List of Abbreviations
ВПС – congenital heart disease
RVOT – right ventricular outflow tract
VSD – ventricular septal defect
ASD – atrial septal defect
CT – computed tomography
LV – left ventricle
PA – pulmonary artery
PAH – pulmonary arterial hypertension
PH – pulmonary hypertension
LA – left atrium
PVR – pulmonary vascular resistance
RV – right ventricle
RA – right atrium
PCC – pulmonary circulation
CIR – circulatory insufficiency
PTBV – percutaneous transluminal balloon valvuloplasty (percutaneous transluminal balloon pulmonary valvuloplasty)
ECG – electrocardiography
EchoCG – echocardiography
Qp/Qs – pulmonary-to-systemic blood flow ratio
Terms and Definitions
Cardiac catheterization is 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.
Pulmonary hypertension is a pathophysiological condition characterized by an increase in mean pulmonary artery pressure > 25 mm Hg, leading to right ventricular heart failure as the most common cause of patient mortality.
Level of evidence (LoE) – the degree of certainty that the estimated effect of a medical intervention is true [1].
Grade of recommendation (GoR) – the degree of certainty that the effect of an intervention is reliable and that following the recommendations will bring more benefit than harm in a specific situation [1].
Echocardiography is an ultrasound imaging method aimed at studying the 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 valve stenosis (PVS) is a congenital heart defect characterized by an obstruction to blood flow between the right ventricle and the pulmonary artery at the level of the pulmonary valve.
1.2 Etiology and pathogenesis of the disease or condition (group of diseases or conditions)
Isolated pulmonary valve stenosis develops as a result of cusp fusion without impaired development of the RV outflow tract. The valve cusps are usually fused at the base and retain a minimal degree of mobility. However, more often they fuse along the entire perimeter, forming a membrane with an opening. In mild cases, the orifice diameter is greater than 1 cm; in severe cases, it is less than 3–4 mm. A bicuspid or funnel-shaped structure of the pulmonary valve is not uncommon. Such valve anatomy occurs in 20% of patients with this pathology [1–3].
Stenosis can also be caused by structural changes in the pulmonary valve cusps, namely dysplasia, in which the cusps are represented by elastic tissue with proliferation of collagen fibers and fibrous tissue [2].
Hemodynamic disturbances in pulmonary stenosis are caused by impaired blood outflow from the RV to the PA. Increased pressure in the RV is the main compensatory mechanism for hemodynamic impairment. In addition to elevated pressure, changes in the cardiac cycle structure toward a prolonged ejection period play a certain role in maintaining the required stroke volume. During systole, blood ejection reaches its maximum, but at the end of contraction, a residual blood volume remains in the ventricular cavity, as a result of which ejection continues into the second phase of systole [3–5].
The main hemodynamic characteristic of this defect is the systolic pressure gradient between the RV and the PA. Meanwhile, systolic pressure in the PA remains within normal limits in most cases.
The resulting RV hypertrophy leads to increased RV stiffness and a reduced cavity volume, causing an increase in diastolic pressure. Parallel to this increase, systolic pressure in the right atrium rises, causing RA dilatation and hypertrophy. These hemodynamic conditions can lead to dilatation of the foramen ovale, which is why a significant shunt at the atrial level, diverting part of the blood into the left heart chambers, is detected in some patients [5].
1.3 Epidemiology of the Disease or Condition
Pulmonary valve stenosis is one of the most common congenital heart defects (CHDs). Pulmonary valve stenosis is usually an isolated defect, accounting for 7 to 12% of all CHDs and 80 to 90% of all RV outflow tract stenoses. About 20% of patients with pulmonary valve stenosis have dysplastic pulmonary valve cusps. In Noonan syndrome, this feature is inherited in an autosomal dominant manner linked to chromosome 12. This group of CHDs is frequently associated with Williams and Alagille syndromes [6].
1.4 Features of Coding the Disease or Condition
Q22.1 – Congenital pulmonary valve stenosis
1.5 Classification of the disease or condition
Most existing classifications of isolated pulmonary stenosis are developed taking into account the systolic pressure in the right ventricle and the pressure gradient across the pulmonary valve, which reflect not only the degree of intracardiac hemodynamic disturbance but also correlate with the clinical manifestations of the defect. Taking this feature into account, E.W. Nugent identified the following groups:
Mild stenosis – systolic pressure gradient between the RV and the PA less than 25 mm Hg;
Moderate stenosis – systolic pressure gradient from 25 to 49 mm Hg;
Moderately severe stenosis – systolic pressure gradient from 50 to 79 mm Hg;
Severe stenosis – systolic pressure gradient greater than 80 mm Hg.
V.I. Pipiya proposed a classification by disease stage:
Stage I – mild stenosis; this stage is characterized by elevated systolic pressure in the RV up to 60 mm Hg, patients have no complaints, and ECG data show only minimal signs of RV overload;
Stage II – pronounced stenosis; systolic pressure in the RV ranges from 61 to 100 mm Hg, with a clinically prominent picture of the defect;
Stage III – severe stenosis with a pressure greater than 100 mm Hg, severe clinical course of the defect, signs of circulatory impairment;
Stage IV – stage of decompensation, where myocardial dystrophy with severe circulatory impairment is dominant, while low pressure values in the RV may be detected.
Valvar pulmonary stenosis [7-10]:
1.1. Valve dysplasia;
1.2. Unicuspid or bicuspid valve;
1.3. Funnel-shaped valve.
Pulmonary stenosis with an intact interventricular septum includes: obstruction at one of the levels along the blood flow path from the RV to the pulmonary circulation, an intact interventricular septum, normal geometric structure of the infundibulum, hypertrophy of the RV myocardium (concentric hypertrophy of the right ventricular outflow tract may develop).
1.6 Clinical presentation of the disease or condition
Valvar pulmonary stenosis is characterized by a prolonged asymptomatic course. The severity of the defect depends primarily on the degree of narrowing of the pulmonary valve annulus. In children with isolated valvar pulmonary stenosis, signs of heart failure are absent in 25% of cases [8-14].
In the presence of clinical manifestations, a characteristic complaint is dyspnea occurring upon physical exertion, and in severe cases, observed even at rest.
Marked stenosis combined with a patent foramen ovale in newborns presents with cyanosis and symptoms of circulatory failure. The condition of such patients can be extremely severe, requiring urgent surgical intervention.
Middle school-aged children frequently complain of chest pain associated with coronary circulatory insufficiency of the hypertrophied RV myocardium.
Cyanosis is not a pathognomonic sign of this stenosis, but it may be present if the foramen ovale remains patent or if pulmonary stenosis is combined with other congenital heart defects.
2. Diagnostics, medical indications and contraindications for diagnostic methods
The diagnosis of valvar pulmonary stenosis is established based on:
Mandatory:
1. Electrocardiography
2. Echocardiography
Minor:
3. Contrast-enhanced computed tomography of the heart
2.1 Complaints and Anamnesis
It is recommended to take the medical history and complaints in all patients with PS (taking medical history and complaints for heart and pericardial disorders) [3,4,15,16,23].
gOR CLOE 4Grade of Recommendation C; Level of Evidence – 4
Comments: Depending on the degree of hemodynamic impairment, significant variability in the clinical course of the defect is observed. The patient's condition depends on the severity of PS, RV hypertrophy, and their changes with age. It is necessary to clarify whether the patients have dyspnea, palpitations, decreased exercise tolerance; poor weight gain in infants and toddlers; and chest pain in older age groups: dilated pulmonary arteries in rare cases can cause retrosternal pain by compressing the left main coronary artery [3,4,15,16,23].
2.2 Physical Examination
It is recommended that all patients with suspected PS undergo an initial consultation with a pediatric cardiologist and/or cardiovascular surgeon to establish a diagnosis (initial examination (inspection, consultation) by a pediatric cardiologist and/or initial examination (inspection, consultation) by a cardiovascular surgeon) [3,4,15,16,23].
gOR CLOE 4Grade of Recommendation C; Level of Evidence – 4
In all patients with PS, it is recommended to perform a physical examination determining the shape of the chest and palpation of the heart region (visual inspection for heart and pericardial disorders; palpation for heart and pericardial disorders) [3,4,15,16,23].
gOR CLOE 4Grade of Recommendation C; Level of Evidence – 4
Comments: The formation of a precordial bulge is characteristic, developing as a result of prolonged pressure from enlarged heart chambers on the anterior chest wall. Examination of the patient reveals distension and pulsation of the neck veins. A systolic thrill is detected in the pulmonary area.
All patients are recommended to undergo cardiac auscultation (auscultation for heart and pericardial disorders) [3,4,15,16,23].
gOR CLOE 5Grade of Recommendation C; Level of Evidence – 5
Comments: auscultation in patients with PS reveals:
- a harsh systolic murmur in the II-III intercostal space, radiating toward the left clavicle and interscapular space;
- the second heart sound over the pulmonary area is often not heard or is sharply diminished;
- A soft diastolic murmur may be detected in pulmonary valve regurgitation caused by leaflet deformation.
2.3 Laboratory Diagnostics
In patients with isolated pulmonary involvement, it is non-specific.
All patients with PS are recommended to undergo a complete blood count (CBC) and urinalysis, including total hemoglobin, hematocrit, erythrocyte, leukocyte, and platelet counts, and erythrocyte sedimentation rate (ESR) as part of initial evaluation, upon hospital admission (including for surgical treatment), during follow-up at least once a year, and in the postoperative period [3,4,96,111-115,117].
gOR CLOE 5Grade of Recommendation C; Level of Evidence – 5
Comments: Performing a complete blood count in all patients with PS during dispensary follow-up and prior to surgical treatment is important for detecting anemia and providing timely correction. In patients with a mean corpuscular volume (MCV) of less than 80 fL, it is advisable to assess serum iron, ferritin, transferrin, and total iron-binding capacity (TIBC) for the timely correction of iron deficiency states. In patients with an MCV greater than 100 fL, it is advisable to determine blood vitamin B12 (cyanocobalamin) and serum folic acid levels for the timely correction of B12- or folate-deficiency states. The aforementioned conditions can negatively affect the clinical and functional status, the course of the patient's disease, and the outcomes of surgical treatment [96-98].
It is recommended to perform a general general-health blood biochemistry panel (levels of potassium, sodium, chlorides, glucose, creatinine, total protein, albumin, urea, total and conjugated bilirubin, uric acid, serum iron, and the activity of aspartate aminotransferase and alanine aminotransferase) to assess renal and hepatic function in all patients with PS as part of the initial evaluation, upon hospital admission (including for surgery), during follow-up at least once a year, and in the postoperative period [4,3,15,73,96].
gOR CLOE 5Grade of Recommendation C; Level of Evidence – 5
It is recommended to measure the N-terminal pro-brain natriuretic peptide (NT-proBNP) level in the blood of all patients with PS as part of the initial evaluation and subsequently as needed for mortality risk stratification, as well as in the event of surgical treatment when necessary in the pre- and/or postoperative period [27,96,98-105,118].
gOR CLOE 4Grade of Recommendation C; Level of Evidence – 4
It is recommended that all patients with PS admitted to the hospital for surgical treatment undergo a coagulogram (exploratory hemostasis assessment) (activated partial thromboplastin time (APTT), international normalized ratio (INR), and fibrinogen level) to rule out congenital and acquired blood coagulation disorders and to predict postoperative thrombosis and bleeding [90,106-107].
gOR CLOE 5Grade of Recommendation C; Level of Evidence – 5
Comments: in the presence of laboratory signs of hemostatic disorders, it is prudent to postpone the surgical intervention until the causes of these disorders are identified and eliminated.
It is recommended that all patients with PS admitted to the hospital for surgical treatment undergo ABO blood grouping, Rhesus factor (Rh antigen D) determination, C, c, E, e, Cw, K, k phenotype determination, and screening for red blood cell antibodies [111,112,119-120].
gOR CLOE 5Grade of Recommendation C; Level of Evidence – 5
It is recommended that all patients with PS admitted to the hospital for surgical treatment undergo testing for Hepatitis B surface antigen (HBsAg) in the blood; antibodies to Hepatitis C virus in the blood; antibodies to Treponema pallidum in the blood; IgM and IgG antibodies to Human immunodeficiency virus HIV-1 in the blood; IgM and IgG antibodies to Human immunodeficiency virus HIV-2 in the blood, to rule out co-existing HIV infection, hepatitis, and syphilis [3,4,113,121,165-168].
gOR CLOE 5Grade of Recommendation C; Level of Evidence – 5
2.4 Instrumental Diagnostics
All patients with PS are recommended to undergo electrocardiography (ECG) to assess right heart overload and hypertrophy, cardiac rhythm, and conduction [3,4,15,16,23-25].
gOR CLOE 4Grade of Recommendation C; Level of Evidence – 4
Comments: In moderate stenosis, ECG changes may be absent. With low RV/PA pressure gradients, the electrical axis of the heart remains normal, and an increased P-wave amplitude is recorded only in the right precordial leads. In patients with moderate to severe stenosis, ECG signs of RV hypertrophy are determined, sometimes combined with right atrial dilation and hypertrophy. The electrical axis of the heart deviates to the right. ST-segment depression and T-wave inversion in the right precordial leads may be observed, indicating extreme RV overload and coronary circulatory insufficiency.
All patients with PS are recommended to undergo targeted chest radiography to determine heart configuration and the state of the pulmonary circulation [3,4,15,16,19,23].
gOR CLOE 4Grade of Recommendation C; Level of Evidence – 4
Comments: The degree of cardiomegaly and the prominence of the pulmonary vascular pattern directly depend on the degree of stenosis at the pulmonary artery level and the duration of the defect [19]. The cardiac shadow is usually enlarged. In the anteroposterior view, the cardiac shadow expands to the right due to right atrial hypertrophy and dilatation, and to the left due to right ventricular hypertrophy, which displaces the left ventricle upward and posteriorly. The left cardiac contour shows a marked bulging and elongation of the pulmonary artery arch. The pulmonary vascular pattern remains unchanged. Expansion of the pulmonary artery trunk combined with a normal or diminished pulmonary vascular pattern are characteristic signs of isolated pulmonary stenosis, especially at the valve level.
All patients with PS are recommended to undergo transthoracic echocardiography (TTE) with Doppler ultrasound, which is the primary diagnostic tool, especially for detecting associated congenital heart disease such as ASD or VSD [123-127,132,138].
gOR CLOE 5Grade of Recommendation C; Level of Evidence – 5
Comments: Echocardiography (TTE) is a key diagnostic method for establishing the diagnosis of pulmonary stenosis and assessing patient severity: it provides information on localization (level of stenosis) and the severity of volume overload in the right chambers of the heart. TTE in the short-axis view at the aortic valve level (M-mode echocardiography of the pulmonary valve) or the subcostal long-axis view of the right ventricular outflow tract allows for detailed anatomy and function of the pulmonary artery. In M-mode echocardiography of the pulmonary artery, the pulmonary valve is visualized with altered cusps: thickened and/or elongated, desynchronization of cusp coaptation during systole (limited systolic motion), accompanied by post-stenotic dilation of the pulmonary artery trunk. Signs of valve dysplasia include thickened immobile cusps, a hypoplastic pulmonary valve annulus, subvalvular narrowing, and the absence of post-stenotic pulmonary artery dilation. Echocardiography differentiates classical valvular pulmonary stenosis from pulmonary valve cusp dysplasia. Color Doppler mapping reveals turbulent flow, and the severity of stenosis can be determined using continuous-wave Doppler. The degree of right ventricular hypertrophy and tricuspid regurgitation is determined in the apical 4-chamber view or the parasternal short-axis view at the aortic valve level.
It is recommended to perform contrast-enhanced cardiac computed tomography in patients with PS to clarify echocardiography findings (if necessary or if echocardiographic results are inconclusive), to diagnose associated congenital heart disease, and when planning surgical treatment [3,4,15,16,127-130,138].
gOR CLOE 5Grade of Recommendation C; Level of Evidence – 5
Comments: CT plays an increasingly important role in the diagnosis of congenital heart disease: it provides excellent spatial resolution and rapid data acquisition, allows visualization of epicardial coronary arteries and collateral arteries, and detects parenchymal lung diseases. Ventricular volumes and function can be assessed in less time compared to MRI. CT is currently more widely available than MRI and therefore plays a special role in emergency situations. Cardiac CT is an alternative to MRI in patients with implanted pacemakers or defibrillators. The high dose of ionizing radiation was previously considered the main drawback of most modern systems. However, recent developments, such as ECG synchronization and new rotational techniques, reduce radiation dose, which may make CT a more attractive imaging method in coming years [24].
It is recommended to perform magnetic resonance imaging of the heart and great vessels in patients with pulmonary stenosis to assess their structural and functional changes [127-130,138].
gOR CLOE 5Grade of Recommendation C; Level of Evidence – 5
Comments: Cardiac MRI improves imaging capabilities, especially for assessing ventricular volume overload. The method provides three-dimensional anatomical reconstructions with high spatial and temporal resolution, volumetric measurements, and evaluation of blood vessels and myocardial fibrosis.
MRI is more informative compared to echocardiography in the quantitative assessment of right ventricular volumes and ejection fraction, right ventricular outflow tract obstruction, pulmonary regurgitation, evaluation of the pulmonary arteries (stenoses, aneurysms) and aorta (aneurysm, dissection, coarctation); systemic and pulmonary veins (anomalous drainage, obstructions, etc.), collaterals and arteriovenous malformations (superior to CT capabilities), in coronary artery disease (CAD) (inferior to CT), evaluation of intra- and extracardiac masses (inferior to CT), quantitative assessment of myocardial mass (left and right ventricles), detection and quantification of myocardial fibrosis/scar (late gadolinium enhancement); and tissue characterization (fibrosis, fat, etc.) [29].
Transvenous cardiac catheterization (cardiac ventriculography) and selective pulmonary angiography are recommended for patients who are simultaneously scheduled for transcatheter valve intervention [3,4,72,131-132,138].
gOR CLOE 5Grade of Recommendation C; Level of Evidence – 5
Comments: If there are signs of elevated systolic pressure in the pulmonary artery according to echocardiography, potential additional causes, such as peripheral pulmonary artery stenoses, should be considered [12-15].
It is recommended to perform coronary angiography in patients with congenital pulmonary stenosis combined with right ventricular outflow tract obstruction to rule out coronary artery anomalies or crossing of the right ventricular outflow tract by a coronary artery [28,34].
gOR CLOE 5Grade of Recommendation C; Level of Evidence – 5
As an alternative to coronary angiography, computed tomographic coronary angiography is recommended to be considered [28].
gOR CLOE 4Grade of Recommendation C; Level of Evidence – 4
2.5 Other Procedures
Before discharge from the hospital, all patients with congenital pulmonary stenosis are recommended to undergo electrocardiogram recording as a postoperative control measure [23,122,133,136-137].
gOR CLOE 5Grade of Recommendation C; Level of Evidence – 5
Before discharge from the hospital, all patients with congenital pulmonary stenosis are recommended to undergo echocardiography as a postoperative control measure [23,134-135].
gOR CLOE 5Grade of Recommendation C; Level of Evidence – 5
3. Treatment, including drug and non-drug therapies, diet therapy, and pain management
3.1 Conservative Treatment
Supportive therapy
The management strategy for patients with this pathology is determined by the severity of the pulmonary artery stenosis. For children who respond to medical therapy, surgical intervention may be postponed.
Most asymptomatic patients do not require drug therapy.
In the presence of right ventricular outflow tract obstruction, patients are recommended to be prescribed medications that reduce heart rate and increase ventricular diastolic filling time (beta-blockers) [16,17].
gOR CLOE 4Grade of Recommendation C; Level of Evidence – 4
When signs of heart failure develop (most commonly right-sided), including in the postoperative period (especially after surgery performed under cardiopulmonary bypass), the administration of loop diuretics, thiazides, and aldosterone antagonists is recommended [19,20,139-141].
Level of Recommendation CLevel of Evidence 3Level of Recommendation C; Level of Evidence – 3
In patients with circulatory failure, it is recommended to resort to conservative therapy using diuretics, beta-blockers, and ACE inhibitors. Treatment should not be prolonged and is used prior to surgical correction of congenital heart disease. It is advisable to combine loop diuretics (furosemide** (solution for intravenous and intramuscular administration) 1-2 mg/kg/day, maximum daily dose 6 mg/kg/day (solid dosage form is off-label in children under 3 years of age)) with potassium-sparing diuretics (spironolactone** (capsules) 1-3 mg/kg/day (solid dosage form is off-label in children under 3 years of age)) [17,18,139-142].
gOR CLOE 5Grade of Recommendation C; Level of Evidence – 5
Patients at high risk of thrombosis following surgical treatment are recommended to receive thromboprophylaxis with low-molecular-weight or unfractionated heparin (ATC group B01AB) [90,107,109,110].
gOR CLOE 5Grade of Recommendation C; Level of Evidence – 5
Comments: The high-risk group for thrombosis includes patients with a history of thrombosis, thrombophilia, infectious complications, and multiple organ failure.
Thromboprophylaxis with unfractionated #sodium heparin** (contraindications: newborns, especially premature or low birth weight infants (due to the presence of benzyl alcohol in the preparation; use with caution in children under 3 years of age) should be initiated with a bolus of 75 IU/kg in children, followed by continuous intravenous infusion at a rate of: 28 IU/kg/h in children under 1 year of age, and 20 IU/kg/h in children older than 1 year. Dose adjustment is made based on laboratory test results (see section 2.3, Appendix A3) [107,109].
Thromboprophylaxis with low-molecular-weight heparins (heparin group) is calculated as follows: #enoxaparin sodium** (off-label in children under 18 years of age) in children under 2 months of age – 0.75 mg/kg, in children over 2 months of age – 0.5 mg/kg, every 12 hours subcutaneously; #dalteparin sodium (the safety and efficacy of #dalteparin sodium in children have not been established) – in children aged 1 month to 2 years, 150 IU/kg, from 2 to 8 years – 125 IU/kg, over 8 years – 100 IU/kg; in adults – 2500 IU every 12 hours subcutaneously. #Fondaparinux sodium may be used in children older than 1 year (off-label in children under 17 years of age due to the lack of efficacy and safety data) at a dose of 0.1 mg/kg, and in adults at 2.5 mg daily subcutaneously. Dose adjustment is made based on the results of laboratory tests [90, 107, 109, 110].
In all patients with pulmonary stenosis who undergo thromboprophylaxis with unfractionated or low-molecular-weight heparin (B01AB heparin group) after surgical treatment, it is recommended to perform laboratory monitoring of drug efficacy [90, 107, 109, 110].
gOR CLOE 5Grade of Recommendation C; Level of Evidence – 5
Comments: When using unfractionated heparin (#heparin sodium**; contraindications: newborns, especially premature infants or those with low body weight due to the presence of benzyl alcohol in the preparation; use with caution in children under 3 years of age), it is necessary to determine the aPTT (activated partial thromboplastin time) (optimal range in children is 60–85 seconds) and study the heparin level in the blood (anti-Xa activity) (optimal range is 0.35–0.7). Until the optimal range is reached, these parameters are determined 4 hours after each change in the infusion rate. Once the optimal range is achieved, monitoring is performed every 12 hours (see Appendix A3). When using low-molecular-weight heparin preparations, it is necessary to study the blood heparin level (determination of anti-Xa activity) (optimal range is 0.5–1.0). Until the optimal range is reached, it is determined 3–4 hours after each injection; once the optimal range is achieved and the patient's clinical and laboratory condition is stable, monitoring is not required [90, 107, 109, 110].
When following up patients after surgical treatment of infundibular pulmonary stenosis with reconstruction of the right ventricular outflow tract, it is recommended to provide infective endocarditis prophylaxis for 6 months [91].
gOR CLOE 5Grade of Recommendation C; Level of Evidence – 5
Comments: For any type of congenital heart defect where synthetic materials/prostheses were used during correction (mechanical bileaflet aortic valve prosthesis***, biological pulmonary valve prosthesis***, cardiovascular synthetic patch***), bacterial endocarditis prophylaxis is indicated for 6 months after surgery or lifelong if residual shunts or valvular regurgitation persist. Endocarditis prophylaxis is performed during dental procedures accompanied by damage to the oral mucosa (tooth extraction, manipulation in the periapical zone of the tooth, etc.). Infective endocarditis prophylaxis consists of oral administration of amoxicillin** (dispersible tablets) 30–60 minutes before the procedure at a dose of 50 mg/kg/day divided into 2–3 doses (in case of allergy to #ampicillin** [contraindicated in children under 1 month], clindamycin** [intravenous and intramuscular solution] is used at a dose of 20 mg/kg) [36].
3.2 Surgical Treatment
Surgical treatment (transluminal balloon pulmonary valvuloplasty, pulmonary valve repair under cardiopulmonary bypass, or pulmonary valve replacement under cardiopulmonary bypass) is recommended for patients with pulmonary valve stenosis when the systolic pressure gradient between the right ventricle and the pulmonary artery is equal to or greater than 50 mm Hg [4, 15].
gOR CLOE 5Grade of Recommendation C; Level of Evidence – 5
Comments: in the neonatal period, indications for defect correction are primarily determined by the patient's clinical condition and the severity of pulmonary stenosis [4, 15, 60].
In the neonatal period, in the absence of normal recovery of right ventricular compliance after previous surgical treatment, it is recommended to create a systemic-to-pulmonary shunt (anastomosis between the subclavian artery and the pulmonary artery) [15, 33].
gOR CLOE 4Grade of Recommendation C; Level of Evidence – 4
In the presence of concomitant hypoplasia of the right ventricle and tricuspid valve, it is recommended that patients undergo one-and-a-half ventricular repair or the Fontan procedure (creation of a cavopulmonary anastomosis) at an age older than one year [63, 64].
gOR CLOE 5Grade of Recommendation C; Level of Evidence – 5
3.3 Endovascular interventions
As a first stage, all patients with pulmonary stenosis, in the absence of contraindications, are recommended to undergo transluminal balloon pulmonary valvuloplasty to reduce the pressure gradient between the right ventricle and the pulmonary artery [6-7, 62, 68-71, 73-82].
gOR CLOE 4Grade of Recommendation C; Level of Evidence – 4
Comments: Contraindications for transluminal balloon valvuloplasty include: hypoplasia of the pulmonary artery annulus (Z-score less than –3), unicuspid pulmonary valve, infundibular pulmonary stenosis. In critical pulmonary stenosis, balloon valvuloplasty is performed during continuous intravenous infusion of prostaglandins to improve right ventricular compliance [10, 12, 44].
3.3 Other Treatment
No.
4. Medical Rehabilitation and Sanatorium-Resort Treatment
It is recommended that patients with pulmonary stenosis adopt lifestyle modifications to increase physical activity for the primary prevention of cardiovascular risk factors (hypertension, obesity, diabetes mellitus), as well as for integration into normal social life, except for children with other risk factors (presence of a pacemaker [dual-chamber rate-responsive implantable pacemaker***], cardioverter-defibrillator [dual-chamber implantable cardioverter-defibrillator***; triple-chamber implantable cardioverter-defibrillator***], channelopathies, etc.) [143-152].
gOR CLOE 5Grade of Recommendation C; Level of Evidence – 5
Comment: Thanks to the introduction of advanced surgical and interventional techniques, including perioperative intensive care, the survival of children with congenital heart disease has noticeably improved over recent decades, leading to an increase in the number of patients reaching adulthood [144]. As patients with congenital heart disease grow older, their heart health can be additionally affected by acquired cardiovascular risk factors (e.g., hypertension, obesity, diabetes) commonly seen in the general population, which increases the risk of metabolic diseases, stroke, and coronary artery disease [145, 146]. The development of atherosclerotic and metabolic diseases manifesting in adulthood is known to typically begin as early as early childhood. Childhood obesity and a sedentary lifestyle are known to be the major contributing factors [147]. This emphasizes the need for primary prevention; therefore, lifestyle interventions are required to increase physical activity (PA) in children with congenital heart disease, as physical activity is indispensable for the physical, emotional, and psychosocial development of children [148–152].
Children with mild pulmonary stenosis are recommended to engage in daily physical activity as defined by the WHO at the level of > 60 minutes, without restrictions, as well as participate in all types of recreational/competitive sports with mandatory assessment of pulmonary stenosis severity at least once a year [154-155].
gOR CLOE 5Grade of Recommendation C; Level of Evidence – 5
Children with moderate pulmonary stenosis are recommended to engage in daily physical activity of moderate and low intensity, as well as dynamic and static sports of low and moderate intensity, excluding competitive sports, provided that exercise testing demonstrates satisfactory exercise tolerance [154-155].
gOR CLOE 5Grade of Recommendation C; Level of Evidence – 5
Children with severe pulmonary stenosis are recommended to engage only in low-intensity dynamic and static sports and physical activity depending on symptoms and exercise test results, excluding competitive sports [154].
gOR CLOE 5Grade of Recommendation C; Level of Evidence – 5
It is recommended that within 6 months after surgical correction of pulmonary stenosis under cardiopulmonary bypass (pulmonary valve repair under cardiopulmonary bypass, pulmonary valve replacement under cardiopulmonary bypass), a patient with a complicated postoperative course (heart failure, infectious complications, repeated surgical interventions during a single hospitalization) be referred to specialized cardiological sanatoriums and children's rehabilitation centers for medical rehabilitation, including climatotherapy, balneotherapy, physical therapy, and kinesitherapy methods [87, 156].
gOR CLOE 5Grade of Recommendation C; Level of Evidence – 5
Comments: The following conditions may occur after pulmonary stenosis correction:
- Residual hemodynamically significant gradient across the pulmonary valve;
- Recurrence of subvalvular obstruction;
- Development and/or progression of pulmonary regurgitation due to the natural history of the defect or after the initial intervention;
- Development of prosthesis-related complications (endocarditis, thrombosis, systemic embolism, periprosthetic regurgitation, or pannus-related obstruction) following mechanical valve replacement (pulmonary valve bioprosthesis ***);
- Development of cardiac arrhythmias, in some cases leading to sudden cardiac death [65,157-161].
It is recommended that physical activity be restricted for all patients for three months following hospital discharge after surgical treatment of the defect under cardiopulmonary bypass (pulmonary valve repair under cardiopulmonary bypass, pulmonary valve replacement under cardiopulmonary bypass) [156].
gOR CLOE 5Grade of Recommendation C; Level of Evidence – 5
Comment: intense physical exertion and competitive sports are prohibited for 6 months after surgical treatment under cardiopulmonary bypass (pulmonary valve repair under cardiopulmonary bypass, pulmonary valve replacement under cardiopulmonary bypass). Further restriction of activity depends on the severity of residual hemodynamic disorders. Physical activity restrictions persist for more than 6 months in cases of: 1) Clinically significant residual right ventricular outflow tract obstruction (peak LV/Ao gradient greater than 50 mm Hg); 2) Marked right ventricular myocardial hypertrophy; 3) Clinically significant ventricular or supraventricular arrhythmias; 4) More than moderate pulmonary regurgitation [86].
It is recommended to include children with congenital pulmonary stenosis after surgical treatment in cardiac rehabilitation programs incorporating aerobic and resistance training [155-156,162].
GCR CLOE 5 Grade of Recommendations C; Level of Evidence – 5
It is recommended to refer children with congenital pulmonary stenosis without hemodynamic impairment and without prognostically significant rhythm and conduction disorders for sanatorium-resort treatment [163-164].
(Level of Evidence C, Grade of Recommendation 5)
Comment: climatic and balneological resorts provide comprehensive sanatorium-resort treatment including: heliotherapy (A20.30.021), climate therapy (A20.30.012), dry-air baths (A20.30.022), local (2-4 chamber) therapeutic baths (A20.30.009).
5. Prevention and Dispensary Observation
The prognosis following surgical or endovascular correction of pulmonary stenosis is favorable, provided the congenital heart defect is corrected in a timely manner. Life expectancy and physical performance can be fully restored. In patients with unoperated, hemodynamically insignificant stenosis, the prognosis for life is favorable [15,20].
It is recommended that all patients with pulmonary stenosis undergo follow-up monitoring (dispensary follow-up visit (examination, consultation) by a pediatric cardiologist) [28,60].
gOR CLOE 4Grade of Recommendation C; Level of Evidence – 4
Comments: The frequency of follow-up visits with a pediatric cardiologist/pediatrician is at one month, 3, 6, and 12 months post-surgery.
Subsequent follow-up of postoperative or non-operated patients is carried out at intervals of 3-36 months [28,60].
Following surgery (open/endovascular), patients are recommended to remain under dispensary observation for one year, and subsequently as clinically indicated. Patients with right ventricular dysfunction, a residual gradient, or pulmonary arterial hypertension should be monitored annually in specialized centers. In patients with a small congenital or residual pressure gradient between the pulmonary artery and the right ventricle with preserved right ventricular function and the absence of associated congenital heart defects or other lesions, follow-up visits at intervals of 3-5 years may be considered. Following endovascular intervention, regular monitoring is required during the first 2 years, and subsequently every 2-4 years depending on the outcome.
To determine the frequency and scope of dispensary follow-up (dispensary visit (examination, consultation) by a pediatric cardiologist) for patients with pulmonary stenosis (both operated and non-operated), it is recommended to be guided by the presence of heart failure symptoms (heart failure functional class), hemodynamic status, presence of cardiac arrhythmias, hypoxemia, etc. (Appendix A4) [28,60].
gOR CLOE 4Grade of Recommendation C; Level of Evidence – 4
It is recommended to perform echocardiography in all patients with pulmonary stenosis during dispensary follow-up visits (dispensary visit (examination, consultation) by a pediatric cardiologist) [23,87,122,133-137].
gOR CLOE 5Grade of Recommendation C; Level of Evidence – 5
It is recommended to perform an ECG in all patients during dispensary follow-up visits (dispensary visit (examination, consultation) by a pediatric cardiologist) [23,87,89,122,133,136-137].
gOR CLOE 5Grade of Recommendation C; Level of Evidence – 5
During dispensary follow-up (dispensary visit (examination, consultation) by a pediatric cardiologist) for all patients with pulmonary stenosis, it is recommended to perform graded exercise stress tests (bicycle ergometry; exercise electrocardiography; exercise echocardiography; single-stage varying-intensity exercise test) and pulse oximetry with a frequency corresponding to their risk group classification (Appendix A4) [28,60].
gOR CLOE 5Grade of Recommendation C; Level of Evidence – 5
6. Organization of Medical Care
Indications for hospital admission to a medical organization:
1) presence of symptoms of heart failure;
2) presence of pulmonary hypertension;
3) elective surgical treatment.
Indications for emergency hospitalization:
1) deterioration of the patient's functional status due to the progression of circulatory insufficiency symptoms or cardiac arrhythmias;
2) pulmonary hypertension requiring therapy selection/adjustment.
Indications for discharging a patient from a medical organization:
1) absence of a significant gradient between the right ventricle and pulmonary artery;
2) absence/compensation of symptoms of circulatory insufficiency.
7. Additional Information
Pregnancy and childbirth
Pregnancy and childbirth are generally well tolerated [93]:
- against the background of corrected pulmonary stenosis;
- in patients with preserved right ventricular function;
- with I-II functional classes according to NYHA;
- in the absence of significant pulmonary hypertension and significant right ventricular outflow tract obstruction;
- in uncorrected stenosis with a pressure gradient of less than 30 mm Hg.
In the presence of cyanosis and pulmonary hypertension, pregnancy is contraindicated for patients with pulmonary stenosis. If pregnancy occurs in this patient group, artificial termination of pregnancy (abortion) is optimal [93-94].
Criteria for Evaluating the Quality of Medical Care
Quality criteria for primary health care provided to children with congenital pulmonary valve stenosis (ICD-10 codes: Q22.1)
No. | Quality assessment criteria | Evaluation of compliance |
|---|---|---|
1. | Initial examination (assessment, consultation) by a pediatric cardiologist and/or initial cardiovascular surgeon (upon diagnosis establishment) | Yes/No |
2. | A dispensary appointment (examination, consultation) by a cardiologist or a dispensary appointment (examination, consultation) by a pediatric cardiologist was performed | Yes/No |
3. | Electrocardiography performed (upon establishing the diagnosis and/or during follow-up) | Yes/No |
4. | Targeted chest X-ray performed (upon diagnosis establishment and when planning surgical treatment) | Yes/No |
5. | Echocardiography performed (at diagnosis establishment and/or during dispensary follow-up) | Yes/No |
Quality criteria for specialized medical care provided to children with congenital pulmonary valve stenosis (ICD-10 codes: Q22.1)
No. | Quality assessment criteria | Evaluation of compliance |
|---|---|---|
1. | Electrocardiography performed (when planning surgical treatment and upon discharge from the hospital) | Yes/No |
2. | Targeted chest X-ray performed (upon diagnosis establishment and when planning surgical treatment) | Yes/No |
3. | Echocardiography was performed (when planning surgical treatment and upon discharge from the hospital) | Yes/No |
4. | Computed tomography of the heart with contrast enhancement and/or magnetic resonance imaging of the heart and great vessels performed (in case of insufficient diagnostic value of echocardiography data, and in the absence of medical contraindications) | Yes/No |
5. | Surgical treatment performed (for severe and critical pulmonary valve stenosis) (provided the patient is a candidate for surgery) | Yes/No |
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Appendix A1. Working Group Composition
Avramenko A.A., "Association of Cardiovascular Surgeons of Russia" (Samara)
Belov V.A., "Association of Cardiovascular Surgeons of Russia" (Perm)
Bogdanov V.N., "Association of Cardiovascular Surgeons of Russia" (Chelyabinsk)
Bokeria L.A., Academician of the RAS, "Association of Cardiovascular Surgeons of Russia", (Moscow)
Boriskov M.V., MD, "Association of Cardiovascular Surgeons of Russia" (Krasnodar)
Borisova N.A., "Russian Scientific Society of Endovascular Diagnostics and Treatment Specialists", (Saint Petersburg)
Brodsky A.G., PhD, "Association of Cardiovascular Surgeons of Russia" (Surgut)
Volkov S.S., PhD, "Association of Cardiovascular Surgeons of Russia" (Moscow)
Gavrilov R.Yu., "Association of Cardiovascular Surgeons of Russia", (Volgograd)
Gorbatikov K.V., MD, "Association of Cardiovascular Surgeons of Russia" (Tyumen)
Gorbatykh Yu.N., MD, "Association of Cardiovascular Surgeons of Russia" (Novosibirsk)
Evtushenko A.V., Russian Society of Cardiology, (Kemerovo)
Zelenikin M.A., DSc in Medical Sciences, "Association of Cardiovascular Surgeons of Russia", (Moscow)
Zelenikin M.M., DSc in Medical Sciences, "Association of Cardiovascular Surgeons of Russia", (Moscow)
Ivanov S.N., MD, Russian Society of Cardiology, (Novosibirsk)
Kalashnikov S.V., PhD, "Association of Cardiovascular Surgeons of Russia" (Moscow)
Kim A.I., DSc in Medical Sciences, "Association of Cardiovascular Surgeons of Russia", (Moscow)
Kovalev I.A., DSc in Medical Sciences, "Association of Pediatric Cardiologists of Russia", (Moscow)
Komissarov M.I., (Saint Petersburg)
Krivoshchekov E.V., MD, "Association of Cardiovascular Surgeons of Russia" (Tomsk)
Kupryashov A.A., MD, "Association of Cardiovascular Surgeons of Russia" (Moscow)
Levchenko E.G., MD (Moscow)
Martynyuk T.V., Russian Society of Cardiology, (Moscow)
Moiseeva O.M., MD, Russian Society of Cardiology, (Saint Petersburg)
Nalimov K.A., "Association of Cardiovascular Surgeons of Russia" (Khabarovsk)
Petrushenko D.Yu., "Association of Cardiovascular Surgeons of Russia" (Kazan)
Plotnikov M.V., PhD, "Association of Cardiovascular Surgeons of Russia" (Astrakhan)
Pursanov M.G., MD (Moscow)
Svobodov A.A., MD, "Association of Cardiovascular Surgeons of Russia" (Moscow)
Sinelnikov Yu.S., Doctor of Medical Sciences, (Perm)
Teplov P.V., "Association of Cardiovascular Surgeons of Russia" (Krasnoyarsk)
Trunina I.I., Doctor of Medical Sciences, "Association of Pediatric Cardiologists of Russia", (Moscow)
Yakovleva A.N., (Saint Petersburg)
There is no conflict of interest.
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 for these clinical guidelines:
Pediatric Cardiologist
Cardiologist
Cardiovascular Surgeon
Surgeon
Ultrasound Diagnostics Physician
Pediatrician
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 IIa IIb | Conflicting evidence and/or a divergence of opinion about the usefulness/efficacy of the given procedure, intervention, treatment Weight of evidence/opinion is in favor of usefulness/efficacy Usefulness/efficacy is less well established by evidence/opinion | It is advisable to use May be considered |
III | Evidence or general agreement that the given procedure, intervention, treatment is not useful/effective, 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 opinion of the experts and/ or small studies, retrospective studies, registries |
Table 3. Scale for assessing the levels of evidence reliability (LER) for diagnostic methods (diagnostic interventions).
LoE | 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 reference method control, or studies with a reference method that is not independent of the studied 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).
LoE | 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 | Only justification of the mechanism of action of the intervention (preclinical studies) or expert opinion is available |
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 on 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
Federal Law No. 323-FZ of November 21, 2011 (as amended on December 28, 2024) "On the Fundamentals of Protecting the Health of Citizens in the Russian Federation" (with rev. and add., effective March 1, 2025)
Order of the Russian Ministry of Health No. 918n of November 15, 2012 (as amended on February 21, 2020) "On Approval of the Procedure for Providing Medical Care to Patients with Cardiovascular Diseases"
Monitoring and dose adjustment of unfractionated heparin (#sodium heparin**) [107]Appendix A4. Classification of patients into groups based on symptoms. Frequency of clinical follow-up for patients.
Depending on symptoms, patients with congenital heart disease (CHD) can be divided into four groups (A, B, C, D):
Group A
- heart failure functional class I;
- absence of anatomical hemodynamic abnormalities;
- absence of cardiac arrhythmias;
- normal liver, kidney, and lung function.
Group B
- heart failure functional class II;
- minimal stenosis and/or valve regurgitation (grade I);
- mild aortic or ventricular dilation;
- LVEF at least 50%, right ventricular ejection fraction at least 40%;
- hemodynamically insignificant intracardiac shunts (Qp/Qs <1.5);
- absence of hypoxemia;
- cardiac arrhythmias not requiring treatment;
- absence of signs of pulmonary hypertension;
- normal renal and hepatic function.
Group C
- heart failure functional class III;
- moderate or significant stenosis and/or valve regurgitation (grades II–III), arterial or venous stenoses, moderate aortic or ventricular dilation;
- LVEF 40–49%, RV EF 35–39%;
- hemodynamically significant intracardiac shunts (Qp/Qs ≥1.5);
- mildly expressed hypoxemia (SatpO2 ≥85%);
- cardiac arrhythmias controlled by therapy;
- pulmonary hypertension (pulmonary hypertension functional class I–II);
- internal organ dysfunction controlled by therapy.
Group D
- heart failure functional class IV;
- significant aortic dilation;
- pronounced hypoxemia (SatpO2 <85%);
- cardiac arrhythmias refractory to therapy;
- pulmonary hypertension (pulmonary hypertension functional class III–IV);
- internal organ dysfunction refractory to ongoing therapy.
Depending on whether a patient with corrected or uncorrected pulmonary artery (PA) stenosis belongs to a particular group, different observation frequencies and scopes of required examinations are recommended:
Table 1. Frequency of patient monitoring by group.
Observation frequency (months) / diagnostic methods | Group A | Group B | Group C | Group D |
|---|---|---|---|---|
Examination by a pediatric cardiologist / cardiologist | 36 | 12 | 6 | 3 |
Electrocardiography | 36 | 12 | 6-12 | 3-6 |
Echocardiography | 36 | 12 | 6-12 | 3-6 |
Pulse oximetry | as needed | as needed | 6 | 3 |
Exercise testing (6-minute walk test [6MWT] [performing an exercise test using an ergometer], cardiopulmonary exercise testing [cardiopulmonary exercise testing with ergospirometry] if indicated) | 36 | 24 | 12-24 | 6-12 |
Appendix B. Physician Action Algorithms
Appendix C. Patient Information
Pulmonary valve stenosis is a relatively common congenital heart defect. The presence of an obstruction to blood flow (stenosis) from the right ventricle into the pulmonary artery may be the sole developmental anomaly of the heart or part of a more complex defect.
These structures begin their intrauterine formation in weeks 4–5. For various reasons during intrauterine development, when the single vessel divides into the pulmonary artery and aorta, abnormal development of the pulmonary valve may occur. Shortly after birth and the establishment of normal circulation in both circulatory loops, a pressure gradient arises between the right ventricle and the pulmonary artery. Consequently, when blood is pumped from the right ventricle into the pulmonary artery, the blood flow encounters an obstruction in the form of stenosis, leading to an insufficient volume of blood entering the pulmonary artery (the patient's lungs). That is, with each heart contraction, a portion of the blood remains in the right ventricle. In this situation, the right ventricle is forced to work with increased workload to overcome the barrier to blood flow into the pulmonary artery.
Valvular pulmonary stenosis may be asymptomatic. In most cases, approximately 55–65%, mild stenoses do not require surgical treatment. However, if the child has reached school age while remaining asymptomatic, surgery may still be offered. The fact is that if the child contracts a childhood infection or even undergoes simple dental extraction in the presence of altered pulmonary valve leaflets, endocarditis—an inflammatory process of the inner lining of the heart chambers—may develop. Although this probability is very low (only 1–2% of cases), it exists [95].
Moderate and severe stenoses represent a completely different and much more dangerous situation. In severe cases, not only the heart but also the pulmonary vessels suffer: the right ventricle becomes overloaded with an increased volume of excess blood, while an insufficient amount of blood reaches the pulmonary artery for systemic oxygenation. The most crucial indicators of this progression are the pressure gradient between the pulmonary artery and the right ventricle. Ultrasound examination (echocardiography) provides these data today. Numerous compensatory mechanisms are activated: the muscle mass of the right ventricle increases, and the pulmonary vessels adapt as well [95].
What should you pay attention to in order to avoid such developments in time?
The main indicator in the neonatal period is weight gain. In older children, pronounced symptoms may be absent against this background. The main method for diagnosing this condition is timely routine screening (dispensary examination) involving cardiac auscultation, as well as ultrasound examination if congenital heart disease is suspected.
With drug therapy, symptoms may disappear or significantly decrease. However, if nothing changes, if heart dimensions increase, and the degree of stenosis progresses, it is necessary to consult surgeons.
The best surgical results are achieved after correcting congenital heart disease before the age of two to two and a half years, when the child shows signs of heart failure. At that stage, all processes are still reversible. The heart rapidly decreases in size [95].
Appendix G. Assessment Scales, Questionnaires, and Other Evaluation Tools
None provided.