List of Abbreviations
CHD — congenital heart disease
ASD – atrial septal defect
ЕОК – European Society of Cardiology
IHD — ischemic heart disease
CT — computed tomography
PH – pulmonary hypertension
LA — left atrium
MRI — magnetic resonance imaging
PVR – pulmonary vascular resistance
ICU – intensive care unit
RV — right ventricle
RA — right atrium
ECG — electrocardiography
EchoCG — echocardiography
TEE – transesophageal echocardiography
Qp/Qs – pulmonary-to-systemic blood flow ratio
** – the drug is included in the list of vital and essential drugs
*** – medical device implanted when providing medical care under the state guarantee program for free medical care to citizens
# - prescription of a medicinal product for indications not approved by state regulatory authorities, not mentioned in the instructions for use.
Terms and Definitions
ESC – European Society of Cardiology. The text of these clinical guidelines additionally indicates the indications for statements in accordance with the recommendations of the European Society of Cardiology (ESC). In accordance with the international document, classes of recommendations and levels of evidence are distinguished.
Outcome – any possible result arising from the exposure to a causal factor, preventive or therapeutic intervention, or any established changes in health status occurring as a consequence of an intervention.
Laboratory diagnostics — a set of methods aimed at analyzing test material using various specialized equipment.
Cardiomegaly – enlargement of the heart caused by hypertrophy of the heart muscle volume or dilation of the heart chambers.
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.
Computed tomography (CT) is a non-invasive diagnostic imaging method that produces a series of submillimeter cross-sectional axial images of human body organs and structures. It is based on the measurement and complex computer processing of differences in X-ray attenuation by tissues of varying density. Various contrast-enhancement techniques using iodine-based radiopaque agents are employed to improve the differentiation of organs from one another, as well as normal and pathological structures. To visualize the structures of the cardiovascular system, CT angiography is used—a technique involving the intravenous bolus injection of a radiopaque agent into the bloodstream using an automated contrast injection system at a predetermined flow rate and injection time. Based on the acquired data and through computer post-processing with 3D reconstruction, a three-dimensional model of the heart and blood vessels is constructed.
Pulmonary hypertension – a pathophysiological condition characterized by an increase in mean pulmonary artery pressure > 25 mm Hg, with the development of right ventricular heart failure as the most frequent cause of patient death.
Magnetic resonance imaging (MRI) is a non-invasive, non-ionizing computerized method for producing tomographic medical images to study internal organs and tissues. It is based on the phenomenon of nuclear magnetic resonance and the measurement of the electromagnetic response of atomic nuclei placed in a strong constant magnetic field in response to excitation by a specific combination of electromagnetic waves; these nuclei are hydrogen atoms present in the human body as part of water and other substances. MR angiography is a method for imaging the lumen of blood vessels using a magnetic resonance scanner. The method makes it possible to evaluate both the anatomical and functional features of blood flow. MR angiography is based on the difference in signal between moving protons (blood) and surrounding stationary tissues, which allows for vascular imaging without the use of any contrast agents (non-contrast magnetic resonance angiography). In certain cases, intravenous bolus contrast enhancement using paramagnetic contrast agents (gadolinium) is applied.
Working group for the development/updating of clinical guidelines – a team of specialists working together and in coordination to develop/update clinical guidelines and bearing joint responsibility for the results of this work.
Statement-recommendation – a provision reflecting the procedure and correctness of performing a particular medical intervention with proven efficacy and safety.
Level of Evidence (LoE) – the degree of certainty that the found effect of a medical intervention is true.
Class of Recommendation (CoR) – the degree of certainty in the reliability of the intervention effect and in the fact that following the recommendations will bring more benefit than harm in a specific situation.
Echocardiography – a method of ultrasound examination of the heart aimed at studying its morphology and function.
1. Brief information on the disease or condition
1.1 Definition of the disease or condition
Atrial septal defect (ASD) – a congenital heart defect (excluding patent foramen ovale) that reflects abnormalities in the development of the primary and secondary interatrial septa, characterized by the presence of an opening between the right and left atria and causing blood shunting between them.
1.2 Etiology and pathogenesis of the disease or condition (group of diseases or conditions)
The formation of the defect is associated with underdevelopment of the primary and secondary interatrial septa and endocardial cushions during the embryonic period. Genetic, physical, environmental, and infectious factors lead to organogenesis disorders. ASD is characterized by pathological blood flow from the LA to the RA. Factors determining the left-to-right blood shunt include the greater 'compliance' of the RV, the anatomical location of the atria, and, albeit slightly, higher pressure in the LA. The volume of the pathological shunt depends on RV 'compliance' and the size of the defect. RV dilation due to its volume overload may be accompanied by the development of tricuspid regurgitation. Constant left-to-right blood shunting leads to pulmonary hypervolemia and PH. Spontaneous closure of a secundum ASD with a diameter of 4–5 mm is observed in 56% of patients, 6–7 mm in 30%, and 8–10 mm in 12%. The size of a secundum ASD can change with age. In 70% of patients, a secundum ASD ≤4 mm decreases in size, in 12% it remains unchanged, and in 18% it increases. If a secundum ASD is >8–12 mm, it decreases in 9% of patients, remains unchanged in 15%, and increases in 76%. Mortality increases in the second half of life: from 0.6–0.7% in 20-year-olds and 4.5% in 40-year-olds to 7.5% in 60-year-olds [40]. Atrial arrhythmias: atrial flutter, atrial fibrillation, sick sinus syndrome are the result of prolonged volume overload of the right heart chambers and usually occur in adulthood [41]. Pulmonary arterial hypertension mainly develops in the 3rd–4th decade of life, and its prevalence among adult patients with ASD ranges from 8 to 10% [42]. Paradoxical embolism, the source of which can be peripheral venous thrombosis, atrial arrhythmias, intravenous infusions of unfiltered solutions, or indwelling venous catheters, is a risk factor in all defects regardless of size [10].
1.3 Epidemiology of the Disease or Condition
ASD accounts for 7.1–8.7% of all congenital heart defects [2]. The incidence of ASD ranges from 0.317 to 0.941 cases per 1,000 live births, depending on the population, diagnostic methods, and timing of epidemiological studies [2,3]. ASD is more common in females (male-to-female ratio = 1:2). In 30–50% of children with congenital heart disease, ASD is part of a combined defect [1].
1.4 Features of Coding the Disease or Condition
Q21.1 – Atrial septal defect.
1.5 Classification of the disease or condition
Classification of ASD [3,4]:
primum atrial septal defect (15% of all ASDs);
secundum atrial septal defect (80% of all ASDs);
common atrium;
sinus venosus defect:
• superior;
• inferior.
coronary sinus defect (unroofed coronary sinus):
• proximal;
• middle;
• distal.
Comments: Coronary sinus defect (defect of the coronary sinus roof, which is not anatomically an ASD) may be accompanied by partial or total anomalous pulmonary venous drainage and/or drainage of a persistent left superior vena cava into the coronary sinus or left atrium.
1.6 Clinical presentation of the disease or condition
Defects of this type have a sparse clinical presentation and are often asymptomatic. Some patients show minimal signs of circulatory failure (fatigue and exertional dyspnea, increased sweating), moderate physical growth retardation (weight and height parameters are below the 10th percentile on the distribution chart for age and gender), and a tendency to recurrent respiratory infections. Symptom severity increases in the 3rd–4th decade of life. Cyanosis may be observed in cases of venoarterial shunting with the development of severe pulmonary hypertension. The presence of cardiac rhythm disturbances – extrasystoles, supraventricular tachycardia, atrial flutter/fibrillation – may be accompanied by complaints of skipped heartbeats and palpitations of varying clinical severity.
2. Diagnostics, medical indications and contraindications for diagnostic methods
Atrial septal defect is suspected during a cardiac examination, chest radiography, and electrocardiography, and is confirmed by transthoracic echocardiography using color Doppler flow imaging.
2.1 Complaints and Anamnesis
When taking a history regarding suspected ASD, it is recommended to clarify information from parents about dyspnea and fatigue occurring in the child after physical exertion, poor weight gain, and frequent respiratory infections to verify the diagnosis [1,3–8].
ESC: no (CoR C, LoE 5)
Comments: In childhood, patients often lack clinical manifestations. Dyspnea and tachycardia are early symptoms of the disease in children with large ASDs, but hemodynamic compensation and regression of the clinical picture usually occur during the first months of life. Heart failure symptoms are minor and may be limited to mild weakness, sweating in the child, and pallor of the skin. In most adult patients, symptoms usually appear before the fourth decade of life: decreased exercise tolerance, exertional dyspnea, and palpitations (supraventricular tachyarrhythmias), and less commonly – pulmonary infections and right-sided heart failure.
When taking the history and anamnesis in suspected ASD, it is recommended to obtain information regarding cardiac arrhythmias and paradoxical embolisms in adult patients to verify the diagnosis [1,3–9].
ESC: no (CoR C, LoE 5)
2.2 Physical Examination
In all patients with suspected ASD, it is recommended to perform cardiac auscultation to diagnose congenital heart disease [1,3–5,7,8].
ESC: no (CoR C, LoE 5)
Comments: Clinical diagnosis of the defect in typical cases is fairly simple and based on a specific auscultatory picture. Cardiac auscultation reveals a systolic murmur of grade 2–3/6 intensity in the second intercostal space to the left of the sternum (projection of the pulmonary valve), fixed splitting of the second sound over the pulmonary artery projection, and a diastolic murmur over the tricuspid valve projection in cases of a significant volume of left-to-right blood shunting. In the event of severe pulmonary hypertension, an accentuated second sound and a diastolic murmur over the pulmonary valve projection are heard.
In adult patients with suspected ASD, it is recommended to perform pulse oximetry at rest and during physical exertion to assess the direction of the systemic-pulmonary shunt [8].
ESC: no (CoR C, LoE 5)
2.3 Laboratory Diagnostics
In patients with uncomplicated ASD, it is non-specific.
It is recommended that all patients with ASD admitted to the hospital for surgical treatment undergo an assessment of acid-base balance and blood gases (pH, BE, pCO2, PO2, Lac - capillary/arterial/venous blood gas analysis) to evaluate the severity of hypoxemia and the degree of metabolic disorders; coagulation testing (screening hemostasis panel: APTT, PT, INR, D-dimer, fibrinogen, antithrombin); international normalized ratio (INR) determination to predict the risk of perioperative bleeding and blood loss volume; ABO blood group and Rhesus D antigen (Rh factor) typing along with C, c, E, e, Cw, K, k phenotype determination, as well as screening for red blood cell antibodies; testing for hepatitis B surface antigen (HBsAg) in the blood; testing for antibodies to hepatitis C virus (HCV) in the blood; testing for antibodies to *Treponema pallidum* in the blood; testing for human immunodeficiency virus HIV-1 IgM and IgG antibodies in the blood; testing for human immunodeficiency virus HIV-2 IgM and IgG antibodies in the blood to rule out associated HIV infection and hepatitis [5,7,30].
No ESC guideline (LoE C, GoR 5)
2.4 Instrumental Diagnostics
In all patients with suspected ASD, it is recommended to perform an electrocardiogram (ECG) to detect right heart strain, evaluate cardiac rhythm and conduction [1,3-5,12].
No ESC guideline (LoE C, GoR 5)
Comments: The ECG often reveals right axis deviation, signs of right atrial (RA) enlargement, incomplete or complete right bundle branch block, and an abnormal P wave axis (sinus venosus defect). ECG signs of accessory atrioventricular pathways and atrioventricular conduction disturbances may be present.
It is recommended that all patients with suspected ASD undergo transthoracic echocardiography (TTE) using color Doppler flow imaging, which is the primary diagnostic tool for establishing the diagnosis of ASD, determining its size, location, volume, and direction of blood shunting [1,3-5,7,8].
No ESC guideline (LoE C, GoR 5)
Comments: In patients with ASD, the maximum size (width) of the defect is measured using color Doppler flow imaging in two mutually perpendicular directions. The presence, number, and direction of blood shunts across the atrial septum (AS) are determined. Left-to-right shunting is typically observed, but it can be bidirectional in the presence of severe pulmonary hypertension or severe tricuspid regurgitation.
Secondary ASD should be distinguished from a patent foramen ovale. In the case of a patent foramen ovale, the septum primum flap is visualized adjacent to the defect, and the color Doppler shunt flow has an "oblique" trajectory.
The hemodynamic significance of the shunt in ASD is assessed using cardiac morphometry, jet diameter, and the pulmonary-to-systemic flow ratio (Qp/Qs). Measuring the Qp/Qs ratio is not meaningful in the presence of high pulmonary vascular resistance (e.g., newborns) or severe pulmonary hypertension.
When selecting patients for endovascular closure, the type of ASD should be determined—it must be secondary—and the rims must be measured: aortic rim (distance between the defect edge and the aorta); atrioventricular valve rim; superior vena cava (SVC) and inferior vena cava (IVC) rims (inferoposterior rim); posterior rim between the defect edge and the posterior atrial wall; and the rim to the upper right pulmonary vein. Rim lengths should be greater than 5 mm for percutaneous transcatheter closure of secondary ASD.
Due to the large blood shunt through the ASD, the left ventricle (LV) is volume-underloaded, and age-related LV diastolic dysfunction can lead to an increase in the left-to-right blood shunt through the ASD, resulting in an even greater enlargement of the RA and RV, which causes late-onset symptoms in elderly individuals. However, following ASD closure, these patients are at an increased risk of developing acute heart failure with pulmonary edema due to underlying LV diastolic dysfunction. Detecting occult LV diastolic dysfunction can help identify patients at risk of developing acute heart failure immediately after ASD closure.
Right ventricular (RV) function (systolic or diastolic) is generally preserved. Alterations may occur, for example, in the presence of pulmonary hypertension.
Transesophageal echocardiography (TEE) is recommended if the defect cannot be clearly localized by transthoracic echocardiography [1,7,8].
No ESC guideline (LoE C, GoR 5)
Comments: TEE provides higher-resolution visualization of the interatrial septum and pulmonary venous connections. TEE is particularly useful when sinus venosus defect or anomalous pulmonary venous drainage is suspected, as well as for more precise measurement of defect size and rims prior to planned endovascular defect correction.
All patients with suspected ASD are recommended to undergo a chest X-ray in the anteroposterior projection to detect pulmonary pathology. If a chest CT scan is performed, a chest X-ray should be omitted to avoid duplicating the CT imaging [1,3-5].
No ESC guideline (LoE C, GoR 5)
Comments: Chest radiography allows for the detection of pulmonary vasculature changes associated with hypervolemia or pulmonary arterial hypertension, signs of RV and/or RA enlargement, and bulging of the pulmonary artery trunk.
It is recommended to perform 24-hour Holter ECG monitoring in adult patients with ASD and concomitant rhythm and/or conduction disturbances to detect the latter and, if necessary, select appropriate therapy [30].
No ESC guideline (LoE C, GoR 5).
It is recommended to perform contrast-enhanced cardiac and great vessel magnetic resonance imaging (MRI) when planning surgical treatment if echocardiographic results are inconclusive, to further characterize certain types of ASD (sinus venosus defect), evaluate RV volume overload, and assess for anomalous pulmonary venous drainage when suspected, in adult patients with newly diagnosed ASD, or in patients with combined pathology [1,3,7,8].
No ESC guideline (LoE C, GoR 5)
It is recommended to perform contrast-enhanced cardiac CT only in cases of a diagnosed concomitant congenital heart defect during preparation for surgical treatment [1,3,7,8].
ESC IIaC (LoE C, GoR 5)
It is recommended to perform cardiac catheterization in patients with ASD when the estimated pulmonary artery systolic pressure is >40 mmHg or in the presence of indirect echocardiographic signs of pulmonary hypertension to determine pulmonary vascular resistance and the reversibility of pulmonary hypertension [1,3,5,7,8].
ESC IC (CQR C, LOR 5
It is not recommended to repeat cardiac catheterization in patients with Eisenmenger syndrome if the initial catheterization confirmed the diagnosis [33].
No ESC guideline (LoE C, GoR 5)
It is recommended to repeat cardiac catheterization in patients with Eisenmenger syndrome if the diagnosis is in doubt or if there have been significant changes in the clinical picture [33].
No ESC guideline (LoE C, GoR 5)
In adult patients with low or intermediate pre-test probability of coronary artery disease (CAD), it is recommended to perform coronary computed tomography angiography (CCTA) when coronary anomalies are suspected [28,29].
ESC IIaC (LoE C, GoR 5)
In patients with a high pre-test probability of CAD, or in men over 40 years of age, or postmenopausal women prior to planned cardiac surgery, it is recommended to perform invasive coronary angiography when coronary anomalies are suspected [28,29].
ESC IC (LoE C, GoR 5)
2.5 Other Procedures
Prior to hospital discharge, all patients are recommended to undergo an electrocardiogram for postoperative monitoring purposes [1,30].
No ESC guideline (LoE C, GoR 5)
Prior to hospital discharge, all patients are recommended to undergo transthoracic echocardiography for postoperative monitoring purposes [1,30].
No ESC guideline (LoE C, GoR 5)
3. Treatment, including drug and non-drug therapies, diet therapy, and pain management
3.1 Conservative management
Medical therapy for isolated forms of ASD is not recommended [1,3,5,7,8].
No ESC guideline (LoE C, GoR 5)
Comments: Patients with secondary ASD are typically asymptomatic and do not require pharmacotherapy.
In the presence of heart failure in infants under one year of age with ASD, the prescription of diuretics is recommended [1,3,5,7,8].
No ESC guideline (LoE C, GoR 5)
Comments: Indications for medical treatment of heart failure in the presence of ASD may arise in premature infants under one year of age with comorbid pulmonary conditions.
In patients with concomitant supraventricular arrhythmias presenting with atrial flutter/fibrillation, it is recommended to administer antiarrhythmic therapy and/or perform interventional treatment [1,12-15,30].
ESC IIaC (LoE C, GoR 5)
In adult patients with an unrepaired ASD who develop Eisenmenger syndrome, other antihypertensive agents are recommended [7,16,31,36].
ESC IB (LoE B, GoR 2)
Anticoagulant therapy is recommended in adult patients with ASD for the prevention of thromboembolic events in the presence of atrial fibrillation/flutter or documented thrombosis/thromboembolism [37,38].
ESC IC (LoE C, GoR 5)
Comments: Anticoagulant therapy with vitamin K antagonists in the absence of supraventricular rhythm disorders, mechanical valves (mechanical bileaflet aortic valve prosthesis***, mechanical bileaflet mitral valve prosthesis***), or vascular grafts (synthetic blood vessel prosthesis***) is not routinely recommended in patients with ASD. The administration of antithrombotic agents is decided on an individual basis in the presence of a pulmonary artery aneurysm with an in-situ thrombus or a history of thromboembolic events. In patients with Eisenmenger syndrome who have atrial fibrillation/flutter as well as documented thrombosis/thromboembolism, anticoagulant therapy may be recommended provided there is a low bleeding risk.
3.2 Surgical Treatment
There are two methods of surgical treatment for ASD: X-ray endovascular using cardiac occluders*** and open surgery (cardiopulmonary bypass).
Surgery for isolated ASD is recommended to be performed by suturing or closure using any certified material (medical device with a registration certificate: synthetic cardiovascular patch***, animal-derived cardiovascular patch***, cardiac occluder***) [3-5].
None in ESC (LoE C, GoR 5).
Contraindications for transcatheter ASD closure [43].
Relative:
young age of patients (<15 kg);
occlusion or congenital absence of the IVC;
blood coagulation disorders, peptic ulcer disease, or other contraindications to platelet aggregation inhibitors or anticoagulants;
pulmonary hypertension with right-to-left shunting;
minimum distance from the coronary sinus, atrioventricular valves, or right pulmonary veins < 5 mm.
Absolute contraindications:
left atrial hypoplasia;
sinus venosus defect;
primum ASD;
intracardiac thrombi;
sepsis;
concomitant cardiac anomalies requiring surgical correction.
Endovascular or surgical treatment under cardiopulmonary bypass for ASD is recommended regardless of symptom presence if there is right ventricular volume overload, no signs of PH according to non-invasive imaging methods, or PVR index < 3 Wood units*m2, with a predominantly systemic-to-pulmonary shunt direction (Qp/Qs > 1.5:1), and a PVR/SVR ratio < 0.3 [1,3-5,7,8,30].
ESC I B (CGR C, LOR 5)
Endovascular or surgical treatment under cardiopulmonary bypass for ASD is recommended for symptomatic patients and asymptomatic patients with right ventricular volume overload, if the systemic-to-pulmonary shunt direction predominates (Qp/Qs > 1.5:1), PVR index is 3–5 Wood units*m2, and PVR/SVR ratio < 0.3 [1,3-5,7,8,30,43].
ESC IIaC (C-GPR, 5 LoE)
It is recommended to consider ASD closure with a fenestrated patch if the systemic-to-pulmonary shunt direction predominates (Qp/Qs > 1.5:1), and the baseline PVR index is ≥ 5 Wood units*m2, but decreases to ≤ 5 Wood units*m2 during targeted therapy [1,3-5,7,8,30,43].
ESC IIbC (Level of Evidence C, Grade of Recommendation 5)
Endovascular or surgical treatment under cardiopulmonary bypass for ASD is recommended for patients with paradoxical emboli (after ruling out other causes) regardless of defect size, provided there is no PAH or LV impairment [1,3-5,7,8,30,43].
ESC IIaC (C-GPR, 5 LoE)
It is recommended to carefully weigh the surgical risk against the potential benefit of ASD closure in elderly patients in whom defect closure with an occluder is not feasible [30].
ESC I C (CGR C, LOR 5)
Comments: The need for reoperation for recurrent ASD is rare. ASDs without signs of RV volume overload do not affect life expectancy and therefore require no closure. Potential complications of open surgical ASD treatment include: residual interatrial shunt, stenosis of the vena cava and pulmonary vein ostia (when correcting sinus venosus ASD), sick sinus syndrome (when correcting sinus venosus ASD), atrial re-entry tachycardias associated with an atrial incision scar, and tricuspid regurgitation. Potential complications of endovascular ASD treatment include: residual interatrial shunt and, rarely, cardiac occluder dislocation***.
Endovascular closure of secundum ASD is recommended as the priority treatment method; if it cannot be performed, open surgery under cardiopulmonary bypass should be preferred [3,18-20].
ESC IC (LoE B, GoR 3)
Comments: Indications for transcatheter closure of secundum ASD are: 1) defects up to 36 mm in size; 2) adequate defect rims of at least 5 mm; 3) the ratio of the left disc diameter of the required cardiac occluder*** to the total interatrial septum diameter must be less than 1. Percutaneous catheter closure of secundum ASD is preferably performed at an age > 1.5–2 years.
Surgical treatment of isolated ASD is not recommended in children under 12 months of age [3-5,8].
No ESC guideline (LoE C, GoR 5)
Comments: The best results are achieved when the procedure is performed at an age < 25 years. ASD closure after 40 years of age does not appear to affect the incidence of arrhythmias during follow-up. However, a positive effect on morbidity is achieved by closing the defect at any age (exercise tolerance, dyspnea, right-sided heart failure), especially when endovascular intervention is feasible [30].
Surgical closure of secundum ASD via an open approach is recommended for patients if concomitant cardiac surgery is considered [3,8,18].
No ESC guideline (LoE C, GoR 5)
Treatment of sinus venosus defect, coronary sinus defect, or primum ASD is recommended to be performed via an open surgical approach [3,8,18].
No ESC guideline (LoE C, GoR 5)
Endovascular and surgical correction of ASD is not recommended for patients with Eisenmenger syndrome, as well as patients with a predominantly systemic-to-pulmonary shunt (Qp/Qs > 1.5:1) if the PVR index is ≥ 5 Wood units*m2 despite ongoing targeted therapy [30-34].
ESC III (LoE C, GoR 5)
In patients with severe irreversible pulmonary hypertension without evidence of left-to-right shunting, ASD closure is not recommended [3,8,16].
ESC IIIC (CGR C, LOR 5)
In patients requiring surgical ASD closure who have a history of symptomatic atrial fibrillation (AF), surgical AF ablation is recommended during surgical ASD closure [30, 35].
ESC IIaC (LoE C, GoR 5)
4. Medical Rehabilitation and Sanatorium-Resort Treatment
Within 3 months after surgical (open or endovascular) intervention, in the presence of residual pulmonary hypertension and/or signs of circulatory failure, the patient is recommended to undergo rehabilitation in a cardiological sanatorium or rehabilitation center [1,24].
None in ESC (LoE C, GoR 5)
Following surgery, the patient is recommended to restrict physical activity for up to 3 months from hospital discharge [1,17,25].
No ESC guideline (LoE C, GoR 5)
Comments: Six months after ASD repair (open or endovascular), patients may be cleared for all sports activities in the absence of: 1) signs of pulmonary arterial hypertension; 2) symptomatic tachyarrhythmias or second- or third-degree AV block; 3) signs of myocardial dysfunction [17,25].
Stage III (outpatient) of medical rehabilitation for children after surgical ASD correction may be carried out in sanatorium-resort settings taking into account the recommendations of a multidisciplinary rehabilitation team (ICF), primarily pediatric cardiologists [45, 46].
Sanatorium-resort treatment is recommended for children after surgical ASD correction in the compensated stage, without hemodynamic impairment, with circulatory failure no higher than grade I, within their residential climate zone, at balneological and climatic health resorts to improve the function of the cardiovascular and respiratory systems, the child's adaptive and compensatory mechanisms, increase immunological reactivity and nonspecific resistance, reduce muscle detraining, and correct the child's psycho-emotional state [47, 49, 50, 51, 55, 56].
None in ESC (LoE 5, GoR C).
Comment. Referral for sanatorium-resort treatment in children after surgical ASD correction is recommended no earlier than 6 months after surgery.
In the rehabilitation of children after surgical correction of ASD, it is important to consider the presence of the main syndromes dominating in the preoperative pathogenesis:
- heart failure,
- pulmonary hypertension,
- hypoxia and arterial hypoxemia syndrome,
- decreased immune reactivity,
- asthenoneurotic syndrome.
Sanatorium-resort treatment is not recommended for children after surgical correction of ASD in the presence of:
- circulatory insufficiency of stage IIA and higher (> II functional class according to ROSS),
- moderate and severe pulmonary arterial hypertension (with sPAP > 35 mm Hg),
- life-threatening tachyarrhythmias and bradyarrhythmias,
- ventricular or atrial tachycardias,
- second- and third-degree AV block,
- decreased left ventricular ejection fraction,
- subacute myocarditis, pericarditis,
- presence of a pacemaker [50, 51],
No ESC guideline (LoE 5, GoR C).
Comment. General contraindications for sanatorium-resort treatment and acute or subacute diseases, including acute infectious diseases until the end of the isolation period, must also be taken into account [47].
Children aged 4 to 14 years inclusive, including those accompanied by a legal representative, and children aged 15 to 18 years unaccompanied are sent to sanatorium-resort institutions managed by the Russian Ministry of Health. If the sanatorium-resort institution has a cardiology department, children aged 7 to 14 years inclusive are admitted without being accompanied by a legal representative, unless the need for accompaniment is due to medical indications [48]. The duration of sanatorium-resort treatment is 21 days.
To assess the initial clinical and functional status of patients with ASD upon admission to sanatorium-resort treatment, it is necessary to have ECG and echocardiography data, and for children with arrhythmic syndrome, Holter monitoring data.
Methods of sanatorium-resort treatment.
1. Climatotherapy (air and sun baths, sea bathing) is recommended for children after surgical correction of ASD in the stage of compensation, without hemodynamic impairment, and with circulatory insufficiency of no higher than stage I in order to increase the nonspecific resistance and immune defense of the child's body [49, 51, 52, 68-72].
No ESC guideline (LoE 5, GoR C).
Comments. Climatotherapy is carried out according to generally accepted methods and taking into account special dosimetric tables: climatotherapy starting with regimen I (sparing, mild) for 3-5 days, then regimen II (sparing-toning);
- air baths are prescribed depending on the season of the year, the regimen in the ward (sanatorium climate ward) or in the open air, daily, for 5–30 minutes, with a course of 15–20 procedures;
- heliotherapy (sun baths) is prescribed according to regimen I (sparing, scattered solar radiation) with dosing from 1 to 4 therapeutic doses and regimen II (sparing-training) with an increase in therapeutic doses from 1 to 8, daily, 1-2 times a day, course of treatment 15-18 procedures;
- sea bathing (thalassotherapy) is carried out according to regimens I (sparing) and II (sparing-training) (when sea water temperature is at least 22°C). It starts with rubbing, dousing, and dipping for 2-3 days.
2. At the sanatorium-resort stage, children after surgical correction of ASD in the stage of compensation, without hemodynamic impairment, and with circulatory insufficiency of no higher than stage I are recommended to receive medical nutrition [49-51, 57, 62].
No ESC guideline (LoE 5, GoR C).
Comments: sanatorium-resort institutions use a standard diet (Pevzner therapeutic diet table no. 15) without exceeding the age norm for carbohydrates, animal fats, fluids, and salt [49, 51, 57].
3. Patients after surgical correction of ASD in the stage of compensation, without hemodynamic impairment, and with circulatory insufficiency of no higher than stage I are recommended to be prescribed a physical activity regimen [51, 58-63, 66, 67, 69].
ESC II B (LoE 5, GoR C).
Comments: the physical activity regimen includes
- morning hygienic gymnastics with self-monitoring of heart rate (HR), therapeutic gymnastics;
- walks in the fresh air along the sea and in the park area;
- therapeutic dosed walking is prescribed adequately to the patient's condition at a pace of 70–80 steps per minute for regimen I and 80-90 steps per minute for regimen II with short rest periods (3-5 min) on a bench. The pulse rate is measured before and after therapeutic walking to monitor exercise tolerance.
Patients with ASD and stage I circulatory insufficiency are recommended to be prescribed physical loads according to regimen I. If patients exhibit worsened tolerance to physical and emotional stress or deteriorated myocardial functional capacities during the sanatorium-resort stage of treatment, it is recommended to transfer the child to regimen I (sparing) until the end of their stay in the sanatorium.
4. In the sanatorium, children after surgical correction of ASD in the stage of compensation, without hemodynamic impairment, and with circulatory insufficiency of no higher than stage I are recommended to be prescribed therapeutic massage of the collar zone [49-51, 64, 65].
ESC II C (LoE 5, GoR C).
Comments: massage techniques used include stroking, friction, and kneading. The procedures are carried out daily, lasting 10-12 minutes, with a course of 8-10 procedures.
5. At the sanatorium-resort stage, children after surgical correction of ASD in the stage of compensation, without hemodynamic impairment, and with circulatory insufficiency of no higher than stage I are recommended to be prescribed antihypoxic physical treatment methods that help increase blood oxygenation, reduce tissue hypoxia, and enhance oxygen utilization by the myocardium and brain:
- sodium chloride baths [49, 51]:
No ESC guideline (LoE 5, GoR C).
Comment: water temperature 36-37°C, sodium chloride concentration 10-20 g/L, procedure duration 8-12 min, every other day, 8-10 procedures per course;
- carbon dioxide baths [49, 51].
No ESC guideline (LoE 5, GoR C).
Comment: water temperature 34-36°C, CO2 concentration 0.6-0.9-1.3 g/L, every other day, procedure duration 6-10 min, course of 8-10 procedures;
- oxygen baths [49, 51].
No ESC guideline (LoE 5, GoR C).
Comments: water temperature 36-37°C, oxygen pressure 0.3 atm, 8-10 min prescribed every other day, course of 8-10 procedures.
6. Children after surgical correction of ASD in the stage of compensation, without hemodynamic impairment, and with circulatory insufficiency of no higher than stage I in a sanatorium are recommended sedative treatments aimed at enhancing inhibitory processes in the central nervous system. Sedative effects are provided by:
- therapeutic massage of the collar zone (according to the above methodology) [49-51, 64, 65],
ESC II C (LoE 5, GoR C).
- conifer baths [49, 51].
No ESC guideline (LoE 5, GoR C).
Comment: used in children over 6 years old, at a temperature of 36-37°C, every other day, for 7-10 min, course of 8-10 procedures;
- iodine-bromine baths [49, 51].
No ESC guideline (LoE 5, GoR C).
Comments: water temperature 36-37°C, 8-10 min (depending on age), every other day, course of 8-12 baths;
- nitrogen baths [49, 51].
No ESC guideline (LoE 5, GoR C).
Comments: water temperature in the bath 36°C, 8-10 min, every other day, course of 8-10 baths.
- electrosleep therapy [49, 51, 68].
ESC not available (LoE 4, GoR B).
Comments: performed using the orbital-occipital method with a current of 6 - 10 mA, lasting 15-20 minutes, daily, course of 12-15 procedures.
7. At the sanatorium-resort treatment stage, children after surgical correction of ASD in the stage of compensation, without hemodynamic impairment, and with circulatory insufficiency of no higher than stage I are recommended carbon dioxide baths (using the above methodology) as cardiotonic methods aimed at stimulating cardiac activity [49-51].
No ESC guideline (LoE 5, GoR C).
Patients after surgical correction of ASD in the stage of compensation, without hemodynamic impairment, and with circulatory insufficiency of no higher than stage I are recommended vasodilator methods aimed at improving microcirculation:
- climatotherapy (using the above methodology) [49, 51, 52, 68-72].
ESC not available (LoE 5, GoR C).
- therapeutic massage of the collar zone (using the above methodology) [49-51, 64, 65],
ESC II C (LoE 5, GoR C).
- conifer baths (using the above methodology) [49, 51].
No ESC guideline (LoE 5, GoR C).
-physical therapy (using the above methodology) [51, 58-63, 66, 67, 69].
ESC II B (LoE 3, GoR B).
- "dry" carbon dioxide baths [51].
No ESC guideline (LoE 5, GoR C).
Comments: carbon dioxide concentration 32%, temperature 32°C, procedure duration 8–20 min (depending on age), daily or every other day, course of 8–10 procedures;
- Sinusoidal modulated current (SMT) therapy [49].
No ESC guideline (LoE 5, GoR C).
Comments: prescribed paravertebrally to the cervical-collar region (CIV–ThII), modes IV and III, stimulation frequency 30 Hz, modulation depth 50–75%, 5 min for each mode, daily, course of 10 procedures;
- galvanization of the collar region (Scherbak's galvanic collar) [49–51];
No ESC guideline (LoE 5, GoR C).
Comments: current intensity 2–8 mA, procedure duration 8–16 min according to the schedule, daily, course of 10 procedures;
- infrared laser therapy [49].
No ESC guideline (LoE 5, GoR C).
Comments: prescribed paravertebrally at the CIV–ThII level, labile technique, frequency 80 Hz, pulse power 2–4 W, procedure duration 2, 3, 4 min (depending on age), daily, course of 5–6 procedures;
- showers: circular and jet showers [49, 51].
No ESC guideline (LoE 5, GoR C).
Comments: water temperature 35–30 °C, duration 2–3 minutes, every other day, course of 10–15 procedures;
- contrast shower [51].
No ESC guideline (LoE 5, GoR C).
Comments: water temperature 36 and 18 °C, duration 8–10 min, every other day, course of 8–10 procedures;
8. For patients after surgical correction of ASD in the compensation stage, without hemodynamic impairment, and with circulatory insufficiency no higher than grade I, it is recommended to use autonomic-correcting methods aimed at correcting autonomic dysfunction. The following are used:
- air baths (using the methodology described above) [49, 51, 52, 68–72].
No ESC guideline (LoE 5, GoR C).
- heliotherapy (using the methodology described above) [49, 51, 52, 68–72].
No ESC guideline (LoE 5, GoR C).
- thalassotherapy (using the methodology described above) [49, 51, 52, 68–72],
ESC guideline not available, (LoE 5, GoR C);
- pine needle baths (using the methodology described above) [49, 51].
No ESC guideline (LoE 5, GoR C).
- hydrokinesitherapy (therapeutic swimming) [51].
No ESC guideline (LoE 5, GoR C).
Comments: therapeutic swimming is performed in warm water of an open water body or pool at a comfortable pace with a gradual increase in distance and duration of stay in the water.
9. Psychotherapy is recommended for patients after surgical correction of ASD in the compensation stage, without hemodynamic impairment, and with circulatory insufficiency no higher than grade I. Individual, small-group, and family sessions are conducted to correct the child's interactions with the social environment, eliminate negative psychoemotional factors, and form an adequate response to stressful situations [51, 73].
ESC guideline not available (LoE 4, GoR C).
Comments: In case of deterioration of the clinical and functional state of patients after surgical correction of ASD at the sanatorium-resort stage of treatment, emergency professional care and further management of the child on baseline drug therapy, a sparing climate-motor and therapeutic regimen are required [51, 73].
5. Prevention and Dispensary Observation
It is recommended to perform pulse oximetry for all patients during follow-up visits to monitor the patient's condition after the performed surgical intervention [30, 44].
ESC IIbB (GoR C, LoE 5)
Echocardiography is recommended for all patients with ASD during follow-up visits [30, 44].
ESC IB (GoR C, LoE 5)
ECG recording is recommended for all patients with ASD during follow-up visits [30, 44].
ESC not available (LoE C, GoR 5)
Patients after surgery (open/endovascular) are recommended to undergo dispensary follow-up for one year, and further according to indications, if the ASD was closed, but the following conditions persist or have developed: pulmonary arterial hypertension, supraventricular arrhythmia, right or left ventricular dysfunction, concomitant heart defects or other heart diseases [7,8].
No ESC guideline (LoE C, GoR 5)
Comments: The frequency of dispensary follow-up by a pediatric cardiologist/cardiologist is at one month, 6, and 12 months after surgery. The dispensary follow-up protocol includes ECG and echocardiography, as well as graded exercise tolerance tests and pulse oximetry, if necessary. Further monitoring, if indicated, is carried out at intervals of 12–60 months [7,8].
It is recommended to perform a complete (clinical) blood count and urinalysis, including hemoglobin, hematocrit, erythrocyte, leukocyte, and platelet counts, and erythrocyte sedimentation rate in all patients with ASD, as well as after surgical correction during dynamic follow-up every 6–12 months and upon hospital admission [30,44].
ESC not available (LoE 5, GoR C)
It is recommended to perform a general routine biochemical blood test (levels of potassium, sodium, glucose, creatinine, total protein, urea, total bilirubin, blood alanine aminotransferase and aspartate aminotransferase activity, blood free triiodothyronine (fT3), free thyroxine (fT4), and thyroid-stimulating hormone (TSH), C-reactive protein) to assess renal and hepatic function and exclude inflammation, in the presence of clinical symptoms, and to determine protein fractions by electrophoresis in all patients with ASD, as well as after surgical correction during dynamic follow-up. Determination of total blood cholesterol, low-density lipoprotein cholesterol (LDL-C), and triglycerides (TG) is recommended to identify risk factors for concomitant atherosclerosis and, if necessary, adjust therapy in all adult patients with ASD, as well as after surgical correction during dynamic follow-up [30,44].
ESC not available (LoE 5, GoR C)
It is recommended to measure the blood level of N-terminal pro-B-type natriuretic peptide (NT-proBNP) in patients with ASD, as well as after surgical correction, when indicated, to assess the prognosis of patients with ASD [30,44].
ESC not available (LoE 5, GoR C)
When monitoring patients after transcatheter ASD closure, it is recommended to use antiplatelet agents, other than heparin, for 6 months [7,18-20,26].
No ESC guideline (LoE C, GoR 5)
Comments: As antiplatelet agents other than heparin, #acetylsalicylic acid** (off-label in children under 18 years of age) at a dose of 5 mg/kg/day once daily (not exceeding 325 mg/day) or #clopidogrel** (off-label in children under 18 years of age) at a dose of 0.2–1.0 mg/kg/day once daily are used [7,26].
Children who have undergone surgical or transcatheter ASD correction are recommended to be vaccinated no earlier than three months later [1].
No ESC guideline (LoE C, GoR 5)
Patients after surgical or transcatheter ASD closure are recommended to receive infective endocarditis prophylaxis for 6 months [27].
ESC IIaC (GoR C, LoE 5)
Comments: For any type of congenital heart disease where synthetic materials/prostheses*** were used during correction, bacterial endocarditis prophylaxis is carried out, when indicated, for 6 months after surgery or for life 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.).
6. Organization of Medical Care
Indications for planned hospitalization:
1) presence of symptoms of heart failure;
2) presence of cardiac arrhythmias;
3) elective surgical treatment.
Indications for emergency hospitalization:
1) deterioration of the patient's functional status due to the onset of hemodynamically significant cardiac arrhythmias;
2) presence of paradoxical systemic vascular embolism, including cerebral embolism.
Indications for patient discharge from the hospital:
1) absence of symptoms of heart failure and cardiac arrhythmias.
7. Additional Information
No.
Criteria for Evaluating the Quality of Medical Care
№ | Quality criteria | ESC | GoR | LoE |
|---|---|---|---|---|
Diagnosis stage | ||||
1 | Patient history and complaints were collected | none | C | 5 |
2 | Heart auscultation was performed | none | C | 5 |
3 | Electrocardiogram recording performed | none | C | 5 |
4 | Echocardiography with color Doppler mapping was performed | none | C | 5 |
5 | Cardiac chamber catheterization was performed in the presence of indirect echocardiographic signs of pulmonary hypertension. | IC | B | 3 |
Conservative and surgical treatment stage | ||||
1 | Diuretics were prescribed in the presence of heart failure symptoms | none | C | 5 |
2 | Other antihypertensive agents were prescribed to adult patients who developed Eisenmenger syndrome as a result of uncorrected ASD. | IB | C | 2 |
3 | Antiarrhythmic therapy was prescribed in the presence of atrial flutter/fibrillation. | IIaC | C | 5 |
4 | Surgical intervention was performed to close the atrial septal defect in the presence of medical indications. | IIaC | C | 5 |
Postoperative Monitoring Stage | ||||
1 | Electrocardiography was recorded prior to hospital discharge. | none | C | 5 |
2 | Echocardiography with color Doppler mapping was performed prior to discharge from the hospital | none | C | 5 |
References
Show references
Myung K. Park. // Pediatric Cardiology for Practitioners. Mosby; 6 edition, 2014.-688 p.
Hoffman JIE, Kaplan S. The incidence of congenital heart disease. J Am CollCardiol. 2002;39:1890–900.
Kouchoukos N.T., Blackstone E.H., Hanley F.L., Kirklin J.K. Kirklin/Barratt-Boyes cardiac surgery: morphology, diagnostic criteria, natural history, techniques, results, and indications. – 4th ed. Philadelphia: Elsevier; 2013.
Kupryashov A.A. Atrial septal defect. Partial anomalous pulmonary venous drainage. In: Bokeria L.A., Shatalov K.V. (eds.). Pediatric Cardiac Surgery. A Guide for Physicians. A.N. Bakulev National Medical Research Center of Cardiovascular Surgery of the Russian Ministry of Health, 2016, pp. 294-312.
Sharykin AS. Congenital heart defects. A guide for pediatricians, cardiologists, and neonatologists. Moscow: Teremok; 2005.
Benson DW, Sharkey A, Fatkin D, et al. Reduced penetrance, variable expressivity, and genetic heterogeneity of familial atrial septal defects.Circulation. 1998; 97: 2043– 8.
Jochen Weil. // Guidelines for the Management of Congenital Heart Diseases in Childhood and Adolescence. Cardiology in the Young. 2017: 27(Suppl. 3): S1–S105.
Karen K. Stout, Curt J. Daniels, Jamil A. Aboulhosn, Biykem Bozkurt, Craig S. Broberg, Jack M. Colman, Stephen R. Crumb, Joseph A. Dearani, Stephanie Fuller, Michelle Gurvitz, Paul Khairy, Michael J. Landzberg, ArwaSaidi, Anne Marie Valente, George F. Van Hare. //2018 AHA/ACC Guideline for the Management of Adults With Congenital Heart Disease: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines. // Circulation. 2019; 139: e698–e800.
Loscalzo J. Paradoxical embolism: clinical presentation, diagnostic strategies, and therapeutic options. Am Heart J. 1986;112: 141–5.
Ward R, Jones D, Haponik EF. Paradoxical embolism.Anunderrecognizedproblem.Chest. 1995;108: 549 –58.
Silka MJ, Rice MJ. Paradoxic embolism due to altered hemodynamic sequencing following transvenouspacing.Pacing ClinElectrophysiol.1991; 14: 499 –503.
Prystowsky EN, Benson DW Jr, Fuster V, et al. Management of patients with atrial fibrillation.A statement for healthcare professionals.From the Subcommittee on Electrocardiography and Electrophysiology, American Heart Association.Circulation.1996; 93: 1262–77.
Sarquella-Brugada G., Blomstrom-Lundqvist C., Deanfield J., Janousek J., Abrams D., Bauersfeld U., Brugada R., Drago F., de Groot N., Happonen J.M., Hebe J., YenHo S., Marijon E., Paul T., Pfammatter J.P., Rosenthal E. // 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.
Clinical Guidelines: Supraventricular Cardiac Arrhythmias in Adults. http://cr.rosminzdrav.ru/#!/schema/242#doc_abbreviation (accessed June 18, 2019).
Clinical Guidelines: Atrial Fibrillation and Flutter in Adults. http://cr.rosminzdrav.ru/#!/schema/888#doc_a1 (accessed June 18, 2019).
Clinical Guidelines: Pulmonary Hypertension. http://cr.rosminzdrav.ru/#!/recomend/136 (accessed June 18, 2019).
GeorgeF. VanHare, MichaelJ. Ackerman, Juli-anneK. Evangelista, RichardJ. Kovacs, RobertJ. Myerburg, KeriM. Shafer, CaroleA. Warnes, ReginaldL. Washington. Eligibility and Disqualification Recommendations for Competitive Athletes With Cardiovascular Abnormalities: Task Force 4: Congenital Heart Disease: A Scientific Statement From the American Heart Association and American College of Cardiology. // Circulation. 2015;132:e281–e291.
Du Z.D., Hijazi Z.M., Kleinman C.S., Silverman N.H., Larntz K. Comparison between transcatheter and surgical closure of secundum atrial septal defect in children and adults: results of a multicenter nonrandomized trial. J. Am. Coll. Cardiol. 2002; 39: 1836–44.
Fischer G, Stieh J, Uebing A, Hoffmann U, Morf G, Kramer HH. Experience with transcatheter closure of secundum atrial septal defects using the Amplatzer septal occluder: a single centre study in 236 consecutive patients.Heart.2003; 89: 199 –204.
Dhillon R, Thanopoulos B, Tsaousis G, Triposkiadis F, Kyriakidis M, Redington A. Transcatheter closure of atrial septal defects in adults with the Amplatzer septal occluder. Heart.1999; 82: 559–62.
Rybka M.M., Khinchagov D.Ya., Mumladze K.V., Lobacheva G.V., Vedernikova L.V. Edited by L.A. Bokeria. Protocols for Anesthetic Management of Cardiac Surgeries Performed in Neonates and Children. Methodological Recommendations. Moscow: Bakulev Scientific Center for Cardiovascular Surgery of RAMS; 2014.
Rybka M.M., Khinchagov D.Ya. Edited by L.A. Bokeria. Protocols of anesthesiological management for cardiac surgery procedures performed in coronary artery disease, valvular heart disease, rhythm disorders, hypertrophic cardiomyopathy, and ascending aortic aneurysms in patients of various age groups. Guidelines. Moscow: Bakoulev Scientific Center for Cardiovascular Surgery of RAMS; 2015.
Rybka M.M., Khinchagov D.Ya., Mumladze K.V., Nikulkina E.S. Edited by L.A. Bokeria. Protocols of anesthesiological management for endovascular and diagnostic procedures performed in cardiac surgery patients of various age groups. Guidelines. Moscow: Bakoulev Scientific Center for Cardiovascular Surgery of RAMS; 2018.
Podzolkov VP, Kassirsky GI (eds.). Rehabilitation of patients after surgical treatment of congenital heart defects. Moscow: Bakulev Scientific Center of Cardiovascular Surgery; 2015.
Boitsov S.A., Smolensky A.V., Zemtsovsky E.V., Kolos I.P., Lidov P.I. Guidelines for the admission of athletes with cardiovascular abnormalities to training and competition. Conference "SPORTMED-2011". 2011, December 8, Expocentre, Moscow.
Therese M. Giglia, M. Patricia Massicotte, James S. Tweddell, Robyn J. Barst, Mary Bauman, Christopher C. Erickson, Timothy F. Feltes, Elyse Foster, Kathleen Hinoki, Rebecca N. Ichord, Jacqueline Kreutzer, Brian W. McCrindle, Jane W. Newburger, Sarah Tabbutt, Jane L. Todd, Catherine L. Webb. // Prevention and Treatment of Thrombosis in Pediatric and Congenital Heart Disease: A Scientific Statement From the American Heart Association. // Circulation.2013;128:2622-2703.
Habib G., Lancellotti P., Antunes M.J., Bongiorni M.G., Casalta J.P., Del Zotti F., Dulgheru R., El Khoury G., Erba P.A., Iung B., Miro J.M., Mulder B.J., Plonska-Gosciniak E., Price S., Roos-Hesselink J., Snygg-Martin U., Thuny F., Tornos Mas P., Vilacosta I., Zamorano J.L. // 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). // European Heart Journal, Volume 36, Issue 44, 21 November 2015, Pages 3075–3128.
Baumgartner H, Falk V, Bax JJ, et al. 2017 ESC/EACTS Guidelines for the management of valvular heart disease. Eur Heart J. 2017;38(36):2739-2791.
Knuuti J, Wijns W, Saraste A, et al. 2019 ESC Guidelines for the diagnosis and management of chronic coronary syndromes. Eur Heart J. 2020;41(3):407-477. doi:10.1093/eurheartj/ehz425.
Baumgartner H , et al. 2020 ESC Guidelines for the management of adult congenital heart disease The Task Force for the management of adult congenital heart disease of the European Society of Cardiology (ESC).Eur Heart J (2020) 00, 183.
Galiè N., Humbert M., Vachiery J.L et al. 2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension: The Joint Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS): Endorsed by: Association for European Paediatric and Congenital Cardiology (AEPC), International Society for Heart and Lung Transplantation (ISHLT) // Eur. Heart.J. – 2016. – Vol. 37. – N. 1. – P. 67–119.
Stout KK, Daniels CJ, Aboulhosn JA, Bozkurt B, Broberg CS, Colman JM, Crumb SR, Dearani JA, Fuller S, Gurvitz M, Khairy P, Landzberg MJ, Saidi A, Valente AM, Van Hare GF.2018AHA/ACC Guideline for the Management of Adults With Congenital Heart Disease: A Report of the American College of Cardiology/AmericanHeart Association Task Force on Clinical Practice Guidelines. Circulation. 2019; 139(14):e698-e800.
Kaemmerer H, Apitz C, Brockmeier K, Eicken A, Gorenflo M, Hager A, de Haan F, Huntgeburth M, Kozlik-Feldmann RG, Miera O, Diller GP. Pulmonary hypertension in adults with congenital heart disease: Updated recommendations from the Cologne Consensus Conference 2018.Int J Cardiol. 2018; 272S:79-88.
Lopes AA, O"Leary PW. Measurement, interpretation and use of haemodynamic parameters in pulmonary hypertension associated with congenital cardiac disease. Cardiol Young 2009; 19(5):431–435.
Calkins H, Kuck KH, Cappato R, et al. 2012 HRS/EHRA/ECAS Expert Consensus Statement on Catheter and Surgical Ablation of Atrial Fibrillation: recommendations for patient selection, procedural techniques, patient management and follow-up, definitions, endpoints, and research trial design.Europace 2012;14:528–606).
Gatzoulis MA, Beghetti M, Galiè N, Granton J, Berger RM, Lauer A, Chiossi E, Landzberg M; BREATHE-5 Investigators. Longer-term bosentan therapy improves functional capacity in Eisenmenger syndrome: results of the BREATHE-5 open-label extension study. Int J Cardiol. 2008; 127(1):27–32.
Kirchhof P, Benussi S, Kotecha D, et al. 2016 ESC Guidelines for the management of atrial fibrillation developed in collaboration with EACTS. Europace. 2016;18(11):1609-1678.
Kirchhof P, Benussi S, Kotecha D, et al. 2016 ESC Guidelines for the management of atrial fibrillation developed in collaboration with EACTS. Eur Heart J. 2016;37(38):2893-2962.
Frost A, Badesch D, Gibbs JSR, Gopalan D, Khanna D, Manes A, Oudiz R, Satoh T, Torres F, Torbicki A. Diagnosis of pulmonary hypertension. EurRespir J. 2019 Jan 24;53(1). pii: 1801904.].
GevaT, MartinsJD, WaldRM. Atrialseptaldefects. Lancet.2014;383(9932):1921-1932
RigbyM.Atrialseptaldefect. In: Diagnosis and Management of Adult Congenital Heart Disease. London: Churchill Livingston, 2003
Schwerzmann M, Pfammatter JP. Approaching atrial septal defects in pulmonary hypertension. Expert Rev CardiovascTher. 2015 Jun;13(6):693-701.
Endovascular Surgery. National Guidelines: in four volumes / edited by B.G. Arakelyan [B. G. Alexyan]. Moscow: 2017, 2250 p.
Jochen Weil. Guidelines for the Management of Congenital Heart Diseases in Childhood and Adolescence.CardiologyintheYoung. 2017: 27(Suppl. 3): S1–S105.
Order of the Ministry of Health of the Russian Federation No. 878n dated October 23, 2019 "On approval of the Procedure for organizing medical rehabilitation of children"
Federal Law No. 323-FZ dated November 21, 2011 (as amended on March 8, 2022) "The fundamentals of protecting the health of citizens in the Russian Federation". Article 40. Medical rehabilitation and sanatorium-resort treatment.
Order of the Ministry of Health of the Russian Federation No. 1029n dated September 28, 2020 "On approval of lists of medical indications and contraindications for sanatorium-resort treatment."
Order of the Ministry of Health of the Russian Federation No. 279n dated May 5, 2016 "On approval of the Procedure for organizing sanatorium-resort treatment."
Special physiotherapy / edited by Professor G.N. Ponomarenko. Moscow: Meditsina; 2005. p. 426-430.
Physical and rehabilitation medicine. National guidelines. Concise edition // Edited by G.N. Ponomarenko. Moscow: GEOTAR-Media, 2017. 512 p. / Chapter 21. Khan MA, Kuyantseva LV. Cardiovascular diseases. p. 447-452.
Sanatorium-resort treatment: national guidelines / edited by A.N. Razumov, V.I. Starodubov, G.N. Ponomarenko. Moscow: GEOTAR-Media, 2021. p. 593-596.
Ponomarenko GN, Kovlen DV. Physical and rehabilitation medicine. Evidence-based clinical guidelines: 3rd ed., rev., suppl. // G.N. Ponomarenko, D.V. Kovlen / Edited by Academician A.N. Razumov. Moscow: Nauka, 2020. p. 10-22.
Sukhareva GE, Golubova TF, Garmash OI, Eliseeva LV. On the issue of sanatorium-resort rehabilitation of children with congenital heart defects in the remote postoperative period at the Yevpatoria resort. Proceedings of the nineteenth annual session of the Bakulev Scientific Center of Cardiovascular Surgery, May 18-20, 2015 // Cardiovascular Diseases: Bulletin of the Bakulev Scientific Center of Cardiovascular Surgery of the Russian Academy of Medical Sciences – May-June 2015. – No. 3. – Vol. 16. – p. 150.
Kurganova AV, Eliseeva LV, Semenyak EG, et al. Issues of organizing sanatorium-resort care for children with unoperated congenital heart defects at the Yevpatoria resort // Bulletin of Physiotherapy and Balneology. – 2019. – Vol. 25. – No. 1. – p. 116-116.
Gierat-Haponiuk K, et al. Effect of complex cardiac rehabilitation on physical activity and quality of life during long-term follow-up after surgical correction of congenital heart disease // Kardiologia Polska (Polish Heart Journal). – 2015. – Vol. 73. – No. 4. – p. 267-273.
Bjarnason-Wehrens B, et al. Cardiac rehabilitation in congenital heart disease // Cardiovascular Prevention and Rehabilitation. – Springer, London, 2007. – p. 361-375.
Order of the Ministry of Health of the Russian Federation No. 330 dated August 5, 2003 (as amended on November 24, 2016) "On measures to improve therapeutic nutrition in medical and preventive institutions of the Russian Federation".
Shlyk N. I. Lechebnaya fizicheskaya kultura pri zabolevaniyakh serdechno-sosudistoy sistemy: uchebno-metodicheskoe posobie / N.I. Shlyk. - Izhevsk: Udmurt University Press, 2014. - P. 66-85.
Takken T. et al. Recommendations for physical activity, recreation sport, and exercise training in paediatric patients with congenital heart disease: a report from the Exercise, Basic & Translational Research Section of the European Association of Cardiovascular Prevention and Rehabilitation, the European Congenital Heart and Lung Exercise Group, and the Association for European Paediatric Cardiology // European journal of preventive cardiology. – 2012. – Vol. 19. – No. 5. – P. 1034-1065. Epifanov V.A. Lechebnaya fizicheskaya kultura i massazh [Electronic resource]: textbook / Epifanov V.A. – 2nd ed., rev. and supp. – M.: GEOTAR-Media, 2013. – 528 p. – Access mode: https://www.rosmedlib.ru/book/ISBN9785970426456.
Karpov V. Y. et al. Congenital Heart Defects in Children and the Main Approaches to Rehabilitation After their Surgical Treatment // Indian Journal of Public Health Research & Development. – 2019. – Vol. 10. – No. 10.
Tataurova V.P., Eliseeva L.V. Dynamics of myocardial functional reserve parameters in children operated on for congenital heart disease // Bulletin of Physiotherapy and Balneology. 2018. - Vol. 24. - No. 1. - P. 129-129.
Kurganova A.V., Eliseeva L.V., Tataurova V.P. Dynamics of heart rate variability in children operated on for congenital heart defects // Bulletin of Physiotherapy and Balneology. 2018. - Vol. 24. - No. 2. - P. 107-107a.
Eliseeva L. V., Tataurova V. P., Semenyak E. G. Sanatorium-resort rehabilitation of children with congenital heart defects // Vestnik fizioterapii i kurortologii. – 2015. – Vol. 21. - No. 2. – P. 123-123.
Eliseeva L.V., Tataurova V.P., Semenyak E.G. Complex sanatorium-resort treatment of children with congenital heart defects in the postoperative period // Electronic collection of materials of the conference dedicated to the 90th anniversary of the Pyatigorsk Scientific Research Institute of Balneology, 2015, P. 81-81.
Eliseeva L. V. et al. Dynamics of clinical, functional, and laboratory parameters in children operated on for congenital heart defects at the stage of sanatorium-resort rehabilitation // Vestnik fizioterapii i kurortologii. – 2016. – Vol. 22. – No. 3. – P. 68-69.
Eliseeva L.V., Tataurova V.P. Effect of sanatorium-resort treatment on the dynamics of myocardial functional reserve parameters in children after surgical correction of an atrial septal defect // Vestnik fizioterapii i kurortologii. – 2021. – Vol. 98. – No. 3-2. – P. 71-72.
Kurganova A. V., Eliseeva L.V. et al. Sanatorium-resort rehabilitation of children with unoperated congenital heart defects // Vestnik fizioterapii i kurortologii. – 2017. – Vol. 23. – No. 3. – P. 109-109.
Golubova T.F., Lyubchik V.N., Eliseeva L.V. Rehabilitation potential of children who underwent surgical correction of congenital heart defects at different age periods // Bulletin of New Medical Technologies. Electronic Edition. 2014. - No. 1. - P. 213-213.
Tikkanen A.U. et al. Paediatric cardiac rehabilitation in congenital heart disease: a systematic review // Cardiology in the young. – 2012. – Vol. 22. – No. 3. – P. 241-250.
Golubova T.F., Kurganova A.V., Eliseeva L.V. Neurophysiological parameters in children operated on for congenital heart defects and their dynamics under the influence of sanatorium-resort rehabilitation // Abstracts of the 10th All-Russian Congress "Pediatric Cardiology 2018", Moscow, December 13-15, 2018, P. 12-13 (electronic version www.cardio-rus.ru).
Kurganova A.V., Eliseeva L.V., Semenyak E.G. Cerebral hemodynamics parameters in children after surgical correction for congenital heart defects at different ages // Vestnik fizioterapii i kurortologii. 2018 -. Vol. 24.- No. 2. P. - 107-108.
Kaladze N.N., Yushchenko A.Yu. Dynamics of quality of life parameters in children with ventricular septal defect during sanatorium-resort treatment // Bulletin of Physiotherapy and Balneology. 2020. - Vol. 26. - No. 2. - P. 26-30.
Eliseeva L.V., Kuznetsov V.G. Dynamics of quality of life and psychoemotional status in children operated on for congenital heart defects under the influence of sanatorium-resort treatment. - Proc. All-Russian Scientific-Practical Conf. with international participation "Rehabilitation and Prevention–2014", October 16–17, 2014, Moscow, P. 79-82.
Appendix A1. Working Group Composition
Avramenko A.A. (Samara)
Alexyan B.G., Academician of the RAS, "Russian Scientific Society of Interventional Radiology and Endovascular Treatment Specialists", (Moscow)
Belov V.A., (Kaliningrad)
Bogdanov V.N. (Chelyabinsk)
Bokeria L.A., Academician of the RAS, "Association of Cardiovascular Surgeons of Russia", (Moscow)
Boriskov M.V., DSc in Medical Sciences, (Krasnodar)
Borisova N.A., "Russian Scientific Society of Endovascular Diagnostics and Treatment Specialists", (Saint Petersburg)
Brodsky A.G., PhD in Medical Sciences, (Surgut)
Volkov S.S., PhD in Medical Sciences, (Moscow)
Gavrilov R.Yu., "Association of Cardiovascular Surgeons of Russia", (Volgograd)
Garmash O.I., MD, (Yevpatoria)
Gladyshev I.V., (Chelyabinsk)
Golubova T.F., MD, Professor, (Yevpatoria)
Gorbatikov K.V., DSc in Medical Sciences, (Tyumen)
Gorbatykh Yu.N., DSc in Medical Sciences, (Novosibirsk)
Gorbachevsky S.V., DSc in Medical Sciences, "Association of Cardiovascular Surgeons of Russia", (Moscow)
Grigoryan A.M., PhD in Medical Sciences, "Russian Scientific Society of Endovascular Diagnostics and Treatment Specialists", (Moscow)
Eliseeva L.V., (Yevpatoria)
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)
Irtyuga O.B., PhD, Russian Society of Cardiology" (Saint Petersburg)
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., DSc in Medical Sciences, (Tomsk)
Krupyanko S.M., MD, Association of Pediatric Cardiologists of Russia, (Moscow)
Kupryashov A.A., DSc in Medical Sciences, (Moscow)
Kurganova A.V., PhD, (Yevpatoria)
Levchenko E.G., (Moscow)
Lezhnev A.A., (Moscow)
Lyubchik V.N., MD, (Yevpatoria)
Martynyuk T.V., Russian Society of Cardiology, (Moscow)
Movsesyan R.R., DSc in Medical Sciences, "Association of Cardiovascular Surgeons of Russia", (Saint Petersburg)
Nalimov K.A., (Khabarovsk)
Nikiforov A.B., (Moscow)
Petrushenko D.Yu., (Kazan)
Plotnikov M.V., Candidate of Medical Sciences, (Astrakhan)
Podoksenov A.Yu., (Tomsk)
Pursanov M.G., Doctor of Medical Sciences, (Moscow)
Svobodov A.A., Doctor of Medical Sciences, (Moscow)
Semenyak E.G., (Yevpatoria)
Sinelnikov Yu.S., Doctor of Medical Sciences, (Perm)
Tataurova V.P., (Yevpatoria)
Teplov P.V. (Krasnoyarsk)
Trunina I.I., Doctor of Medical Sciences, "Association of Pediatric Cardiologists of Russia", (Moscow)
Chernogrivov A.E., MD, "Association of Cardiovascular Surgeons of Russia", (Penza)
Shekhmametyev R.M., (Perm)
Shlyakhto E.V., Academician of the Russian Academy of Sciences, Russian Society of Cardiology (Saint Petersburg)
Schmaltz A.A., Doctor of Medical Sciences, (Moscow)
Yakovleva A.N., (Saint Petersburg)
Alexandrova S.A., PhD (Moscow) All-Russian Public Organization for the Promotion of the Development of Diagnostic and Therapeutic Radiology "Russian Society of Roentgenologists and Radiologists"
Baryshnikova I.Yu., PhD in Medical Sciences (Moscow) All-Russian Public Organization for the Promotion of the Development of Radiation Diagnostics and Therapy "Russian Society of Roentgenologists and Radiologists"
Bergen T.A., MD, PhD (Novosibirsk), All-Russian Public Organization for Assistance to the Development of Radiation Diagnostics and Therapy "Russian Society of Radiologists"
Rychina I.E., PhD (Moscow), All-Russian Public Organization for Assistance to the Development of Radiation Diagnostics and Therapy "Russian Society of Radiologists"
Sinitsyn V.E., MD, PhD, Professor (Moscow), All-Russian Public Organization for the Promotion of the Development of Diagnostic Radiology and Therapy "Russian Society of Roentgenologists and Radiologists"
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"
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
Functional Diagnostics Physician
Pediatrician
Endovascular radiologist / X-ray endovascular diagnostic and treatment physician
Radiologist
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 | Should be considered apply May be considered apply |
III | Evidence or general agreement that the given procedure, intervention, treatment is not useful/effective, and in some cases may be harmful | Not recommended to use |
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
On the Basics of Protecting the Health of Citizens in the Russian Federation (Federal Law No. 323-FZ of November 21, 2011)
Procedure for Providing Medical Care to Patients with Cardiovascular Diseases (Order of the Russian Ministry of Health No. 918n of November 15, 2012)
Transthoracic echocardiography performance technique
Requirements:
In newborns: transducers with a frequency of 7.5–12 MHz; in adults: 2.5–7 MHz.
Contraindications: none.
Limitations: cases of an inadequate acoustic window
Important indicators when analyzing and interpreting the obtained images:
Performing transthoracic echocardiography
Comprehensive echocardiography
to rule out associated congenital heart disease, assess heart dimensions and contractile function, and evaluate valve competence (mitral and aortic):
Left ventricular (LV) end-diastolic dimension and quantitative assessment of LV contractile function are determined in M-mode from the left parasternal approach in the LV long-axis view or LV short-axis view at the level of the tips of the mitral valve (MV) leaflets, which serve as a reference point for placing the cursor perpendicular to the MV leaflet coaptation line. Measurements are taken from the posterior surface of the interventricular septum to the anterior surface of the LV posterior wall.
Visual assessment of the LV: from the apical four-chamber view in comparison with the right heart chambers.
Right atrium (linear dimensions compared to the left atrium in the 4-chamber heart view, area [normally less than 11 cm²/m²]).
End-diastolic dimension (EDD) of the right ventricle (RV) in the LV short-axis or 4-chamber view relative to the LV end-diastolic dimension (a ratio greater than 1 indicates enlargement). In adults, a basal EDD greater than 47 mm (in men) and greater than 43 mm (in women), a mid-cavity dimension greater than 35 mm (in women) and 42 mm (in men), and a longitudinal dimension greater than 87 mm (in men) and 80 mm (in women) in the apical 4-chamber view indicate RV enlargement.
RV linear dimensions: measured in the apical 4-chamber view, where the interventricular septum acts as the bisector of the scanning sector and the right upper pulmonary vein is visualized. Flattening of the interventricular septum in diastole may be visualized.
Assessment of RV systolic function: tricuspid annular plane systolic excursion (TAPSE; in adults, TAPSE < 17 mm indicates severe RV dysfunction; in children, Z-score calculation is required), RV fractional area change (normal > 35%), peak systolic velocity of the tricuspid annulus by tissue Doppler imaging (in adults, S’ is normally > 9.5 cm/s; S' < 9.5 cm/s is a predictor of RV ejection fraction < 40% [5, 24, 25]), which serves as a marker of global contractility.
Aortic valve annulus diameter, dimensions of the aortic root and ascending aorta: from the left parasternal long-axis approach.
ASD: assessed from the subcostal approach. A modified 4-chamber view along the long axis of the atria, a bicaval view focusing on the inferior vena cava (IVC) or superior vena cava (SVC) are used. The short-axis view at the level of the aortic valve from the left parasternal approach can be useful. Measurement of the maximum size (width) of the defect is performed in two mutually perpendicular directions using color Doppler flow imaging (the defect may have an oval shape). Proper ultrasound machine settings are required to eliminate excessive color blooming over the interatrial septum tissue, which can lead to overestimation of the true defect size. Small defects measure less than 5 mm.
Determination of the hemodynamic significance of shunting:
Color Doppler flow imaging is used to determine the presence, magnitude, and direction of blood shunting across the interatrial septum (IAS). For better visualization of low-velocity shunt flows at the IAS level, the color scale velocity can be lowered (to 25–40 cm/s) and adjusted for color display (decrease color gain). Pulsed-wave Doppler mode can also be used to detect bidirectional shunting in addition to color Doppler flow imaging.
Assessment of the pulmonary-to-systemic flow ratio (Qp/Qs). The diameters of the aortic and pulmonary valve annuli and the velocity time integrals across the aorta and pulmonary artery should be measured using pulsed-wave Doppler mode. Semilunar valves must be free of stenosis and significant regurgitation. Measuring the Qp/Qs ratio is not meaningful in the presence of high pulmonary vascular resistance (e.g., newborns) or severe pulmonary hypertension; there are no clear recommendations for moderate pulmonary hypertension (e.g., the clinical significance of Qp/Qs in patients with pneumonia, bronchopulmonary dysplasia, or chronic obstructive pulmonary disease is unclear).
Assessment of right atrial and right ventricular dilatation, pulmonary trunk enlargement, and accelerated blood flow across the pulmonary valve due to mismatch between the fibrous ring diameter and the increased blood volume flowing through it.
Secundum ASD should be differentiated from a patent foramen ovale. In the case of a patent foramen ovale, the septum primum flap is visualized adjacent to the defect, and the color Doppler shunt flow has an "oblique" trajectory. Secundum ASD may be associated with so-called IAS aneurysm, i.e., bulging of the septum due to thinning or severe stretching to a depth of more than 10 mm. When an aneurysm is present, its base and depth, as well as the number and diameter of ASDs located within it, are measured.
Selection for endovascular closure: the ASD type must be determined—it should be secundum—and the margins must be measured: aortic margin (distance between the defect edge and the aorta); margin to the atrioventricular valve; margin to the SVC and IVC (inferoposterior); posterior margin—between the defect edge and the posterior atrial wall; and margin to the right upper pulmonary vein. Margin lengths should be greater than 5 mm for percutaneous transcatheter closure of secundum ASD.
Chiari network represents a remnant of the venous valve and appears as a net-like structure in various locations within the right atrium (RA), including near the IVC and coronary sinus orifices. It should be differentiated from an RA membrane and a prominent Eustachian valve. It is important to note the presence of additional structures in the RA, especially in patients scheduled for endovascular ASD closure, as they may interfere with the passage of guidewires and catheters through the RA.
Pulmonary hypertension. In patients with ASD, severe pulmonary hypertension develops relatively late, following prolonged volume overload and a period of relative compensation. Moreover, the development of severe pulmonary hypertension is frequently accompanied by significant RV dilatation and dysfunction, resembling idiopathic pulmonary arterial hypertension. Echocardiographic assessment of the probability of pulmonary hypertension is performed using the methods described in the relevant guidelines.
Appendix B. Physician Action Algorithms
Appendix C. Patient Information
Atrial septal defect (ASD) is the second most common congenital heart disease. In this anomaly, an opening exists from birth in the septum dividing the right and left atria into two separate chambers.
The presence of a septal defect results in a left-to-right shunt, i.e., from the left atrium to the right, causing a large volume of blood to enter the latter. Consequently, the right atrium experiences volume overload, and its pressure increases over time. Long-standing large atrial defects lead to pulmonary circulation overload, right ventricular failure, and pulmonary hypertension, thereby causing damage to the heart and lungs. The neonatal period and infancy pass smoothly in the vast majority of children; they do not require pharmacotherapy or surgical correction. Small defects may close spontaneously during the first year of life or in early childhood, require no treatment, and do not affect the child's development. Symptoms of overload typically appear with large defects in children older than 1 year and manifest as delayed physical development, dyspnea, tachycardia, reduced exercise tolerance, and frequent bronchopulmonary infections. Cardiac arrhythmias may also develop due to overstretching of the right atrial cavity. In adults, persistent atrial defects can lead to transient ischemic attacks. In the presence of lower extremity varicose veins or a tendency toward thrombosis, venous blood from the right atrium may shunt into the left atrium and pass via the left ventricle into the aorta, which gives rise to vessels supplying the brain. Most women with ASD can carry a pregnancy to term; however, in cases of large defects or complications such as heart failure, pulmonary hypertension, or arrhythmias, the risk of a complicated pregnancy increases sharply. Quality of life and life expectancy in patients with an atrial septal defect decline sharply in adulthood, typically around the age of 30. In rare cases, signs and symptoms of the disease may appear much later.
To prevent such a "natural" history of the anomaly, surgical closure of the defect is recommended. The choice of surgical intervention depends on the location of the atrial septal defect and its association with other congenital heart anomalies, and is determined by the physician.
They are classified into:
- "Secundum" ASD: located in the center of the atrial septum, with rims present on all sides;
- "Primum" ASD: located in the lower part of the septum, adjacent to the atrioventricular valves, and typically part of a more complex congenital heart disease;
- Sinus venosus defect: localized in the upper part of the septum, relatively rare;
- Coronary sinus defect: a defect between the coronary sinus (part of the cardiac venous system) and the left atrium.
Several types of surgical interventions are available to repair an atrial septal defect:
- "Non-surgical" (transcatheter) defect closure: access via a blood vessel without body incisions. In this method, the defect is closed using a special umbrella-like device—an occluder***—which is advanced in a folded state via a catheter and deployed in the atrium after crossing the defect;
- ASD repair under cardiopulmonary bypass: open-heart surgery during which the defect is closed with a patch.
Today, both approaches are widely used with excellent outcomes. The choice of closure method depends on a number of factors and is determined jointly by the cardiologist and cardiovascular surgeon. In any case, the intervention is non-emergent, and the timing of the surgery can be planned in advance. Following successful surgery, symptoms disappear and quality of life is unaffected.
Appendix G. Assessment Scales, Questionnaires, and Other Evaluation Tools
Not applicable.