.

PA with VSD is a complex congenital heart defect characterized by the absence of a direct connection between the right ventricle and the pulmonary artery system, with pulmonary blood flow maintained by a patent ductus arteriosus (PDA) and/or collaterals. The guidelines cover confirmation of anatomy and hemodynamics, selection of early primary or staged surgical repair, and long-term postoperative follow-up.

Key points

Anatomical basis of the defect: absence of the pulmonary artery at one or more levels, large subaortic VSD, additional sources of pulmonary blood flow, RV hypertrophy, and dextroposition of the aortic root. → in the text
Clinically, arterial hypoxemia with progressive cyanosis predominates; hypercyanotic spells are usually absent. Prolonged hypoxemia can lead to clubbing and watch-glass nails. → in the text
Prognosis is determined by the sources of pulmonary blood flow: in ductus-dependent hemodynamics, mortality up to 12 months reaches 90%, and the overall median survival in PA with VSD is 6 months to 2 years. → in the text
Any anatomical variant of PA with VSD is an indication for surgery; the intervention is performed as early as possible, most often in the first year of life. → in the text
In favorable anatomy, radical correction is performed: VSD closure, creation of a right ventricle-to-pulmonary artery conduit/pathway, and, if necessary, unifocalization of pulmonary blood flow. → in the text
Long-term postoperative follow-up is required, including ECG and echocardiography, annual pulse oximetry, and periodic Holter monitoring; in the presence of residual complications, the frequency of examinations is increased. → in the text

Diagnostics

Gather history and complaints from every patient: specify dyspnea, cyanosis of the lips and fingers, delayed physical development, fatigue, and exercise tolerance. If dyspnea and cyanosis worsen, evaluate the severity of clinical deterioration. Evidence level C, reliability level of evidence 5. → in the text
Perform a physical examination: assess the severity of cyanosis and chest shape, palpate the precordial area, measure heart rate and respiratory rate, and perform auscultation. A diminished second heart sound over the pulmonary area and continuous (systolic-diastolic) murmurs of a PDA, major aortopulmonary collateral arteries (MAPCAs), or a systemic-to-pulmonary shunt may be present. Evidence level C, reliability level of evidence 5. → in the text
Perform a complete blood count, pulse oximetry or dynamic capillary blood saturation monitoring, biochemical blood test, lactate, NT-proBNP, and coagulogram. Lactate >2.2 mmol/L, pro-BNP >125 pg/mL, and signs of metabolic acidosis specified in the guidelines are considered predictors of the need for emergency surgery. Evidence level C, reliability level of evidence 5. → in the text
Perform an ECG and chest X-ray. Typical ECG findings include rightward electrical axis deviation from +100° to +180°, RV hypertrophy, signs of right atrial overload, and incomplete right bundle branch block; chest X-ray evaluates pulmonary vascular markings, heart size, and characteristics of the aorta and pulmonary arteries. Evidence level C, reliability level of evidence 5. → in the text
Perform transthoracic echocardiography with color Doppler mapping: assess chamber dimensions, VSD, aortic dextroposition, RV hypertrophy, pulmonary trunk and branches, and signs of atresia. For types III and IV, echocardiographic evaluation of the pulmonary artery system is not recommended. Evidence level C, reliability level of evidence 5. → in the text
To clarify anatomy and hemodynamics, perform transvenous cardiac catheterization with ventriculography and angiocardiography; identify pulmonary artery branches, sources, and distribution of collaterals. When planning surgery, if echocardiographic and angiocardiographic data are insufficient, perform contrast-enhanced cardiac CT; MRI is used for ambiguous results and structural-functional assessment. Evidence level C, reliability level of evidence 5. → in the text

Treatment

Radical repair includes VSD closure and reconstruction of the right ventricular outflow tract (RVOT) into the pulmonary artery system, with pulmonary blood flow unifocalization if necessary. It is performed primarily in favorable anatomy, usually within the first year of life, or as a second stage following palliative surgery. Evidence level C, reliability level of evidence 5. → in the text
Favorable criteria for radical correction include: aortic oxygen saturation >70–75%, hemoglobin <180–190 g/L, McGoon index >1.5, Nakata index >250 mm²/m², pulmonary artery native index (PANI) >250 mm²/m², left ventricular end-diastolic volume (LVEDV) >40 mL/m², ventricular ejection fraction >50%, absence of multiple large collaterals, and presence of true pulmonary arteries in all bronchopulmonary segments. Evidence level C, reliability level of evidence 5. → in the text
Palliative tactics are chosen in cases of saturation <70%, hemoglobin >190 g/L, newborn weight <2.5 kg, LVEDV <40 mL/m², McGoon index <1.2, Nakata index or PANI <250 mm²/m², pronounced collateralization, or severe comorbid pathology. Evidence level C, reliability level of evidence 5. → in the text
Palliative options include systemic-to-pulmonary shunting, RVOT reconstruction without VSD closure, central shunt, PDA stenting, pulmonary branch stenting or stenting of MAPCA stenoses, interventions on the valve membrane in type I PA with VSD, and MAPCA unifocalization. Evidence level C, reliability level of evidence 5. → in the text
If the LVEDV index is <40 mL/m² or the Nakata index is 150–250 mm²/m², a systemic-to-pulmonary shunt is performed as the first stage; if the Nakata index is <150 mm²/m², RVOT reconstruction without VSD closure is performed. Evidence level C, reliability level of evidence 5. → in the text
With persistent PaO₂ <7 kPa (50 mm Hg) or saturation <70%, long-term oxygen therapy is administered; standard flow is 2–4 L/min, sometimes up to 10 L/min is required. Oxygen therapy alleviates the condition but does not alter pulmonary hemodynamics and the natural history of the defect. Evidence level C, reliability level of evidence 5. → in the text
Newborns with critical pulmonary stenosis and a closing PDA are prescribed alprostadil to maintain ductal patency while monitoring heart rate, respiratory rate, and blood pressure. Loop diuretics are used in the presence of a large PDA or multiple large MAPCAs causing pulmonary overcirculation and heart failure; forced diuresis should be avoided. Evidence level C, reliability level of evidence 5. → in the text
Following radical repair, balloon angioplasty with stenting is performed for pulmonary artery or conduit stenosis; embolization is performed for MAPCAs that are not the sole source of blood supply to a segment. In the presence of pulmonary regurgitation with RV dilation, RV end-diastolic volume >60 mL/m², tricuspid regurgitation, or QRS duration >160 ms, pulmonary valve replacement is indicated. Evidence level C, reliability level of evidence 5. → in the text

When to refer and how to monitor

Planned hospitalization is indicated in the presence or worsening of cyanosis and dyspnea, as well as to clarify the morphological type of the defect and select the treatment strategy. → in the text
Emergency hospitalization is indicated for a sharp increase in cyanosis or dyspnea, dependence of a newborn on intravenous prostaglandin infusions, and progressive cardiovascular failure. → in the text
Following surgical repair with a complicated postoperative course — residual VSD, pulmonary stenosis, circulatory failure, infection, or reoperations during the same hospital stay — patients are referred for rehabilitation at a specialized cardiological center within 6 months. → in the text
Postoperative follow-up is scheduled at 1, 3, 6, and 12 months, then at intervals of 3–36 months up to 18 years of age; patients with RV dysfunction, residual shunt, RVOT obstruction, or aortic regurgitation require annual follow-up at a specialized center. → in the text

Frequently asked questions

Which investigations confirm PA with VSD?

The diagnosis is established based on ECG, transthoracic echocardiography, cardiac chamber catheterization with angiocardiography, and contrast-enhanced cardiac CT. These studies clarify pulmonary artery anatomy, VSD, hemodynamics, and sources of collateral blood supply. → in the text

When can radical correction be performed?

Decision-making is based on clinical status and operable anatomy: aortic saturation >70–75%, hemoglobin <180–190 g/L, McGoon index >1.5, Nakata index and PANI >250 mm²/m², LVEDV >40 mL/m², and ventricular ejection fraction >50%. In favorable anatomy, surgery is usually performed during the first year of life. → in the text

When is a staged or palliative operation needed?

It is chosen in cases of severe hypoxemia, hemoglobin >190 g/L, newborn weight <2.5 kg, pulmonary artery hypoplasia, small left ventricle, multiple MAPCAs, or severe comorbid conditions. With a Nakata index <150 mm²/m², RVOT reconstruction without VSD closure is performed as the first stage, and with an index of 150–250 mm²/m², a systemic-to-pulmonary shunt is performed. → in the text

How to support the child before surgery?

In persistent hypoxemia with PaO₂ less than 7 kPa or oxygen saturation less than 70%, oxygen therapy is administered, usually at 2–4 L/min. For a closing PDA in a newborn, alprostadil is used; for pulmonary overload and heart failure, loop diuretics are used, avoiding forced diuresis and carefully monitoring systemic blood pressure. → in text

How to monitor the patient after correction?

After discharge, an echocardiogram and pleural ultrasound are performed at 2 weeks, followed by annual ECG and echocardiography, annual pulse oximetry, and Holter monitoring once every 2 years. Chest MRI is performed every 2–5 years, and in case of residual complications, exercise tolerance is additionally assessed. → in text

Status: Applied · approved by the Scientific and Practical Council of the Russian Ministry of Health
Database ID: 47, version 3 (ID 47_3)
Publication date: October 1, 2024
Age category: children
ICD-10 codes: Q25.5
Specialties: Cardiology Cardiovascular Surgery Diagnostic Radiology and Therapy Pediatrics
Developers: Association of Cardiovascular Surgeons of Russia
All-Russian Public Organization "Association of Pediatric Cardiologists of Russia"
All-Russian Public Organization for Promotion of the Development of Diagnostic Radiology and Therapy "Russian Society of Roentgenologists and Radiologists"
Official text: Russian Ministry of Health Guidelines — 47_3

List of Abbreviations

AV - arteriovenous

ACG - angiocardiography

PAA - pulmonary artery atresia

ALT - alanine aminotransferase

AST - aspartate aminotransferase

MAPCA - major aortopulmonary collateral arteries

VA - venoarterial shunt

CHD - congenital heart disease

PAP - pulmonary artery pressure

VSD - ventricular septal defect

ASD - atrial septal defect

CT — computed tomography

PA - pulmonary artery

PH - pulmonary hypertension

MRI — magnetic resonance imaging

PDA - patent ductus arteriosus

PVR - pulmonary vascular resistance

SVR - systemic vascular resistance

RA - right atrium

RV - right ventricle

SISC - systemic cardiac index

TOF - tetralogy of Fallot

ECG - electrocardiography

EchoCG – echocardiography

Sat O2 - blood oxygen saturation

PISC - pulmonary cardiac index

EPRC index - effective pulmonary blood flow index

QRS - electrocardiogram ventricular complex

RC - radical correction

NYHA - New York Heart Association Functional Classification

Terms and Definitions

Pulmonary artery atresia - absence of a connection between the right ventricle and the pulmonary arterial system, depending on the type of defect.

Cardiac catheterization - an invasive procedure performed for therapeutic or diagnostic purposes in cardiovascular pathology by introducing catheters into the heart chambers or the lumen of great vessels.

Collateral vessels - abnormal vessels branching off from the aorta or its branches and providing additional blood flow to the lungs.

Ventricular septal defect - an anatomical connection between the right and left ventricles of the heart.

Endocarditis - inflammation of the inner lining of the heart; it is a frequent manifestation of other conditions.

Echocardiography - an ultrasound method aimed at investigating morphological and functional changes of the heart and its valvular apparatus.

1. Brief information on the disease or condition

1.1 Definition of the disease or condition

Pulmonary artery atresia is the congenital absence of a direct connection between the RV and the PA system. This is a rather rare CHD, accounting for 2–3% of all CHDs. The defect occurs both in combination with VSD and with various complex CHDs, such as transposition of the great vessels, tricuspid atresia, single ventricle of the heart, etc. The anatomical criteria for the defect are as follows: 1) absence of the PA at various levels; 2) large subaortic VSD; 3) presence of additional sources of collateral pulmonary blood flow; 4) RV hypertrophy; 5) dextroposition of the aortic root; 6) normal relationship between the aorta and the PA trunk [1, 2, 3].

1.2 Etiology and pathogenesis of the disease or condition (group of diseases or conditions)

PAA with VSD is a complex congenital heart defect belonging to the conotruncal defect group. The pulmonary arterial tree consists of three main segments of different embryological origin: 1. The PA trunk is formed as a result of the division of the truncus arteriosus, 2. The right and left PAs are formed from the 6th pair of aortic arches, 3. The intrapulmonary arterial vessels originate from the lung buds. The absence or impaired development of one or more segments explains the variety of anatomical variants of the defect. Due to a disturbance of the arterial conus, the aortopulmonary and interventricular septa fail to form, leading to the formation of a large VSD.

At the same time, the child's condition mainly depends on the size of the patent ductus arteriosus (PDA) and the presence of major aortopulmonary collateral arteries. Hemodynamic disturbances in this defect are determined mainly by the absence of a direct connection between the RV and the PA system, making direct flow of venous blood from the RV impossible. Blood flow to the lungs occurs via a bypass route from the RV through the VSD into the left ventricle (LV); subsequently, mixed arterialized blood enters the aorta and only then reaches the lungs via the PDA or collateral vessels. The blood oxygen saturation in the aorta, collateral arteries, and pulmonary artery is identical [1, 2, 3, 4].

Associated anomalies may include ASD, atrioventricular septal defect (more commonly in patients with Down syndrome), and a right-sided aortic arch. Coronary artery anomalies are possible, often being conal branches from the right coronary artery crossing the RV outflow tract [1, 2, 3].

1.3 Epidemiology of the Disease or Condition

The incidence is 3–5% of all CHDs. Mortality: The patient's life prognosis depends on the nature of pulmonary blood flow. Mortality in children with ductus-dependent circulation is up to 90% by 12 months of age. In patients with multiple sources of pulmonary blood flow and moderately pronounced cyanosis, mortality reaches 50% by 3–5 years of age. With enhanced pulmonary blood flow and the presence of major aortopulmonary collateral arteries, patients die as pulmonary hypertension develops, predominantly in the third decade of life. Overall, the median survival for patients with PAA and VSD ranges from 6 months to 2 years [1, 2, 3, 5].

1.4 Features of Coding the Disease or Condition

Q25.5 Pulmonary atresia with ventricular septal defect

1.5 Classification of the disease or condition

According to J. Somerville's classification [6], four types of the defect are distinguished:

I. PA valve atresia. The trunk, right, and left pulmonary arteries are fully formed and

patent;

II. Atresia of the PA valve and trunk. Both pulmonary arteries are preserved and may have a common or

separate origin;

III. Atresia of the valve, trunk, and one pulmonary artery. The other pulmonary artery is formed and patent;

IV. Atresia of the valve, trunk, and both pulmonary arteries. Blood flow to the lungs is maintained via a network of collateral vessels.

There is a more modern classification of the defect adopted at the 3rd International Nomenclature Conference for Pediatric Cardiac Surgery in New Orleans in 1999 [7].

Classification of the lesser circulation:

Type A - true pulmonary arteries are present, collateral arteries are absent, pulmonary blood flow is maintained via the PDA;

Type B - presence of both true pulmonary and collateral arteries;

Type C - true pulmonary arteries are absent, pulmonary blood flow is maintained via collateral arteries.

Classification of the defect depending on the type of atrioventricular and ventriculoarterial connection:

concordant atrioventricular and ventriculoarterial connections;
concordant atrioventricular and discordant ventriculoarterial connections;
discordant atrioventricular and concordant ventriculoarterial connections;
discordant atrioventricular and ventriculoarterial connections.

Types of collateral pulmonary circulation in pulmonary atresia with ventricular septal defect (PA/VSD):

major aortopulmonary collateral arteries (MAPCAs);
patent ductus arteriosus (PDA);
mediastinal arteries;
bronchial arteries;
fistula between the left coronary artery and the pulmonary artery trunk;
mixed forms. The patent ductus arteriosus, MAPCAs, and bronchial arteries play the most significant role in maintaining pulmonary blood flow [3, 8, 7].

1.6 Clinical presentation of the disease or condition

The clinical presentation is dominated by signs of arterial hypoxemia; however, dyspneic-cyanotic spells are absent, which distinguishes this condition from tetralogy of Fallot. Along with generalized cyanosis, clubbing of the fingers ("drumstick fingers") and toenails ("watch-glass nails") are observed. A left parasternal chest deformity in the form of a cardiac bulge is sometimes noted. In acyanotic forms of the defect, life expectancy is longer than in the cyanotic form. Pronounced cyanosis in most cases appears when the child begins to walk. As a rule, cyanosis progressively increases and reaches maximum intensity by early school age. By puberty, the patients' condition improves slightly and may remain stable without deterioration for a long time [3, 9].

The working capacity of patients who reach adulthood is significantly reduced, and they are generally poorly suited for physical labor. The course of the underlying disease can suddenly worsen due to various complications, which frequently become the cause of patient mortality. The most typical complications, in particular cerebrovascular accidents in the form of thrombosis or hemorrhage, are caused by hypoxemia, embolism, polycythemia, and increased blood viscosity [8, 10].

2. Diagnostics, medical indications and contraindications for diagnostic methods

The diagnosis of PA/VSD is established based on:

electrocardiography,
transthoracic echocardiography,
transvenous cardiac chamber catheterization and angiocardiography,
contrast-enhanced cardiac computed tomography.

2.1 Complaints and Anamnesis

It is recommended to take the medical history and conduct symptom assessment in all patients with pulmonary atresia and ventricular septal defect [2, 3, 10].

(Level of Evidence C, Grade of Recommendation 5)

Comments: During the history and symptom taking, it is recommended to identify in all patients the presence of dyspnea, bluish discoloration of the lips and fingertips, and delayed physical development. Attention should be paid to the degree of fatigue, exertional dyspnea, occasionally at rest, and cyanosis. With increasing dyspnea and cyanosis, the patients' condition worsens, and they can take only a few steps without stopping.

2.2 Physical Examination

It is recommended to perform a physical examination in all patients with PA/VSD, determining the presence and severity of cyanosis, chest shape, palpation of the precordial area, and counting the heart rate and respiratory rate [5, 11, 12].

(Reliability of Evidence Level C, Confidence Level in Recommendations 5).

Comments: patients with PA/VSD are generally hyposthenic. Their chest is typically cylindrical in shape and lacks a cardiac bulge. The skin and visible mucous membranes are cyanotic. Long-standing arterial hypoxemia is characterized by the development of clubbing ("drumstick fingers") and ("watch-glass nails").

It is recommended to perform cardiac auscultation in all patients [2, 3, 10].

(Reliability of Evidence Level C, Confidence Level in Recommendations 5).

Comments: Auscultation reveals very sparse findings. The second heart sound (S2) over the pulmonary area is weakened, and the first heart sound (S1) is accentuated. If a PDA is functioning, a characteristic systolic-diastolic murmur may be detected. A systolic-diastolic murmur of major aortopulmonary collateral arteries is detected over the lung fields. If the patient has undergone a systemic-pulmonary shunt, a systolic-diastolic murmur is auscultated over the projection of the shunt [2, 3, 8].

2.3 Laboratory Diagnostics

In patients with PA/VSD, they are non-specific.

It is recommended to perform a complete blood count (CBC) in all patients with PA/VSD [2, 3, 12, 13].

(Reliability of Evidence Level C, Confidence Level in Recommendations 5).

Comment: The test is performed to assess the patient's condition, indirectly estimate the level of pulmonary blood flow, and determine the urgency of examination and treatment.

It is recommended to monitor capillary blood oxygen saturation dynamically or perform pulse oximetry in all patients with PA/VSD [2, 3, 10, 57].

(Reliability of Evidence Level C, Confidence Level in Recommendations 5).

Comment: The test is performed to assess the patient's condition, the degree of arterial hypoxemia, indirectly estimate the level of pulmonary blood flow, and determine the urgency of examination and treatment.

It is recommended to perform a general somatic blood biochemistry panel in all patients (creatinine, glucose, sodium, potassium, AST, ALT, total protein, albumin, urea, total bilirubin, free and conjugated bilirubin), as well as blood lactic acid levels [2, 3, 8, 14, 15, 60, 61].

(Reliability of Evidence Level C, Confidence Level in Recommendations 5).

Comment: The test is performed to assess the patient's condition, indirectly estimate pulmonary blood flow, and evaluate the biochemical profile of the blood. A lactic acid (lactate) level above 2.2; signs of metabolic acidosis — a drop in blood pH down to 4.0; and pro-BNP above 125 pg/mL are predictors of the need for urgent surgical intervention.

It is recommended to measure blood levels of N-terminal pro-brain natriuretic peptide (NT-proBNP) in all patients [58, 59].

(Reliability of Evidence Level C, Confidence Level in Recommendations 5).

Comment: The test is performed to assess the patient's condition and indirectly estimate pulmonary blood flow. A pro-BNP level above 125 pg/mL is a predictor of the need for urgent surgical intervention.

It is recommended to perform a coagulogram (screening hemostasis test) (determination of the international normalized ratio [INR], activated partial thromboplastin time, blood fibrinogen level) to assess the status and functional efficiency of various links of blood systems such as coagulation and anticoagulation, especially in patients with pronounced cyanosis [2, 3, 14, 16, 60, 61].

(Reliability of Evidence Level C, Confidence Level in Recommendations 5).

Comment: The test is performed to assess the patient's condition, indirectly estimate pulmonary blood flow, and evaluate the blood coagulation system.

2.4 Instrumental Diagnostics

It is recommended that all patients undergo an electrocardiogram (ECG) to verify the diagnosis, detect myocardial conduction disorders, right atrial and right ventricular hypertrophy, and electrical axis deviation [2, 3, 5, 8, 11].

(Reliability of Evidence Level C, Confidence Level in Recommendations 5).

Comments: PA/VSD is characterized by rightward electrical axis deviation (from +100 to +180°), right ventricular myocardial hypertrophy, incomplete right bundle branch block, and signs of right atrial overload.

It is recommended that all patients undergo chest radiography to assess the condition of the lungs, heart chamber sizes, and anatomical features of this congenital heart disease [2, 3, 8, 12].

(Reliability of Evidence Level C, Confidence Level in Recommendations 5).

Comments: PA/VSD is characterized by increased translucency of the lung fields due to decreased pulmonary blood flow. When sufficiently large pulmonary artery branches are present, enhanced pulmonary vasculature is detected, associated with atypical shadows of collateral vessels. In some cases, asymmetry of the pulmonary pattern is revealed, where it is enhanced on one side and depleted on the other. A moderately enlarged transverse heart shadow, concavity of the pulmonary artery segment, an elevated heart apex due to right ventricular enlargement, and an accentuated cardiac waist are observed. In oblique projections, enlargement of the right heart chambers, reduction in left ventricular size, and a widened ascending aorta shadow are determined.

It is recommended that all patients undergo transthoracic echocardiography (TTE) with color Doppler mapping for a detailed assessment of intracardiac anatomy and hemodynamics [10, 17, 19].

(Reliability of Evidence Level C, Confidence Level in Recommendations 5).

Comments: In PA/VSD, it is necessary to evaluate the dimensions of the right and left heart chambers, the location and size of the VSD, the degree of aortic dextroposition, and the severity of right ventricular hypertrophy. In type I of the defect, the pulmonary artery trunk and branches are determined depending on the degree of their hypoplasia. In type II, the bifurcation, confluence site, and both pulmonary artery branches are identified. In types III and IV, assessing the pulmonary artery system via echocardiography is not recommended. The sizes of the left heart chambers and signs of the atretic proximal pulmonary artery — a thickened membrane and a blindly ending right ventricular outflow tract — are determined.

It is recommended that all patients undergo transvenous cardiac catheterization (A06.10.008 Cardiac ventriculography) and angiocardiography (A06.12.029 Panaortography) to determine intracardiac anatomy, pulmonary artery branch abnormalities, the presence and distribution of collateral arteries in the lungs, and the severity of hemodynamic disturbances [10, 17, 21, 22].

(Reliability of Evidence Level C, Confidence Level in Recommendations 5).

Comments: The study is necessary to identify hemodynamic disturbances present in patients with PA/VSD: systolic pressure in both ventricles is equal, and blood oxygen saturation is reduced. A catheter can be advanced into the pulmonary artery only in the presence of a PDA.

The angiocardiography (ACG) protocol for patients with pulmonary atresia with ventricular septal defect (PA/VSD) should include right ventriculography in the anteroposterior projection with cranial angulation (to visualize the anatomy of the pulmonary artery branches) and lateral projection (to visualize the severity of combined stenosis), pulmonary arteriography, and aortography (to determine the presence of additional sources of pulmonary blood supply).

During right ventriculography, the occluded right ventricular outflow tract is opacified, and the contrast medium passes through the VSD into the ascending aorta. Aortography is recommended to study the sources of pulmonary blood supply, allowing for the determination of their presence, size, origin level, course, and distribution within the lungs, as well as establishing their relationship with the true pulmonary arteries. Following palliative interventions, ACG is recommended to assess the degree of patient preparation for radical correction. The assessment includes the degree of development of the pulmonary artery system, potential deformation of pulmonary artery branches in the area of systemic-to-pulmonary shunt placement, and the degree of left ventricular enlargement.

It is recommended that all patients undergo calculation of the following parameters: 1. McGoon index - the ratio of the sum of the diameters of the pulmonary artery ostia to the diameter of the descending aorta ((A1+B1)/dAo) - normal value $\ge$ 1.5. 2. Pulmonary arterial index (Nakata index) - the ratio of the sum of the cross-sectional areas of the right and left pulmonary arteries to the body surface area, normal value $\ge$ 330 $\pm$ 30 mm²/m². 3. Full neopulmonary arterial index (FNAI) - the ratio of the sum of the cross-sectional areas of the right and left pulmonary arteries and major aortopulmonary collateral arteries (MAPCAs) to the body surface area. 4. The ratio of the number of lung segments supplied by the true pulmonary arteries to the number of segments supplied by MAPCAs. [2, 3, 12, 23, 62]

(Reliability of Evidence Level C, Confidence Level in Recommendations 5).

Comment: To determine the feasibility of a radical operation, it is recommended to calculate pulmonary artery size parameters, evaluate indications for correcting pulmonary artery branch stenoses, closing systemic-to-pulmonary shunts, and addressing MAPCAs via various endovascular interventions.

It is recommended that all patients undergo contrast-enhanced cardiac computed tomography to clarify echocardiography and ACG findings (if necessary, or if the results of instrumental methods are inconclusive) when planning surgical treatment [2,3,12,23].

(CQR C, LVE 5)

Comment: The study should be performed when it is impossible to obtain adequate visualization during angiocardiography, in order to image the main trunk, branches, and peripheral branches of the pulmonary artery, and to determine the degree of pulmonary and tricuspid regurgitation. In newborns and infants under one year of age, contrast-enhanced cardiac computed tomography is the method of choice for diagnosing intracardiac defect anatomy, pulmonary artery stenoses, and associated congenital heart defects.

It is recommended to perform magnetic resonance imaging of the heart and great vessels to assess their structural and functional changes (if necessary, or if the results of instrumental methods are inconclusive) [2, 3, 10, 23].

(Level of Evidence C, Grade of Recommendation 5)

Comments: Cardiac MRI improves visualization capabilities, particularly for assessing ventricular volume overload and quantifying shunts. The method provides high spatial and temporal resolution three-dimensional anatomical reconstructions, volumetric measurements, vascular assessment, and myocardial fibrosis evaluation. MRI is used in adults in the following clinical situations:

- as an additional method when echocardiographic data are ambiguous or borderline, e.g., left ventricular volumes and ejection fraction;

- MRI is more informative compared to echocardiography for the quantitative assessment of right ventricular volumes and ejection fraction, right ventricular outflow tract obstruction, pulmonary regurgitation, evaluation of pulmonary artery and aortic stenoses, collaterals and arteriovenous malformations, in coronary artery disease (CAD), quantitative assessment of myocardial mass (left and right ventricles), detection and quantification of myocardial fibrosis/scar (gadolinium-enhanced imaging reveals late enhancement); tissue characterization (fibrosis, fat, etc.) [2, 3, 8].

2.5 Other Procedures

It is recommended that all patients undergo transesophageal echocardiography prior to the completion of the main correction stage to assess the quality of the performed surgical intervention [3, 5, 11, 24].

It is recommended that all patients undergo electrocardiography registration prior to hospital discharge to monitor the postoperative status [3, 5, 11, 24].

(Level of Evidence C, Grade of Recommendation 5)

It is recommended that all patients undergo echocardiography prior to hospital discharge to monitor the postoperative status [3, 5, 11, 24].

(Reliability of Evidence Level C, Confidence Level in Recommendations 5).

3. Treatment, including drug and non-drug therapies, diet therapy, and pain management

3.1. Conservative management

Maintenance therapy

Conservative management of unoperated patients is individualized and performed by an experienced pediatric cardiologist or cardiologist [2, 3, 10]. Treatment tactics are determined by the severity of hypoxemia.

It is recommended that in the absence of decreased blood oxygen saturation and with a satisfactory weight gain in the infant, surgical intervention be performed once a body weight of 3 kg is reached [3, 5, 9].

(Reliability of Evidence Level C, Confidence Level in Recommendations 5).

It is recommended to administer long-term oxygen therapy to patients with a persistent decrease in blood partial pressure of oxygen below 7 kPa (less than 50 mm Hg) or blood oxygen saturation below 70% according to pulse oximetry [2, 3, 10].

(Reliability of Evidence Level C, Confidence Level in Recommendations 5).

Comments: Most patients with PA/VSD exhibit severe resting arterial hypoxemia due to right-to-left blood shunting. Oxygen therapy can alleviate patients' condition, improve exercise tolerance and quality of life, but it cannot improve pulmonary hemodynamics or influence the clinical course of the defect [2, 3, 8]. The standard oxygen therapy regimen is 2–4 L/min, and in some cases up to 10 L/min using home-use systems is required.

It is recommended to administer alprostadil** therapy to newborns with critical pulmonary stenosis and a closing patent ductus arteriosus (PDA) to maintain PDA patency [8, 10].

(Reliability of Evidence Level C, Confidence Level in Recommendations 5).

It is recommended to monitor vital signs during alprostadil** therapy (heart rate [A02.10.002 Measurement of heart rate], respiratory rate [A02.09.001 Measurement of respiratory rate], blood pressure [A02.12.002 Measurement of blood pressure on peripheral arteries]) [20, 24-30].

(Reliability of Evidence Level C, Confidence Level in Recommendations 5).

It is recommended to use "loop" diuretics in patients with a large PDA, multiple large major aortopulmonary collateral arteries with pulmonary overcirculation, and symptoms of heart failure, including chronic heart failure [27-30].

(Reliability of Evidence Level C, Confidence Level in Recommendations 5).

Comments: Clinical data demonstrate a clear improvement in symptoms with the use of "loop" diuretics in patients with circulatory failure. When prescribing diuretics, forced diuresis should be avoided, and systemic blood pressure should be closely monitored to prevent hemoconcentration in response to decreased intravascular blood volume, hypokalemia, and prerenal failure.

3.2. Surgical treatment

Radical correction

Any anatomical variant of PA/VSD is an absolute indication for surgery, which should be performed as early as possible, most frequently during the child's first year of life.

It is recommended to perform radical correction (A16.10.032 Radical correction of pulmonary atresia) - VSD closure, creation of a right ventricular outflow tract to the pulmonary artery system, and, if necessary, unifocalization of the pulmonary blood supply to the maximum possible number of lung segments - as a primary operation in patients with favorable pulmonary artery anatomy, usually during the first year of life, or as a second stage of correction following a palliative intervention if the clinical assessment indicates favorable anatomy and hemodynamics [2, 3, 32].

(Reliability of Evidence Level C, Confidence Level in Recommendations 5).

It is recommended to consider the following indications when planning radical correction for PA/VSD:

1. Satisfactory clinical and functional status of patients:

A. hemoglobin level less than 180-190 g/L (if values are below these thresholds, radical correction of the defect can be performed in children)

B. aortic blood oxygen saturation greater than 70-75%

2. Anatomical criteria for operability of the defect:

A. Satisfactory development of the pulmonary artery system:

the ratio of the sum of the pulmonary artery ostia diameters to the descending aorta diameter ((A1+B1)/dAo) greater than 1.5,

the ratio of the sum of the cross-sectional areas of the right and left pulmonary arteries to the body surface area (pulmonary arterial index) greater than 250 mm²/m²,

full neopulmonary arterial index (FNAI) - the ratio of the sum of the cross-sectional areas of the right and left pulmonary arteries and MAPCAs to the body surface area - greater than 250 mm²/m².

B. Absence of decreased left ventricular volumetric parameters (left ventricular end-diastolic volume greater than 40 mL/m²)

C. Ventricular ejection fraction greater than 50%

D. Absence of multiple, large collateral arteries

D. presence of true pulmonary arteries in all bronchopulmonary segments [2, 3, 11, 32].

(Reliability of Evidence Level C, Confidence Level in Recommendations 5).

Comments: Indications for radical repair are determined based on the assessment of the patient's general condition, anatomy, and hemodynamics. Radical repair can be performed as a primary surgery in favorable PA anatomy, usually in the first year of life, or as a second stage of correction following a palliative intervention if clinical evaluation indicates favorable anatomy and hemodynamics. In patients with a pulmonary artery index of 150–250 mm²/m^2,complete neopulmonary arterial index 150–250 mm²/m² and LV EDV index of 30–40 mL/m2, radical repair may be associated with a higher risk [3].

It is recommended to determine the surgical approach taking into account the anatomical variant of right ventricular outflow tract obstruction [3, 5, 11, 12].

(Reliability of Evidence Level C, Confidence Level in Recommendations 5).

It is recommended for type 1 defect with a well-developed pulmonary artery trunk to perform radical correction with transannular patch plasty of the right ventricular outflow tract and pulmonary artery trunk (A16.10.032 Radical correction of pulmonary atresia), extending the incision to the stenotic pulmonary artery branch if necessary. [3, 5, 11, 12]

(Reliability of Evidence Level C, Confidence Level in Recommendations 5).

It is recommended in cases of hypoplasia of the proximal pulmonary artery trunk and type 2 defect to perform conduit implantation (A16.10.039.001 Radical correction of truncus arteriosus using a conduit under cardiopulmonary bypass). [3, 5, 11, 12]

(Reliability of Evidence Level C, Confidence Level in Recommendations 5).

It is recommended during proximal conduit implantation to take into account the possible anomalous origin of the coronary arteries (crossing of the RV outflow tract by a major coronary artery). Both valved and non-valved synthetic vascular prostheses, xenografts, or homografts can be used as a conduit. [3, 5, 11, 12]

(Reliability of Evidence Level C, Confidence Level in Recommendations 5).

It is recommended for all patients with pulmonary atresia and ventricular septal defect (PA-VSD) to undergo radical correction (A16.10.032 Radical correction of pulmonary atresia)—VSD closure and reconstruction of the RV outflow tract when the Nakata index is greater than 250 mm²/m² and the left ventricular end-diastolic volume index is greater than 40 mL/m2 [3, 5, 11, 12].

(Reliability of Evidence Level C, Confidence Level in Recommendations 5).

It is recommended for all patients with PA-VSD in the presence of pulmonary branch stenoses, absence of bifurcation, or pulmonary artery interruption to perform their plasty using auto- or xenopericardium (Cardiovascular patch of animal origin***) [3, 5, 11, 12].

(Level of Evidence C, Grade of Recommendation 5)

It is recommended during radical correction to eliminate all identified congenital heart defects in the patient [1, 2, 3, 31].

(Reliability of Evidence Level C, Confidence Level in Recommendations 5).

It is recommended to perform palliative surgeries in patients with PA-VSD in the presence of:

1. Arterial blood oxygen saturation less than 70%

2. Hemoglobin level greater than 190 g/L

3. Newborns with a body weight of less than 2.5 kg;

4. Concomitant pathology:

• congenital extracardiac anomalies (CNS, lungs, liver, kidneys);

• infectious complications;

• acute cerebrovascular accident

5. Unsatisfactory defect anatomy:

• Decreased LV volumetric parameters — LV end-diastolic volume index less than 40 mL/m^2, ratio of LV EDD/EDV less than 70–75% of age norm;

• Mitral valve size less than 2–2.5 Z-score;

6. Hypoplasia of the pulmonary vascular bed:

• Ratio of the sum of pulmonary artery ostial diameters to the descending aorta diameter ((A1+B1)/dAo) less than 1.2.

• Ratio of the sum of cross-sectional areas of the right and left pulmonary arteries to body surface area (pulmonary artery index) less than 250 mm2/m2.

• Full neo-pulmonary arterial index (FPAI) less than 250 mm²/m².

7. Pronounced collateral blood supply to the lungs [2, 3, 11].

(Reliability of Evidence Level C, Confidence Level in Recommendations 5).

Comment: The goal of palliative treatment is to increase pulmonary blood flow and prepare for radical correction of the defect. As a result of repeated surgeries, arterial blood oxygen saturation increases, hemoglobin levels decrease, LV volume increases (greater than 40 mL/m2), the pulmonary artery system develops, and pulmonary artery pressure rises. Subsequently, patients may undergo radical correction of the defect.

The following types of palliative surgeries are recommended for patients with PA-VSD:

1. Creation of systemic-to-pulmonary shunts (A16.12.046 Creation of anastomosis between subclavian artery and pulmonary artery)

2. Reconstruction of RV outflow tracts without VSD closure (A16.10.007.001 Creation of connection between right ventricle and pulmonary artery)

3. Creation of a central shunt (A16.12.045 Creation of anastomosis between aorta and pulmonary artery)

4. Patent ductus arteriosus (PDA) stenting,

5. Stenting of pulmonary artery branches (A16.12.028.004 Stenting of pulmonary arteries)

6. Stenting of major aortopulmonary collateral arteries,

7. Perforation, transluminal balloon angioplasty, stenting of the membrane in the pulmonary valve projection in type 1 defect

8. Unifocalization of major aortopulmonary collateral arteries (A16.12.069.001 Unifocalization of pulmonary blood flow) [8, 10, 12, 20, 30].

(Level of Evidence C, Grade of Recommendation 5)

Comment: The choice of a specific type of palliative treatment depends on the surgeon's preference, multidisciplinary team decision, expertise of the endovascular surgery team, patient's clinical condition, age, history of previous palliative interventions, and degree of pulmonary artery development. Various types of systemic-to-pulmonary shunts are most frequently performed. Reconstruction of the right ventricular outflow tract without VSD closure is performed in cases of pulmonary artery hypoplasia when a systemic-to-pulmonary shunt is not feasible. PDA stenting is performed in critically ill newborns. Perforation, transluminal balloon angioplasty, and stenting of the membrane in the pulmonary valve projection for type 1 defect are performed in severely ill patients with a favorable defect morphology

Staged surgical treatment of PA-VSD

It is recommended to perform staged surgical treatment in patients under the following conditions [2, 3, 10, 31]:

1. Arterial blood oxygen saturation less than 70%

2. Hemoglobin level greater than 190 g/L

3. Multiple major aortopulmonary collateral arteries (MAPCAs)

4. Hypoplasia of the pulmonary artery system (Nakata index less than 150–200, McGoon ratio less than 1.0, FPAI less than 150 mm²/m²).

(Reliability of Evidence Level C, Confidence Level in Recommendations 5).

Comments: The goal of staged surgical treatment is VSD closure, creation of an adequate connection between the RV and the pulmonary artery system, elimination of extracardiac sources of pulmonary blood flow using embolization or direct ligation of MAPCAs, and restoration of true pulmonary blood flow to the maximum possible number of broncho-pulmonary segments using unifocalization techniques [3, 17, 33].

The first stage usually involves reconstruction of the right ventricular outflow tract without VSD closure. All components of the multilevel stenosis are excised in a graded manner, and the RV outflow tract and pulmonary artery trunk are patched. In type 2 defect, an artificial pulmonary artery trunk is implanted. The greatest growth of the pulmonary arteries occurs within the first 6 months after surgery. All subsequent surgical procedures should aim at complete elimination of multiple extracardiac sources of pulmonary blood flow, restoration of the most complete true pulmonary blood flow to lung lobes and segments, adequate enlargement of underdeveloped segments or replenishment of missing segments of the central pulmonary arteries, and creation of a single source of pulmonary blood flow from the right ventricle [29, 32, 35].

It is recommended for all patients with PA-VSD and an LV end-diastolic volume index less than 40 mL/m² or Nakata index less than 250 mm²/m² but greater than 150 mm²/m² to perform a systemic-to-pulmonary shunt (A16.12.046 Creation of anastomosis between subclavian artery and pulmonary artery) as the first stage of surgical treatment [3, 5, 11, 12].

(Level of Evidence C, Grade of Recommendation 5)

It is recommended for all patients with PA-VSD and a Nakata index less than 150 mm²/m² to perform reconstruction of the RV outflow tract without VSD closure (A16.10.007.001 Creation of connection between right ventricle and pulmonary artery) as the first stage of surgical treatment [3, 5, 11, 12].

(Level of Evidence C, Grade of Recommendation 5)

Surgical interventions in patients with type III PA-VSD.

It is recommended to perform radical correction of the defect (A16.10.032 Radical correction of pulmonary atresia) if the patient meets operability criteria [2, 3, 10].

(Reliability of Evidence Level C, Confidence Level in Recommendations 5).

Comments: Indications for radical correction are determined based on the assessment of the patients' general condition, anatomy, and hemodynamics. Radical correction can be performed as a primary surgery in patients with favorable pulmonary artery anatomy, usually during the first year of life, or as a second stage of correction after a palliative intervention. Indications for radical correction of the defect are outlined in the "Surgical Treatment" section.

It is recommended to perform a palliative intervention (systemic-to-pulmonary shunt (A16.12.046 Creation of an anastomosis between the subclavian artery and pulmonary artery) or reconstruction of the RV outflow tract without VSD closure (A16.10.007.001 Creation of a communication between the right ventricle and pulmonary artery) if the patient does not meet the anatomical or hemodynamic criteria for operability of the defect [2, 3, 10].

(Reliability of Evidence Level C, Confidence Level in Recommendations 5).

Comments: The goal of palliative treatment is to increase pulmonary blood flow and prepare the patient for radical repair of the defect. As a result of repeated surgeries, arterial blood oxygen saturation increases, hemoglobin levels decrease, LV volume increases, the PA system develops, and pressure in the PA increases. Subsequently, patients may undergo radical repair of the defect. Indications for radical repair of the defect are described in the section "Palliative interventions".

It is recommended to perform unifocalization of pulmonary blood flow followed by radical correction in patients with major aortopulmonary collateral arteries [36-38].

(CRA C, LOR 4).

It is not recommended to perform one-stage radical correction in patients with MAPCAs and hypoplasia of the PA system, as the surgery is associated with a rather high mortality rate [39-41].

(Reliability of Evidence Level C, Confidence Level in Recommendations 5).

Comments: In type III PA/VSD, the anatomy of the defect is characterized not only by the absence of communication between the RV and the PA system, but also by the absence of the PA trunk and one of its branches. Previously, exclusively various types of systemic-to-pulmonary shunts were performed; currently, unifocalization of pulmonary blood flow is performed followed by radical correction. This intervention is still associated with a fairly high mortality rate, numerous postoperative complications, and the need for reoperations [3, 31].

Surgical interventions in patients with type IV PA/VSD.

It is recommended to perform staged unifocalization of pulmonary blood flow in patients with type IV PA/VSD prior to radical correction (A16.10.032 Radical correction of pulmonary atresia) [42-44].

(Reliability of Evidence Level C, Confidence Level in Recommendations 5).

Comments: These palliative procedures are mainly performed using a synthetic graft (PTFE) or xenopericardium patch to create central PA branches. Each procedure is supplemented by the creation of a systemic-to-pulmonary shunt using a PTFE graft.

It is recommended for patients with type IV PA/VSD, in the presence of local stenoses of collateral vessels, to perform plastic repair of the stenosis site using a xenopericardial patch (Cardiovascular patch of animal origin***) [2, 3, 10].

(Reliability of Evidence Level C, Confidence Level in Recommendations 5).

It is recommended to perform an anastomosis between the collateral and subclavian arteries using a synthetic graft in patients [2, 3, 12].

(Reliability of Evidence Level C, Confidence Level in Recommendations 5).

Comments: In patients with numerous stenosed collateral arteries 2–3 mm in diameter, it is impossible to create central pulmonary arteries due to the small diameter of the vessels; therefore, such patients undergo procedures aimed at eliminating or "bypassing" the stenoses. Both procedures are aimed at improving the somatic condition of the patients, i.e., increasing the level of pulmonary blood flow and, accordingly, blood oxygen saturation.

It is recommended to perform A16.10.032 Radical correction of pulmonary atresia in patients after completion of unifocalization procedures [2, 3, 8, 45].

(CRA C, LOR 4).

It is recommended for all patients in whom surgical treatment is not feasible to undergo angioplasty (A16.12.026.012 Transluminal balloon angioplasty) and stenting of collateral arteries (A16.12.028.013 Stenting of major aortopulmonary collateral arteries) [11, 20, 44, 45].

(Reliability of Evidence Level C, Confidence Level in Recommendations 5).

Comments: are performed in some patients as a palliative procedure, during which balloon angioplasty of the MAPCA stenosis area or stent implantation into the collateral artery supplying a large lung segment is carried out. After the procedure, blood oxygen saturation increases.

It is recommended to perform endovascular interventions in patients with type IV PA/VSD under the following conditions:

1. Severe condition of patients, hypoxemia, hemoglobin level above 180 g/L.

2. Impossibility of performing standard surgical procedures on collateral arteries (complex patterns of vascular distribution in the lungs; previous interventions in the pleural cavities; morphological changes in the lungs corresponding to pulmonary hypertension).

3. Absence of contraindications for anticoagulant therapy.

4. The collateral artery supplies more than 3 lung segments.

5. Mean pressure in the collateral vessel distal to the stenosis is less than 15 mm Hg.

6. Stent placement must not obstruct blood flow to the side branches of the collateral artery.

7. Absence of severe peripheral stenoses of MAPCAs.

8. Adult age, when open surgical procedures carry a higher risk [11, 20, 46-48].

(Reliability of Evidence Level C, Confidence Level in Recommendations 5).

Surgical interventions in the long-term period after radical correction of the defect.

It is recommended for all patients after radical correction of PA/VSD in the presence of PA stenoses to undergo transluminal balloon angioplasty of the pulmonary arteries with stenting [3, 10, 20].

(Level of Evidence C, Grade of Recommendation 5)

It is recommended for all patients after radical correction of PA/VSD in the presence of stenoses of implanted RV-PA conduits to undergo transluminal balloon angioplasty (A16.12.026.013 Transluminal balloon angioplasty of pulmonary arteries) with stenting (A16.12.028.011 Conduit stenting) [3, 10, 22].

(Level of Evidence C, Grade of Recommendation 5)

It is recommended for all patients after radical correction of PA/VSD in the presence of major aortopulmonary collateral arteries to undergo their embolization, provided that the MAPCAs are not the sole source of blood supply to the respective lung segments [3, 10, 22].

(Level of Evidence C, Grade of Recommendation 5)

Comment: Embolization of collateral arteries helps to reduce pulmonary blood flow volume, lower systolic PA pressure, and decrease RV volume overload.

It is recommended for all patients after radical correction of PA/VSD who have pulmonary valve regurgitation combined with RV dilatation (RV EDV greater than 60 mL/m²), tricuspid regurgitation, or QRS complex prolongation greater than 160 ms to undergo pulmonary valve replacement (A16.10.003.030 Pulmonary valve replacement under cardiopulmonary bypass) [3, 10, 20].

(Level of Evidence C, Grade of Recommendation 5)

4. Medical Rehabilitation and Sanatorium-Resort Treatment

It is recommended within 6 months after surgical correction of PA/VSD under cardiopulmonary bypass in patients with a complicated postoperative course (residual VSDs, pulmonary artery stenoses, heart failure, infectious complications, reoperations during the same hospitalization) to undergo rehabilitation in a specialized cardiological medical facility [2, 3, 10, 47, 49].

(Reliability of Evidence Level C, Confidence Level in Recommendations 5).

Comments: The following conditions may occur after defect correction:

- Residual shunt or VSD recanalization may occur in 10% of cases after defect correction. In such cases, reoperation is indicated for a hemodynamically significant shunt [2, 3, 10, 47, 49].

- Residual pulmonary artery stenosis may occur in 15% of cases after defect correction. In such cases, reoperation is indicated for a hemodynamically significant stenosis [2, 3, 10, 47, 49].

- Pulmonary valve regurgitation may occur in 16% of cases after defect correction. Reoperation is indicated for hemodynamically significant regurgitation [2, 3, 10, 47, 49].

- Tricuspid regurgitation may occur in 18% of cases after defect correction. In such cases, reoperation is indicated for hemodynamically significant regurgitation [2, 3, 10, 47, 49].

- RV outflow tract aneurysm may occur in 2% of cases after defect correction. In the case of a false aneurysm, emergency surgery is indicated due to the risk of rupture. In the case of a true aneurysm, reoperation is indicated for large aneurysm size, concomitant severe stenoses of PA branch ostia, and tricuspid regurgitation [2, 3, 10, 47, 49];

- Aortic regurgitation. It may be a consequence of the initial defect anatomy (cusp prolapse in subarterial or perimembranous VSD), or involvement of the aortic valve cusp in the suture line. Its frequency reaches 5-20% [2,5,16]. Aortic regurgitation may progress, and its incidence increases with age. It usually progresses slowly, and this process is highly individual. Monitoring and (if necessary) surgical treatment are carried out in accordance with established guidelines [2, 3, 10, 47, 49].

- Rhythm and conduction disorders (ventricular and supraventricular arrhythmias, bundle branch block, atrioventricular blocks, and sudden cardiac death) may occur both immediately after surgical correction and at later periods [48, 49].

- Neurological disorders, such as encephalopathy and seizure syndrome, occur in 0.5–1% of cases and require ongoing follow-up by a neurologist at the patient's place of residence [50].

Following palliative correction:

- Anastomosis hypofunction, insufficient dilation of the right ventricular outflow tract

- accompanied by pulmonary hypovolemia and pronounced hypoxemia;

- Anastomosis hyperfunction, excessive dilation of the right ventricular outflow tract

- accompanied by significant pulmonary hypervolemia, lung congestion, left ventricular failure, and subsequently pronounced hypoxemia;

- Unsuccessful unifocalization procedure - exclusion of some lung segments from the blood supply - progressive hypoxemia.

It is recommended to restrict physical activity for all patients following surgical correction for three months from the date of hospital discharge [2, 3].

(LoE C, GoR 5).

Comments: One year after radical correction, patients may be cleared for participation in all competitive sports in the absence of: 1) Signs of VSD recanalization, 2) Severe tricuspid regurgitation, 3) Severe pulmonary valve regurgitation, 4) Pulmonary artery stenosis, 5) Symptomatic tachyarrhythmias or second- or third-degree AV block; 6) Signs of myocardial dysfunction; 7) Ventricular or atrial tachycardia; 8) Right ventricular pressure elevation exceeding 50% of the systolic BP level.

5. Prevention and Dispensary Observation

The prognosis after surgical correction of pulmonary atresia with ventricular septal defect is favorable provided the congenital heart disease is corrected in a timely manner. Life expectancy and physical performance may be limited in the presence of iatrogenic residual complications [2, 3, 4, 47].

It is recommended to provide dispensary follow-up for patients after surgical intervention [3, 47].

(Reliability of Evidence Level C, Confidence Level in Recommendations 5).

Comments: The frequency of dispensary visits (examinations, consultations) with a pediatric cardiologist is at 1 month, 3, 6, and 12 months postoperatively. The dispensary follow-up protocol includes ECG and echocardiography, as well as pulse oximetry when necessary. Subsequent monitoring by specialists is carried out at intervals of 3–36 months until the age of 18, after which the patient transitions to the adult outpatient clinic for continued follow-up [1, 10, 11].

Following surgery, it is recommended to remain under dispensary observation for one year, and thereafter as clinically indicated. Patients with right ventricular dysfunction, residual shunt, right ventricular outflow tract obstruction, or aortic regurgitation should be followed annually at specialized centers.

When determining the frequency of follow-up, one should be guided by the hemodynamic status, presence of iatrogenic residual complications, symptoms of heart failure, cardiac arrhythmias, hypoxemia, etc.

Depending on whether a patient with a corrected or uncorrected defect belongs to a particular functional class (FC), varying frequencies of follow-up and scopes of required diagnostic examinations are recommended:

Follow-up frequency (months) / Diagnostic methods	FC I	FC II	FC III	FC IV
Pediatric cardiologist appointment (examination, consultation)	36	12	6	3
Electrocardiography	12	12	6-12	3-6
Echocardiography	12	12	6-12	3-6
Pulse oximetry	12	12	6	3
Assessment of the cardiovascular system's response to physical exertion (6-minute walk test, exercise stress testing using a cycle ergometer when necessary)	36	24	12-24	6-12

It is recommended to provide a dispensary appointment (examination, consultation) with a pediatric cardiologist for adolescents before and after surgery, with subsequent transfer to dispensary follow-up by an adult cardiologist [16].

(Reliability of Evidence Level C, Confidence Level in Recommendations 5).

It is recommended to perform pulse oximetry in all patients once a year [2, 3, 11].

(Level of Evidence C, Grade of Recommendation 5)

Comment: The test is performed to assess the patient's condition, indirectly evaluate pulmonary blood flow, and check for potential veno-arterial blood shunting.

It is recommended to perform an ECG in all patients once a year following surgical intervention [2, 3, 11].

(Level of Evidence C, Grade of Recommendation 5)

Comments: An ECG is performed to evaluate cardiac rhythm and QRS complex duration. In patients who underwent radical correction via a transventricular approach, complete right bundle branch block is registered, and QRS complex duration reflects the degree of right ventricular enlargement. A QRS duration of 180 ms or more is a risk factor for sudden cardiac death. The detection of atrial flutter or fibrillation, or episodes of ventricular tachycardia, indirectly indicates pronounced hemodynamic disturbances. ECG analysis reveals sinus rhythm in 92% of cases and coronary sinus rhythm in 8%. A decrease in the degree of rightward axis deviation is noted, along with a reduction in signs of right ventricular hypertrophy, right atrial enlargement, and right bundle branch block.

It is recommended to perform 24-hour Holter ECG monitoring (A05.10.008 Holter monitoring of cardiac rhythm) in all patients once every 2 years [50, 51].

(Level of Evidence C, Grade of Recommendation 5)

Comments: Holter monitoring is necessary to rule out cardiac arrhythmias, which are frequent after radical correction, the most severe being sustained monomorphic ventricular tachycardia originating from the right ventricular outflow tract. Approximately 45% of patients have clinical symptoms of arrhythmias, while sudden death accounts for 5–6% of the evaluated patient cohort. For patients with nonsustained ventricular tachycardias without hemodynamic compromise, specific treatment is generally not administered, or medications suppressing ectopic focus activity (beta-blockers) are prescribed. Patients with syncopal or presyncopal conditions receive more "aggressive" treatment. This includes antiarrhythmic therapy and the implantation of implantable cardioverter-defibrillators***, which is the sole method for preventing sudden cardiac death in this category of patients.

In patients with atrial re-entry tachycardia after radical correction, conservative treatment has low efficacy; all such patients require intracardiac electrophysiological study and radiofrequency ablation.

In high-grade atrioventricular blocks, implantation of a cardiac pacemaker*** is required

It is recommended to perform echocardiography and pleural ultrasound in all patients 2 weeks after hospital discharge [3, 47].

(Reliability of Evidence Level C, Confidence Level in Recommendations 5).

Comment: Echocardiography is used to monitor the degree of pulmonary and aortic regurgitation, check for VSD recanalization, and assess the severity of right ventricular outflow tract and pulmonary artery stenosis. Pleural ultrasound is necessary to rule out fluid accumulation and to evaluate the adequacy of postoperative conservative therapy. The obtained data allow for timely adjustment of conservative therapy or evaluation of the need for reoperation.

It is recommended to perform echocardiography in all patients once a year [8, 10].

(Reliability of Evidence Level C, Confidence Level in Recommendations 5).

Comments: Echocardiography is necessary to assess the patient's postoperative status. The presence and severity of residual right ventricular outflow tract and pulmonary artery stenosis, the significance of pulmonary regurgitation, and the presence and severity of tricuspid regurgitation are determined. The integrity of atrial and ventricular septal defect closures, the size of the right atrium, and the degree of aortic root dilation are evaluated. Measurement of the right ventricular myocardial performance index can be a useful adjunct in assessing right ventricular systolic function.

It is recommended to perform transvenous cardiac catheterization and angiocardiography (A06.10.008 Cardiac ventriculography) in patients after radical surgery to identify residual complications [3, 11, 20, 53].

(LoE C, GoR 5).

Comments: The study is performed to assess the development of the pulmonary artery system, potential deformation of pulmonary artery branches in the area of the systemic-pulmonary anastomosis, right ventricular outflow tract obstruction, pulmonary regurgitation, and pulmonary artery and aortic stenoses; and to diagnose collateral arteries and arteriovenous malformations.

It is recommended that all patients undergo chest MRI (A05.30.006 Magnetic resonance imaging of chest organs) once every 2–5 years to assess intracardiac hemodynamics and detect residual complications following the correction of pulmonary atresia with ventricular septal defect [8, 10, 12, 43, 55].

(Reliability of Evidence Level C, Confidence Level in Recommendations 5).

Comment: The study can be useful for evaluating right ventricular volume, its systolic function, and the severity of pulmonary regurgitation and residual congenital heart defects, especially in cases of pulmonary artery stenosis and aortic dilation.

It is recommended to perform exercise tolerance testing — bicycle ergometry, treadmill test (A12.10.005 Bicycle ergometry) in patients with residual complications after radical correction [2, 3, 56].

(Reliability of Evidence Level C, Confidence Level in Recommendations 5).

Comments: The test is necessary for an objective assessment of cardiac functional capacity and the presence of potential arrhythmias. The study is performed using a bicycle ergometer or treadmill test.

It is recommended for all patients after surgical treatment to undergo antibacterial prophylaxis of infective endocarditis before dental scaling/procedures for 6 months [2, 3, 55, 56].

(Reliability of Evidence Level C, Confidence Level in Recommendations 5).

Comments: For any type of congenital heart disease where synthetic materials/prostheses were used during correction, indications-based prophylaxis of bacterial endocarditis is carried out for 6 months postoperatively or for life if residual shunts or valve regurgitation persist. Endocarditis prophylaxis is performed during dental interventions [2, 3].

It is recommended that children who have undergone surgery for pulmonary atresia with ventricular septal defect (PA-VSD) receive vaccinations no earlier than three months later [2, 3].

(Reliability of Evidence Level C, Confidence Level in Recommendations 5).

6. Organization of Medical Care

Indications for hospital admission to a medical organization:

1. Presence of cyanosis, dyspnea (the presence of cyanosis and dyspnea, especially their intensification, is

an indication for planned hospitalization to determine the management regarding surgical treatment of the patient);

2. Diagnosis of the defect type (the presence of a defect is an indication for hospitalization for

the purpose of diagnosis and selection of the treatment method for congenital heart disease).

Indications for emergency hospitalization:

1. Sharp intensification of cyanosis, dyspnea;

2. Dependence on intravenous prostaglandin infusions in the newborn;

3. Progressive cardiovascular failure.

Indications for patient discharge from a medical organization:

1. Elimination of all defects,

2. Age-appropriate normal blood pressure and heart rate values;

3. Relief of arterial hypoxemia;

4. Sinus heart rhythm;

5. Absence of cardiac arrhythmias;

6. Absence of heart failure;

7. Adequate urine output level;

8. Increased exercise tolerance;

9. Heart failure functional class 1–2 at discharge.

10. Following palliative interventions: Hemodynamics independent of prostaglandin infusion after staged correction, oxygen saturation level > 275% in room air after staged correction.

7. Additional Information

It is recommended that patients with PA-VSD avoid excessive physical activity that provokes potentially dangerous symptoms such as severe dyspnea and fatigue, dizziness, syncope, and chest pain [2, 3, 10].

(Reliability of Evidence Level C, Confidence Level in Recommendations 5).

It is recommended to administer influenza and pneumococcal immunizations to all patients to prevent disease progression amidst intercurrent infections [2, 3, 10].

(CRA C, LVE 5).

Pregnancy and childbirth

Pregnancy and childbirth are usually well tolerated:

- with corrected heart defects;

- with preserved right ventricular function;

- in NYHA functional classes I–II NYHA;

- in the absence of significant VSD recanalization, significant right ventricular outflow tract obstruction, and severe tricuspid regurgitation [2, 5, 7].

Pregnancy is not recommended in the presence of cyanosis with uncorrected heart defects and residual complications after radical correction [2, 3].

(Reliability of Evidence Level C, Confidence Level in Recommendations 5).

Comments: Pregnancy is strictly contraindicated in uncorrected heart defects due to high maternal and infant mortality [2, 5]. Pregnancy in patients with cyanosis, in the absence of pulmonary hypertension, is associated with significant risks for the mother and fetus.

Criteria for Evaluating the Quality of Medical Care

№	Quality criteria	ESC	GoR	LoE
Diagnosis stage
1	Anamnesis and complaints were collected	none	C	5
2	Heart auscultation was performed	none	C	5
3	Electrocardiography was performed	none	C	5
4	Echocardiography with color Doppler mapping was performed	IC	C	5
5	Transvenous cardiac chamber catheterization and angiocardiography (A06.10.008 Ventriculography of the heart) were performed when indicated	IC	C	5
Conservative and surgical treatment stage
1	Loop diuretics were prescribed in the presence of heart failure symptoms	none	C	5
2	Surgical treatment for radical correction of pulmonary atresia was performed	IC	C	5
Postoperative monitoring phase
1	Electrocardiography was performed prior to discharge from the hospital	none	C	5
2	Echocardiography was performed before hospital discharge	none	C	5

References

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Rybka M.M., Khinchagov D.Ya., Mumladze K.V., Nikulkina E.S. Edited by L.A. Bokeria. Protocols for anesthetic management of X-ray endovascular and diagnostic procedures performed in cardiac surgery patients of various age groups. Guidelines. M.: A.N. Bakulev National Medical Research Center of Cardiovascular Surgery of RAMS; 2018.
Marino PL. The ICU Book. 2nd ed. Baltimore: Williams & Wilkins; 1998.
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Rehman R, Marhisham MC, Alwi M. Stenting the complex patent ductus arteriosus in tetralogy of Fallot with pulmonary atresia: challenges and outcomes. Future Cardiol. 2018 Jan;14(1):55-73.
Barbero-Marcial M., Atik A., Baucia J. et al. Reconstruction of stenotic or nonconfluent pulmonary arteries simultaneously with a Blalock-Taussig shunt. J. Thorac.Cardiovasc. Surg. 1988; 95: 82-9.
Davies B., Mussa S., Davies D., Stickley J., Jones T.J., Barron D.J. Unifocalization of major aortopulmonary collateral arteries in pulmonary atresia with ventricular septal defect is essential to achieve excellent outcomes irrespective of native pulmonary artery morphology. J. Thorac. Cardiovasc. Surg. 2009. 138. 1269-1275.
Honjo O., Al-Radi o., MacDonald C., Tran K.C., Sapra P. The fucnctional intraoperative pulmonary blood flow study is a more sensitive predictor than preoperative anatomy for right ventricular pressure and physiologic tolerance of ventricular septal defect closure after complete unifocalization in patients with pulmonary atresia, ventricular septsl defect and major aortopulmonary collaterals. Circulation. 2009. 120 (11 Suppl.): S46-52.
Reddy V.M., Liddicoat J.R., Hanley E.L. Midline one-stage complete unifocalization and repair of pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals. J. Thorac. Cardiovasc. Surg. 1995. 109. 832-840.
Mainwaring RD, Patrick WL, Roth SJ, Kamra K, Wise-Faberowski L, Palmon M, Hanley FL. Surgical algorithm and results for repair of pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals. J Thorac Cardiovasc Surg. 2018 Sep;156(3):1194-1204.
Mainwaring RD, Patrick WL, Ma M, Hanley FL. An analysis of patients requiring unifocalization revision following midline unifocalization for pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals. Eur J Cardiothorac Surg. 2018 Jul 1;54(1):63-70.
Soquet J, Barron DJ, d"Udekem Y. A Review of the Management of Pulmonary Atresia, Ventricular Septal Defect, and Major Aortopulmonary Collateral Arteries. Ann Thorac Surg. 2019 Aug;108(2):601-612. doi: 10.1016/j.athoracsur.2019.
Ikai A. Surgical strategies for pulmonary atresia with ventricular septal defect associated with major aortopulmonary collateral arteries. Gen Thorac Cardiovasc Surg. 2018 Jul;66(7):390-397.
Zhu J., Kato A., Saedi A., Parker R. Pulmonary flow study predicts survival in pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries. J. Thorac. Cardiovasc. Surg. 2016. 152 (6). 1494-1503.
Barron DJ, Botha P. Approaches to Pulmonary Atresia With Major Aortopulmonary Collateral Arteries. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 2018 Mar;21:64-74.
Sinha R, Gooty V, Jang S, Dodge-Khatami A, Salazar J. Validity of Pulmonary Valve Z-Scores in Predicting Valve-Sparing Tetralogy Repairs-Systematic Review f. Children (Basel). 2019 May 4;6(5):67.
Cohen MS. Assessing the borderline ventricle in a term infant: combining imaging and physiology to establish the right course. Curr Opin Cardiol. 2018 Jan;33(1):95-100.
Mainwaring R.D., Sheikh A.Y., Punn R., Redy V.M., Hanley F.L. Surgical outcomes for patients with pulmonary atresia major aortopulmonary collaterals and Alagille syndrome. Eur. J. Cardiothorac. Surg. 2012. 42. 235-240.
Zahn E.M., Lima V.C., Benson L.N., Freedom R.M. Use of endovascular stents to increase pulmonary blood flow in pulmonary atresia with ventricular septal defect. Am. J. Cardiol. 1992; 70 (1): 411-2.
Podzolkov V.P., Alekyan B.G., Kokshenev I.V., Cheban V.N. Reoperations after correction of congenital heart defects. M.: A.N. Bakulev National Medical Research Center of Cardiovascular Surgery of RAMS; 2013.
Agrawal H, Alkashkari W, Kenny D. Evolution of hybrid interventions for congenital heart disease. Expert Rev Cardiovasc Ther. 2017 Apr;15(4):257-266.
Podzolkov VP, Kassirsky GI (eds.). Rehabilitation of patients after surgical treatment of congenital heart defects. Moscow: Bakulev Scientific Center of Cardiovascular Surgery; 2015.
Priori S.G., Blomstrom-Lundqvist C., Mazzanti A. et al. 2015 ESC Guidelines for the management of patients with ventricular arrhythmias and the prevention of sudden cardiac death: The Task Force for the Management of Patients with Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death of the European Society of Cardiology (ESC). Endorsed by: Association for European Paediatric and Congenital Cardiology (AEPC). ESC Scientific Document Group. Eur Heart J. 2015 Nov 1; 36 (41): 2793-2867.
Brugada J., Blom N., Sarquella-Brugada G. et al. Pharmacological and non-pharmacological therapy for arrhythmias in the pediatric population: EHRA and AEPC-Arrhythmia Working Group joint consensus statement. European Heart Rhythm Association; Association for European Paediatric and Congenital Cardiology. Europace. 2013 Sep; 15 (9):1337-82.
Ammash N, Warnes CA. Cerebrovascular events in adult patients with cyanoticcongenital heart disease. J Am Coll Cardiol 1996; 28: 768-772.
Grosse-Wortmann L, et al. Preoperative total pulmonary blood flow predicts right ventricular pressure in patients early after complete repair of tetralogy of Fallot and pulmonary atresia with major aortopulmonary collateral arteries. J Thorac Cardiovasc Surg. 2013. PMID: 23414777
Hoffman JI, Kaplan S. //The incidence of congenital heart disease. //J Am Coll Cardiol. 2002;39:1890-900.
Habib G., Lancellotti P., Antunes M.J. et al. 2015 ESC Guidelines for the management of infective endocarditis: The Task Force for the Management of Infective Endocarditis of the European Society of Cardiology (ESC) Endorsed by: European Association for Cardio-Thoracic Surgery (EACTS), the European Association of Nuclear Medicine (EANM). Eur Heart J Volume 36, Issue 44, 21 November 2015, Pages 3075-3128.
Garrocho-Rangel A, Echavarria-Garda AC, Rosales-Bёrber MA, Flores-Velazquez J, Pozos-Guillёn A. Dental management of pediatric patients affected by pulmonary atresia with ventricular septal defect: A scoping review. Med Oral Patol Oral Cir Bucal. 2017 Jul 1;22(4):e458-e466.
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Skudarnov E.V. et al. Clinical and anamnesic characteristics of congenital heart defects in children // Russian Pediatric Journal. – 2022. – Vol. 3. – No. 1. – P. 288.
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Appendix A1. Working Group Composition

Arnautova I.V., MD, "Association of Cardiovascular Surgeons of Russia", (Moscow)
Belov V.N., MD, "Association of Cardiovascular Surgeons of Russia", (Kaliningrad)
Bokeria L.A., Academician of RAS, "Association of Cardiovascular Surgeons of Russia", (Moscow)
Boriskov M.V., MD (Krasnodar)
Volkov S.S., Ph.D. (Moscow)
Gorbatikov K.V., MD (Tyumen)
Gorbatykh Yu.N., MD, "Association of Cardiovascular Surgeons of Russia", (Novosibirsk)
Gorbachevsky S.V., Prof., "Association of Cardiovascular Surgeons of Russia", (Moscow)
Gushchin D.K., PhD, (Moscow)
Ermolenko M.L., MD, "Association of Pediatric Cardiologists of Russia", (Moscow)
Zelenikin M.A., Prof., "Association of Cardiovascular Surgeons of Russia", (Moscow)
Zelenikin M.M., Prof., "Association of Cardiovascular Surgeons of Russia", (Moscow)
Kalashnikov S.V., PhD, (Moscow) "Association of Cardiovascular Surgeons of Russia",
Kim A.I., Prof., (Moscow) "Association of Cardiovascular Surgeons of Russia",
Kokshenev I.V., Prof., (Moscow) "Association of Cardiovascular Surgeons of Russia",
Krivoshchekov E.V., MD, (Tomsk)
Krupyanko S.M., MD, "Association of Pediatric Cardiologists of Russia", (Moscow)
Kupryashov A.A., MD, "Association of Cardiovascular Surgeons of Russia", (Moscow)
Movsesyan R.R., MD, "Association of Cardiovascular Surgeons of Russia", (Saint Petersburg)
Nikiforov A.B., Junior Research Fellow, (Moscow)
Petrushenko A.V., Ph.D. (Kazan)
Plotnikov M.V., Ph.D. (Astrakhan)
Podzolkov V.P., Academician of the Russian Academy of Sciences, "Association of Cardiovascular Surgeons of Russia", (Moscow)
Pursanov M.G., MD, "Russian Scientific Society of Interventional Radiology and Endovascular Diagnostics Specialists", (Moscow)
Rychina I.E., PhD, (Moscow) All-Russian Public Organization for Assisting the Development of Diagnostic Imaging and Therapy "Russian Society of Radiologists and Roentgenologists"
Sabirov B.N., MD, "Association of Cardiovascular Surgeons of Russia", (Moscow)
Svobodov A.A., MD, "Association of Cardiovascular Surgeons of Russia", (Moscow)
Sinelnikov Yu.A., MD, (Perm)
Sinitsyn V.E., MD, Prof., (Moscow) All-Russian Public Organization for Assisting the Development of Diagnostic Imaging and Therapy "Russian Society of Radiologists and Roentgenologists"
Trunina I.I., Doctor of Medical Sciences, "Association of Pediatric Cardiologists of Russia", (Moscow)
Tumanyan M.R., Prof., "Association of Pediatric Cardiologists of Russia", (Moscow)
Chernogrivov A.E., MD, "Association of Cardiovascular Surgeons of Russia", (Penza)
Shatalov K.V., MD, "Association of Cardiovascular Surgeons of Russia", (Moscow)
Schmalz A.A., MD, "Association of Cardiovascular Surgeons of Russia", (Moscow) No conflict of interest.
Yurpolskaya L.A., Doctor of Medical Sciences (Moscow) All-Russian Public Organization for Assistance to the Development of Radiology and Therapy "Russian Society of Radiologists"

All members of the Working Group have confirmed that they have no financial support or conflicts of interest to disclose.

Appendix A2. Methodology for Developing Clinical Guidelines

Target audience of the developed clinical guidelines:

Pediatric Cardiologist
Cardiologist
Cardiovascular Surgeon
Ultrasound Diagnostics Physician
Pediatrician
Cardiovascular surgeon / Endovascular surgeon.

During the development of the clinical guidelines, international scales for the levels of recommendation persuasiveness and levels of evidence reliability were used (Tables 1 and 2), as well as a new system of scales for the levels of evidence reliability (LER) and levels of recommendation persuasiveness (LRP) for therapeutic, rehabilitative, preventive, and diagnostic interventions (Tables 3, 4, and 5), introduced in 2018 by the Center for Healthcare Quality Assessment and Control of the Russian Ministry of Health. The National Guidelines were formulated based on ESC recommendations, taking into account national specifics, examination and treatment features, and the availability of medical care. For this reason, the text of these clinical guidelines simultaneously uses two scales for evaluating the reliability of evidence for recommendation theses: the ESC levels of evidence reliability with LRP and LER. ESC recommendation classes have been added to assess the necessity of executing the recommendation thesis.

Table 1. Classes of recommendations according to the European Society of Cardiology (ESC) guidelines.

ESC Class of Recommendation	Definition	Suggested wording
I	Evidence and/or general agreement that a given diagnostic procedure, intervention/treatment is beneficial, useful, effective	Recommended / Indicated
II Ila Ilb	Conflicting data and/or opinions regarding the efficacy/usefulness of a diagnostic procedure, intervention, or treatment. The majority of data/opinions are in favor of the efficacy/usefulness of a diagnostic procedure, intervention, or treatment. Efficacy/usefulness of a diagnostic procedure, intervention, or treatment is less well established.	Recommended / May be applied
III	Data or a unanimous consensus that a diagnostic procedure, intervention, or treatment is useless/ineffective, and in some cases may be harmful.	Not recommended

ESC Class of Recommendation

Definition

Suggested wording

Evidence and/or general agreement that a given diagnostic procedure, intervention/treatment is beneficial, useful, effective

Recommended / Indicated

Ila

Ilb

Conflicting data and/or opinions regarding the efficacy/usefulness of a diagnostic procedure, intervention, or treatment. The majority of data/opinions are in favor of the efficacy/usefulness of a diagnostic procedure, intervention, or treatment. Efficacy/usefulness of a diagnostic procedure, intervention, or treatment is less well established.

Recommended / May be applied

III

Data or a unanimous consensus that a diagnostic procedure, intervention, or treatment is useless/ineffective, and in some cases may be harmful.

Not recommended

Table 2. Levels of evidence reliability according to the European Society of Cardiology (ESC) guidelines.

	ESC Level of Evidence
A	Data derived from multiple randomized clinical trials or meta-analyses
B	Data derived from a single randomized clinical trial or large non-randomized studies
C	Consensus of expert opinion and/or small studies, retrospective studies, registries

Table 3. Scale for assessing the levels of evidence reliability (LER) for diagnostic methods (diagnostic interventions).

	Definition
1	Systematic reviews of studies with reference standard control or systematic reviews of randomized clinical trials with meta-analysis
2	Individual studies with reference method control or individual randomized clinical trials and systematic reviews of studies of any design, excluding randomized clinical trials with meta-analysis
3	Studies without consecutive control using a reference standard, or studies with a reference standard not independent of the test method, or non-randomized comparative studies, including cohort studies
4	Non-comparative studies, case report
5	Only justification of mechanism of action or expert opinion is available

Table 4. Scale for assessing the levels of evidence reliability (LER) for methods of prevention, treatment, and rehabilitation (preventive, therapeutic, and rehabilitative interventions).

	Definition
1	Systematic review of RCTs incorporating meta-analysis
2	Individual RCTs and systematic reviews of studies of any design except RCTs incorporating meta-analysis
3	Non-randomized comparative studies, including cohort studies
4	Non-comparative studies, case reports or case series, case-control studies
5	There is only a justification of the mechanism of action of the intervention (preclinical studies) or expert opinion

Table 5. Scale for assessing the levels of recommendation persuasiveness (LRP) for methods of prevention, diagnostics, treatment, and rehabilitation (preventive, diagnostic, therapeutic, and rehabilitative interventions).

GoR	Definition
A	Strong recommendation (all considered efficacy criteria (outcomes) are important, all studies are of high or acceptable methodological quality, and their conclusions on the outcomes of interest are consistent)
B	Conditional recommendation (not all considered efficacy criteria (outcomes) are important, not all studies are of high or acceptable methodological quality, and/or their conclusions on the outcomes of interest are inconsistent)
C	Weak recommendation (lack of adequate quality evidence (all considered efficacy criteria (outcomes) are unimportant, all studies are of low methodological quality, and their conclusions on the outcomes of interest are inconsistent)

Procedure for updating clinical guidelines

The mechanism for updating clinical guidelines provides for their systematic revision at least once every three years, as well as upon the emergence of new evidence-based medicine data regarding the diagnosis, treatment, prevention, and rehabilitation of specific diseases, or in the presence of justified additions/comments to previously approved clinical guidelines, but no more frequently than once every 6 months.

Appendix A3. Reference Materials

Procedure for the Provision of Medical Care to Patients with Cardiovascular Diseases (Order of the Russian Ministry of Health No. 918n of November 15, 2012)
Order of the Russian Ministry of Health dated February 28, 2019, No. 103n "On Approval of the Procedure and Timelines for the Development and Revision of Clinical Practice Guidelines, Their Standard Form, and Requirements for Their Structure, Composition, and Scientific Validity of Information Included in Clinical Practice Guidelines" (Registered with the Ministry of Justice of Russia on May 8, 2019, No. 54588);
Order of the Ministry of Health of the Russian Federation No. 203n dated May 10, 2017, "On approval of criteria for assessing the quality of medical care";
Order of the Ministry of Health of the Russian Federation No. 804n dated October 13, 2017 "On approval of the nomenclature of medical services";
Order of the Ministry of Health of the Russian Federation No. 700n dated October 7, 2015 "On the nomenclature of specialties of specialists with higher medical and pharmaceutical education";
Order of the Russian Ministry of Health No. 4n dated June 6, 2012 (as amended on September 25, 2014) "On approval of the nomenclature classification of medical devices".

Appendix B. Physician Action Algorithms

A. Algorithm for primary diagnosis and surgical treatment of pulmonary atresia with ventricular septal defect

B. Algorithm for patient evaluation following radical correction of pulmonary atresia with ventricular septal defect

Appendix C. Patient Information

Pulmonary atresia is the congenital absence of a direct connection between the right ventricle and the pulmonary arterial tree. It is a relatively rare congenital heart defect, accounting for 2–3% of all congenital heart defects. The defect occurs both in combination with a ventricular septal defect and with various complex congenital heart defects, such as transposition of the great arteries, tricuspid atresia, single ventricle, etc. The anatomical criteria of the defect are as follows: 1) absence of the pulmonary artery at various levels; 2) large ventricular septal defect; 3) presence of additional sources of collateral pulmonary blood flow; 4) right ventricular hypertrophy; 5) dextroposition of the aortic root; 6) normal spatial relationship between the aorta and the pulmonary artery trunk. At the same time, the child's condition mainly depends on the size of the patent ductus arteriosus and the presence of major aortopulmonary collateral arteries. Hemodynamic disturbances in this defect are determined primarily by the absence of a direct connection between the right ventricle and the pulmonary artery system, making direct flow of venous blood from the right ventricle impossible. Pulmonary blood flow is maintained through a bypass from the right ventricle through the ventricular septal defect into the left ventricle, after which mixed arterialized blood enters the aorta and only then reaches the lungs via the patent ductus arteriosus or collateral vessels. Oxygen saturation of the blood in the aorta, collateral arteries, and pulmonary artery is identical. The patient's life expectancy depends on the nature of pulmonary blood flow. Mortality in children with ductus-dependent circulation is 90% by 12 months of age. In patients with multiple sources of pulmonary blood flow and moderately pronounced cyanosis, mortality reaches 50% by 3–5 years of age. In patients with increased pulmonary blood flow and major aortopulmonary collateral arteries, death occurs with the development of pulmonary hypertension, predominantly in the third decade of life. Overall, the median survival of patients with pulmonary atresia and ventricular septal defect ranges from 6 months to 2 years.

After discharge from a specialized center, it is recommended to strictly follow the instructions specified in the discharge documents (discharge summary).

Follow-up by a pediatric cardiologist or cardiologist at the place of residence is recommended at least once every 6 months, strictly adhering to their prescriptions and appointments.

Follow-up by a pediatric cardiologist or cardiologist at a specialized center is recommended at least once every 12 months.

For any invasive procedures (dental, cosmetic, and other procedures involving or carrying the risk of disrupting the integrity of the skin and mucous membranes), antibiotic prophylaxis is strictly recommended to prevent infective endocarditis.

Any planned invasive procedures must be coordinated with the pediatric cardiologist or cardiologist providing follow-up care at the place of residence.

Changes in the doses and regimens of certain medications, as well as the prescription of additional or alternative medications, are carried out solely by the attending physician.

If side effects from medications occur, it is recommended to discuss this with the attending physician as soon as possible.

Excessive physical exertion should be avoided.

If the following symptoms occur or rapidly progress, an unscheduled consultation with a pediatric cardiologist or cardiologist is recommended as soon as possible: fatigue, shortness of breath, cyanosis, edema, abdominal distension, arrhythmias, syncope, neurological deficits (loss of vision, hearing, speech, numbness of a limb, paresis and paralysis, including transient ones), acute respiratory infections, fever of unknown origin.

Appendix G. Assessment Scales, Questionnaires, and Other Evaluation Tools

No.

Scales and calculators from this guideline

NYHA functional classClassification of chronic heart failure severity

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Answers based on Russian clinical practice guidelines right inside ChatGPT and Claude Connect the WhiteCoat Pro connector, and the assistant will answer based on the guidelines for «Pulmonary Atresia with Ventricular Septal Defect» and other active Ministry of Health guidelines with a reference to the section, rather than retelling foreign guidelines. Address: mcp.pervoklassno.ru/mcp. How to configure ChatGPT, Claude, Gemini, and Perplexity for Russian clinical practice guidelines

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