Abnormal drainage of the pulmonary veins in adults. Abnormal pulmonary vein drainage

Partial anomalous drainage of the pulmonary veins is a defect in which one or more pulmonary veins drain into the right atrium or its tributaries - the superior, inferior vena cava, coronary sinus, left innominate vein. Both the right and left pulmonary veins may drain abnormally.

This anomaly was first described by Winslow in 1739. By 1942, Brody had collected 67 published cases. With the advent of the surgical era, the defect was thoroughly studied using hundreds of examples.

NADLV accounts for less than 1% of all congenital heart disease. According to the results of 1800 sections, PADLV was detected in 0.6-0.7% of cases. It is less common in clinical studies and its exact frequency is unknown. The defect is usually combined with ASD and accounts for 14.7% of all ASD.

Etiology

The etiology of the anomaly is unknown. Embryogenesis is based on partial early obliteration of the common pulmonary vein and the preservation of one of the pulmonary-systemic venous canals draining the pulmonary veins.

Anatomy

There are various options for CHADLV.

The right pulmonary veins drain into derivatives of the right cardinal system - into the right atrium, SVC, usually in combination with a venous sinus defect, as well as into the IVC, often with an intact interatrial septum.

The left pulmonary veins drain into derivatives of the left cardinal system - into the left innominate vein through the left rudimentary SVC or into the coronary sinus. This type of anomaly is usually accompanied by ASD and other anomalies.

Since the embryonic visceral plexus is a midline structure, cross-drainage of the left-sided pulmonary veins into derivatives of the right cardinal system and vice versa is possible.

All forms of PAD have common anatomical features:

expansion and hypertrophy of the right atrium and ventricle;

dilatation of the pulmonary artery.

The chambers of the left heart are not enlarged.

Drainage of the right pulmonary veins into the SVC

The usual form of this anomaly is the drainage of the veins of the upper and middle lobes into the SVC. The upper lobe is drained by one large or two or three smaller veins in the SVC below the azygos vein. The middle lobe vein drains into the SVC at its junction with the right atrium. The vein of the lower right lobe usually flows into the left atrium, and occasionally into the right atrium. The lower part of the SVC - between the azygos vein and the right atrium - is 2 times wider than normal.

In most cases, a venous sinus type ASD is present. The defect has no upper and partially posterior edge. Sometimes a secondary ASD occurs, and rarely a primary one. Occasionally the interatrial septum is intact.

Drainage of the right pulmonary veins into the IVC

All right pulmonary veins or the right middle and lower lobes of the lung drain into the IVC immediately above or below the diaphragm. The configuration of the pulmonary veins, unlike the norm, resembles a spruce. The interatrial septum is usually intact. This anomaly, called Turkish saber or scimitar syndrome by Neill et al., is combined with other anomalies such as hypoplasia of the right lung, anomalies of the bronchial system, dextroposition of the heart, hypoplasia of the right pulmonary artery, and abnormal blood supply to the right lung from the aorta. Additional heart defects that are common include:

coarctation of the aorta;

Drainage of the left pulmonary veins into the innominate vein

The left innominate vein is usually the site of the anomalous drainage of the left pulmonary veins. Veins carrying blood from the left upper lobe or the entire left lung enter the innominate vein through a derivative of the left cardinal system, the anomalous vertical vein. It is also called persistent HDL, however, since it does not have a direct connection with the heart, this name is not correct. Usually there is an ASD, rarely it is intact.

Other sites of abnormal pulmonary vein connection

Infrequent sites of anomalous drainage of the left pulmonary veins include the coronary sinus, IVC, SVC, right atrium, and left subclavian vein. Unusual drainage sites for the right pulmonary veins are the coronary sinus and azygos vein.

In rare cases, the veins of both lungs drain abnormally, except for a small portion of the pulmonary venous system, which drains normally into the left atrium. This variant of the defect is called a subtotal anomalous venous connection. Functionally, patients with this defect closely resemble patients with TADLV.

Associated heart defects and syndromes

PADLV, like TADLV, is often accompanied by atrial isomerism and heterotaxy of internal organs. Turner and Noonan syndromes are often associated with PAD. With Turner syndrome, it occurs in 3.6% of cases in the form of a venous sinus defect, with an intact interatrial septum - in 14.3%. In more than half of the cases, there is a 45 XO karyotype. PADLV was not observed in the mosaic type of this syndrome. Noonan syndrome with normal chromosomes is accompanied by PAD in 6% of cases. Rarely, partial and total anomalous pulmonary venous drainage accompany tetralogy of Fallot. Of the 1810 cases of tetralogy we operated on, abnormal drainage of the pulmonary veins was never encountered.

Hemodynamics

Hemodynamic disturbances are the same as with an interatrial defect - pulmonary blood flow is increased due to recirculation of blood through the lungs. Its volume depends on the number of anomalous veins, the presence and size of the ASD and the magnitude of the PVR.

PALV with intact interatrial septum

If one vein drains into the right atrium or its tributaries, the left-to-right shunt volume accounts for about 20% of the total pulmonary blood flow. This small amount of hypervolemia does not manifest itself clinically. If all but one pulmonary vein drains abnormally, the physiology and clinical manifestations are consistent with TADPV. If the veins of one lung drain abnormally, pulmonary blood flow depends on the magnitude of PVR and the compliance of the atrium. In patients with drainage of the right pulmonary veins into the IVC with an intact interatrial septum, the blood flow through them accounts for 24-32% of the total pulmonary blood flow. In this syndrome, blood flow depends on the presence of pathology of the parenchyma of the right lung and concomitant anomaly of the arterial blood supply, which leads to an increase in vascular resistance in this lung. In the case when the abnormal connection of the veins of one lung is not accompanied by another pathology, the blood flow in this lung is increased and accounts for up to 66% of the total pulmonary blood flow. This is due to the greater distensibility of the right atrium, into which the anomalous veins drain. The distensibility of the left atrium is less, so a normal amount of blood flows to it. The pressure in the right atrium is lower than in the left. Because the pressure in both pulmonary arteries is equal, the pressure gradient in the abnormally draining lung is higher than in the normal lung. Pulmonary vascular resistance is the same in both lungs, so the volume of blood flow in the abnormally draining lung is greater.

Due to the absence of blood shunting through the intact interatrial septum, the pressure in the pulmonary artery is not increased unless there are associated defects.

The intact atrial septum isolates blood flowing from the abnormally draining lung from the systemic circulation. Therefore, disease or surgical resection of the opposite lung can be fatal.

CHADLV with ASD

With a small defect, the hemodynamics are practically the same as the PAD with an intact septum. A large defect has a significant impact on the state of intracardiac circulation, increasing the volume of blood shunting at the atrial level. The ratio of the volume of pulmonary blood flow to systemic blood flow reaches 3.5:1. The relative contribution of each lung to the shunt current varies. The proportion of blood from the right lung that is shunted from left to right is on average 84%, from the left lung - 54%. In a venous sinus defect, there is a small right-to-left discharge from the SVC into the left atrium through the adjacent septal defect. Shunting of blood from right to left also occurs with secondary ASD. Adult patients with uncomplicated PAD may develop pulmonary hypertension.

Clinic and diagnostics

The clinical picture of abnormal pulmonary venous drainage associated with ASD is generally no different from the clinical picture of isolated ASD. Isolated PAD may be characterized by slightly different symptoms; in particular, there may be no splitting of the second heart sound. To diagnose PAD, a combined transthoracic and transesophageal echocardiography study is most often used. X-ray angiographic examination is indicated in doubtful cases, if there is an enlargement of the right chambers of the heart and an intensification of the pulmonary pattern on the radiograph, as well as symptoms of right ventricular overload on the ECG.

Clinical signs depend on the number of pulmonary veins draining into the right heart. Abnormal drainage of one pulmonary vein does not manifest itself clinically. Even with abnormal drainage of half of the pulmonary veins, children are usually asymptomatic. Objective clinical signs correspond to ASD, in which there is a wide, fixed splitting of the second tone. In the absence of a defect, tone II is normal. There is a systolic murmur in the second intercostal space on the left, and sometimes a mid-diastolic murmur of relative tricuspid insufficiency.

The ECG shows signs of right ventricular hypertrophy, right bundle branch block. In some cases, the ECG may be normal.

The radiograph is essentially consistent with that of an ASD. There are also specific signs indicating the location of the confluence of the pulmonary veins. Thus, if the pulmonary veins flow into the SVC, its lower portion is dilated, which is often manifested by an expansion of the heart shadow above the contour of the right atrium or double density within the upper edge of the atrium. If the pulmonary veins drain into the azygos vein, it is dilated and appears on x-ray as a rounded bulge in the superior mediastinum on the right side of the heart.

The confluence of the left pulmonary veins into the left innominate vein is reflected on the radiograph as a supracardial shadow formed by the vertical vein on the left, the innominate vein above and the SVC on the right. However, this sign is not as pronounced as with TADLV.

Echocardiography can detect PAD only if suspicion is based on general clinical data. Failure to visualize all four pulmonary veins in the presence of right atrium and right ventricular dilatation is strongly suggestive of PAD, especially in the presence of an ASD. PADLV can be found in any type of ASD and persistent HDL. A sinus venosus defect with PAD is best identified from a subcostal position or from a high right parasternal position. The SVC can be seen overhanging the interatrial communication.

The abnormal connection of the pulmonary veins to the SVC is best seen along the short axis from the suprasternal notch or the long high axis of the SVC. These same positions make it possible to visualize the confluence of the pulmonary veins into the left innominate vein. The pulmonary veins, which drain directly into the right atrium, are better visible from the apical four-chamber or subcostal position. Subcostal short- and long-axis positions at the level of the IVC and its junction with the right atrium should be used when scimitar syndrome is suspected. Abnormal drainage of the pulmonary veins into the coronary sinus is better identified by scanning the posterior left atrioventricular groove.

Cardiac catheterization is used only in unclear cases to diagnose concomitant defects and changes in the pulmonary parenchyma in scimitar syndrome. The oxygen surge allows one to determine the level of pulmonary vein drainage, excluding abnormal drainage into the IVC because blood flow in the right lung is reduced, as well as the flow of highly oxygenated blood into the IVC from the renal veins. The presence of an intact atrial septum is diagnosed by the inability of the catheter to pass into the left atrium and by the difference between the pressure in the right atrium and the wedge pressure.

Selective angiography is of limited value if the pulmonary veins drain into or close to the right atrium. But if they drain into the systemic veins, angiography can reliably determine the location of the anomalous connection - in the IVC, left innominate vein or azygos vein. With selective pulmonary arteriography, the sites of the abnormal connection are visible in the pulmonary venous phase of the contrast agent flow. If the catheter can be retrogradely advanced into an abnormally draining pulmonary vein, the administration of a contrast agent allows detailed anatomy of the pulmonary venous blood path to the right heart to be seen.

Differential diagnosis

PAD is difficult to distinguish from uncomplicated ASD. Preoperative accurate diagnosis is of no practical importance, since during the intervention it is easy for the surgeon to navigate the details of the defect.

TADLV has a dramatically different clinical picture with severe pulmonary congestion and heart failure leading to death within 6 months. life. Cyanosis is also a distinctive feature.

Natural course

In the 2nd and 3rd decades of life, dyspnea on exertion and cyanosis appear due to pulmonary hypertension and increased pulmonary arterial resistance.

Presurgical management

Limitation of physical activity is not required, prevention of bacterial endocarditis is not indicated.

Surgery

Indications for operations are determined by the volume of left-right blood discharge. Intervention is indicated if pulmonary blood flow is 1.5 times or more higher than systemic blood flow. The optimal age for surgery is 2-5 years.

Operations are performed under infrared conditions. The technique of intervention depends on the location of the confluence of the pulmonary veins.

When draining into the right atrium, the ASD is expanded if necessary and, using an autopericardial patch, the ostia of the pulmonary veins are diverted into the left atrium. If the septum is intact, it is partially excised. The free edge of the ASD can be directly sutured to the wall of the right atrium over the orifices of the pulmonary veins. If necessary, a flap of the interatrial septum or a patch from the autopericardium is cut out.

The right superior pulmonary veins drain into the SVC at its junction with the right atrium. It is recommended to cannulate the superior vena cava through its wall immediately above the confluence of the pulmonary veins, but we do not see any great technical difficulties with standard cannulation through the right atrial appendage. Sometimes several veins drain the upper lobe of the right lung high in the SVC. Careful dissection of the entire vein allows identification of these pulmonary veins, selection of the cannulation site, and planning of the method of correction. When isolating the lateral surface of the vena cava, it is important not to damage the phrenic nerve. At the surgeon's option, the vena cava cannulate through the appendage and wall of the right atrium. When draining the pulmonary veins into the cavoatrial junction, an intra-atrial tunnel is created from the autopericardium, connecting the mouths of the right pulmonary veins with the venous sinus defect. An important condition is to maintain a constant diameter of the formed tunnel throughout its entire length, so that there is no obstruction to the outflow of blood from the lung. A patch from the autopericardium is sewn in such a way that its smooth surface faces inward of the left atrium to prevent the formation of mural thrombi on the left side of the heart.

Part of the tunnel may lie within the SVC, so the posterior atrial incision used to correct this anomaly is closed with a pericardial patch or right atrial wall flap.

If the pulmonary veins enter the SVC high or if the SVC is small in diameter, as is common in the presence of persistent HDV, the Warden procedure is performed. This method involves retracting the lower segment of the vena cava into the left atrium through a septal defect and moving the upper part of the divided vena cava into the right atrium appendage. A short incision into the right atrium allows exposure of the septal defect and cavoatrial junction. The lateral edge of the SVC opening is sutured to the lower edge of the ASD. A pericardial flap can also be used to retract the SVC into the left atrium. The superior vena cava is divided above the level of the confluence of the pulmonary veins and the cardiac end is sutured, being careful not to narrow the superior pulmonary vein. The cephalic end of the SVC is mobilized and anastomosed with the right atrial appendage, trying to avoid narrowing of the anastomosis. The anastomosis can be performed with a straight venous cannula or using a cannula with a curved tip. In the presence of a wide HDV, the right narrow vena cava can not be cannulated, periodically squeezing it with a band while performing the anastomosis.

Correction of abnormal drainage of the left pulmonary veins into the coronary sinus is carried out in the same way as with total drainage: the anterior wall of the coronary sinus is dissected and a patch fixed to the edge of the ASD and to the lower edge of the sinus is taken into the left atrium. Due to the proximity of the AV node, the sutures are placed superficially.

Abnormal drainage of the left pulmonary veins into the innominate vein is often a component of mixed forms of PAPV in which there is also anomalous drainage of the right pulmonary veins. Isolated drainage of the left upper lobe vein into the innominate vein does not significantly increase pulmonary blood flow, so its correction is not advisable. Moreover, if other significant anomalies are being corrected, lengthening the operation by eliminating this form of abnormal drainage is not justified. However, if all venous drainage from the left lung is into the innominate vein, which is hemodynamically consistent with a large ASD, correction is indicated. The operation can be performed from the left side as a closed procedure. Domestic surgeons performed this intervention under IR conditions, since drainage was an operational finding during various open operations undertaken for a different reason. The apex of the left appendage was cut off, a longitudinal incision was made to the left atrium, and the pulmonary end of the crossed vertical vein was anastomosed with the left atrium, trying to avoid its twisting. Surgical techniques should ensure free flow of venous blood into the left atrium. For this purpose, some authors suggest making a longitudinal incision in the vertical vein and suturing the edges of the incision in the transverse direction to reduce the likelihood of its bending at the site of entry into the pericardial cavity. To eliminate the “pouch effect,” the left atrial appendage is not crossed, but only incised to half its circumference. The vertical vein is incised to increase the length of the suture line. If the operation is performed without IR, the left branch of the pulmonary artery should be closed with tape.

Mortality does not exceed 1%. Long-term results are similar to those after ASD correction and depend on the state of the pulmonary vascular bed at the time of surgery. With low PVR and early operations, life expectancy and performance are normal.

Complications

Obstruction of the SVC has been described after correction of a venous sinus defect with abnormal drainage. This form of drainage is characterized by supraventricular arrhythmias. After correction of the venous sinus defect, 3% of patients have clinical signs of SVC obstruction, and 30-40% have sick sinus syndrome. This complication can be prevented by preserving the sinus node artery. Postoperative arrhythmias are rare in other forms of PAD.

In the long term, the possibility of pulmonary vein obstruction must be taken into account. It leads to decreased blood flow to the affected lung. This complication can be detected by angiography or ventilation perfusion scintigraphy. Doppler echocardiography can reveal differences in flow signals in the right and left pulmonary arteries. Venous obstruction results in very little systolic antegrade flow and diastole return flow compared to normal pulmonary artery. Pulmonary venous obstruction may also result from narrowing of the superior or inferior vena cava. Successful expansion of the vena cava using balloon angioplasty or stent placement has been described.

Anomalous drainage of the pulmonary veins is a defect in which all or some of the pulmonary veins drain into the right atrium (instead of the left) or the venous vessels leading to it. The first description of the vice dates back to 1739.

Most children have a concomitant atrial septal defect, the cavity of the right atrium and ventricle is enlarged, and the lumen of the pulmonary artery is expanded. There is also a combination with other congenital heart defects (transposition of the aorta and pulmonary artery, ventricular septal defect, single ventricle, pulmonary stenosis, dextrocardia).

With total anomalous drainage, there is no communication at all between the pulmonary veins and the left atrium. In this case, all the blood from the pulmonary circulation enters the right atrium or the vessels leading to it.

There are 4 types of this complex congenital heart defect:

1) the pulmonary veins can flow into the superior vena cava system, which collects venous blood from the upper extremities and head, occurs in 55% of cases;

2) the confluence of the pulmonary veins into the right atrium or into the coronary sinus, occurs in 30% of cases;

3) the pulmonary veins can flow into the inferior vena cava system, which collects blood from lower limbs, internal organs, occurs in 12% of cases;

4) in 3% of cases there may be various combinations of this condition.

With partial anomalous drainage, manifestations of the defect may be absent for a long time or resemble those of an atrial septal defect. The main symptoms of the defect are atypical: fatigue, heart pain, shortness of breath during exercise, there may be repeated pneumonia, and retardation in physical development. With age, the child develops a cardiac hump, the boundaries of the heart (determined by the doctor) expand, more to the right. When listening to the work of the heart, a characteristic murmur is detected. Heart failure rarely develops.

With total abnormal drainage of the pulmonary veins, attention is drawn to a bluish discoloration of the skin, which can appear at any age of the child, most often towards the end of the first year of life. However, the severity of the bluish coloration is insignificant, and therefore it can only be noticed when screaming. With this type of abnormal drainage of the pulmonary veins, children are more likely to lag behind in physical development, and a cardiac hump appears earlier (mainly on the right side).

With partial anomalous drainage no characteristic symptoms on the ECG. Phonocardiography confirms auscultation data (listening to the work of the heart). Also, additional examination methods are radiography of organs. chest, Ultrasound of the heart, catheterization of the heart cavities (in doubtful cases). The most valuable diagnostic technique is selective angiocardiography, in which a contrast agent is injected into the trunk of the pulmonary artery. After filling it, an X-ray of the chest organs is performed and the course and place of confluence of all pulmonary veins are identified, while the superior vena cava, as well as the right and left parts of the heart are contrasted.

In case of complete anomalous drainage of the pulmonary veins, if the condition of the newborn is critical, a closed septotomy (dissection of the interatrial septum and creation of a communication between the right and left parts of the heart) is usually performed. Complete surgical correction of the defect depends on its type. The mouths of the pulmonary veins are moved into the left atrium using a patch. The results of the operation are usually good.

About 80% of children with total anomalous pulmonary venous drainage die in the first years of life. The later the first symptoms of the disease appear, the better the prognosis. For signs of heart failure, cardiac glycosides (digoxin) and diuretics are prescribed. Effective drug therapy makes it possible to postpone surgical treatment until an older age, since correction of the defect at an early age is accompanied by a fatal outcome of 35 to 50%. When correcting partial ADLV, various operations are used, depending on the type of defect.

Updated: 2019-07-09 23:44:02

Atherosclerosis is a disease of blood vessels in which cholesterol, calcium salts are deposited on their walls, and connective tissue grows.

Anomalous pulmonary venous drainage (ADPV) is a defect in which all (total) or individual (partial) pulmonary veins drain into the right atrium or the vena cava leading to it. The first description of the defect belongs to J. B. Winslow (1739) and J. G. Wilson (1798). The frequency of ADLV is 0.5-2% of all congenital heart disease in combination with ASD in 10-15%. It is likely that a significant number of cases of partial ADLV remain undiagnosed due to the lack of clinical manifestations.
Anatomy, classification. Anomalous drainage of the veins of the right lung is more common. The following variants of ADLV can be distinguished (Fig. 12): 1) supracardial level - pulmonary

a - supracardial level: abnormal drainage of JIB into the SVC; b - cardiac level: abnormal drainage of the PV into the RA; c - infracardial level: abnormal drainage of the PV into the IVC; 1 - atrial septal defect.
These veins flow into the left innominate, superior vena cava or one of their branches; 2) cardiac level - drainage of all or part of the pulmonary veins into the cavity of the right atrium or coronary sinus; 3) infracardial - part of the pulmonary veins flows into a venous vessel located below the heart; 4) mixed.
Most patients have a concomitant secondary ASD (often a venous sinus defect) and a patent foramen ovale. Autopsy shows enlarged cavities of the right atrium and ventricle, dilated pulmonary arteries and vena cava; the left chambers of the heart and the aorta are not changed. Usually at the confluence there are several mouths of the pulmonary veins; sometimes they gather into one trunk and open at a common mouth. In 20% of cases, other congenital heart defects occur (VSD, transposition of the aorta and pulmonary artery, tetralogy of Fallot, single ventricle, pulmonary stenosis, dextrocardia).
Hemodynamics. With partial ADLV, hemodynamic disturbances are similar to those with ASD and are determined by the number of abnormally draining veins, the size of the left-to-right shunt and the size of the ASD accompanying congenital heart disease. If one pulmonary vein is drained and there is no ASD, the defect remains asymptomatic. Pulmonary artery pressure with partial APPV long time remains normal, since blood discharge occurs at the level of the atria and there is no “transfer pressure” factor (from the left ventricle and aorta to the right ventricle and pulmonary artery, as with VSD and PDA).
Clinic, diagnostics. With partial ADLV, clinical manifestations may be absent for a long time or resemble those of a secondary ASD. The main symptoms of the defect are
typical: fatigue, heart pain, shortness of breath on exertion, possible repeated pneumonia, retardation in physical development. With age, a cardiac hump appears, the boundaries of cardiac dullness expand more to the right. The auscultatory picture is similar to that of secondary ASD: soft systolic murmur at the site of the pulmonary artery projection, clefting

tones. Heart failure in children rarely develops, usually in cases where more than 50% of the pulmonary veins are abnormally drained; in adults, deterioration in general condition appears earlier than in cases of isolated ASD.

The patient's life is possible only if there is a concomitant ASD. If there is no ASD, but only a patent ductus arteriosus, patients die at an early age, since the left parts of the heart do not participate in the blood circulation; death also occurs in cases of premature (before or shortly after birth) closure of the oval window. There are many varieties of this complex congenital heart disorder, and therefore various classifications have been proposed. The simplest classification is R. Darling et al. (1957), they distinguish four types of defect: type I - supracardial - the flow of all pulmonary veins with a common trunk (collector) through the anomalous vein into the superior vena cava, through the vertical vein - into the left innominate, through the anomalous vein - into the azygos (47% ); Type II - cardiac - the flow of all pulmonary veins into the right atrium or coronary sinus (30%);

type - infracardial - the flow of all pulmonary veins into the portal or inferior vena cava (18%); Type IV - mixed’ - various combinations of these three types (5%) (Fig. 13). Before flowing into the heart or the vessel leading to it, the pulmonary veins are collected into a single chamber - a collector, which is then connected to the systemic circulation at different levels. With this type of ADPV, dilatation of the right chambers and the pulmonary artery trunk are noted, while the left parts of the heart are usually normal.

With total ADPV, there may be anatomical causes of obstruction in the pulmonary venous drainage: most often this is associated with a narrowing of the mouths of abnormally draining pulmonary veins or the opening of the collector, less often there is external pressure on the anomalous vein (at the point of passage through the diaphragm, between the left pulmonary artery and the left main bronchus). In 80% of cases, obstruction of the pulmonary veins accompanies the infradiaphragmatic variant of ADPV.

The most common concomitant congenital heart disease with total ADPV are the common atrium, a single ventricle, hypoplasia of the left, transposition of the great vessels (this combination creates a functional correction of the defect). In 25-30% of cases, extracardiac malformations of the gastrointestinal tract (abnormal rotation of the intestine, diverticula, umbilical hernia), spleen (agenesia, multiple accessory spleens), genitourinary

1-supracardial level; 2-cardiac level (all pulmonary veins flow into the coronary sinus); 3 - infracardiac level.

(congenital kidney cysts, hydronephrosis) and musculoskeletal systems.

Hemodynamics. With total ADLV, blood enters the right atrium from both circulations; the magnitude and direction of discharge are determined by the ratio of the resistances of the systemic and pulmonary circulations and the diastolic relaxation of the ventricles. A large amount of arterial blood enters the right atrium from the collector into which the pulmonary veins flow. In the atrium, arterial blood mixes with venous blood, from here, through the ASD, part of the blood (smaller) enters the left atrium and then into the systemic circulation; more blood is directed to the pulmonary circle, contributing to a decrease in the left chambers of the heart and the development of pulmonary hypertension. If the ASD reaches a significant size and the discharge of blood into the systemic circulation is sufficient, then the left ventricle does not become hypoplastic, and pulmonary hypertension does not exceed grades I-II. and patients can live to adulthood. In cases of pulmonary vein obstruction, venous pulmonary hypertension develops; in the absence of physiological involution in the structure of the pulmonary vessels, congenital pulmonary hypertension is also possible. Thus, total ADLV is characterized by the circulation of mixed blood with a slight decrease in the oxygen content in the systemic circulation, overload of the right parts of the heart, and pulmonary hypertension (arterial, venous, congenital).

Clinic, diagnostics. The clinical course of total ADPV is determined by the anatomical and hemodynamic features of the defect, in particular the level of pulmonary resistance, the degree of pulmonary venous obstruction, the size of the interatrial communication, and the condition of the right ventricular myocardium. The first signs of this type of defect often appear from the first days and months of life, manifestations of cardiac

insufficiency, repeated pneumonia and acute respiratory viral infections, cough, retardation in physical development. Cyanosis can appear at any age, often towards the end of the first year of life. As a rule, it is insignificant and noticeable only when screaming; pronounced cyanosis from the first days of life is characteristic of pulmonary venous obstruction. The cardiac hump (mostly on the right side) appears earlier than in isolated DMG1G1. Systolic tremor is usually absent. On auscultation, the first sound in the area of the tricuspid valve is increased (a sign of increased blood flow through it). The II tone above the pulmonary artery is accentuated, split, and there is often a III tone; in the second intercostal space on the left, a medium-intensity systolic murmur is heard (as with an ASD); a mesodiastolic murmur is possible at the lower edge of the sternum on the left. If the collector drains into the superior vena cava and there is pulmonary venous obstruction, a prolonged systolic murmur may be heard above the clavicle on the right or left.

In adult patients with total ADPV and pulmonary hypertension, a systolic ejection sound is heard in the second intercostal space on the left (see Chapter 2). Right ventricular failure in combination with clinical symptoms of ASD and moderate cyanosis helps to suspect total ADVC in children. In adult patients with this defect, the clinical picture is indistinguishable from ASD.

The ECG (Fig. 14) shows a significant deviation of the electrical axis of the heart to the right (Z.aAQRS from +90 to +210°), an increase in the right atrium (high wave Pn.v), and the right ventricle (in V4R and V| qR form ', rR', rSR', in the left precordial leads the R waves are low, the S waves are deep; these signs are especially pronounced with high pulmonary hypertension for

ma R to Vi). WPW syndrome type B may be observed. With an increase in the load on the right ventricle, a shift of the ST interval below the isoline appears with deep negative T waves in leads II, III, aVF, Vt-Ve.

Phonocardiography confirms the auscultation data and has all the signs of ASD.

On the radiograph with total ADLV, the pulmonary pattern is significantly enhanced both in the arterial and venous beds, moderate or significant cardiomegaly is noted, caused by an enlargement of the right chambers, the left sections are of normal size, and sometimes an enlarged shadow of the superior vena cava is visible. For the supracardial form of the defect, a cardiac shadow in the form of a “figure eight” or “snow woman” is typical, where the lower part is the heart itself, and the additional formation at the top right is a collector that collects blood from all pulmonary veins and opens into the left or right hollow or innominate veins (Fig. 15). The latter are dilated, as they accommodate a large volume of pulmonary blood flow. Sometimes the shape of the heart imitates an enlarged thymus gland. When the pulmonary veins flow into the coronary sinus or inferior vena cava, there are no characteristic radiological manifestations of the defect.

M-echocardiography does not reveal the defect; indirect signs are the relatively small size of the left atrium and left ventricle, dilatation of the right atrium and ventricle, paradoxical movement of the interventricular septum. When the pulmonary veins flow into the coronary sinus, two-dimensional echocardiography makes it possible to determine the space formed by the venous collector behind the shadow of the heart, which is confirmed by intravenous administration of ultrasound contrast.

Probing of the heart cavities with total ADPV reveals high blood oxygen saturation in all parts of the heart (90-96%); in the pulmonary artery the saturation is sometimes the same as in the aorta. The highest saturation is observed in the right atrium or in the vein into which the pulmonary vein collector drains. Pressure in the pulmonary artery can be increased due to changes (fetal, sclerotic) in the pulmonary arterioles, increased pulmonary blood flow, obstruction of the pulmonary veins (pulmonary capillary pressure also increases at the same time). The pressure in the atria is the same or higher in the right than in the left. In such cases, small ASDs and a severe course of the disease are observed from the first days. Urgent atrioseptostomy (Rush Kind procedure) is often indicated during catheterization.

The introduction of a contrast agent into the pulmonary artery allows you to immediately make the correct diagnosis (Fig. 16). The contrast agent is detected in the pulmonary vein collector (PVC), through the vertical vein (VV) enters the left innominate vein (SV), then into the superior vena cava and the right atrium.

Differential diagnosis. In newborns and children in the first months of life, this defect is differentiated from mitral or aortic atresia, mitral stenosis, triatrial heart, pulmonary vein stenosis, transposition of the great vessels, lymphangiectasia; at an older age - with VSD.

Course, treatment. About 80% of children with total ADVC die in the first years of life. This is especially true for infracardial (infradiaphragmatic) and other variants of the defect with pulmonary venous obstruction. The main causes of death of patients: heart failure, pneumonia, premature closure of the oval window. The later the first symptoms of the disease appear, the better the prognosis. Observations are described when patients lived up to 30 years; in such cases, there is good mixing of blood at the level of the atria due to the sufficient size of the ASD, there is no venous obstruction, the level of pressure and pulmonary resistance is low, the condition of the right ventricular myocardium is satisfactory. In the presence of heart failure, the administration of cardiac glycosides and diuretics (see Chapter 22) is indicated. Effective drug therapy makes it possible to postpone surgical treatment until an older age, since correction of the defect at an early age is accompanied by a mortality rate of 35 to 50% [Falkovsky G. E. et al., 1986; Clarke D. et al., 1977].

In case of total ADLV in the right atrium (cardiac form), the operation is performed using artificial circulation and hypothermia and consists of enlarging the interatrial defect and applying a patch in such a way as to direct blood flow from the mouths of the pulmonary veins through the defect into the left atrium. In cases where the collector is drained through the coronary sinus, to create adequate drainage, part of the septum located between the coronary sinus and the oval window is excised, the posterosuperior wall of the coronary sinus is incised, and the patch displaces the wall and the coronary sinus into the left atrium. For supracardial and infracardial types of defect, an anastomosis is performed between the pulmonary vein collector and the left atrium [Burakovsky V.I. et al., 1989].

In case of severe hypoplasia of the left ventricle, correction of the ASD with a perforated patch or two-stage surgical treatment is used, including: stage 1 - anastomosis between the collector and the left atrium (allows development of the left chambers of the heart), stage II - ligation of the vertical

vein through which the pulmonary veins drain into the right superior vena cava.

Mortality after surgery in children under 6 years of age, according to

Galloway et al. (1985) was 10%. It is higher in the infracardial form, drainage of the pulmonary veins into the superior vena cava, especially into the right (than when they flow into the coronary sinus or directly into the right atrium), with pulmonary hypertension, hypoplastic left ventricle and left atrium.

When the operation is performed in childhood, the long-term results are usually good, since sclerotic changes do not have time to develop in the vessels of the pulmonary circulation and in the myocardium of the ventricles of the heart. If the anastomosis shows signs of functional obstruction, repeat surgery is indicated.

In this rather rare type of defect, the pulmonary veins do not flow into the left atrium, but through abnormal connections into the venous system. All four pulmonary veins can flow through a common collector into either the superior vena cava, the coronary sinus, or the portal vein system. Very rarely they are connected directly to the right atrium.

Since with this defect, blood from both circulations ultimately enters the right atrium, systemic circulation is maintained by blood passing through the foramen ovale, unless, of course, the ductus arteriosus is functioning or the pressure in the vessels of the pulmonary circulation does not exceed that in vessels of a large circle. The hemodynamic features of this type of defect, similar to those of LVH without atresia of the mitral or arterial valve, do not have a negative impact on the development of the fetus. The left parts of the fetal heart receive a smaller amount of blood relative to the pulmonary blood flow (which depends on the size of the foramen ovale), which comes not through the pulmonary veins, but from the right atrium. An important point, which determines the natural course of the disease and symptomatology, is the concomitant obstruction of the pulmonary veins, which invariably occurs when the pulmonary veins enter the systemic circulation below the diaphragm. Obstruction of the veins in this subphrenic type of defect is caused by the volume and length of the collector, a decrease in the caliber of the ductus venosus after childbirth, or stenosis of the collector at the site of its connection to the systemic vein. Thus, after the birth of a child, oxygenated blood from the lungs passes through the portal vein and liver before entering the right atrium. On the contrary, with the supradiaphragmatic type of defect, oxygenated blood from the lungs enters directly either into the coronary sinus, or into the superior vena cava through the common left vertical vein, or into the right atrium, mixing with systemic venous blood. It has been established that with this type of defect, venous obstruction of varying degrees can occur.

In general, in children with severe obstruction of the pulmonary veins, symptoms appear earlier with the subdiaphragmatic type of defect. In this case, the child may develop pulmonary edema and atelectasis, develop interstitial emphysema and enlarge the lymphatic ducts. In addition, hypertrophy of the median layer of the pulmonary veins and changes in the pulmonary arterioles are unchanged. Moreover, with severe obstruction of the pulmonary veins, the bronchial veins dilate, playing the role of collaterals. The walls of small pulmonary arteries thicken, and muscle mass peripheral arterioles. The right side of the heart and pulmonary artery are usually dilated, while the left side of the heart is relatively small.

Symptoms of the disease in newborns whose pulmonary veins are not obstructed appear later than with venous obstruction. At the same time, the child's pulmonary blood flow is noticeably increased. Typically, in children in whom symptoms appear in the early postnatal period, the communication between the atria is small, i.e., it is through the foramen ovale rather than through a true defect in the interatrial septum. The prognosis depends on its size.

The incidence of total anomalous pulmonary venous drainage is 2-4% of all congenital heart defects that appear during the newborn period; the defect is equally common in both boys and girls.

In children with this defect, blood from the vessels of both the greater and lesser circles enters the right atrium, and from it into the vessels of the lungs and systemic blood flow through the hole in the interatrial septum and the left ventricle. In this regard, the level of pO2 in the vessels of the systemic circle and blood oxygenation are less than normal. Their absolute value depends on pulmonary blood flow. With pulmonary vein obstruction, pulmonary blood flow is reduced and pulmonary congestion and edema may develop, which worsens hypoxemia and cyanosis. Without radical surgery, the child quickly dies. In this case, pulmonary hypertension is always observed and signs of heart failure appear early. The subdiaphragmatic type of defect is easy to assume if the level of pO2 in the blood of the umbilical vein is higher than in the aortic vein.

Total anomalous pulmonary venous drainage

The word "abnormal" means "wrong." With this defect, the pulmonary veins (and there are four of them), which should flow into left atrium, do not fall into it, i.e. don't connect with him. There are a lot of options for their wrong fit.

There is “partial” anomalous drainage - this is when one or two of the four veins flow into the right atrium (the most common option), and in the vast majority of cases it is combined with atrial septal defects, and we talked about this in the chapter on ASD.

Complete or total anomalous pulmonary venous drainage (TAPVD) is quite different. With this defect, all four pulmonary veins from both lungs are connected into one wide collector vessel. This collector of arterial blood oxidized in the lungs does not fuse with the left atrium, as it should, but connects to the venous system of the body, usually through a large vein. Arterial blood thus bypasses the heart and enters the great veins and the right atrium. Only here, having passed through the atrial septal defect, will it end up where it should be initially - in the left atrium, and then makes its usual journey through the systemic circulation. It is difficult to imagine that this could even happen. But children with this defect are born full-term, and the heart copes with this situation for some time. However, this time may be very short.

Firstly, the life of a child depends on the size of the interatrial communication - the smaller it is, the more difficult it is for arterial blood to reach its destination in the left half of the heart.

Secondly, in this left half of the heart, a significant part of the blood is simply venous, i.e. unoxidized, and it will again be pumped into the large circle. Thus, partially venous blood begins to circulate in the child’s arteries, and he becomes “blue”, i.e. the color of the skin, and especially the tips of the fingers and mucous membranes (lips, mouth) is bluish. This is cyanosis, and we’ll talk about its causes, manifestations and consequences later.

With complete anomalous drainage, the cyanosis may not be very pronounced, but it is there, and it is usually noticeable soon after birth.

In most cases, the condition of children with complete anomalous pulmonary venous drainage is “critical” from the very beginning of life. If nothing is done, they will die within a few days or months.

Surgical treatment exists, and the results today are quite encouraging. The operation is quite complex, it is performed on an open heart and consists in the fact that the common collector of the pulmonary veins is sutured to the left atrium, and the hole in the atrial septum is closed with a patch. Thus, after the operation, normal blood circulation is restored in two separated circles.

Sometimes an emergency option is acceptable - expansion of the defect during probing as the first, life-saving stage, which allows you to somewhat delay the main intervention.

We will not touch here on many details related to various types vice and methods for its correction. But we only want to emphasize that children with this defect need immediate specialized help, which is completely real today.

The long-term results of the operation are quite good - after all, the main defect has been eliminated. However, children should be under the supervision of cardiologists because complications such as rhythm disturbances or narrowing of the pulmonary veins at the suture sites are possible (this occurs because the heart continues to grow after such a major operation). And again we want to emphasize: this child is not disabled. He must lead an absolutely normal life, and the sooner the operation is performed, the faster everything will be forgotten.

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Total anomalous pulmonary venous drainage in newborns

All pulmonary veins drain into the right heart.

Suyaracardiac (the pulmonary veins drain into the ascending vertical vein, the innominate vein);

Cardiac (pulmonary veins drain into the right atrium, coronary sinus);

Infracardial (pulmonary veins drain into intra-abdominal veins).

PATHOPHYSIOLOGY

Despite the different anatomy of the defect, from the point of view of circulatory pathophysiology, it can be classified into two types depending on the presence or absence of pulmonary vein obstruction. Of no small importance for the continuation of life in the postnatal period is the presence of communications between the RA (right atrium) and the LA (left atrium) (ASD, LLC).

The hemodynamics of the non-obstructive variant of TADPV are similar to large ASD. Blood from the pulmonary veins enters the RA, then into the RV (right ventricle) and, in the presence of an ASD, into the LA. In this case, the amount of blood that returns through the ASD to the left atrium is determined by the size of the defect and the degree of distensibility of the pancreas. However, under any conditions, a significantly larger portion of the blood enters the pancreas and circulates in the pulmonary circulation (pulmonary circulation), causing volume overload of the pancreas and severe hypervolemia of the pulmonary circulation. The pressure in the PA (pulmonary artery) remains low. Due to the fact that mixing of venous and arterial blood coming from the pulmonary veins occurs at the level of the RA, the saturation in the PA and aorta is identical.

With the obstructive variant of TADPV, the outflow of oxygen-enriched blood from the pulmonary veins is difficult. This leads to the development of pulmonary venous hypertension and then to increased pressure in the PA and RV. A situation is created that resembles the hemodynamics of mitral valve stenosis. In this case, there is a high risk of developing pulmonary edema, because hydrostatic pressure in the capillaries becomes significantly higher than the osmotic pressure of the blood. As long as the existing ASD provides a sufficient volume of right-to-left shunting of blood, the pancreatic cavity remains small. The left chambers of the heart, as in the case of the non-obstructive variant of TADLV, remain “underloaded” and have a relatively small size. The level of blood oxygen saturation in the aorta and pulmonary artery is equal, but its values are significantly lower than in the non-obstructive version of the defect. The degree of arterial desaturation will be inversely proportional to the volume of blood flow through the ICC.

The prognosis for patients with TADLV is extremely unfavorable. Without surgical correction, two thirds of patients with the non-obstructive form of the disease die by the end of the first year of life.

The cause of death is often pneumonia. With the obstructive variant, life expectancy is months.

CLINIC

A. Clinical manifestations of the disease:

Obstructive variant of TADLV:

Characterized by rapidly progressing cyanosis from birth, which intensifies with feeding, which is associated with compression of the pulmonary veins by the esophagus;

Dyspnea and signs of pulmonary edema in the neonatal period.

Moderate cyanosis from birth;

Retarded physical development, frequent bronchopulmonary infections;

Signs of heart failure (tachycardia, shortness of breath, hepatomegaly).

b. Physical examination:

Obstructive variant of TADLV:

Loud II sound at the base of the heart;

Non-obstructive version of TADLV:

The second tone at the base of the heart is split (the pulmonary component of the second sound is accentuated);

Weak or moderate intensity (no more than 3/6) systolic murmur of relative stenosis of the pulmonary artery valve in the 2nd m.r. to the left of the sternum;

Mesodiastolic murmur of relative stenosis of the TC (tricuspid valve) along the left edge of the sternum in the lower third (with a significant flow from the RA).

DIAGNOSTICS

Electrocardiography

Obstructive variant of TADLV:

pancreatic hypertrophy (R-type);

Less commonly, RA hypertrophy.

Non-obstructive version of TADLV:

RV hypertrophy according to the type of right bundle branch block in lead Vj (a consequence of volume overload of the RV);

Less commonly, RA hypertrophy.

Diagnosis of TADPV is based on the absence of all pulmonary veins draining into the left atrium in typical locations.

Sharp dilatation of the right heart;

Dilatation of the pulmonary artery;

Significant reduction in left cameras;

Dilated vertical vein and superior vena cava;

Presence of ASD with right-left shunt.

TREATMENT AND OBSERVATION

1. Observation and treatment of patients with uncorrected TADLV

The obstructive variant of TADLV is an emergency indication for surgery. Before the patient is admitted to the cardiac surgery clinic, the following actions must be taken:

A. IV infusion of prostaglandin E 1 preparations, which will maintain the duct open and ensure the discharge of blood from the PA into the aorta. This will reduce the risk of developing pulmonary edema and increase the volume of blood flow in the BCC.

V. Correction of metabolic acidosis.

Non-obstructive version of TADLV:

A. Intensive therapy for heart failure (diuretics, digoxin).

b. If metabolic acidosis occurs, it is corrected.

When a diagnosis of TADLV with the presence of a small/restrictive ASD is made, the Rashkind procedure is performed to increase the volume of blood flow in the BCC. The indication for the procedure is the presence of restrictive communication between the atria - a pressure gradient of more than 6 mm Hg.

Indications for surgical treatment:

Establishing a diagnosis of TADLV is an absolute indication for surgery.

Contraindications to surgical treatment:

The presence of absolute contraindications for concomitant

High venous resistance of pulmonary vessels.

Surgical tactics

The timing of surgery is determined by the presence or absence of pulmonary venous obstruction.

In case of obstructive TADLV, intervention is performed immediately within the first hours after diagnosis. This is usually the first hours of a child's life.

For non-obstructive TADLV, surgical treatment can be delayed and performed during the first months of life.

Surgical technique

Under IR conditions. The common collector of the PV is identified at the place of its confluence. Usually the collector is of sufficient length (even in the case of infracardiac drainage) to move it into the left atrium. The collector is opened widely, eliminating areas of narrowing. The left atrium is widely opened. A wide anastomosis is formed between the PV collector and the left atrium. Additional elements of the venous system (vertical vein, etc.) are ligated.

Specific complications of surgical treatment

Residual pulmonary hypertension;

Heart rhythm disturbances (sick sinus syndrome), atrial tachycardia;

Reduced cardiac output due to residual PV outflow tract obstruction.

Postoperative follow-up

Monitoring is carried out every month. The duration of observation is determined individually. Late complications of surgical correction of the defect are monitored: pulmonary vein stenosis, cardiac arrhythmias.
If NRS is registered in the postoperative period, in addition to the examination, it is recommended that SMECG be performed every 6 months or more often. If indicated, antiarrhythmic therapy, RFA, or pacemaker implantation are performed.
Prevention of bacterial endocarditis is carried out according to indications if there is pulmonary vein stenosis.
Admissibility of physical education after correction of the defect. Physical activity is limited in patients with pulmonary vein stenosis.

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Total anomalous pulmonary venous drainage (TAPVD)

Total anomalous drainage of the pulmonary veins is a defect in which there is no direct connection of the pulmonary veins with the left atrium. The pulmonary veins drain abnormally into the right atrium or its tributaries. Almost all patients have a patent foramen ovale or ASD.

TADLV was first described by Wilson in 1798. In 1951, Muller performed the first successful operation. It was palliative in nature. Without using open heart technology, an anastomosis was performed between the pulmonary vein collector and the left ear. In 1956, Lewis, Varco and co-authors reported successful correction of the defect under conditions of moderate hypothermia created by surface cooling and temporary occlusion of the venous inflow to the heart. In the same year, Burroughs and Kirklin performed correction of the defect under IR conditions. Experience in subsequent years showed that mortality in infants was significantly higher than in older children. Over time, reports began to appear in the literature about the success of operations in severe patients with obstructive type of TADLV. Improved outcomes were associated with the introduction of hypothermic perfusion with reduced blood flow and circulatory arrest.

The incidence of TADLV, according to various studies, varies from 0.83 to 2.8%.

Morphogenesis

The association of TADLV formation with known external teratogenic agents has not been clearly established, although exposure to lead, paints, solvents, and pesticides may be a likely cause. The genetic nature of TADLV has not been identified, but a monogenic mode of inheritance cannot be excluded due to the many reports of familial cases among consanguineous brothers and sisters. The gene for this familial form of TADLV is associated with chromosome 4q13-q12. In the same region, the vascular endothelial growth factor receptor gene was found, which is associated with familial and, probably, sporadic forms of TADLV.

TADLV is combined with a number of congenital syndromes - asplenia, polysplenia, cat eye syndrome. With cat eye syndrome, 50% of patients have congenital heart defects such as TADLV, tetralogy of Fallot, VSD, and ATK.

Anatomy

According to the clinical classification of Darling et al., depending on the level of drainage, the defect is represented by four options:

Supracardial. Makes up 50% of all TADLV. The common collector of the pulmonary veins, located behind the left atrium, drains into the SVV through the left vertical and left innominate veins.

Intracardiac. It is observed in 20% of patients with TADLV. The common collector of the pulmonary veins drains into the coronary sinus or they separately flow into the right atrium with four orifices.

Infracardiac. Occurs in 20% of cases. The common collector of the pulmonary veins drains into the portal vein, ductus venosus, hepatic vein and the IVC. The common pulmonary vein, through a vertical vein that pierces the diaphragm in the area of the esophageal opening, connects with the portal veins and the IVC through the ductus venosus or hepatic sinusoids.

Mixed. Occurs in 10% of patients. This type is a combination of the previous options.

The patient’s life is possible only if there is a concomitant ASD or an open oval window, which are an integral part of the defect. For the same reason, persistent patent ductus arteriosus in young children is not a complicating anomaly.

Regardless of the location of the abnormal connection of the pulmonary veins, the heart in patients with TADPV has common anatomical signs: dilatation and hypertrophy of the right ventricle and right atrium, dilatation of the pulmonary artery. The left side of the heart is relatively undeveloped, especially the left atrium. Its volume is reduced to 50% of normal.

Connection with the right atrium

The junction is usually located in the inferoposterior part of the right atrium. The common pulmonary vein or separately two, three or four pulmonary veins drain into the right atrium. Narrowing of anomalous connections of the pulmonary veins is rare. Concomitant heart defects are often observed.

Connection with the right common cardinal system

The pulmonary veins on both sides form a common collector located behind the left atrium. A single venous trunk departs from the right side of this collector, which ascends, passes in front of the root of the right lung and flows posteriorly into the SVC. In rare cases, it connects with v.azygos. Domestic surgeons encountered this variant of TADLV in one patient. An exact diagnosis was not made before surgery. External and intracardial examination did not reveal the junction of the collector with the right parts of the heart, despite the fact that the SVC was significantly dilated. After creation of an anastomosis between the pulmonary vein collector and the left atrium and weaning from CPB, cardiac output was reduced, which was manifested by arterial hypotension. During a test short-term clamping of the SVC at the site of its entry into the right atrium arterial pressure rose adequately. It became clear that there was a leak of blood along a short path from the left atrium to the SVC. The entire posterior wall of the SVC was isolated and a dilated azygos vein was detected. After her dressing, normal hemodynamics were restored.

Connection to the left common cardinal system

Left innominate vein.

In this, the most common variant of TADLV, the pulmonary veins on both sides form a common collector behind the left atrium. The vertical vein, arising from the left side of the collector, usually passes in front of the left pulmonary artery and main bronchus, ascends into the superior mediastinum, passes in front of the aortic arch and joins the left innominate vein proximal to the junction of the left jugular and subclavian veins. The left innominate vein merges normally with the SVC. Less commonly, the vertical vein runs between the left pulmonary artery and the left main bronchus. These structures compress it externally, causing obstruction of the pulmonary venous outflow. The venous trunk connecting the manifold to the left innominate vein is called the persistent left superior vena cava, or anomalous vertical vein.

The vessel connecting the pulmonary vein collector directly to the SVC, bypassing the innominate vein, passes between the right pulmonary artery and the trachea and is compressed by them. One of the options for obstruction of the vertical vein is its discrete or diffuse narrowing.

The abnormal venous pathway is within the pericardium. The pulmonary veins merge with a common trunk, which connects with the coronary sinus in the area of the atrioventricular groove. The coronary sinus then follows the normal course into the right atrium, and the opening is, as usual, located between the IVC and the tricuspid valve. The veins of the heart drain normally into the coronary sinus. The latter is covered with fibers of the left atrium. The coronary sinus shares a common wall with the left atrium along almost its entire length. There may be narrowings at the junction of the common pulmonary vein with the coronary sinus or within it.

Connection with the umbilical-vitelline system

The distal junction is located below the diaphragm. The pulmonary veins on both sides form a common collector behind the left atrium. The common venous trunk arises from the middle part of the collector, descends anteriorly to the esophagus and penetrates the diaphragm through the esophageal opening. Most often, the anomalous vein connects with the portal vein at the confluence of the splenic and superior mesenteric veins. Less commonly, the anomalous trunk connects to the ductus venosus, to one of the hepatic veins, or directly to the inferior vena cava. In this type of TADPV, there is usually obstruction of the pulmonary venous return.

Sites of anatomical obstruction of pulmonary venous drainage

Many patients with supra- and intracardial types of TADPV and most patients with the infracardial type have pulmonary hypertension due to obstruction of the pulmonary venous return in the form of narrowing of the pulmonary vein orifices, the collector orifice, or external pressure on the anomalous vein. The presence of obstruction in the anomalous pulmonary venous channel has a decisive influence on the hemodynamic status and clinical manifestations.

Obstruction at the level of the interatrial septum.

A small interatrial communication may obstruct the flow of blood into the left atrium and thereby impair the outflow of blood from the pulmonary veins. The life expectancy of patients with TADLV is apparently related to the size of the ASD. Patients with large defects live longer than patients with restrictive interatrial communications.

Narrowing of the pulmonary veins and their mouths is especially common with atrial isomerism. Histological examination reveals thickening of the media and adventitia, as well as proliferation of the intima.

Obstruction of abnormal venous pathways.

One of the causes of obstruction is external pressure. Thus, with supracardial TADPV, the vertical vein may pass behind the left pulmonary artery and be compressed in the narrow space between the bronchus, pulmonary artery and patent ductus arteriosus or ligament. It may become compressed between the right pulmonary artery and trachea. Internal narrowing also occurs with the typical passage of a vertical vein anterior to the left pulmonary artery. With infracardial TADLV, compression of the vertical vein occurs in the esophageal opening. In cases where the vertical vein connects with the ductus venosus, its natural narrowing disrupts the outflow of blood from the pulmonary veins. Finally, when the anomalous vein connects with the portal vein or one of its tributaries, the hepatic sinusoids compress the pulmonary venous conduit, obstructing pulmonary venous return.

Atresia of the common pulmonary vein

Rarely, common pulmonary vein atresia occurs, which is a fusion of all pulmonary veins that has no connection with the heart or systemic veins. The short life expectancy is possible due to the small connections between the bronchial and pulmonary veins. With this pathology, dilatation of the pulmonary lymphatic vessels is pronounced. Common pulmonary vein atresia is the most severe form of obstructive TADPV, which manifests as severe pulmonary edema and severe cyanosis immediately after birth. Diagnosis is made using echocardiography and angiography. Surgery is successful only in exceptional cases. ECMO is used before and after surgery until the lung condition improves.

Associated heart defects

TADLV is combined with many congenital heart diseases, especially often in patients with atrioabdominal heterotaxy:

tetralogy with an absent pulmonary valve;

interruption of the aortic arch;

corrected and complete transposition of the great vessels;

ventricle with two outlets;

hypoplastic left heart syndrome;

Turkish saber syndrome.

Lung microstructure

The histological picture depends on the presence or absence of pulmonary venous obstruction. In the absence of obstruction in the muscular arteries and arterioles, there is pronounced hypertrophy of the medial layer. Intimal growth is rarely seen in infants but is commonly seen in older children and adults.

TADPV with obstruction is characterized by medial hypertrophy in the walls of anomalous venous channels, extrapulmonary veins, and small pulmonary veins. It has been shown that the walls of the pulmonary arteries and veins with abnormal drainage are significantly thicker than normal and with VSD and pulmonary hypertension. Thickening of the medial layer in arteries and veins is more pronounced in the presence of pulmonary venous obstruction. Thickening of the muscle layer in small pulmonary arteries in TADPV correlates with the magnitude of pulmonary artery pressure. At the same pressure in the pulmonary artery, media thickening is always more pronounced in TADPV than in VSD. The thickness of the middle layer of the pulmonary veins is also closely related to the level of pulmonary arterial hypertension in patients with TADPV. Intimal proliferation is usually present in the arterioles, and necrotizing arteritis is often noted.

The interalveolar spaces are characterized by edema and erythrocyte extravasation. Subpleural and interlobular lymphatic vessels are dilated. Lymphangiectasia is observed in 62% of patients with TADLV. It is accompanied by interstitial emphysema, which is one of the causes of postoperative mortality.

Hemodynamics

The right atrium receives blood from both circulations. Arterial blood enters the right atrium from a collector into which the pulmonary veins flow. In the atrium, it mixes with the venous blood, from here, through the VSD or open oval window, part of the blood enters the left atrium and then into the systemic circulation; the other part is sent to a small circle. The nature of blood circulation depends on the distribution of mixed venous blood between the pulmonary and systemic circles. Blood circulation is primarily determined by the size of the interatrial communication. If it is restrictive, the amount of blood entering the left atrium is small and systemic blood flow is reduced. The decrease in systemic blood flow is partially compensated by an increase in right atrial pressure. Since all systemic and pulmonary veins drain into the right atrium, the pressure in both venous systems is increased.

With a wide open foramen ovale or ASD, blood moves freely between the two atria. Under these conditions, the distribution of mixed venous blood is determined by the ratio of the resistances of the systemic and pulmonary circulation and the distensibility of the ventricles. In patients with obstruction of the pulmonary veins, venous and, in connection with this, arterial pulmonary hypertension develops. In the absence of involution of the fetal structure of the pulmonary arteries, primary pulmonary hypertension sometimes occurs.

TADLV is characterized by overload of the right heart and pulmonary hypertension.

TADLV without venous obstruction

At birth, the distribution of blood flow between the greater and lesser circles is approximately equal, since vascular resistance is almost equal. During the first few weeks of life, the pulmonary vascular bed matures, pulmonary arterial resistance decreases, and the volume of mixed venous blood entering the pulmonary circulation gradually increases. Pulmonary blood flow is 3-5 times higher than systemic blood flow. Systemic blood flow is normal. Because the mixed venous blood pool receives 3 to 5 parts fully arterialized blood to one part desaturated systemic venous blood, oxygen saturation of blood in the right atrium can exceed 90%. As a rule, the blood in the right atrium is well mixed, so the blood saturation in the right ventricle, pulmonary artery, left atrium, left ventricle and aorta is equal to that in the right atrium.

With TADLV, dilatation and hypertrophy of the right ventricle and dilatation of the pulmonary artery are noted.

Pulmonary artery pressure in infants varies from mildly elevated to systemic. In the few children who survive into adolescence or beyond, pulmonary artery pressure is slightly elevated. Over time, medial hypertrophy and intimal proliferation develop in the pulmonary arterioles, so more pronounced hypertension is observed in the 3rd and 4th decades of life.

TADLV with pulmonary venous obstruction

The increased pressure in the pulmonary venous channels is transmitted to the pulmonary capillary bed. When the hydrostatic pressure in the capillaries exceeds the osmotic pressure of the blood, pulmonary edema develops. Mechanisms aimed at preventing pulmonary edema are increased lymphatic flow, alternative pulmonary venous bypass channels, decreased pulmonary capillary wall permeability, and reflex arteriolar spasm. The latter leads to decreased pulmonary blood flow, pulmonary hypertension, increased pressure in the right ventricle, hypertrophy and right ventricular failure. A decrease in the volume of pulmonary blood flow leads to a decrease in the proportion of oxygenated blood in mixed venous blood and a decrease in saturation in the arteries of the systemic circulation.

Clinic, diagnostics

The clinical course of TADLV is determined by the anatomical and hemodynamic features of the defect, in particular:

level of pulmonary resistance;

degree of pulmonary venous obstruction;

the size of the interatrial communication;

condition of the right ventricular myocardium;

the presence of a functioning ductus botallus.

TADLV without pulmonary venous obstruction

These babies have no symptoms at birth. Soon, half of the children experience shortness of breath, coughing, feeding difficulties, recurring respiratory infections and heart failure. For others, symptoms appear by the first year of life.

Cyanosis can appear at any age. Initially, cyanosis is not expressed and intensifies in the presence of heart failure, as well as as a result of gradually developing secondary changes in the pulmonary vessels. By the first year of life, 75-85% of children die, most in the first 3 months. Babies are lagging behind in physical development, irritable, and their faces darken when they cry or are stressed. Dyspnea, rapid breathing and tachycardia almost always occur. The cardiac hump, predominantly right-sided, appears earlier than with isolated ASD.

The auscultatory picture is characterized by the presence of many heart sounds. The first tone is loud and clear, followed by the expulsion tone. The second tone is widely split and does not change with breathing. The pulmonary component of tone II is accentuated. The third tone is almost always heard, maximally at the apex. In older patients, a fourth heart sound is almost always heard.

Heart murmurs may occasionally be absent. No shaking is felt. A soft blowing systolic murmur is usually heard in the pulmonary artery. Often the noise is clearly audible on the xiphoid process and along the lower edge of the sternum on the left. Murmurs result from turbulent flow in the pulmonary outflow tract and tricuspid valve insufficiency. In half of the cases, a diastolic murmur of increased blood flow through the tricuspid valve is heard along the left edge of the sternum below. When draining the collector into the left innominate vein, a prolonged systolic murmur can be heard on the right or left at the base of the heart. Unlike functional venous murmur, pathological murmur does not increase in diastole and does not change when changing position or pressing on the jugular veins.

In heart failure, the liver is enlarged and there is peripheral edema. In some children who have survived infancy, the terminal phalanges of the fingers are thickened.

A typical sign is a high, pointed P wave in lead II or in the right leads, reflecting enlargement of the right atrium. The electrical axis is tilted to the right. There are always signs of right ventricular hypertrophy, manifested by high wave voltage in the right leads and incomplete right bundle branch block.

The purpose of ultrasound examination of the TADLV is to confirm the clinical diagnosis and localize the site of abnormal drainage. Doppler studies can detect the presence of obstruction in individual pulmonary veins and along the vertical vein. A sign common to all forms of TADLV is volume overload of the right ventricle. The right ventricle is dilated. The right atrium and pulmonary arteries are also dilated, the interatrial septum bulges to the left. Paradoxical movement of the interventricular septum is noted.

In addition to signs of volume overload of the right ventricle, suspicion of TADPV is caused by the inability to trace the entry of the pulmonary veins into the left atrium along the high parasternal or suprasternal short axis. From this, as well as the subcostal position, an echo-free space can be detected, which is the common pulmonary venous canal behind the left atrium with the pulmonary veins flowing into it. Once the common venous collector has been identified, its drainage site should be located. In the supracardial form of TADLV, the ascending vertical vein drains into a dilated systemic venous structure, most often the left innominate vein or the left innominate vein. Doppler examination of the vertical vein reveals the direction of blood flow upward and the opposite direction of blood in the superior vena cava.

When draining the TADLV into the coronary sinus, the latter is dilated and can be detected along the long parasternal axis or from the apical four-chamber position.

Doppler examination of the vertical vein reveals the direction of blood flow upward, in contrast to the opposite movement in the superior vena cava.

If the common pulmonary vein collector drains into the coronary sinus, the latter is dilated and can be detected from the four-chamber apical position. The dilated coronary sinus bulges anteriorly and superiorly into the left atrium as it is located in the atrioventricular groove. If the collector drains directly into the right atrium, it is located higher than when draining into the coronary sinus.

In infradiaphragmatic TADLV, the common pulmonary vein collector drains into the portal venous system, but may connect to the hepatic veins. The pulmonary veins converge into a common canal, which is often small and located below the left atrium and just above the diaphragm. It is visible from the subcostal position. The common descending vein lies between the aorta and the IVC. These vessels can be differentiated by the direction and nature of blood flow during Doppler echocardiography: in the aorta - pulsating laminar in the direction from the heart, in the inferior vena cava - almost constant towards the heart. Flow in the descending vertical vein resembles that in the IVC, but directed away from the heart.

To diagnose mixed TADLV, it is necessary to use different sensor positions. The most common type of mixed drainage is a combination of drainage into the coronary sinus and the left innominate vein.

Doppler examination is the best method to assess the presence of pulmonary venous obstruction. Unobstructed pulmonary venous flow is characterized by almost constant biphasic or triphasic flow, with a short period of reversal in early diastole. Obstruction within one of the components of the pulmonary venous pathway is manifested by high-velocity direct monophasic flow. Obstruction of the pulmonary venous return may be masked if pulmonary blood flow is reduced. It can be unmasked by introducing prostaglandin E1. Discrete narrowing or compression along the pulmonary venous pathway between structures such as the branch of the pulmonary artery and the bronchus can be seen directly.

Color Doppler studies provide information about the direction and average velocity of flow through the anomalous connection of the pulmonary veins. This allows you to quickly identify the junction of the pulmonary venous channel with the systemic vein. The obstruction creates flow turbulence and appears as a mosaic colored jet. Once the site of turbulence is detected, the degree of narrowing is assessed by the peak value of the spectral flow velocity during Doppler echocardiography.

For each type of TADLV, the possibility of associated defects, such as endocardial cushion defects, pulmonary atresia, single ventricle, and TMA, is carefully examined. Errors in diagnosing TADPV are more likely with anomalies of the atrial situs, mixed drainage of the pulmonary veins and associated defects.

Antenatal echocardiography is increasingly used to diagnose heart defects in high-risk cases. The normal connection of the pulmonary veins to the left atrium can be determined from the apical four-chamber position of the fetal heart. Because pulmonary blood flow is reduced in the fetus, identification of abnormal pulmonary venous drainage is difficult. An indirect sign of TADPV is the dilation of the right ventricle and pulmonary artery, which is not proportional to the size of the left ventricle and aorta, especially when other defects that create the same picture are excluded. Despite the difficulties of prenatal diagnosis, there are isolated reports in the literature of cases of an unmistakable diagnosis of TADLV confirmed after birth. Conversely, cases of TADLV first detected in the neonatal period are presented, despite prenatal echodiagnosis.

TADLV can be diagnosed by non-invasive MRI. This method detects the pulmonary veins entering the chamber behind the left atrium. The common pulmonary vein can be seen to connect to the left innominate vein, coronary sinus, or right atrium.

Common to all types of TADLV are signs of increased pulmonary blood flow. The right atrium and right ventricle are dilated and hypertrophied, the pulmonary artery arch is bulging. The chambers of the left heart are not dilated. The junction of the pulmonary venous collector may affect the configuration of the cardiac shadow. With abnormal drainage into the innominate vein, the shadow of the heart resembles a figure eight or a snowman. The upper part of the figure eight is formed by the vertical vein on the left, the left innominate vein on top and the superior vena cava on the right. The characteristic radiological signs are usually absent in the first few months of life and are often seen in older children and adults. With abnormal drainage of the pulmonary veins into the superior vena cava, its dilatation is manifested by bulging of the right upper edge of the heart shadow.

Indications for cardiac catheterization are currently limited due to the high sensitivity and 99% specificity of echocardiographic diagnosis of TADLV. Catheterization is performed to clarify important details that were not clarified during an echo CG study:

identification of associated defects;

detecting locations of anomalous connections;

localization of venous obstruction.

The site of an anomalous connection between the pulmonary and systemic veins can be detected by an oxygen “spike” at the level of the left innominate vein, right superior vena cava, or coronary sinus. Abnormal drainage into the right atrium is identified by increased oxygenation at the level of the right atrium, as with other left-to-right shunts into the right atrium. In TADLV, oxygen saturation in the right atrium fluctuates between 80 and 95%. Approximately the same level of saturation in the right ventricle, pulmonary artery, left atrium, left ventricle and systemic arteries. When the pulmonary venous return is abnormally drained into the left innominate vein or SVC, mixed blood flows predominantly into the right ventricle, and blood from the inferior vena cava is shunted primarily into the left atrium, so the blood saturation in the pulmonary artery may be higher than in the systemic arteries.

Right ventricular and pulmonary artery pressures range from mildly elevated to systemic levels. Pressure in the right atrium does not fully reflect the adequacy of interatrial communication. The presence of equal pressure values in both atria is not a reliable sign of a non-obstructive interatrial defect. This phenomenon is explained by the almost identical distensibility of the two ventricles, so the filling pressures are almost equal even with restrictive interatrial communication. Positive pressure gradient of only 2 mm Hg. Art. or more may indicate a small ASD, but may also occur with a larger message. The only reliable way to assess the size of the interatrial communication is to measure it with a balloon catheter.

Selective pulmonary arteriography significantly clarifies the results of EchoCG. The passage of the contrast agent through the pulmonary arteries and its appearance in the pulmonary venous canals gives a clear idea of the anatomy of the defect. In TADLV in the left innominate vein, a vertical vein can be seen arising from the pulmonary vein collector and connecting to the left innominate vein. Next, you can monitor the flow of contrast agent into the SVC.

If there is abnormal drainage into the coronary sinus, contrast material collects in the common pulmonary vein and enters the coronary sinus in the posterior part of the heart. In the anteroposterior projection, the coronary sinus appears as an ovoid formation in the middle and lower part of the right atrium. The anatomical details of the coronary sinus are better defined in the lateral projection.

When the pulmonary veins drain directly into the right atrium, extracardiac structures are not visualized and contrast material quickly enters the right atrium.

If the catheter enters an abnormal vein, the blood saturation reaches 100%. Selective angiography is useful, but it should be remembered that the catheter may obstruct sites of pulmonary vein obstruction.

TADLV with pulmonary vein obstruction

Pulmonary venous obstruction always occurs with infracardial TADLV; in 50% of cases - with supracardial drainage, less often - with drainage into the coronary sinus or into the right atrium. Regardless of the location of the obstruction, the clinical picture is similar. Symptoms usually do not appear during the first 12 hours of life, which makes it possible to differentiate this defect from respiratory distress syndrome. Symptoms of obstructive TADLV include progressive shortness of breath, feeding difficulties, and heart failure.

Children die within 2 days - 4 months. life. Infracardial drainage is characterized by cyanosis and shortness of breath, which worsens with straining and swallowing due to increased intra-abdominal pressure or compression of the common pulmonary vein by the esophagus, as is observed with a hiatal hernia.

Although symptoms are severe, cardiovascular signs may be minimal. The heart is not dilated, the right ventricle is not elevated. The second tone is usually split and the pulmonary component is accentuated. There is no cardiac murmur, or it is soft, blowing over the pulmonary artery. Usually, moist rales are heard in the lower parts of the lungs. Hepatomegaly is almost always observed, accompanied by peripheral edema.

The ECG shows signs of right ventricular hypertrophy. In contrast to TADPV without venous obstruction, there is usually no evidence of right atrium enlargement.

If there are clinical signs of obstructive TADPV, it is necessary to carefully examine the individual pulmonary veins, the pulmonary vein collector and its drainage into the right-sided structures of the heart in order to locate the site of narrowing. It is characterized by a high-speed, constant and monophasic flow of venous blood.

The size of the heart shadow is almost normal. The pulmonary pattern is enhanced and is characterized by diffuse fan-shaped compactions radiating from the roots. The boundaries of the heart shadow are unclear. There are no specific radiological signs of TADLV with obstruction.

Detection of highly oxygenated blood in the superior or inferior vena cava is of great diagnostic importance. However, this fact should be interpreted with caution, since, on the one hand, due to a decrease in pulmonary blood flow, the saturation of mixed pulmonary and systemic venous blood may be low, on the other hand, in newborns with TADLV in the portal venous system, the blood from the umbilical vein is completely oxygenated, which confirms diagnosis. Right ventricular pressure is usually equal to or greater than systemic pressure. Atrial pressure is normal, which contrasts with high pressure in the pulmonary arterial vascular bed.

Pulmonary angiogram is a reliable method for diagnosing TADPV in the portal venous system with venous obstruction. Sites of obstruction may also be found in other locations of abnormal pulmonary venous drainage. Given the significant delay in the passage of the contrast agent through the pulmonary vascular bed, it appears in the pulmonary venous system with a delay of up to 12 s after administration.

If the catheter is inserted into an abnormal venous channel, it may pass through the area of narrowing with the risk of significant or complete obstruction of venous outflow, so the injection of contrast agent must be done quickly and manually.

Differential diagnosis

TADPV without obstruction should be differentiated from large VSD, ductus arteriosus, OSA, AVSD, and single ventricle without pulmonary stenosis. Unlike TADLV, with all these anomalies there are radiological and ECG signs of hypertrophy of the left atrium and left ventricle. Not typical for TADLV various noises hearts characteristic of comparative defects.

In older children and adult patients, differentiation should be made from ASD, common atrium and PAD. With TADLV, there is usually at least moderate cyanosis, which does not occur in individuals in the first or second decades of life with various defects with an arteriovenous shunt at the atrial level.

TADVC with obstruction must be differentiated from other causes of pulmonary venous obstruction and from hypoplastic left heart syndrome, tricuspid atresia, pulmonary atresia, coarctation, TMA, respiratory distress syndrome, and persistent fetal circulation. Severe cardiomegaly usually accompanies valvular atresia and coarctation of the aorta and is a clear distinguishing feature that is absent in obstructive TADPV. An enhanced arterial vascular pattern is characteristic of TMA and hypoplastic left heart syndrome. Decreased pulmonary vascularity is typical of pulmonary and tricuspid atresia. Obstruction of pulmonary venous return may result from mitral regurgitation or left ventricular failure. These causes are easily diagnosed based on characteristic clinical signs, in particular left ventricular hypertrophy and dilatation. The absence of left ventricular hypertrophy is characteristic of mitral stenosis, which is also a cause of impaired blood outflow from the lungs. The presence of left atrial hypertrophy indicates that the narrowing is located close to the mitral valve. This may be mitral stenosis or left atrial supravalvular ring stenosis, in which the pressure in the left atrium is increased.

In patients with right ventricular hypertrophy, TADPV must be differentiated from stenosis of individual pulmonary veins, triatrial heart, and atresia of the common pulmonary vein. In all these cases, the pressure in the arterial bed of the lungs is increased, and the pressure in the left atrium is normal.

Identification of obstructive defects is carried out using selective angiography. Important diagnostic criteria are the detection of a right-to-left shunt and the duration of filling of the left atrium.

EchoCG is especially informative in differentiating TADLV from persistent fetal circulation and respiratory distress syndrome.

Natural course

The prognosis of TADPV depends on the size of the interatrial communication, the presence of pulmonary venous obstruction, and pulmonary arterial resistance in children fortunate enough to survive several years.

In a review by Keith et al, including all types of TADLV, 50% of children with this diagnosis died by the 3rd month of life and 80% by the first year. Burroughs and Edwards data are similar. Patients with inadequate interatrial communication have an even worse prognosis. The prognosis for patients with obstruction of an abnormal venous channel is catastrophic. They usually die within the first few weeks of life; the oldest child lived for 4.5 months. Some patients survive infancy due to increased pulmonary arterial resistance. This adaptive mechanism may provoke the physician to attempt a risky surgical intervention. Pronounced changes in the intima of the pulmonary arterioles are detected already at 8 months of age.

Abnormal drainage of the pulmonary veins in adults. Abnormal pulmonary vein drainage

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Total anomalous pulmonary venous drainage in newborns

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Total anomalous pulmonary venous drainage (TAPVD)

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