List of Abbreviations
aPTT — activated partial thromboplastin time;
SVC — superior vena cava;
CHD – congenital heart disease;
ASD — atrial septal defect;
CT — computed tomography;
CTA — computed tomography angiography;
PA — pulmonary artery;
LA — left atrium;
IAC — interatrial communication;
MRI – magnetic resonance imaging;
IVC – inferior vena cava;
NSAIDs — nonsteroidal anti-inflammatory and antirheumatic drugs;
HRD — heart rhythm disorders;
PFO — patent foramen ovale;
TV — transverse vein;
RV — right ventricle;
RA — right atrium;
RFA — radiofrequency ablation;
Holter ECG – 24-hour electrocardiogram monitoring;
TAPVR – total anomalous pulmonary venous return;
ECG – electrocardiogram;
ECMO – extracorporeal membrane oxygenation
PPM – permanent pacemaker;
EchoCG – echocardiography;
Terms and Definitions
Cardiac catheterization (angiocardiography) – an invasive therapeutic and/or diagnostic procedure performed in a specialized X-ray surgical operating room by inserting diagnostic or intervention catheters into the heart chambers or the lumen of great vessels via puncture of peripheral arteries or veins.
Total anomalous pulmonary venous return, supracardiac type – a developmental anomaly of the great vessels in which all pulmonary veins drain via a common trunk (collector) into the superior vena cava (SVC) or its major tributaries (left brachiocephalic vein, azygos vein, persistent left SVC).
Total anomalous pulmonary venous return, cardiac type – a developmental anomaly of the great vessels in which all pulmonary veins drain via a common trunk (collector) into the coronary sinus or the right atrium.
Total anomalous pulmonary venous return, infracardiac type – a developmental anomaly of the great vessels in which all pulmonary veins drain via a common trunk (collector) into the inferior vena cava (IVC), hepatic veins, or portal vein (or ductus venosus). The pulmonary venous collector (vertical vein) lies in the posterior mediastinum beneath the pericardium, penetrates the diaphragm at the esophageal hiatus, typically becoming obstructed at this site and determining the obstructive nature of pulmonary venous drainage.
Total anomalous pulmonary venous return, mixed type – a developmental anomaly of the great vessels in which all pulmonary veins drain via separate or grouped orifices into various systemic great veins and/or directly into the right atrium.
Level of Certainty of Evidence (LoE) – the degree of confidence that the estimated effect of a medical intervention is true [1].
Level of evidence (LoE) – the degree of certainty in the validity of the intervention effect and that following the recommendations will bring more benefit than harm in a specific situation [1].
1. Brief information on the disease or condition
1.1 Definition of the disease or condition
Total anomalous pulmonary venous return (TAPVR) – a group of developmental anomalies of the pulmonary veins characterized by the absence of a direct hemodynamic connection between the pulmonary veins and the left atrium [1].
Morphological criteria of the defect [2]:
1. Enlargement of the right ventricle and right atrium, dilation of the pulmonary artery;
2. Reduction of the left heart chambers depending on the size of the interatrial communication;
3. In all forms of TAPVR except the mixed type, pulmonary veins from both sides form a common collector located posterior to the heart;
4. In any form of TAPVR, the pulmonary venous collector may have local obstruction;
5. In the infracardiac form of TAPVR, the site where the pulmonary venous collector enters the systemic veins is located below the diaphragm;
6. Obstruction of pulmonary venous return is more commonly found at the site of pericardial penetration by the collector to the left of the midline in supracardiac TAPVR with drainage via the left brachiocephalic vein;
7. Obstruction of pulmonary venous return is more commonly found at the junction of the collector with the ductus venosus or at the diaphragmatic level in infracardiac TAPVR;
Obstruction of pulmonary venous return in any form of TAPVR carries an adverse prognostic significance due to its negative impact on intracardiac, pulmonary, and systemic hemodynamics and the clinical course of this defect [3].
Congenital heart defects associated with TAPVR:
Tetralogy of Fallot;
single ventricle;
transposition of the great arteries;
corrected transposition of the great arteries;
coarctation of the aorta;
interrupted aortic arch;
atrioventricular septal defect;
hypoplastic left heart syndrome;
ventricular septal defect;
truncus arteriosus;
double outlet right ventricle;
heterotaxy syndrome.
1.2 Etiology and pathogenesis of the disease or condition (group of diseases or conditions)
TAPVR develops as a result of an embryonic failure in establishing the hemodynamic connection between the pulmonary-venous plexus and the posterior wall of the primitive left atrium (LA) [21].
In all forms of TAPVR, pulmonary venous return mixes with systemic venous return and flows into the right atrium; consequently, the viability of the fetus and newborn depends on the presence and size of the fetal interatrial communication through which a right-to-left blood shunt and volume loading of the systemic (left) ventricle occur. The interatrial communication is usually represented by a patent foramen ovale, though combination with a secundum or primum atrial septal defect is possible [22].
The severity of pulmonary venous return impairment is determined by the presence or absence of pulmonary venous collector obstruction. The degree of systemic hypoperfusion is determined by the size of the interatrial communication. Obstruction of the pulmonary venous collector is accompanied by pulmonary venous hypertension, which progresses as fetal pulmonary-arterial resistance naturally decreases [23].
In the absence of pulmonary venous collector obstruction, pulmonary blood flow is increased, which is accompanied by the development of pulmonary hypertension via a shunting mechanism at the level of great veins and low-pressure heart chambers.
1.3 Epidemiology of the Disease or Condition
According to various studies, the incidence of TAPVR ranges from 0.5 to 1.1 per 10,000 live births, which accounts for 0.4–0.9% of all patients with congenital heart defects [49].
Among surgically correctable defects in infants during their first year of life, TAPVR accounts for 2.9% [27].
Among anatomical forms, the supracardiac type is the most frequent (44–49%) [49].
In the natural course of TAPVR, the prognosis is unfavorable, especially in obstructive anatomical forms. Only 20% of children born with TAPVR survive to 1 year of age. Of the patients who die in the first 3 months of life, 50% die within the first week after birth [28].
1.4 Features of Coding the Disease or Condition
Q26.2 - Total anomalous pulmonary venous connection.
1.5 Classification of the disease or condition
Clinical classification by R. Darling et al. [30]:
Total anomalous pulmonary venous return, supracardiac type (44–49%) – the common pulmonary venous collector, located posterior to the heart, drains into the SVC system. Collector obstruction is observed in 45% of cases [49].
Total anomalous pulmonary venous return, cardiac type (16–21%) – the common pulmonary venous collector drains into the coronary sinus or directly into the right atrium. Obstruction occurs in 20% of patients [49].
Total anomalous pulmonary venous return, infracardiac type (26–28%) – the common pulmonary venous collector drains into the portal vein, ductus venosus, hepatic vein, or IVC. The pulmonary venous collector penetrates the diaphragm in the region of the esophageal hiatus. The infracardiac type carries the highest risk of pulmonary venous flow obstruction (85%) [49].
Total anomalous pulmonary venous return, mixed type (5–9%) – this type represents any combination of pulmonary venous drainage at two or more levels. This form is frequently associated with other complex congenital heart defects and heterotaxy syndrome [49].
1.6 Clinical presentation of the disease or condition
The clinical course of TAPVR is determined by the anatomical and hemodynamic features of the defect, in particular the degree of pulmonary venous return obstruction, the degree of restriction of the interatrial communication, the magnitude and rate of natural decrease in total pulmonary vascular resistance, the state of the right ventricular myocardium, the degree of left ventricular cavity restriction, and, in the presence of restrictive systemic blood flow, the presence of a functioning arterial duct.
TAPVR without pulmonary venous obstruction
At birth, symptoms are absent. Soon after, half of the infants develop dyspnea, cough, feeding difficulties, recurrent respiratory infections, and heart failure. In the remaining patients, symptoms appear by the first year of life. Cyanosis may appear at any age. Initially, cyanosis is mild and increases in the presence of heart failure, as well as a result of gradually developing secondary pulmonary vascular changes [31].
TAPVR with pulmonary venous obstruction
Symptoms usually do not manifest during the first 12 hours of life, which helps differentiate this defect from respiratory distress syndrome. Symptoms of obstructive TAPVR include progressive dyspnea, feeding difficulties, and signs of heart failure. Infants die between 2 days and 4 months of life [32]. Infracardiac drainage is characterized by cyanosis and dyspnea that worsen with exertion and swallowing due to increased intra-abdominal pressure or esophageal compression of the pulmonary venous collector.
2. Diagnostics, medical indications and contraindications for diagnostic methods
The diagnosis of total anomalous pulmonary venous return is established based on:
1. Transthoracic echocardiography
2. Contrast-enhanced cardiac computed tomography / contrast-enhanced cardiac magnetic resonance imaging
3. Cardiac catheterization
2.1 Complaints and Anamnesis
At the stage of diagnosis clarification, it is recommended to take history and assess complaints in all patients (patients' parents) with suspected TAPVR to verify the diagnosis [39,48].
LoE C, GoR 5
Comments: during the history-taking and symptom assessment, it is recommended to question the patient (or the patient's parents) about the presence of dyspnea at rest or during physical exertion (feeding), increased fatigue, delayed physical development, susceptibility to colds, the presence and severity of cyanosis, and a history of syncopal and presyncopal episodes.
2.2 Physical Examination
In all patients with TAPVR, it is recommended to perform a physical examination including palpation of the cardiac area and abdomen to determine the severity of the condition. [38,48,49]
LoE C, GoR 5
Comments: The apex beat is enhanced in the majority of patients. Patients with the non-obstructive form of TAPVR show signs of right ventricular heart failure (hepatomegaly, peripheral edema) which manifest at 3-7 weeks of life. The obstructive form of TAPVR is characterized by the manifestation of symptoms of right ventricular heart failure within the first week of life.
During cardiac auscultation, it is recommended to check for heart murmurs in all patients to identify pathological murmurs [50,51,52].
LoE C, GoR 5
Comments: In patients with the non-obstructive form of TAPVR, a soft, blowing systolic murmur is auscultated over the pulmonary artery. The first (I) sound is loud and distinct, followed by an ejection click; the II sound is split and does not change with the respiratory phases. The pulmonary component of the II sound is accentuated. The third (III) sound is heard almost always, maximally at the apex. Murmurs are caused by turbulent flow in the pulmonary outflow tract and tricuspid regurgitation. In half of the cases, a diastolic murmur of increased blood flow through the tricuspid valve is heard low down along the left sternal border.
Patients with the obstructive form of TAPVR often have no pathological murmurs.
2.3 Laboratory Diagnostics
In patients with TAPVR, they are non-specific.
All patients with TAPVR are recommended to undergo an acid-base balance and blood gas analysis to assess the severity of hemodynamic and respiratory disorders and the magnitude of metabolic changes [38,53,63].
LoE C, GoR 5
All patients with TAPVR are recommended to undergo a coagulogram (exploratory hemostasis assay) (activated partial thromboplastin time, prothrombin time, prothrombin index, blood fibrinogen level, blood antithrombin III activity, as well as the international normalized ratio [INR]) to predict the risk of intra- and postoperative bleeding [46,52,53,64].
LoE C, GoR 5
All patients with TAPVR are recommended to undergo a general (clinical) urinalysis and a complete blood count with hemoglobin level and hematocrit evaluation, red blood cell, leukocyte, and platelet count, and erythrocyte sedimentation rate (ESR) measurement as part of the initial examination, during follow-up at least once a year, upon hospital admission, and in case of surgical treatment in the pre- and/or postoperative period if necessary [41,48,52,53,64].
LoE C, GoR 5
Before surgical intervention, all patients with TAPVR are recommended to undergo AB0 blood group typing, Rhesus factor (Rh-antigen D) determination, C, c, E, e, Cw, K, k antigen phenotyping, screening for irregular anti-erythrocyte antibodies, hepatitis B surface antigen (HBsAg) testing in blood, hepatitis C virus antibody testing in blood, Treponema pallidum antibody testing in blood, human immunodeficiency virus HIV-1 IgM/IgG antibody testing in blood, and human immunodeficiency virus HIV-2 IgM/IgG antibody testing in blood to rule out associated HIV infection and hepatitis [2,8,64,68,69,71].
LoE C, GoR 5
All patients with TAPVR, as part of the initial examination, upon hospital admission, in case of surgical treatment in the pre- and/or postoperative period if necessary, and at least once a year during follow-up visits, are recommended to undergo a general biochemical blood test (assessment of blood potassium, sodium, chlorides, glucose, creatinine, total protein, albumin, urea, total bilirubin, free and conjugated bilirubin, lactate dehydrogenase, aspartate aminotransferase, and alanine aminotransferase activity) to evaluate renal and hepatic function, and blood electrolyte levels [2,8,64,70.].
LoE C, GoR 5
2.4 Instrumental Diagnostics
All patients with suspected TAPVC are recommended to undergo transthoracic echocardiography (TTE) [65].
Level of Evidence B, Grade of Recommendation 3
Comments: In the supracardiac form of TAPVC, regardless of the site of connection of the collector, dilatation of the superior vena cava with accelerated (turbulent) blood flow is observed. In the infracardiac form of TAPVC, the collector (descending anomalous vein) assessed by pulsed-wave Doppler mode is characterized by a venous flow spectrum directed towards the abdominal cavity. Flow turbulence in color Doppler flow imaging mode, increased velocity, and loss of phase variability in the continuous-wave and pulsed-wave Doppler spectrum characterize pulmonary-venous flow obstruction [2].
All patients are recommended to undergo chest X-ray to determine the size and configuration of the heart and the condition of the pulmonary circulation. [2,51,52,54]
LoE C, GoR 5
Comments: Various forms of total anomalous pulmonary venous connection are characterized by different radiographic findings. In cases of drainage into the SVC, due to dilatation of its lower part, opacity is revealed, which is usually localized over the right atrium. When draining into the azygos vein, opacity in the form of a round convexity is localized at the mediastinal level, above the right cardiac contour. Drainage into the left brachiocephalic vein is manifested by a "figure-of-eight" dilatation formed by the transverse and vertical veins and the SVC on the right. In the obstructive form, pulmonary vascular marking is enhanced and right ventricular dilatation is present; heart dimensions are sharply increased, and the cardiothoracic ratio (CTR) can reach high values [2,49,50].
All patients are recommended to undergo electrocardiography (ECG) to determine right heart overload, assess cardiac rhythm and conduction. [51,52]
LoE C, GoR 5
Comments: A tall peaked P wave in lead II or right atrial leads, characteristic of right atrial enlargement, is a frequent finding. Right ventricular hypertrophy is typical for both forms of TAPVC, usually manifesting as incomplete right bundle branch block [2].
Patients with TAPVC are recommended to undergo contrast-enhanced computed tomography of the heart and vessels (CT angiography) to clarify the anatomy of the heart defect when planning surgical treatment [2,38,53,54].
LoE C, GoR 5
Comments: Computed tomography of the heart and vessels with contrast enhancement allows clarification of the defect morphology, especially in complex and obstructive forms of TAPVC.
2.5 Other Procedures
Before discharge from the hospital, all patients are recommended to undergo electrocardiography for postoperative monitoring [2,8,72,75].
LoE C, GoR 5
Before discharge from the hospital, all patients are recommended to undergo a transthoracic echocardiogram for postoperative monitoring [2,8,72-74].
LoE C, GoR 5
3. Treatment, including drug and non-drug therapies, diet therapy, and pain management
3.1 Conservative Treatment
Patients with TAPVC presenting with clinical decompensation before surgery are recommended mechanical ventilation with positive end-expiratory pressure to stabilize and prepare them for upcoming surgical treatment [2,37,49,50,51].
LoE C, GoR 5
All patients with TAPVC in case of decompensated heart failure at the pre- and postoperative stages are recommended cardiotonic support (#dopamine** 1–15 mcg/kg/min, #epinephrine** (solution for injection, route of administration: intravenous infusion) at a dose of 0.01–1 mcg/kg/min [49], #levosimendan** 0.05–0.2 mcg/kg/min (concentrate for solution for intravenous infusion) [49], and if blood pressure maintenance is necessary, #norepinephrine** (concentrate for solution for intravenous infusion) at a dose of 0.01–1 mcg/kg/min [49], the duration of drug administration is determined individually in each specific case) and diuretic therapy, in cases where circulatory decompensation is accompanied by edematous syndrome (#furosemide** (solution for intravenous and intramuscular injection, route of administration: intravenous) at a dose of 1 mg/kg/day (but not exceeding 20 mg/kg/day) [49], spironolactone** (tablets; route of administration orally as a suspension) at a dose of 0.5–3 mg/kg/day) [49].
LoE C, GoR 5
Patients with the obstructive form of TAPVC are recommended postoperative inhalation therapy with dinitrogen monoxide**(NO2). [50,67]
GoR C, LoE 4
Comments: According to several authors, this approach helps reduce the potential risk of postoperative pulmonary hypertensive crises.
It is recommended to administer prostaglandins in the preoperative period to patients with TAPVC and restrictive systemic hemodynamics [51,60,67].
GoR C, LoE 4
Comments: In situations with low systemic output due to a restrictive interatrial communication and a small patent ductus arteriosus, dilating the ductus with prostaglandins can lead to increased right-to-left shunting. However, due to their pulmonary vasodilatory properties, prostaglandins may decrease pulmonary vascular resistance and worsen the clinical condition of patients with obstructive forms of TAPVC.
3.2 Surgical Treatment
A diagnosis of TAPVC is an absolute indication for surgical treatment. The timing of correction is determined by anatomical and hemodynamic features (obstruction of the pulmonary venous confluence, restrictive interatrial communication) [2,38,54].
Patients with the obstructive form of TAPVC are recommended to undergo emergency surgical correction [2,38,49,50].
LoE C, GoR 5
Comments: Temporary clinical stabilization of the patient is not a reason to delay surgery.
In cases of restrictive interatrial communication (when urgent surgical correction is not feasible), patients are recommended to undergo balloon atrioseptostomy [76-79].
LoE C, GoR 5
Comments: The indication for the procedure is the presence of a restrictive interatrial communication with a pressure gradient greater than 6 mm Hg.
When performing anatomical correction of TAPVC regardless of the anatomical type of the anomaly, it is recommended to direct the pulmonary venous return into the left atrium by creating a free communication between the pulmonary venous confluence and the left atrium, interrupting the connection between the pulmonary venous confluence and the systemic venous circulation, and closing the ASD [80-83].
Level of Evidence C, Consensus Level 5
When correcting the supracardiac form of TAPVC using a superior approach, patients are recommended to create an extracardiac anastomosis between the common pulmonary vein and the roof of the left atrium [37,54].
LoE C, GoR 5
Comments: This approach is convenient and does not distort the natural position of the heart and venous structures.
When correcting the supracardiac form of TAPVC with high drainage of the pulmonary veins into the SVC, it is recommended to perform the Warden procedure [52,54,84-86].
LoE C, GoR 5
When correcting the cardiac form of TAPVC, patients are recommended to incise the "roof" of the coronary sinus to ensure free outflow of pulmonary venous blood into the left atrium [2,87-89].
Level of Evidence C, Consensus Level 5
Comments: To reduce the incidence of postoperative bradyarrhythmias, the "fenestration" technique has been proposed, which involves deeply incising the shared wall between the coronary sinus and the left atrium while separately closing the coronary sinus orifice in the right atrium and the fossa ovalis. This technique preserves the tissue between the coronary sinus and the fossa ovalis, where important internodal conduction pathways are located.
When correcting the infracardiac form of TAPVC, patients are recommended to disconnect the confluence from the IVC system, which can help prevent the development of pulmonary venous stenosis [2,51,54,89-94].
LoE C, GoR 5
Comment: If stenosis develops in the late postoperative period, the Lacour-Gayet "sutureless" repair is used; its essence is that the LA is detached from the pulmonary veins, and a communication is created by suturing a patch of pericardium in situ to avoid direct sutures on the pulmonary vein [93-94].
Intraoperatively, it is recommended to eliminate the patency of the vertical vein if, during its trial clamping after defect correction, left atrial pressure does not rise and blood pressure does not drop [90,91,94-97].
LoE C, GoR 5
In cases where the patient's condition cannot be stabilized and emergency radical surgery cannot be performed, preoperative extracorporeal membrane oxygenation (ECMO) is recommended [51,60,61,62].
Level of Evidence C, Consensus Level 4
Comments: ECMO serves as a means to oxygenate the patient's blood, decompress the pulmonary circulation, and increase systemic perfusion in the most extreme cases. Furthermore, survival rates are lower in patients with TAPVC who require preoperative ECMO compared to those who require ECMO after surgical correction [60,61,62].
3.3 Other Treatment
No.
4. Medical Rehabilitation and Sanatorium-Resort Treatment
Regular follow-up by a local pediatrician and a community cardiologist is recommended, with cardiologist evaluations at least once every 6 months after surgical treatment, after which the follow-up frequency is determined on an individual basis [2,57,71,98].
LoE C, GoR 5
Within 6 months after surgical correction of TAPVC under cardiopulmonary bypass in a patient with a complicated postoperative course (heart failure, infectious complications, re-interventions during the same hospitalization), it is recommended to undergo rehabilitation in a specialized cardiac inpatient facility [57].
GoR C, LoE 4
Comments: The following specific complications may occur after TAPVC correction:
- Residual pulmonary hypertension
- Cardiac arrhythmias: sick sinus syndrome, supraventricular tachycardias
- Residual pulmonary venous outflow obstruction resulting in low cardiac output [84-96].
5. Prevention and Dispensary Observation
Dispensary observation of all patients is recommended every 6–12 months; the duration of follow-up is determined individually [57,99-100].
LoE C, GoR 5
Comments: Dispensary observation includes: collection of complaints and medical history; personal history; external examination; palpation, percussion, and auscultation; blood pressure measurement; electrocardiography; echocardiography. Monitoring for late complications of surgical defect correction is performed (pulmonary venous stenosis, cardiac arrhythmias) for their timely management. Echocardiography is performed at least once every three months during the 1st year after surgery.
Echocardiography is recommended for all patients after TAPVC correction during follow-up visits [2,57,70-71,100-101].
LoE C, GoR 5
Comments: For the purpose of indirectly assessing anastomotic patency, presence/absence of gradients, and evaluating myocardial contractility.
ECG is recommended for all patients after TAPVC correction during follow-up visits [38,57,58].
LoE C, GoR 5
Comments: For the purpose of detecting cardiac arrhythmias characteristic of this patient group.
If cardiac arrhythmias are registered in the postoperative period, 24-hour Holter ECG monitoring is recommended every 6 months or more frequently, in addition to standard evaluation, to clarify the nature of the arrhythmia [2,57,75,84,89].
Children who have undergone TAPVC correction surgery are recommended to receive vaccinations no earlier than three months postoperatively [2,8,56,102-104].
LoE C, GoR 5
Infective endocarditis prophylaxis is recommended in patients with residual pulmonary venous stenoses [38,57,66,100].
LoE C, GoR 5
Comments: For any type of congenital heart defect where synthetic materials/prostheses*** were used during correction, bacterial endocarditis prophylaxis is indicated for 6 months after surgery or lifelong if residual shunts, stenosis, or valvular regurgitation persist. Endocarditis prophylaxis is performed, for example, during dental procedures accompanied by damage to the oral mucosa (tooth extraction, manipulation in the periapical zone of the tooth, etc.). The standard antibiotic prophylaxis regimen involves oral administration of amoxicillin** 50 mg/kg (maximum 2.0 g) one hour before the procedure; if oral administration is not possible, ampicillin** is administered at the same dose intramuscularly or intravenously 30 minutes before the procedure. In case of allergy to beta-lactam antibacterials: penicillins (J01C), #cephalexin** 50 mg/kg (maximum 2.0 g) orally 1 hour before the procedure, or #clarithromycin** 15 mg/kg (maximum 500 mg) orally 1 hour before the procedure, or clindamycin 20 mg/kg (maximum 600 mg) orally 1 hour before the procedure is prescribed [66].
6. Organization of Medical Care
Indications for planned hospitalization:
1. Presence of heart failure symptoms;
2. Presence of pulmonary hypertension;
3. Planned surgical treatment.
Indications for emergency hospitalization:
1. Obstructive form of total anomalous pulmonary venous connection
2. Deterioration of the patient's functional status due to progressive circulatory failure symptoms or cardiac arrhythmias;
Indications for patient discharge from the hospital:
1. Absence/compensation of circulatory failure symptoms;
2. When the patient needs to be transferred to another healthcare organization;
3. Upon the written request of the patient or their legal representative, provided that discharge does not threaten the patient's life.
7. Additional Information
The most significant factors determining early and long-term mortality rates in TAPVC are the presence of pulmonary venous obstruction and associated cardiac anomalies [51].
Postoperative pulmonary venous obstruction also has a significant negative impact on mortality, which approaches 40% in the first 3 years after surgery, and is additionally the main cause of reoperations in TAPVC.
Criteria for Evaluating the Quality of Medical Care
№ | Quality criteria | GoR | LoE |
|---|---|---|---|
Diagnosis stage | |||
1 | Patient history and complaints were collected | C | 5 |
2 | Heart auscultation was performed | C | 5 |
3 | Electrocardiogram recording performed | C | 5 |
4 | Chest X-ray performed | C | 5 |
5 | Echocardiography performed | C | 5 |
6 | Cardiac chamber catheterization was performed if indicated | C | 5 |
Conservative and surgical treatment stage | |||
1 | Mechanical ventilation with positive end-expiratory pressure was performed as necessary. | C | 5 |
2 | Cardiotonic agents were prescribed, excluding cardiac glycosides in cases of circulatory failure decompensation. | C | 5 |
3 | Anatomical correction of total anomalous pulmonary venous connection was performed. | C | 5 |
Postoperative monitoring phase | |||
1 | Electrocardiography was recorded prior to hospital discharge. | C | 5 |
2 | Echocardiography was performed before hospital discharge | C | 5 |
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Appendix A1. Working Group Composition
Working Group Members
1. Avramenko A.A. (Samara)
2. Alekyan B.G., Academician of the RAS (Moscow) "Russian Scientific Society of Endovascular Diagnosis and Treatment Specialists"
3. Arakelyan V.S., Prof. (Moscow)
4. Bogdanov V.N. (Chelyabinsk)
5. Boriskov M.V., MD, PhD (Krasnodar)
6. Bokeria L.A., Academician of the RAS, "Association of Cardiovascular Surgeons of Russia", (Moscow)
7. Brodsky A.G., MD, PhD (Surgut)
8. Gavrilov R.Yu., (Volgograd), "Association of Cardiovascular Surgeons of Russia"
9. Grigoryan A.M., MD, PhD (Moscow) "Russian Scientific Society of Endovascular Diagnosis and Treatment Specialists"
10. Gorbatikov K.V., MD, PhD (Tyumen)
11. Gorbatykh Yu.N., MD, PhD (Novosibirsk)
12. Esayan A.A., MD, PhD (Moscow)
13. Kalashnikov S.V., MD, PhD (Moscow)
14. Kim A.I., Prof. (Moscow), "Association of Cardiovascular Surgeons of Russia"
15. Kovalev I.A., MD, PhD (Moscow), "Association of Pediatric Cardiologists of Russia"
16. Kokshenev I.V., Prof. (Moscow), "Association of Cardiovascular Surgeons of Russia"
17. Krivoshchekov E.V., MD, PhD (Tomsk), "Association of Cardiovascular Surgeons of Russia"
18. Kupryashov A.A., Prof. (Moscow)
19. Levchenko E.G., MD, PhD (Moscow)
20. Movsesyan R.R., Corresponding Member of the RAS, (Saint Petersburg), "Association of Cardiovascular Surgeons of Russia"
21. Nalimov K.A., MD, PhD (Khabarovsk)
22. Petrushenko D.Yu., MD, PhD (Kazan)
23. Plotnikov M.V., MD, PhD (Astrakhan), "Association of Cardiovascular Surgeons of Russia"
24. Podzolkov V.P., Academician of the RAS (Moscow), "Association of Cardiovascular Surgeons of Russia"
25. Svobodov A.A., Prof. (Moscow)
26. Sinelnikov Yu.S., MD, PhD (Perm)
27. Teplov P.V., (Krasnoyarsk)
28. Trunina I.I., Prof. (Moscow) "Association of Pediatric Cardiologists of Russia"
29. Shatalov K.V., Prof. (Moscow), "Association of Cardiovascular Surgeons of Russia"
30. Shakhnazaryan E.A. (Moscow)
No conflict of interest. All members of the Working Group confirmed the absence of financial support/conflict of interest that needs to be disclosed.
Appendix A2. Methodology for Developing Clinical Guidelines
Target audience for these clinical guidelines:
Pediatricians;
Cardiologists;
Pediatric cardiologists
Cardiovascular surgeons.
Methods used for collecting/selecting evidence: electronic database search.
Description of methods used to assess the quality and strength of evidence: the evidence base for the recommendations includes publications indexed in the PubMed and Scopus databases. The search depth was 20 years.
Methods used to assess the quality and strength of evidence
expert consensus;
assessment of the quality of recommendations in accordance with the rating scheme (Table S1, S2).
assessment of the strength of evidence in accordance with the rating scheme (Table S3).
Table S1. Scale for assessing 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 controlled trials with meta-analysis |
2 | Individual studies with reference standard control or individual randomized controlled trials and systematic reviews of studies of any design, except randomized controlled trials, with meta-analysis |
3 | Studies without consecutive reference standard control or studies with a reference standard not independent of the test method, or non-randomized comparative studies, including cohort studies |
4 | Non-comparative studies, case report |
5 | Only justification of mechanism of action or expert opinion is available |
Table S2. Scale for assessing levels of evidence reliability (LER) for prevention, treatment, and rehabilitation methods (preventive, therapeutic, and rehabilitative interventions)
LoE | Definition |
|---|---|
1 | Systematic review of RCTs with meta-analysis |
2 | Individual RCTs and systematic reviews of studies of any design except RCTs, with meta-analysis |
3 | Non-randomized comparative studies, including cohort studies |
4 | Non-comparative studies, case reports or case series, case-control studies |
5 | There is only a rationale for the mechanism of action of the intervention (preclinical studies) or expert opinion |
Table А3. Grading system for levels of convincing evidence (LCE) for preventive, diagnostic, treatment, and rehabilitation methods (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 evidence of proper quality; all considered efficacy criteria [outcomes] are unimportant, all studies have low methodological quality, and their conclusions regarding outcomes of interest are inconsistent) |
To minimize potential bias, each study was evaluated independently. Any discrepancies in evaluations were discussed by the full panel of authors. If a consensus could not be reached, an independent expert was involved.
Methods used for formulating recommendations: expert consensus.
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 health protection 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).
"On the Classification and Criteria Used in the Implementation of Medicosocial Expertise of Citizens by Federal State Institutions of Medicosocial Expertise" (Order of the Russian Ministry of Health No. 1024n of December 17, 2015)
Order of the Russian Ministry of Health No. 103n dated February 28, 2019, "On approval of the procedure and timelines for the development of clinical guidelines, their revision, the standard template of clinical guidelines, and requirements for their structure, composition, and scientific validity of the information included in clinical guidelines" (Registered with the Russian Ministry of Justice on May 8, 2019, No. 54588).
Order of the Ministry of Health of the Russian Federation No. 203n dated May 10, 2017, "On approval of the criteria for assessing the quality of medical care".
Order of the Ministry of Health of the Russian Federation No. 804n dated October 13, 2017, "On approval of the nomenclature of medical services".
Order of the Ministry of Health of the Russian Federation No. 700n dated October 7, 2015, "On the nomenclature of specialties of specialists with higher medical and pharmaceutical education"; Order of the Russian Ministry of Health No. 1183n dated December 20, 2012 (as amended on August 1, 2014) "On approval of the Nomenclature of positions of medical workers and pharmaceutical workers".
Order of the Russian Ministry of Health No. 4n dated June 6, 2012 (as amended on September 25, 2014) "On approval of the nomenclature classification of medical devices".
Appendix B. Physician Action Algorithms
Appendix C. Patient Information
What is total anomalous pulmonary venous connection?
Anomalous pulmonary venous connection is a rare congenital heart defect caused by a disruption in the intrauterine development of pulmonary venous return to the heart. Its incidence is less than 1% of all congenital heart defects. The causes are not yet fully understood.
In a healthy heart, there are four pulmonary veins returning blood from the lungs to the left atrium. Two veins bring oxygenated blood from the right lung, and two from the left. In a child with anomalous pulmonary venous connection, one or more veins drain into the right atrium or its tributaries. If all pulmonary veins drain incorrectly, the diagnosis of total anomalous pulmonary venous connection (TAPVC) is established. Three drainage variants are possible. The most common is the supracardiac type; in this form, all pulmonary veins drain into the superior vena cava via a so-called "collector" (an anomalous vein). The next most common drainage type is intracardiac, in which the veins drain into the coronary sinus, a structure located in the right atrium. The least common is the infracardiac drainage type, in which the pulmonary veins drain into the inferior vena cava. Sometimes a mixed type of drainage is observed, where veins return to more than one anomalous site described above. Life support is made possible by the presence of an opening in the wall between the right and left atria—an atrial septal defect.
How does this defect manifest?
The time of onset and severity of symptoms depend on the number of anomalously draining veins and the presence of stenoses (segments of veins blocking normal blood flow). Typically, most children with TAPVC are asymptomatic at birth, and in 50% of children, symptoms appear in the first month of life. These include congestive heart failure (tachypnea, edema, poor weight and height gain) as well as cyanosis (bluish discoloration of the lips, nails, etc.). Venous obstruction can occur at any of the three anomalous sites of entry and lead to severe respiratory failure.
This heart defect can be diagnosed prenatally (before birth) using ultrasound as early as the 18th week. After birth, the child is transferred to a cardiac surgery inpatient facility, where additional diagnostic methods aimed at clarifying the anatomy of the defect are performed. These include: echocardiography, radiography, electrocardiography, and cardiac catheterization.
How to treat?
TAPVC is a complex heart defect, and if left untreated, most children do not survive the first year of life. The essence of treatment is surgery, by which the surgeon redirects the pulmonary veins into the left atrium. The atrial septal defect is closed with a patch. The timing of the surgery depends on the presence or absence of obstruction in the path of venous return. If obstruction is present, surgery is performed on an emergency basis; otherwise, it is performed within the first 6 months of life.
In the long-term period, good results are noted in the majority of children. Possible postoperative complications include:
- obstruction at the site of the "connection" of the veins with the left atrium,
- rhythm disorders,
- respiratory and heart failure,
- infections,
- conditions requiring repeated surgical interventions (bleeding, etc.)
Additionally, children who have undergone heart surgery have a high risk of developing infective endocarditis (a heart infection resulting from bacteria entering through the bloodstream). This can occur during dental procedures, gastrointestinal procedures, and respiratory tract procedures. To prevent this complication, antibacterial drugs must be administered before procedures.
Appendix G. Assessment Scales, Questionnaires, and Other Evaluation Tools
No