List of Abbreviations
PAPVR – partial anomalous pulmonary venous return
AV – arteriovenous
ACG – angiocardiography
ALT – alanine aminotransferase
AST – aspartate aminotransferase
VA – venoarterial
SVC – superior vena cava
CHD – congenital heart disease
PAP – pulmonary artery pressure
ASD – atrial septal defect
VT – ventricular tachycardia
CHD – coronary heart disease
CS – coronary sinus
CT – computed tomography
PA – pulmonary artery
PV – pulmonary veins
PH – pulmonary hypertension
LV – left ventricle
LA – left atrium
PVR – pulmonary vascular resistance
MRI – magnetic resonance imaging
IVC – inferior vena cava
PDA – patent ductus arteriosus
PVR – pulmonary vascular resistance
SVR – systemic vascular resistance
RV – right ventricle
RA – right atrium
ART – atrial reentry tachycardia
SVT – supraventricular tachycardia
HF – heart failure
CVS – cardiovascular system
SSS – sick sinus syndrome
AFL – atrial flutter
AF – atrial fibrillation
LDL-C – low-density lipoprotein cholesterol
PAPVR – partial anomalous pulmonary venous return
TEE – transesophageal echocardiography
ECG – electrocardiography
EchoCG – echocardiography
Qp/Qs – pulmonary-to-systemic blood flow ratio
QRS – electrocardiographic ventricular complex
Sat O2 – blood oxygen saturation
Terms and Definitions
ESC – European Society of Cardiology. The text of these clinical guidelines additionally indicates 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 exposure to a causal factor, prophylactic or therapeutic intervention, all established changes in health status occurring as a consequence of the intervention.
Cardiac catheterization – an invasive procedure performed for therapeutic or diagnostic purposes in cardiovascular pathology by inserting catheters into the heart cavity or the lumen of great vessels.
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.
Scimitar syndrome – an infracardiac form of partial anomalous drainage of the right pulmonary veins into the inferior vena cava.
Thesis-recommendation – a statement reflecting the procedure and correctness of performing a particular medical intervention with proven efficacy and safety.
Partial anomalous pulmonary venous return – a congenital heart anomaly in which one or several (not all) pulmonary veins drain into the right atrium rather than the left atrium, or into one or more systemic venous returns—the coronary sinus, superior, or inferior vena cava.
Level of evidence reliability – the degree of confidence that the observed effect of a medical intervention is true.
Level of убедительности рекомендаций (Class of Recommendation, CoR) – the degree of certainty in the reliability of the intervention effect and that adherence to recommendations will bring more benefit than harm in a specific clinical situation.
Endocarditis – inflammation of the inner lining of the heart, a frequent manifestation of other diseases.
Echocardiography – an ultrasound method aimed at studying morphological and functional changes of the heart and its valve apparatus.
1. Brief information on the disease or condition
1.1 Definition of the disease or condition
Partial anomalous pulmonary venous return (PAPVR) is a congenital heart anomaly in which one or several (not all) pulmonary veins (PV) drain not into the left atrium, but into the right atrium, or the coronary sinus (persistent left superior vena cava), or the superior or inferior vena cava (or their major tributaries). Mixed drainage of several PVs into the aforementioned anatomical structures is possible.
1.2 Etiology and pathogenesis of the disease or condition (group of diseases or conditions)
During embryonic development, blood flow from the buds of the future lungs drains into a single venous visceral plexus, along with the common cardinal, umbilical, and vitelline embryonic veins. As the lung buds develop, the magnitude of pulmonary venous return into this plexus increases, whereas the return from the cardinal and other embryonic veins, conversely, decreases. During further differentiation, part of this plexus gives rise to the venous pulmonary vascular bed, and another part to the portal venous system of the liver. The formed embryonic pulmonary vein eventually connects hemodynamically with the future left atrium (LA), later expanding (in the form of a sinus) and differentiating into four pulmonary veins characteristic of normal anatomy. By the time of birth, the metamorphosis of the posterior wall of the left atrium in the region of entry of the embryonic pulmonary vein is expressed in the formation of 4 separate ostia (two right and two left) of the pulmonary veins. A rare variant of the norm may be partial persistence of a single pulmonary vein in the form of expansion (ampulla) of the posterior wall of the LA, into which 4 pulmonary veins drain.
In case of impaired embryonic development, pulmonary veins may completely (the common pulmonary vein transforms into a collector in total anomalous pulmonary venous return) or partially retain their hemodynamic connection with the cardinal veins and/or the developing portal venous system, having lost connection with the LA. As a result, venous return from the lungs is partially (in PAPVR) or completely (in total anomalous drainage) carried out into the great systemic veins, the sinus part of the right atrium (part of the upper right cardinal vein), or the portal vein [1-3].
1.3 Epidemiology of the Disease or Condition
PAPVR is a rare congenital heart defect, detected in 0.6–0.9% of autopsy studies. It is typically associated with an interatrial communication. However, the interatrial septum may be intact in rare cases [3].
1.4 Features of Coding the Disease or Condition
Congenital malformations of great veins (Q26):
Q26.3 – Partial anomalous pulmonary venous return.
1.5 Classification of the disease or condition
PAPVR is conventionally named (classified) by indicating which pulmonary vein(s) anomalously drain and where. The presence or absence of an atrial septal defect (ASD) or patent foramen ovale is also specifically indicated.
Two anatomical forms of PAPVR have trivial designations:
Accounting for about 65% of all cases, the so-called PAPVR with a "sinus venosus" atrial septal defect, in which the right upper lobe and (less commonly) middle lobe PVs drain into the ostial portion of the superior vena cava (SVC), and another mandatory morphological component is a "sinus venosus" type ASD [4].
A rare clinical and morphological variant of PAPVR, characterized by a much more severe natural history and poor prognosis, which predetermines the specifics of treatment and its outcomes, is the so-called "scimitar syndrome" ("Turkish sabre" syndrome). The name of this form is associated with a radiological phenomenon in the form of a characteristic shadow in the right part of the posteroanterior chest radiograph, caused by enlarged descending veins usually from the lower lobe and sometimes the middle lobe of the right lung. Much less commonly, all right pulmonary veins descend and drain into the ostial part of the inferior vena cava (IVC) immediately below or above the diaphragm. Characteristic clinical and morphological signs of scimitar syndrome are: dextrocardia; hypoplasia of the right lung and the right pulmonary artery system; large aortopulmonary collateral arteries to the lower lobe of the right lung, which are usually the dominant source of its blood supply and predetermine the development of secondary changes in the pulmonary muscular arterioles (pulmonary hypertension). An ASD or patent foramen ovale is found in 70% of patients [5,6].
Up to 90% of all cases consist of anomalous drainage of the right PVs. The rarest forms are isolated drainage of one or both left pulmonary veins into an accessory left superior vena cava or the left brachiocephalic vein via a vertical vein [7].
1.6 Clinical presentation of the disease or condition
The clinical presentation of PAPVC is predetermined by the magnitude of the left-to-right blood shunt at the venoatrial level, which in turn depends on the number and size of the anomalously draining pulmonary veins. In rare cases of partial anomalous drainage of more than two pulmonary veins, the clinical course may be influenced by the presence and size of an interatrial communication, similarly to total anomalous pulmonary venous connection. In patients with scimitar syndrome, the manifestation of the defect is significantly influenced by the degree of obstruction of the anomalously draining pulmonary veins at their entry site into the IVC, as well as the severity of collateral pulmonary blood flow in the right lung and the degree of its hypoplasia.
In typical cases of anomalous drainage of 1–2 pulmonary veins with or without an interatrial communication, the left-to-right shunt at the venoatrial level determines a mild, prolonged progression of pulmonary hypervolemia with clinical manifestation in older childhood. Frequently, such forms of PAPVC are an accidental finding during routine examination of "healthy" children or manifest through the development of supraventricular rhythm disturbances caused by right atrial dilation [7].
The clinical presentation of PAPVC consists of decreased exercise tolerance, a predisposition to respiratory infections, mild to moderate manifestations of circulatory failure, and uncharacteristic dyspnea upon exertion. Heart rhythm disturbances are observed against the background of right atrial dilation.
It is important to note that clinical manifestation of PAPVC during the first year of life is possible in anomalous drainage of more than two pulmonary veins, in patients with a practically complete absence of the interatrial septum (functionally single atrium), but is also characteristic of patients with scimitar syndrome. Already in the first months of life, these patients lag behind in physical development, and exhibit dyspnea, cyanosis upon exertion, and pronounced manifestations of heart failure. The presence of a large number of aortopulmonary and bronchial collaterals and/or obstruction in the pathway of anomalous venous outflow from the right lung into the IVC and/or secondary changes in the hypoplastic right lung supplement the clinical picture in these patients with manifestations of severe respiratory failure, metabolic disorders of blood gas composition and acid-base balance, and symptoms of right lung edema (moist rales, hemoptysis) [8,9].
2. Diagnostics, medical indications and contraindications for diagnostic methods
The diagnosis of PAPVC is verified based on the summary data of patient/parent complaints, clinical manifestations of the defect, and laboratory and instrumental research methods.
The diagnosis of PAPVC is established on the basis of:
Electrocardiography;
Transthoracic echocardiography;
Contrast-enhanced computed tomography of the heart, or magnetic resonance imaging of the heart and great vessels (with contrast);
Transvenous heart catheterization (angiography of the pulmonary artery and its branches).
2.1 Complaints and Anamnesis
When taking the medical history of patients with suspected PAPVC, it is recommended to determine the presence of congenital heart disease in close relatives and to clarify the features of the mother's pregnancy in order to facilitate the verification of the congenital heart defect [9-11].
ESC/ROS IIa, LoE C, GoR 5
When eliciting complaints from patients with suspected PAPVC, it is recommended to check for the presence of dyspnea, rapid and/or irregular heartbeat, fatigue (in infants — excessive sweating, feeding difficulties) upon physical exertion; delayed physical development rates; and a predisposition to respiratory infectious diseases [9-11].
ESC IIa C, LoE C, GoR 5
Comments: in childhood, symptoms of circulatory failure are usually absent or mild, and manifest as weakness and sweating of the child during physical exertion.
2.2 Physical Examination
It is recommended to perform a physical examination in all patients with suspected PAPVC to verify the diagnosis [9-11].
ESC IB, LoE C, GoR 5
Comments: Cardiac auscultation reveals a systolic murmur with its maximum intensity in the second to third intercostal space to the left of the sternum. In large blood shunts, some patients have a soft diastolic murmur heard over the tricuspid valve; the second heart sound over the pulmonary artery is widely split regardless of respiratory phases. In the presence of pulmonary hypertension, the pulmonary component of the second sound is accentuated. Children often have an asthenic build with noticeable pallor of the skin.
2.3 Laboratory Diagnostics
There are no specific laboratory diagnostics for PDA.
It is recommended for all patients with PAPVC to undergo a general (clinical) urinalysis and a general (clinical) blood test including the study of blood hemoglobin level and hematocrit evaluation, red blood cell, white blood cell, and platelet counts in the blood, and erythrocyte sedimentation rate testing as part of the initial examination, during follow-up monitoring at least once a year and upon admission to the hospital, as well as in the case of surgical treatment if necessary in the pre- and/or postoperative period for all patients with PAPVC [13-16].
ESC/ROS IC, LoE C, GoR 5
Comment: Performing a general (clinical) blood test for all patients with PAPVC during dispensary follow-up and prior to surgical treatment is important from the standpoint of detecting anemia and timely medical correction. In patients with decreased hemoglobin and hematocrit levels, it is advisable to further study serum iron levels, serum ferritin levels, serum transferrin levels, and total iron-binding capacity of serum for the timely correction of iron deficiency states capable of affecting the clinical and functional status and the course of the patient's disease [16].
It is recommended for all patients with PAPVC admitted to the hospital for surgical treatment to undergo a coagulogram (exploratory hemostasis study) (activated partial thromboplastin time, blood fibrinogen level, and determination of the international normalized ratio (INR)) to exclude congenital and acquired disorders in the blood coagulation system, and to predict postoperative thrombosis and bleeding [15,20,51,52].
ESC/ROS IC, LoE C, GoR 5
It is recommended to perform a general general-medical biochemical blood test (study of blood potassium level, blood sodium level, blood chloride level, blood glucose level, blood creatinine level, total blood protein level, blood albumin level, blood urea level, total blood bilirubin level, free and conjugated blood bilirubin levels, lactate dehydrogenase activity in the blood, aspartate aminotransferase activity in the blood, alanine aminotransferase activity in the blood, and C-reactive protein level in blood serum) to assess renal and hepatic function and exclude an inflammatory process in all patients with PAPVC as part of the initial examination, upon admission to the hospital, in the case of surgical treatment if necessary in the pre- and/or postoperative period, and at least once a year at a follow-up visit during dynamic monitoring [13,15].
ESC/ROS IC, LoE C, GoR 5
Comment: If there are signs of thyroid dysfunction, it is necessary to additionally test the levels of free triiodothyronine (FT3) in the blood, free thyroxine (FT4) in blood serum, and thyroid-stimulating hormone (TSH) in the blood.
It is recommended for all patients with PAPVC admitted to the hospital for surgical treatment who are expected to receive transfusions of donor blood and/or its components to determine the main ABO blood groups, Rh blood system D antigen (Rh factor), phenotype regarding C, c, E, e, Cw, K, k antigens, and to screen for anti-erythrocyte antibodies [18,19,53,54].
ESC/ROS II AC, LoE C, GoR 5
It is recommended for all patients admitted to the hospital for surgical treatment to determine hepatitis B virus surface antigen (HBsAg) in the blood; antibodies to hepatitis C virus in the blood; antibodies to Treponema pallidum in the blood; IgM and IgG antibodies to human immunodeficiency virus HIV-1 in the blood; IgM and IgG antibodies to human immunodeficiency virus HIV-2 in the blood to exclude association with HIV infection, hepatitis, and syphilis [11,15,18-20,55,60-61,77-79].
ESC/ROS II AC, LoE C, GoR 5
It is recommended to study the blood cholesterol level, blood triglycerides level, and low-density lipoprotein cholesterol (LDL-C) level in the blood to identify risk factors for concomitant atherosclerosis and, if necessary, adjust therapy in patients with PAPVC over 18 years of age (usually patients over 40 years of age) as part of the initial examination, as well as in the case of surgical treatment if necessary in the preoperative period [15].
ESC IIIC, LoE C, GoR 5
Рекомендуется blood testing for N-terminal pro-brain natriuretic peptide (NT-proBNP) in all PAPVR patients presenting with signs of heart failure, and subsequently as needed, for mortality risk stratification, as well as in the event of surgical treatment during the pre- and/or postoperative period, if required [17,50,56-59].
ESC/SSC IIa C, Level of Evidence C, Grade of Recommendation 2
Comments: Signs of metabolic acidosis — a drop in blood pH down to 7.0; elevated NT-proBNP levels exceeding 125 pg/mL are predictors of the need for emergency surgical intervention.
2.4 Instrumental Diagnostics
The primary imaging modality in the diagnosis of PAPVR is echocardiography (EchoCG).
Recommendations apply to all patients with suspected PAPVR and during follow-up dispensary monitoring to detect cardiac rhythm and conduction disorders, hypertrophy/overload of the right atrium and right ventricle, and electrical axis deviation, by performing a resting electrocardiogram (ECG) (decoding, description, and interpretation of 12-lead ECG data), as well as in the event of surgical treatment in the pre- and postoperative periods, if required [13,14].
ESC/SSC I B, Level of Evidence C, Grade of Recommendation 5
Comments: PAPVR is typically characterized by right axis deviation (+100 to +180°), RV myocardial hypertrophy, incomplete right bundle branch block, and signs of right atrial overload.
It is recommended for all patients with suspected PAPVR and during follow-up to perform targeted chest radiography for diagnostic purposes, both as the primary method and to rule out other heart and great vessel diseases, as well as extracardiac pathology (pathology of other mediastinal organs, lungs, pleura), to clarify the anatomical features of this congenital heart disease, the state of pulmonary circulation, and to detect pulmonary congestion, as well as in the event of surgical treatment in the pre- and/or postoperative periods, if required [20,32].
ESC/SSC IB, Level of Evidence C, Grade of Recommendation 5
Comments. Chest radiography allows for the detection of changes in the pulmonary arterial bed associated with hypervolemia or pulmonary arterial hypertension, signs of RV and/or RA enlargement, and bulging of the pulmonary artery arch.
Most PAPVR patients do not exhibit any specific radiographic pattern. The radiographic pattern in patients with Scimitar syndrome is specific, primarily due to the shadow of the descending anomalously draining dilated pulmonary veins resembling the silhouette of a Turkish saber (hence the name). In addition, these patients may show signs of right lung hypoplasia, atelectasis of its segments, varying degrees of prominence of the pulmonary vascular pattern, and displacement of the cardiac shadow to the right due to compensatory hyperinflation of the healthy (left) lung.
It is recommended to perform 24-hour Holter ECG monitoring in all PAPVR patients with concomitant rhythm and/or conduction disorders to detect the latter and, if necessary, select therapy, as well as in the case of surgical treatment in the pre- and/or postoperative periods, if required [15,21,26,27].
ESC/SSC IIa C, Level of Evidence C, Grade of Recommendation 5
It is recommended for all patients with suspected PAPVR and during dispensary follow-up to perform transthoracic echocardiography (TTE) with color Doppler mapping for a detailed assessment of intracardiac anatomy and hemodynamics, as well as in the event of surgical treatment in the pre- and/or postoperative periods, if required [15,22,23].
ESC/SSC I A, Level of Evidence C, Grade of Recommendation 5
Comments: PAPVR can be suspected based on the absence of the characteristic blood flow from the right or left pulmonary veins into the left atrium on color Doppler mapping. In PAPVR, dilation and accelerated blood flow are noted in the superior vena cava.
Difficulties may arise in diagnosing PAPVR of the left pulmonary veins into the coronary sinus in the presence of a persistent left superior vena cava and mixed forms of PAPVR. When assessing pulmonary venous flow using Doppler mode, stenosis of the vein at the site of its entry into the right atrium or venae cavae may be identified.
Echocardiography is necessary to assess the dimensions of the right and left heart chambers, the location and size of the atrial septal defect (ASD), the severity of RV, right atrial, and pulmonary trunk dilation. Subcostal (subxiphoid), parasternal, and apical views are used to visualize the pulmonary venous ostia. Systolic pressure in the right ventricle is determined from tricuspid regurgitation using continuous-wave Doppler mode. Calculation of the pulmonary-to-systemic blood flow ratio (Qp/Qs) is mandatory, with values greater than 1.5 considered significant. [22,23].
Transesophageal echocardiography is recommended for patients with PAPVD when clinically indicated and when transthoracic echocardiography visualization is suboptimal, in order to perform a detailed assessment of the valvular apparatus and ASD anatomical features, rule out infective endocarditis, evaluate the results of reconstructive surgery intraoperatively in cases of surgical treatment, and in the pre- and postoperative periods when necessary [24,25].
ESC/SSC IIa B, Level of Evidence C, Grade of Recommendation 5
Comments: Transesophageal EchoCG should not be used for routine or serial evaluation of PAPVR when transthoracic echocardiographic visualization is adequate. Transesophageal echocardiography is most effective in the presence of a sinus venosus ASD.
Transvenous cardiac catheterization and angiography of the pulmonary artery and its branches are recommended for all patients to clarify intracardiac anatomy (if echocardiographic data are ambiguous) prior to surgery, determine the degree of hemodynamic impairment, and measure pressure in the right heart chambers and vessels to assess the severity of pulmonary hypertension [13,14].
ESC/SSC IIa C, Level of Evidence C, Grade of Recommendation 5
Comments: The study is necessary to detect hemodynamic disorders present in PAPVR patients, such as elevated RV pressure.
Coronary angiography is indicated for men over 40 years of age and postmenopausal women regardless of age prior to surgical treatment if coronary artery disease is suspected.
Patients with Scimitar syndrome are recommended to undergo transvenous cardiac catheterization and angiography of the pulmonary artery and its branches with assessment of pulmonary venous return during the venous phase, along with selective angiography of large aortopulmonary collaterals [11,12,31].
ESC/RCS IIa C, LoE C, GoR 5
Comments: For the majority of PAPVR patients, contrast-enhanced CT or contrast-enhanced MRI is sufficient as an additional imaging modality. Determining the surgical strategy in patients with Scimitar syndrome requires angiography to assess the degree of right pulmonary artery hypoplasia, evaluate collateral pulmonary blood flow, and study the anatomy of the right pulmonary veins.
Contrast-enhanced cardiac computed tomography (if available) is recommended for all PAPVR patients upon initial presentation (especially in cases of inconclusive echocardiographic results), as well as in the event of surgical treatment in the pre- and/or postoperative periods, if required [28,62-65].
ESC/SSC IIa C, Level of Evidence C, Grade of Recommendation 2
Comments: The procedure must be performed in all cases to detail the anomalous drainage of pulmonary veins into the systemic venous return (innominate and superior vena cava, coronary sinus, persistent left superior vena cava, inferior vena cava, portal vein). In newborns and infants, contrast-enhanced cardiac computed tomography is the modality of choice for diagnosing the intracardiac anatomy of the anomaly, pulmonary artery stenoses, and detecting associated congenital heart disease.
It is recommended that all patients with PAPVR undergo cardiac and great vessel magnetic resonance imaging to assess structural and functional changes (if necessary, or if the results of other imaging modalities are inconclusive), as well as in the event of surgical treatment, and when clinically indicated in the pre- and/or postoperative periods [29,30].
ESC/RCS IIa C, LoE C, GoR 5
Comments: Cardiac MRI enhances imaging capabilities, particularly for assessing right heart chamber overload, provides anatomical reconstructions with high spatial and temporal resolution, and allows for volumetric measurements, vascular assessment, and myocardial fibrosis evaluation. MRI is used in adults in the following clinical situations: as an adjunct method when echocardiographic data are ambiguous or borderline, such as LV and RV volumes and ejection fractions, which can be critical for treatment planning in adult PAPVR patients when evaluating the volume of overload and valvular regurgitation.
MRI is more informative compared to echocardiography for the quantitative assessment of RV volumes and ejection fraction, detection and quantification of myocardial fibrosis/scar (late gadolinium enhancement); tissue characterization (fibrosis, fat, etc.). The degree of fibrosis is associated with risk factors for cardiac rhythm and conduction disorders [30].
2.5 Other Procedures
It is recommended that all patients with PAPVD undergo electrocardiography prior to hospital discharge for postoperative monitoring to detect potential rhythm and conduction disorders [15,20,21,26,27].
ESC/RCS IIa C, LoE C, GoR 5
It is recommended that all patients with PAPVD undergo echocardiography prior to hospital discharge for postoperative monitoring to assess the efficacy of surgical correction of the defect [15,20,22-25].
ESC/RCS IIa C, LoE C, GoR 5
3. Treatment, including drug and non-drug therapies, diet therapy, and pain management
3.1 Conservative Treatment
Anomalous drainage of 1-2 pulmonary veins is usually pauci- or asymptomatic and does not require pharmacotherapy.
Pharmacotherapy is not recommended in asymptomatic patients with PAPVD [15].
No ESC recommendation, LoE C, GoR 5
Comments: Conservative treatment is prescribed for patients with PAPVD who develop signs of heart failure, supraventricular tachyarrhythmias, pulmonary hypertension, or when it is necessary to delay surgical correction of the defect. Treatment is individualized and performed by an experienced pediatric cardiologist or adult cardiologist [15].
It is recommended to prescribe loop diuretics to patients (typically adults) with PAPVD upon the development of signs of heart failure, including chronic heart failure associated with other cardiovascular diseases in adulthood [11,12,15,33,36,37].
ESC/HRS IIaB, LOC C, LE 5
Comments: The initial single dose of furosemide** in children is calculated as 1 mg/kg body weight/day, with a possible increase up to a maximum of 6 mg/kg/day if indicated, provided the drug is administered no more frequently than every 6 hours (tableted forms are contraindicated in children under 3 years of age). The initial dose of furosemide** for adults is 20–80 mg/day, divided into 2–3 doses.
Clinical data demonstrate marked symptomatic improvement with loop diuretics in patients with heart failure. Diuretic administration should avoid forced diuresis, and systemic blood pressure, electrolyte levels, blood creatinine, urea, and hematocrit must be closely monitored to prevent hemoconcentration in response to reduced intravascular blood volume, hypokalemia, and prerenal failure [15].
Diuretics are recommended for adult patients with overt heart failure, including chronic heart failure, for the relief of edema [15,38,39].
ESC/HRS IIaB, LOC C, LE 5
Comments: The choice of diuretic therapy in adult patients after surgical correction of PAPVD or with uncorrected PAPVD is not limited to loop diuretics (furosemide**, torasemide) but also includes aldosterone antagonists and other potassium-sparing agents; thiazide and thiazide-like diuretics may also be used under careful monitoring of blood electrolyte composition and diuresis dynamics. Existing data on the efficacy of valsartan/sacubitril** (angiotensin II receptor blockers in combination with other agents) in patients with systolic dysfunction and complex congenital heart disease cannot yet be safely translated into clinical practice [39].
It is recommended that patients over 25 years of age with PAPVD who develop signs of heart failure (single or biventricular dysfunction, potentially associated with other cardiovascular conditions) receive treatment in accordance with general ESC guidelines for chronic heart failure [15,39].
ESC/HRS IIaB, LOC C, LE 5
It is recommended that all patients with PAPVD and flutter/fibrillation with rapid ventricular conduction (and potential development of ventricular tachycardia, which can cause sudden cardiac death) undergo closer monitoring and, when indicated, specialized therapy [66-70].
ESC/HRS IIbC, LOC C, LE 5
Comments: For patients with nonsustained ventricular tachycardias without hemodynamic compromise, specific treatment is generally omitted, or drugs suppressing ectopic focus activity (beta-blockers) are prescribed. Patients with syncopal or presyncopal episodes receive more "aggressive" treatment. This includes antiarrhythmic therapy and implantable cardioverter-defibrillator placement, which is the sole method for preventing sudden cardiac death in this patient population [15,26].
It is recommended that all patients older than 25 years (or younger if indicated) with PAPVD and signs of pulmonary hypertension based on instrumental diagnostics (echocardiography, transvenous cardiac catheterization determining PVR and vasoreactivity testing) be evaluated for pre-operative pulmonary arterial hypertension (PAH)-specific therapy according to PAH assessment criteria and treatment indications in the ESC pulmonary hypertension guidelines [15,37,40,41].
ESC IC, LOC A, LE 4
Comments: Patients with PAPVD developing pulmonary hypertension require particularly thorough evaluation. Determining PVR is essential.
It has been demonstrated that with a PVR of less than 5 Wood units, ASD closure is safe and associated with a reduction in pulmonary hypertension and clinical improvement [15,41]. However, even within this patient group, the degree of improvement diminishes as PVR increases.
In patients with a PVR ≥5 Wood units, the likelihood of improvement is minimal, while complete ASD closure may even worsen prognosis [15,37,40]. When deciding on ASD closure in patients with a PVR ≥5 Wood units, vasoreactivity testing is not recommended. It appears safer to initiate PAH therapy, re-evaluate hemodynamic parameters during follow-up, and consider defect closure with a fenestrated patch only if PVR decreases to <5 Wood units in the presence of a significant left-to-right shunt. If these conditions are not met, ASD closure should be avoided [15,37].
3.2 Surgical Treatment
It is recommended to prioritize surgical correction of PAPVD in childhood before the development of complications (supraventricular tachyarrhythmias, biventricular dysfunction, pulmonary hypertension) [4,15,33,34,71].
ESC IIaC, LOC C, LE 5
Comments: A positive impact on morbidity is achieved with defect correction at any age (improved exercise tolerance, reduced dyspnea, right heart failure), but once complications develop, quality of life differs significantly from patients with an uncomplicated course. Therefore, early correction (timely intervention) is preferred [15].
Surgical treatment of PAPVD is recommended for patients with a pulmonary-to-systemic blood flow ratio (Qp/Qs) greater than 1.5 [4,7,10, 11,13,41-44,71].
ESC/HRS IC, LOC C, LE 4
Comments: The optimal age for intervention upon diagnosis is the 2nd–3rd year of life; however, with clinical manifestation of the defect, surgery may be performed before 1 year of age. Correction of scimitar syndrome with lung hypoplasia is indicated to prevent pulmonary sequestration via lung resection, regardless of shunt magnitude, at an early age [41,42].
There are three surgical options for correcting anomalous drainage of the right pulmonary veins into the superior vena cava or right atrium:
Intraatrial baffling using a synthetic cardiovascular patch*** or an animal-derived cardiovascular patch***. The atrial septal defect is closed (or created/additionally enlarged if necessary) with a patch in such a way as to redirect blood flow from the orifices of the anomalously draining pulmonary veins through the ASD into the left atrium, while avoiding stenosis of the anomalous pulmonary vein orifices, the superior vena cava orifice, and restriction of the interatrial communication.
Another correction method combines the aforementioned approach with additional patch enlargement of the superior vena cava orifice.
The third approach (the Warden procedure) is used when anomalous pulmonary veins drain relatively high into the superior vena cava, making baffling unfeasible. It involves transecting the superior vena cava such that its proximal segment, including the orifices of the anomalously draining pulmonary veins, forms a cuff through which pulmonary venous return descends to the right atrium and then flows under a patch (sutured along the edge of the superior vena cava orifice) through the ASD into the left atrium. The distal segment of the transected superior vena cava, including (if possible) the azygos vein orifice, is anastomosed to the apex of the right atrial appendage, potentially utilizing various methods of additional cavoatrial junction augmentation [4,7,43,44].
It is recommended that adult patients with partial anomalous pulmonary venous return (PAPVR) and atrial flutter/atrial fibrillation (AFL/AF) be evaluated regarding the advisability of cryoablation of arrhythmogenic zones or radiofrequency ablation of arrhythmogenic zones (modified maze procedure) prior to or during surgery. Closure of an atrial septal defect (ASD) may limit access to the left atrium if an electrophysiological intervention is performed at a later stage [15,69-70,72].
ESC/HRS IIbC, LOC C, LE 5
Comments: catheter ablation for AFL/AF can be considered provided that the procedure is performed in specialized centers and ablation endpoints can potentially be achieved (e.g., inability to induce arrhythmia, presence of conduction block across ablation lines) [15,26].
In patients with scimitar syndrome, surgical treatment is recommended immediately upon diagnosis, regardless of age and the severity of clinical manifestations [7,8,11,45-47].
No ESC recommendation, LoE C, GoR 5
Comments: There are 2 options for surgical correction of PAPVR in scimitar syndrome:
Tunneling with an extended patch (synthetic cardiovascular patch*** or animal-derived cardiovascular patch***) from the orifice of the anomalously draining pulmonary vein from the inferior vena cava to the ASD in the left atrium. This method carries the risk of obstruction and/or thrombosis of the intraatrial tunnel, stenosis of the inferior vena cava orifice, and the development of cardiac arrhythmias. The lower the pulmonary vein drains, the longer the tunnel must be formed, and the higher the risk of complications.
Transection of the anomalously draining pulmonary vein from the inferior vena cava and its reimplantation into the right atrial wall closer to the ASD or directly into the left atrial wall. Additional augmentation of the transplanted pulmonary vein may be performed to prevent kinking or stenosis of the anastomosis. ASDs in such cases are closed taking into account the location of the transplanted pulmonary vein anastomosis.
The issue of the necessity, timing, and methods of closing aortopulmonary collaterals (if present) is considered separately, depending on their hemodynamic and clinical significance. Collaterals may be closed interventionally (embolization) or surgically ligated.
The issue of preserving or resecting the compromised lower/middle lobe of the right hypoplastic lung is considered separately. Pronounced inflammatory processes, sequestration, marked deformation, and bronchial patency disorders in the affected lobe(s) of the right lung are grounds for considering lobectomy [7,8].
3.3 Other Treatment
No.
4. Medical Rehabilitation and Sanatorium-Resort Treatment
It is recommended that within 6 months after surgical correction of PAPVR under cardiopulmonary bypass, a patient with a complicated postoperative course undergoes rehabilitation in a specialized cardiological inpatient facility [15,20,21,29,33,40,48,49].
No ESC recommendation, LoE C, GoR 5
Comments:
The following conditions may occur after radical correction of the defect:
Postcardiotomy syndrome and cardiac tamponade may develop several weeks after surgical correction of PAPVR. Therefore, symptoms such as fever, fatigue, vomiting, chest pain, or abdominal pain may appear. Immediate echocardiography is required to rule out this complication. Monitoring and (if necessary) treatment in accordance with established guidelines [20,21,29].
Residual interatrial shunting may occur in 5% of cases following defect correction. Reoperation is indicated for hemodynamically significant blood shunting [33,48].
Stenoses of the ostia and obstructions of systemic and pulmonary veins may occur after defect correction. In such cases, reoperation is indicated for a hemodynamically significant degree of venous lumen obstruction. Catheter intervention is possible if technically feasible and the stenosis morphology is appropriate [15,33].
Low-velocity venous blood flow increases the risk of thrombosis in the veins on which the intervention was performed, especially in scimitar syndrome, which requires the prompt prescription of anticoagulant therapy [15].
Cardiac rhythm and conduction disturbances (supraventricular tachyarrhythmias, sick sinus syndrome, and sudden cardiac death) may occur both immediately after surgical correction and at later follow-up periods [26,27].
Residual pulmonary arterial hypertension (PAH) may persist in up to 5% of cases after defect correction. Monitoring and (if necessary) prescription of PAH-specific therapy are carried out based on established PH guidelines [15,37,40].
Neurological disorders – encephalopathy, seizure syndrome, etc., occur in up to 2% of cases and require ongoing follow-up by a neurologist at the patient's place of residence [36].
It is recommended to restrict physical activity for all patients immediately after surgical correction [15,27,33,48,49].
No ESC recommendation, LoE C, GoR 5
Comments: It is optimal to restrict physical activity for 3–6 months from the date of discharge from the hospital in an uncomplicated postoperative course.
1 year after radical correction, patients may be cleared for all competitive sports in the absence of:
signs of a residual ASD,
significant ostial stenosis and obstruction of the systemic and pulmonary veins,
postoperative venous thrombosis,
symptomatic supraventricular tachyarrhythmia and sick sinus syndrome,
signs of ventricular myocardial dysfunction,
residual PAH [15,27].
5. Prevention and Dispensary Observation
The prognosis after surgical correction of PAPVC is favorable provided that the CHD is corrected in a timely manner. Life expectancy and physical performance may be limited in the presence of iatrogenic residual complications [15,20,33].
It is recommended that all patients with PAPVC undergo lifelong follow-up after surgical correction of the defect by a pediatric cardiologist until the age of 18, and by a cardiologist/general practitioner or internist after the age of 18, to determine visit frequency, monitor compliance with prescribed recommendations, modify therapy in a timely manner, assess the risk of cardiovascular complications, refer for additional examinations, and, if necessary, for hospitalization [15,33,39].
ESC/RSC IIa, LoE C, GoR 5
Comments: The frequency of follow-up by a pediatric cardiologist and an adult cardiologist is at 3 (if indicated), 6, and 12 months after surgery. Regular follow-up is advisable during the first 2 years, and then, depending on the results of the medical intervention, every 12–60 months until the age of 18; the patient then transitions to the adult outpatient clinic, where follow-up continues at the same frequency, if indicated, by a cardiologist, general practitioner, or internist. The follow-up protocol includes ECG, echocardiography, cardiac rhythm Holter monitoring, and, if necessary, pulse oximetry.
Patients with ventricular dysfunction, residual atrial shunt, vena cava and pulmonary vein stenosis and occlusion, venous thrombosis, cardiac rhythm and conduction disorders, PAH, and neurological disorders must be followed up annually in specialized centers [15,33].
When determining the frequency of follow-up, one should be guided by the hemodynamic status, presence of iatrogenic residual complications, symptoms of heart failure, presence of cardiac rhythm and conduction disorders, severity of PAH, hypoxemia, etc.
Depending on whether a patient with a corrected or uncorrected defect belongs to a particular functional class, varying follow-up frequencies and scopes of required instrumental examinations are recommended [39]:
Follow-up frequency (months) / Diagnostic methods | FC I within 24 months after surgery, then upon progression of the functional class. | FC II | FC III | FC IV |
Pediatric cardiologist examination | 12 | 12 | 6 | 3 |
Electrocardiography | 12 | 12 | 6-12 | 3-6 |
Holter ECG monitoring (if cardiac arrhythmias are present) | 12 | 12 | 6 | 6 |
Echocardiography (TEE if necessary) | 12 | 12 | 6-12 | 3-6 |
MRI/CT of the heart and great vessels with contrast (if necessary) | 36 | 36 | 12 | 12 |
Pulse oximetry | 12 | 12 | 6 | 3 |
Determination of the type of cardiovascular response to physical exertion (6-minute walk test, and if necessary, exercise testing using an ergometer) | 36 | 24 | 12-24 | 6-12 |
It is recommended that all adolescent patients with PAPVC undergo follow-up before and after surgery by a pediatric cardiologist, with subsequent transfer of the patients to an adult cardiologist [20,39].
ESC/RCS IC, LoE C, GoR
It is recommended that all patients with PAPVC have their clinical status and exercise tolerance assessed during dynamic follow-up [11,12,15,33].
ESC IIA, LoE C, GoR 5
Comments: The test is necessary to determine the optimal physical activity volume, objectively assess cardiac functional capacity, and detect potential arrhythmias. The test is performed using a cycle ergometer test, treadmill test, or possibly a 6-minute walk test with dyspnea assessment using the 10-point Borg scale and pulse oximetry.
It is recommended that adult patients with PAPVC undergo pulse oximetry once a year [4,15,73-75].
ESC/RSC IC, CQR C, LOR 5
Comment: The test is performed to assess the patient's condition, indirectly evaluate pulmonary blood flow, and check for a potential veno-arterial shunt.
It is recommended that all patients with partial anomalous pulmonary venous return (PAPVR), as well as those following its surgical correction, undergo a general (clinical) urinalysis (to assess renal dysfunction) and a complete blood count (evaluating total hemoglobin, red blood cells, white blood cells, platelets, and hematocrit), along with an erythrocyte sedimentation rate (ESR) test (to rule out anemia and inflammatory processes) at least once a year during follow-up [13,14,15].
ESC/RCS IC, LoE C, GoR 5
It is recommended that adult patients with PAPVR after surgical correction, as well as at follow-up control visits, undergo a general routine blood biochemistry panel to assess renal and hepatic function and rule out inflammatory processes [15].
ESC/ROS IC, LoE C, GoR 5
Comments: routine blood biochemistry testing in adult patients with PAPVR may also be performed to identify risk factors for concomitant atherosclerosis for subsequent therapy adjustment if necessary [15].
It is recommended that all patients with PAPVR older than 25 years (at an earlier age if heart failure develops), as well as those after surgical treatment when indicated, undergo blood testing for N-terminal pro-B-type natriuretic peptide (NT-proBNP) to stratify mortality risk [15,50,56-59].
ESC/RCS IIaC, LoE C, GoR 5
It is recommended that all patients after surgical treatment of PAPVR undergo 12-lead resting electrocardiography (ECG), with interpretation and description of ECG data once a year during a control visit and additionally upon the onset of arrhythmia, as well as an ECG once a year when prescribing or modifying treatment that affects intracardiac electrical conduction [15,26,33].
ESC/RSC IB, LoE C, GoR 5
Comments: An ECG should be performed to assess heart rhythm and heart rate. Patients who have undergone radical correction may present with sick sinus syndrome (SSS) with a daytime heart rate dropping below 40 bpm and pauses exceeding 3 seconds, as well as atrial re-entrant tachycardia (ART)/atrial fibrillation (AF) with rapid ventricular conduction and a potential progression to ventricular tachycardia, which is a risk factor for sudden cardiac death. The detection of atrial flutter (AFl), AF, episodes of ventricular tachycardia, or SSS indirectly indicates severe intracardiac hemodynamic disturbances.
It is recommended to perform 24-hour Holter ECG monitoring in all patients with PAPVR and concomitant heart rhythm and/or conduction disturbances in order to detect the latter and, if necessary, select and adjust therapy once a year [15,26,38].
ESC/RCS IIa C, LoE C, GoR 5
Comments: Holter ECG monitoring is necessary to rule out heart rhythm and conduction disturbances that may occur after radical correction of PAPVR. The most severe of these include SSS with a daytime heart rate below 40 bpm and pauses exceeding 3 seconds, as well as ART/AF with rapid ventricular conduction and potential development of ventricular tachycardia, which can cause sudden cardiac death. For patients with short episodes of nonsustained ventricular tachycardia without hemodynamic instability, specific treatment is generally not administered, or drugs suppressing ectopic focus activity (beta-blockers) are prescribed. Patients with syncopal or presyncopal conditions receive more "aggressive" treatment. This includes antiarrhythmic therapy and implantable cardioverter-defibrillator (ICD) placement, which is the sole method for preventing sudden cardiac death in this category of patients.
In patients with ART and AF after radical correction of PAPVR, conservative treatment has low efficacy. In such cases, all patients should undergo an intracardiac electrophysiological study and radiofrequency ablation of arrhythmogenic zones [26, 38].
In case of SSS with a daytime heart rate below 40 bpm and pauses exceeding 3 seconds, pacemaker implantation is required [15,26].
It is recommended that all patients with PAPVR undergo transthoracic echocardiography (TTE) following surgical correction of the defect [15,33,71,76].
ESC/RSC IA, LoE C, GoR 5
Comment: Transthoracic echocardiography and pleural ultrasound are optimally performed 2 to 4 weeks after discharge from the hospital in cases with a complicated pre-operative and postoperative course, as well as during follow-up every 6 months for 2 years after surgery. For longer-term follow-up, they should be performed at least once every 3 years in an uncomplicated course. In cases with a complicated pre-operative and postoperative course, echocardiographic and pleural ultrasound monitoring is performed every 3 to 12 months annually, depending on the heart failure functional class.
Transthoracic echocardiography is used to monitor the hemodynamic significance of a residual atrial septal defect (ASD) and stenosis of the ostia and obstruction of systemic and pulmonary veins, as well as supraventricular tachyarrhythmia and SSS, to evaluate blood flow in the venae cavae and venous collaterals following postoperative venous thrombosis, to determine myocardial dysfunction of both ventricles, and to assess the severity of residual pulmonary hypertension. Pleural ultrasound is necessary to rule out fluid accumulation and to assess the adequacy of conservative therapy administered after the intervention. The data obtained allow for timely adjustment of conservative therapy or addressing the need for reoperation when indicated [15,33].
It is recommended that patients with PAPVR and those following its surgical treatment undergo targeted chest X-ray based on strict indications and during routine preventive examinations [15,20,21].
ESC/RCS none, LoE C, GoR 5
It is recommended that all patients with PAPVR undergo contrast-enhanced cardiac and great vessel MRI/CT prior to surgery to diagnose the drainage site of anomalously draining pulmonary veins, and in the postoperative period when residual complications are detected following defect correction [15,21].
ESC/RCS IIa C, LoE C, GoR 5
Comments: Contrast-enhanced cardiac and great vessel MRI/CT are informative for determining the drainage topography and the presence of restriction at the site of anomalous pulmonary venous drainage, verifying stenosis or obstruction after surgical correction of the defect and thrombosis of the venae cavae and pulmonary veins, and assessing the volume and function of both ventricles in the presence of dysfunction [15,21].
It is recommended that all patients older than 25 years after surgical treatment of PAPVR, as well as during follow-up upon the onset and progression of life-threatening heart rhythm disturbances, undergo contrast-enhanced cardiac magnetic resonance imaging to assess structural and functional characteristics of the heart and the extent of fibrosis [15].
ESC/RSC IIaC, LoE C, GoR 5
It is recommended that all patients older than 25 years (or at an earlier age if indicated) with PAPVR undergo transvenous cardiac catheterization and pulmonary angiography of the main trunk and its branches at the pre-operative hospital stage and in the postoperative period if signs of pulmonary hypertension are present (estimated pulmonary artery pressure >40 mmHg or when this parameter cannot be determined) to assess pulmonary vascular resistance (PVR) and decide on surgery and the initiation of PAH-specific therapy before surgery or after surgical correction of the defect, in accordance with the PAH assessment criteria and indications for PAH-specific therapy outlined in the ESC Guidelines on Pulmonary Hypertension [15,37,40].
ESC IC, LoE C, GoR 4
It is recommended that adult patients with PAPVR with a complicated pre-operative and postoperative course undergo physical tolerance testing (cycle ergometry and/or 6-minute walk test or treadmill test) [15,37].
ESC/RCS none, LoE C, GoR 5
It is recommended that adult patients with PAPVR receive antibiotic prophylaxis for infective endocarditis for 6 months after surgical treatment [15,37].
ESC/RCS none, LoE C, GoR 5
Comments: For any type of congenital heart disease corrected using synthetic materials/prostheses, bacterial endocarditis prophylaxis is indicated for 6 months postoperatively or for life if residual shunts or valvular regurgitation persist. Endocarditis prophylaxis is administered during dental procedures, accompanied by damage to the oral mucosa (tooth extraction, manipulation in the periapical zone of the tooth, etc.) [15,37].
6. Organization of Medical Care
Stages of medical care:
Primary health care;
Specialized outpatient care;
Specialized inpatient medical care and/or high-tech inpatient care;
Postoperative rehabilitation stage, dynamic follow-up;
Type of medical care: specialized medical care, primary health care, emergency medical care;
Conditions of medical care: inpatient, outpatient, outside a medical organization;
Form of medical care: elective, emergency.
Indications for elective hospitalization:
Presence of congenital heart disease;
Presence of symptoms of circulatory failure;
Presence of pulmonary hypertension;
Presence of supraventricular rhythm and cardiac conduction disorders;
Detection of scimitar syndrome regardless of the patient's age and clinical presentation;
Diagnostic uncertainty and the need for special investigative methods (employing diagnostic procedures that cannot or should not be performed in an outpatient setting) to clarify the cause and severity of PAPVR;
Planned surgical intervention for PAPVD.
Indications for emergency hospitalization:
Paroxysm of supraventricular or ventricular arrhythmias, or severe cardiac conduction disturbances with syncopal and presyncopal states;
Decompensation of chronic heart failure;
Presence of paradoxical systemic embolism, including cerebral embolism.
Indications for patient discharge from the hospital:
Diagnosis of PAPVD established using special diagnostic methods, with determination of its severity and further management strategy;
Completed surgery for surgical correction of PAPVD with a completed postoperative follow-up period and management of perioperative complications;
Optimized medical therapy for CHF, antiarrhythmic and PAH-specific therapy in case of refusal of surgical intervention, if indicated;
Terminated paroxysm of supraventricular arrhythmia;
Terminated paroxysm of ventricular arrhythmia and/or implanted cardioverter-defibrillator;
Terminated syncopal and presyncopal episode in sick sinus syndrome and/or implanted pacemaker;
Compensation of heart failure symptoms.
7. Additional Information
It is recommended that all adult patients with PAPVD avoid excessive physical activity that provokes the onset of potentially dangerous symptoms such as severe dyspnea and fatigue, dizziness, syncope, and chest pain [15,37].
ESC/RSC I C, LoE C, GoR 5
It is recommended that all adult patients with PAPVD receive influenza and pneumococcal vaccination to prevent disease progression against the background of intercurrent infection before and after treatment [15,37].
ESC/RCS IIaC, LOR C, LOE 5
It is recommended that all adult patients with PAPVD with a history of infective endocarditis, as well as residual PH, receive infective endocarditis prophylaxis before and after treatment [15,37].
ESC/RCS IIaC, LOR C, LOE 5
Pregnancy and childbirth
Pregnancy and childbirth are usually well tolerated:
after defect correction;
in female patients with preserved function of both ventricles;
for functional classes I–II NYHA;
in the absence of significant stenosis of the venae cavae and pulmonary veins, significant cardiac rhythm and conduction disorders, PH, and other concomitant cardiovascular diseases [15,34].
Patients with PAPVC without PH belong to the low-risk group for maternal mortality or severe morbidity during pregnancy and after delivery, although there may be an increased risk of paradoxical embolism in the presence of ASD. In such cases, patients are followed up by a multidisciplinary team including a cardiologist specializing in care of adult patients with CHD, an obstetrician-gynecologist and an anesthesiologist, with involvement of other specialists as needed, including medical genetics specialists [15,34]. Pregnancy in patients with significant dysfunction of one or both ventricles, in the presence of PH, is associated with a significant risks for both mother and fetus. In such cases, management and treatment approach follows the ESC Guidelines for the management of cardiovascular diseases during pregnancy, 2018 [15,34].
Criteria for Evaluating the Quality of Medical Care
№ | Quality criteria | ESC Class and Level | GoR | LoE | Yes/No |
|---|---|---|---|---|---|
Diagnosis stage | |||||
1 | Collection of complaints and medical history was performed | IB | C | 5 | Yes/No |
2 | Physical examination was performed | IB | C | 5 | Yes/No |
3 | 12-lead resting ECG was registered in all patients with suspected PAPVD and during follow-up of patients with an established diagnosis to detect cardiac rhythm and conduction disorders, right atrial and right ventricular overload, electrical axis deviation, and, if necessary, in the pre- and/or postoperative period in case of surgical treatment | IB | C | 5 | Yes/No |
4 | Targeted chest X-ray was performed in all patients with suspected PAPVD and during follow-up of patients with an established diagnosis for diagnosing the primary condition, ruling out other heart and great vessel diseases as well as extracardiac pathology (pathology of other mediastinal organs, lungs, pleura), clarifying the anatomical features of CHD, the state of the pulmonary circulation, and detecting pulmonary congestion, and, if necessary, in the pre- and/or postoperative period in case of surgical treatment | IB | C | 5 | Yes/No |
5 | Transthoracic echocardiography with color Doppler mapping was performed in all patients with suspected or previously diagnosed PAPVD for a detailed assessment of intracardiac anatomy and hemodynamics, and, if necessary, in the pre- and/or postoperative period in case of surgical treatment | IA | A | 3 | Yes/No |
6 | Contrast-enhanced cardiac and great vessel MRI/CT was performed to determine the drainage topography and the presence of restriction at the level of anomalous pulmonary venous drainage prior to surgical treatment, and to detect stenosis/obstruction/thrombosis of the venae cavae and pulmonary veins in the postoperative period, as well as to assess the volume and function of both ventricles in case of dysfunction | IC | C | 5 | Yes/No |
7 | 24-hour Holter monitoring was performed in all patients with PAPVD and concomitant cardiac rhythm and/or conduction disorders to detect the latter, and if necessary, to select and adjust therapy, and, if necessary, in the pre- and/or postoperative period in case of surgical treatment | IIaA | C | 5 | Yes/No |
8 | Transvenous cardiac catheterization, pulmonary angiography, and selective angiography of its branches were performed to clarify intracardiac anatomy, topical diagnosis of the venae cavae and pulmonary veins, presence of restriction at the site of anomalous pulmonary venous drainage, and, if indicated, to determine pulmonary vascular resistance (PVR) and perform a vasoreactivity test in pulmonary hypertension prior to surgical treatment | IC | C | 5 | Yes/No |
Surgical Treatment Stage | |||||
1 | Radical correction of PAPVD was performed if indicated | IC | C | 5 | Yes/No |
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Appendix A1. Working Group Composition
Bokeria L.A., Academician of the RAS (Moscow), Association of Cardiovascular Surgeons of Russia
Azarinova I.V., Ph.D. (Tomsk), Association of Pediatric Cardiologists of Russia, Russian Society of Cardiology
Boriskov M.V., MD (Krasnodar)
Volkov S.S., Ph.D. (Moscow)
Gorbatikov K.V., MD (Tyumen)
Gorbatykh Yu.N., MD (Novosibirsk)
Gorbachevsky S.V., Prof. (Moscow)
Zelenikin M.M., Prof. (Moscow), Association of Cardiovascular Surgeons of Russia
Kim A.I., Prof. (Moscow)
Kalashnikov S.V., PhD (Moscow)
Kokshenev I.V., Prof. (Moscow)
Kupryashov A.A., D.Sc. (Moscow)
Movsesyan R.R., D.Sc. (Saint Petersburg), Association of Cardiovascular Surgeons of Russia
Nikiforov A.B. (Moscow)
Petrushenko A.V., Ph.D. (Kazan)
Plotnikov M.V., Ph.D. (Astrakhan)
Podzolkov V.P., Academician of the RAS (Moscow)
Sokolov A.A., Prof. (Tomsk), Russian Society of Cardiology
Tumanyan M.R., Prof. (Moscow)
Shatalov K.V., MD, PhD (Moscow)
Shmalts A.A., D.Sc. (Moscow)
No conflict of interest. All members of the Working Group confirmed the absence of financial support/conflicts of interest that need to be reported.
Appendix A2. Methodology for Developing Clinical Guidelines
Target audience of the developed clinical guidelines:
Pediatrician
Cardiologist
Pediatric Cardiologist
Cardiovascular Surgeon
Ultrasound diagnostics physician
General Practitioner
General practitioner.
X-ray Endovascular Diagnostics and Treatment Physician
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 May be considered |
III | Evidence or general agreement that the given procedure, intervention, treatment is not useful/effective, and in some cases may be harmful | Not recommended |
Table 2. Levels of evidence reliability according to the European Society of Cardiology (ESC) guidelines.
ESC Level of Evidence | |
|---|---|
A | Data derived from multiple randomized clinical trials or meta-analyses |
B | Data derived from a single randomized clinical trial or large non-randomized studies |
C | Consensus of opinion of the experts and/ or small studies, retrospective studies, registries |
Table A1. Levels of Evidence (LoE) scale 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 A2. Levels of Evidence (LoE) scale for prevention, treatment, and rehabilitation methods (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 A3. Grades of Recommendation (GoR) scale for prevention, diagnostic, treatment, and rehabilitation methods (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 evaluated efficacy criteria [outcomes] are unimportant, all studies have low methodological quality, and their conclusions regarding the outcomes of interest are inconsistent) |
Procedure for updating clinical guidelines
The clinical guideline update mechanism provides for their systematic revision at least once every 3 years, as well as upon the emergence of new evidence-based data on the diagnosis, treatment, prevention, and rehabilitation of specific conditions, 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 dated November 21, 2011);
Procedure for Providing Medical Care to Patients with Cardiovascular Diseases (Order of the Ministry of Health of Russia No. 918n dated November 15, 2012);
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 for clinical guidelines, and requirements for their structure, composition, and scientific validity of the information included in clinical guidelines" (Registered with the Ministry of Justice of the Russian Federation on May 8, 2019, No. 54588);
Order of the Ministry of Health of the Russian Federation No. 203n dated May 10, 2017, "On approval of criteria for assessing the quality of medical care";
Order of the Ministry of Health of the Russian Federation No. 804n dated October 13, 2017 "On approval of the nomenclature of medical services";
Order of the Ministry of Health of the Russian Federation No. 700n dated October 7, 2015 "On the nomenclature of specialties of specialists with higher medical and pharmaceutical education"; Order of the Russian Ministry of Health No. 205n dated May 2, 2023 (as amended on December 4, 2023) "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
Partial anomalous pulmonary venous drainage is usually combined with an interatrial communication (atrial septal defect, patent foramen ovale) and incorrect anomalous drainage of one or two (less frequently three) pulmonary veins into the right atrium, superior or inferior vena cava, or smaller systemic veins instead of the left atrium. The presence of an opening in the interatrial septum results in a left-to-right shunt. With an atrial septal defect, blood from the left atrium partially flows into the right atrium with each cardiac contraction. Consequently, the right heart chambers become overloaded because they have to handle an extra volume of blood that has passed through the lungs. Therefore, the pulmonary vessels are congested with blood. This leads to a predisposition to respiratory infections (bronchitis, pneumonia). The pressure in the atria is low, and the right atrium is the most compliant heart chamber. Therefore, as it enlarges, it copes with the load quite easily for a certain period of time.
Neonates, infants, and young children in the vast majority of cases grow and develop completely normally. Parents may notice their tendency to frequent colds, which can result in bronchitis or pneumonia, and this should alert the parents. Sometimes children with partial anomalous pulmonary venous drainage grow with weight deficiency and differ somewhat from their healthy peers.
Heart-related complaints may and typically do appear in adolescence. These are complaints of perceived heart rhythm irregularities ("skipped beats"). Over time, they become more frequent, and sometimes lead to a point where the patient becomes unable to tolerate normal, routine physical exertion. To avoid such a "natural" course of the congenital heart defect, surgical treatment of the heart is recommended: the atrial septal defect is closed with a patch made from the child's pericardium, and the anomalously draining pulmonary veins are redirected into the left atrial cavity using a tunnel-shaped patch in the right atrial cavity, which directs the oxygenated blood from the lungs to the left heart chambers. Typically, the intervention is elective rather than urgent. However, surgery should be performed at an earlier age if lower respiratory tract infections (bronchitis, pneumonia) become more frequent, the size of the right heart chambers increases, and the risk of developing bronchial asthma rises.
Performing the surgery at an earlier age has a less negative impact on the emotional and psychological state of the child/patient and, consequently, on their close relatives.
After discharge from a specialized center, it is recommended to strictly follow the instructions specified in the discharge documents (discharge summary).
For any invasive procedures (dental, cosmetological, and other procedures that involve or carry a risk of compromising the integrity of the skin and mucous membranes), it is strongly recommended to provide antibacterial prophylaxis for the prevention of infective endocarditis within 6 months after surgery.
Cases of planned invasive procedures should be coordinated with the cardiologist supervising the patient at their place of residence.
Changes in the doses and regimens of certain medications, as well as the prescription of additional or alternative medications, are carried out solely by the attending physician.
If side effects from medications occur, it is recommended to discuss them with the attending physician as soon as possible.
An unscheduled cardiologist consultation is recommended as soon as possible if the following symptoms occur or sharply progress: fatigue, dyspnea, cyanosis, edema, arrhythmias, syncope, neurological deficits (loss of vision, hearing, speech, limb numbness, paresis and paralysis, including transient ones), acute respiratory diseases, fever of unknown origin.
Appendix G. Assessment Scales, Questionnaires, and Other Evaluation Tools
No.