The recovery period after radical surgery for tetralogy of fallot. Possible complications in patients after radical correction of tetralogy of fallot

In recent years, more and more children are being born with malformations of the cardiovascular system. There are many reasons for such pathologies. Poor ecology, poor nutrition of the expectant mother, regular stressful situations, etc. have a particularly negative impact on health. Tetralogy of Fallot is considered one of the most severe heart defects. Even in the last century, people with this diagnosis were considered doomed. Heart operations were performed only to prolong life for a short time and alleviate the condition.

But medical science did not stand still; scientists all over the world tried to invent new methods of curing patients. Currently, the introduction of new technologies makes it possible to successfully cope with such pathologies, provided that the operation is performed in early childhood.

From the name it is clear that tetralogy of Fallot is not one, but several heart pathologies:

when the membrane part is most often missing;
the volume of the right ventricle is significantly higher than normal;
decrease in the lumen in the pulmonary trunk;
right displacement of the aorta.

Tetralogy of Fallot of the heart is associated with childhood diseases, since the disease is congenital and manifests itself in infancy. The patient's life expectancy depends on the degree of heart damage. If the operation is postponed for any reason, then for the young patient this can lead to dire consequences, even death.

Tetralogy of Fallot in children can be complicated by a fifth anomaly - atrial septal defect, which turns the disease into a pentade of Fallot.

Reasons

The causes of tetralogy of Fallot are tissue hypoxia. The disease is also called a “blue” defect due to the specific coloring of the skin due to oxygen starvation of the tissue (). Hypoxia occurs due to a defect in the ventricular septum, as a result of which the blood flow changes and blood depleted of oxygen enters the systemic circulation.

The situation is further aggravated by the fact that a narrowing occurs in the area of the pulmonary trunk, and then a sufficient amount of venous blood cannot go into the lungs. A significant amount of it is retained in the right ventricle and in the venous part of the systemic circulation.

This mechanism of venous stagnation provokes chronic heart failure, the symptoms of which are:

severe manifestation of cyanosis;
changes in tissue metabolism;
fluid retention in various cavities;
swelling.

To prevent such development, cardiac surgery is strongly recommended to the patient.

Tetralogy of Fallot in newborns manifests itself immediately in the form of an increase in CHF, although in the youngest patients the development of acute CHF is also possible.

The appearance of the baby depends on the severity of the symptoms, as well as on the size of the septal abnormality. The dependence is directly proportional, that is, the stronger the violation, the higher the rate of manifestation of symptoms. The first symptoms of heart disease can be observed already at about one month of age.

The main symptoms of tetralogy of Fallot:

cyanosis of the skin during crying, breastfeeding, and later at rest;
delayed physical development, which manifests itself in the fact that the child sits up later, crawls, holds his head, and so on);
thickening of the fingertips, resembling drumsticks;
thickening of the nail plates;
flattening of the chest;
decrease in muscle mass;
dental growth disorders;
scoliosis of the spine;
sharply developing flat feet.

During attacks, some characteristic features also appear:

breathing problems that become frequent and deep;
blue-violet skin tone;
suddenly dilated pupils;
severe weakness;
loss of consciousness to hypoxic coma;
convulsions.

Older guys squat down during attacks, as during this position the condition is alleviated. The duration of the attack is up to five minutes. After such situations, children talk about severe weakness. Severe cases lead to a heart attack.

Help

We must always remember that postoperative mortality reaches up to 10%, but without surgical intervention, the life of children lasts no more than thirteen years. When the operation is performed before the age of five, up to 90% of patients do not have developmental delays at the age of fourteen.

Forecast

The prognosis after tetralogy of Fallot surgery is positive for 80% of children. These children lead a normal lifestyle, absolutely no different from their peers.

For all patients after such heart surgery, disability is issued for two years, after which a re-examination is done.

A competent specialist will identify Tetralogy of Fallot at the stage of intrauterine development during an ultrasound scan. For accurate diagnosis, the level of the ultrasound machine must be high.

At the consultation, the woman is asked to make a decision on terminating or prolonging the pregnancy. If only a defect is detected without other organ pathologies, then the pregnant woman is explained the methods of treating tetralogy of Fallot. It is important to always remember that this pathology is operable and can be corrected. The main thing is that such a disease is not a death sentence for a child.

Corrections for tetralogy of Fallot:

Cardiac enlargement on chest x-rays should help identify the causes of hemodynamic disorders.
The development of arrhythmias (atrial or ventricular) should prompt a search for hemodynamic causes.
In case of arterial hypoxemia, it is advisable to search for a patent foramen ovale or ASD with a right-to-left shunt.
Dilatation or dysfunction requires a search for residual hemodynamic disturbances of the RV, which usually reveals significant pulmonary regurgitation and tricuspid regurgitation.
Some patients may have LV dysfunction. This may be a consequence of prolonged CPB and insufficient protection of the myocardium, or injury to the coronary artery during surgery. This may be secondary to severe pancreatic dysfunction.

10.7. Recommendations for clinical examination and postoperative management

Class I

Regular clinical examination is indicated for all patients after radical correction. Particular attention should be paid to the degree of pulmonary regurgitation, pressure in the RV, its size and function, and the degree of tricuspid regurgitation. The frequency of examinations is determined based on the severity of hemodynamic disorders, and should be at least once a year (Evidence level: C).
10.8. Recommendations for the rehabilitation of patients after radical correction of tetralogy of Fallot

Class I

1. Patients after radical correction of tetralogy of Fallot should be examined annually by a cardiologist who is an expert in congenital heart disease (Level of Evidence: C)

2. Patients with tetralogy of Fallot need to undergo echocardiography annually, MRI once every 2-3 years by specialists with experience in working with congenital heart disease (Level of evidence: C)

3. Hereditary pathology testing (eg, 22qll) should be offered to all patients with tetralogy of Fallot (Level of Evidence: C)
All patients should undergo regular annual follow-up with a cardiologist who is an expert in congenital heart disease. In some cases, depending on complications and residual congenital heart disease, examination may be performed more frequently. An ECG should be performed annually to evaluate heart rhythm and QRS duration. Echocardiography and MRI should be performed by a specialist competent in diagnosing complex congenital heart disease. Holter monitoring is carried out if there is an assumption of cardiac arrhythmias (Therrien J., 2001, Landzberg M.J., 2001).

10.9. Recommendations for probing and ACG in patients after radical correction of tetralogy of Fallot

Class I

Probing and ECG in patients with tetralogy of Fallot should be performed in regional centers for the treatment of patients with congenital heart disease (Level of Evidence: C)
Routine examination of coronary artery anatomy should be performed before any intervention on the outflow tract of the pancreas (Level of Evidence: C)

Class IIb

1. After radical correction of tetralogy of Fallot, probing and ACG can be performed to determine the causes of LV or RV dysfunction, fluid retention, chest pain, and cyanosis.

2. After radical correction of tetralogy of Fallot, catheterization and ACG can be performed before possible repair of residual stenoses of the pulmonary artery or systemic pulmonary anastomoses or BAR (Level of Evidence: B)

In these cases, transcatheter interventions may include:

a. Removal of residual VSD or aortopulmonary collateral arteries (Level of Evidence: C)

b. Transluminal balloon angioplasty or stenting for pulmonary stenosis (Level of Evidence: B)

V. Elimination of residual ASD (Level of Evidence: B)

1. Invasive testing in patients after correction of tetralogy of Fallot is indicated if the following data cannot be obtained by other methods: assessment of hemodynamics, assessment of pulmonary blood flow and resistance, assessment of the anatomy of the RV outflow tract or pulmonary stenosis, assessment of the coronary arteries before any repeat surgical procedure, assessment of the ventricular functions and presence of residual VSD, degree of mitral or aortic insufficiency, assessment of the amount of blood discharge through a patent foramen ovale or ASD, assessment of pulmonary regurgitation and right ventricular failure.

10.10. Repeated operations

10.10.1. Open operations

Violations of intracardiac hemodynamics and rhythm disturbances are indications for repeated operations in adult patients. The main reasons for repeated operations are residual stenoses of the outflow tract of the pancreas, the trunk and branches of the pulmonary artery, and recanalization of the VSD. PA valve insufficiency was also one of the main reasons for reoperations in these patients. The patients' condition improved significantly after implantation of an artificial prosthesis in the position of the pulmonary valve.

1. Surgeries in patients after radical correction of tetralogy of Fallot should be performed by qualified surgeons with experience in the treatment of congenital heart disease (Level of Evidence: C)

2. Pulmonary valve replacement is indicated for severe pulmonary regurgitation and decreased exercise tolerance (Level of Evidence: B)

3. Anomalies of the coronary arteries, the presence of a coronary artery in the outflow tract of the pancreas should be established before surgery (Level of Evidence: C)

1. PA valve replacement is indicated after radical correction of tetralogy of Fallot and severe pulmonary regurgitation, as well as in case of:

a. Progressive pancreatic dysfunction (Level of Evidence: B)

b. Progressive pancreatic dilatation (Level of Evidence: B)

V. Development of rhythm disturbances (Level of evidence: C)

d. Progression of tricuspid regurgitation (Level of Evidence: C)

2. Collaboration between surgeons and interventional cardiologists is required to perform transluminal balloon angioplasty and stenting of PA stenoses after surgery (Level of Evidence: C)

3. Surgical treatment is indicated for patients after radical correction of tetralogy of Fallot with residual obstruction of the pancreatic outflow tract:

a. Peak systolic pressure gradient greater than 50 mmHg (Level of Evidence: C)

b. RV to LV systolic pressure ratio greater than 0.7 (Level of Evidence: C)

V. Marked pancreatic dilatation with pancreatic dysfunction (Level of Evidence: C)

d. Residual VSD with left-to-right shunt volume greater than 1.5:1 (Level of Evidence: B)

e. Severe aortic valve regurgitation (Level of Evidence: C)

f. Combination of residual defects leading to pancreatic dilatation or dysfunction (Level of Evidence: C)

Long-term mortality after radical correction of tetralogy of Fallot ranges from 0 to 14%. Survival rate 10-20 years after radical correction is 86% (Stark J., Deleval M., 2006, Kirklin J., 2013).

Repeat surgery is indicated for symptomatic patients with severe pulmonary regurgitation or asymptomatic patients with severe pulmonary regurgitation with evidence of significant RV dilatation or dysfunction. Patients with conduits often require reintervention due to conduit stenosis or valve insufficiency. Some patients progress to aortic valve regurgitation, which requires surgery.

Surgical procedures after radical correction of tetralogy of Fallot include: replacement of the pulmonary valve, elimination of pulmonary stenosis, elimination of an aneurysm of the outflow tract of the pancreas, elimination of recanalization of the VSD, replacement or plastic surgery of the tricuspid valve, replacement of the aortic valve, replacement of the ascending aorta, RFA of arrhythmogenic zones, implantation of a cardioverter - defibrillator at high risk of sudden death.

Closure of the patent foramen ovale is recommended, especially if there is cyanosis, episodes of paradoxical embolism, or the need for a permanent pacemaker or cardioverter-defibrillator.

10.10.3. Endovascular interventions

Currently, it is difficult to imagine surgical treatment of tetralogy of Fallot without the use of endovascular surgery methods. One of the directions is the correction of stenoses of the branches of the pulmonary artery, which can be either congenital or acquired (after performing various types of systemic-pulmonary anastomoses). Uncorrected branch stenosis after surgery leads to the persistence of residual high pressure in the right ventricle and the development of acute heart failure, decreased perfusion of the corresponding lung, and deterioration in quality of life.

Balloon angioplasty may be an effective way to reduce obstruction to pulmonary blood flow, thereby increasing pulmonary vascular capacity and reducing PVR. Criteria for selecting patients for angioplasty: 1. The presence of severe hypoplasia or stenosis of at least one of the pulmonary arteries, 2. The diameter of the narrowed segment is less than 7 mm, 3. The pressure in the pancreas relative to the systemic pressure is more than 0.60. If 2 or more criteria are present, balloon angioplasty is recommended. The mechanism of angioplastic action is as follows. When the balloon is inflated, the intima and muscular lining of the vessel rupture, and the fibrous part of the media is stretched. The site of rupture and stretching of copper and intima is filled with connective tissue within 1-2 months. These processes must be pronounced for long-term and successful expansion of the narrowed area. If the rupture concerns only the intima, then angioplasty is usually unsuccessful (Lock J.E., 1983). However, balloon dilatation of PA narrowings is not always successful; the percentage of restenosis in the postoperative period is high, which led to the creation of intravascular endoprostheses (stents). The goal of endoprosthetics is to implant a frame to eliminate elastic return, narrow the vessel and support the vascular wall with the elimination of stenosis.

The approach to transcatheter closure of residual muscular VSD or VSD recanalization remains an effective alternative to surgical closure (Knanth A.L., 2004).

1. Probing is indicated in patients with repaired tetralogy of Fallot to eliminate a residual ASD or a VSD with a left-to-right shunt greater than 1.5:1 if the anatomy of the defect is suitable for transcatheter closure. (Level of Evidence: C)

2. Probing after radical correction of tetralogy of Fallot should be planned with the participation of qualified cardiologists and surgeons competent in the diagnosis and treatment of congenital heart disease. There is considerable experience in closing residual defects using occluders, but experience with percutaneous implantation of a stent valve in the pulmonary artery position is limited, and the effectiveness/safety remains uncertain, although this technique appears promising.

10.11. Recommendations for heart rhythm disturbances

Class I

1. Annual examination with the study of complaints, ECG, assessment of RV function, and an exercise tolerance test is recommended for patients with pacemakers and cardioverter-defibrillators (Level of Evidence: C)

1. Periodic ECG monitoring and Holter monitoring may be useful as part of routine postoperative management. The frequency of testing should be individualized depending on the hemodynamics and clinical prognosis of the arrhythmia (Level of Evidence: C)

1. An electrophysiological study will help determine the true cause of the arrhythmia (Level of Evidence: C)
Rhythm disturbances occupy a special place among the complications of the long-term postoperative period. Sudden death due to various arrhythmias is observed in 3-5% of patients who have undergone radical correction. A wide range of arrhythmias can lead to sudden death: complete atrioventricular block, ventricular and supraventricular arrhythmias.

Many investigators have attempted to determine the mechanism and risk factors for sudden arrhythmia death in this group. Previously, it was believed that rhythm disturbances were associated with impaired AV conduction, with the opinion that injury to conducting tissues during surgery could lead to sudden death in the long term due to a sharp deterioration in conduction. Currently, the emphasis has shifted from AV block to ventricular tachycardia as a more common mechanism of sudden death in patients after correction of tetralogy of Fallot (Deanfield J.F., 1983, Dunnigan A., 1984).

Risk factors for the development of arrhythmias in the long term after radical correction of tetralogy of Fallot are the following: 1) previously performed systemic-pulmonary anastomosis, 2) older age at the time of surgery, 3) High pressure in the RV or its overdistension due to residual PA stenosis or severe pulmonary regurgitation, 4) high degree of ectopic foci during Holter monitoring, 5) inducible ventricular tachycardia during electrophysiological study. There is a correlation between ventricular tachycardia and QRS duration greater than 180 ms. The most significant degree of QRS prolongation was noted precisely among patients with dysfunction and expansion of the RV (the so-called mechano-electrical interaction) (Dore A., 2004, Busso G., 2005).

Determining the risk of developing arrhythmias in asymptomatic patients after radical correction of tetralogy of Fallot is still a subject of debate. Most clinicians rely on annual examination, ECG, Holter monitoring, and exercise testing to record ventricular premature beats, as well as periodic echocardiography and MRI to monitor RV function.

The presence of complaints, that is, atrial flutter, dizziness or an episode of syncope, should increase the suspicion of the presence of arrhythmias in patients and require electrophysiological studies and cardiac catheterization. The performed electrophysiological study can give a prognosis regarding the risk of possible development of ventricular tachycardia. This method can also identify abnormal pathways as an additional factor in the development of arrhythmias. Episodes of ventricular tachycardia or cardiac arrest are now controlled by implantable cardioverter defibrillators.

Congenital heart disease tetralogy of Fallot consists of a combination of narrowing of the outflow tract of the right ventricle (pulmonary artery stenosis), ventricular septal defect, displacement of the aorta to the right, and right ventricular hypertrophy.

With an additional atrial septal defect of a secondary type, they speak of the pentade of Fallot.

The combination of pulmonary artery stenosis, atrial septal defect, and right heart hypertrophy is called Fallot's triad. In 50% of cases, hypertrophy in the area of the Crista supraventricularis leads to infundibular stenosis of the pulmonary artery involving part of the myocardium. The degree of stenosis depends on the contractility of the myocardium (beta blockers or sedatives may have a positive effect in this case).

In 25-40% of cases, valvular pulmonary artery stenosis is present. Due to reduced blood flow through the pulmonary artery, it can be hypoplastic.

Due to severe obstruction in tetralogy of Fallot (the pressure in the pulmonary circulation is already initially higher than in the systemic circulation), a right-to-left shunt occurs. A ventricular septal defect is most often the size of the aortic diameter, promotes pressure balance, and lies below the right velum of the aortic valve. The relationship between aortic root position and VSD is described as aortic jump. The origin of the aorta usually lies at the site of the defect. The degree of jump may vary. With a strong jump, the right ventricle can immediately throw blood through the VSD into the aorta. The degree of obstruction of the right ventricular ejection and aortic jump determine to a large extent the hemodynamic relationships.

Treatment of heart defect Tetralogy of Fallot

Surgery for tetralogy of Fallot is always indicated, since without surgery only 10% of children reach adulthood. If symptoms progress rapidly in infancy and hypoplastic pulmonary vessels are present, palliative intervention is first undertaken: connection between A. subclavia and ipsilateral A. pulmonalis - Blalock-Taussig-Shunt (aortopulmonary window > increased blood flow in the pulmonary vessels, blood oxygenation improves, hypoplastic pulmonary vessels dilate and the underdeveloped left ventricle is trained).

Corrective intervention is carried out after 2-4 years; with early development of the pulmonary vessels, it is possible during the 1st year of life.

Operation. expansion of the inlet section of the right ventricle, closure of the ventricular septal defect, movement of the aorta into the left ventricle.

Prognosis for heart defect tetralogy of Fallot. mortality during surgery: 5-10%. Late results after correction in more than 80% of cases are good. Frequent late complications of this heart defect: heart rhythm disturbances.

Tetralogy of Fallot

Tetralogy of Fallot has 4 anatomical signs:

1) pulmonary artery stenosis - valvular and in most patients the muscular panel (infundibular) is also connected

2) a high VSD of significant size, the upper edge of which is formed by the cusps of the aortic valve

3) dextroposition of the aorta, that is, displacement of the aorta in such a way that it seems to sit astride the interventricular septum and receives blood from both ventricles

4) hypertrophy of the right ventricle.

If there is also a patent foramen ovale or an ASD with right-to-left ejection of blood, the deficiency is called the pentade of Fallot.

Mechanisms of hemodynamic disorders. The nature of primary hemodynamic disorders is determined by the severity of valvular and subvalvular muscular stenosis of the pulmonary artery, which often progresses with age. The direction of blood ejection depends on it, but not the systolic pressure in the right ventricle, which, due to the impressive size of the VSD and the dextroposition of the aorta, is always equal to the pressure in the left ventricle and aorta.

With severe stenosis, pulmonary blood flow is reduced. A significant portion of the blood is discharged from the right ventricle into the aorta, where it is mixed with arterial blood from the left ventricle and causes the development of cyanosis. The ejection of blood from right to left especially increases during exercise, when venous blood flow to the heart increases significantly, and pulmonary blood flow practically does not increase due to pulmonary stenosis. Hemoglobin oxygen saturation in arterial blood can decrease by up to 60%.

If the pulmonary artery stenosis is moderate, then at rest the discharge through the VSD occurs from left to right, and the pulmonary blood flow increases, but there is no cyanosis. This tetralogy of Fallot is called white. During exercise, blood flow to the heart increases, but pulmonary blood flow through pulmonary stenosis does not change. Excess venous blood is discharged into the aorta, which is accompanied by the appearance of cyanosis.

Hemodynamic disturbances are compensated primarily by hypertrophy of the right ventricle, which, however, does not reach the same severity as with isolated pulmonary artery stenosis due to lower pressure in the ventricle.

Pozasertsev’s compensation for hemodynamic disorders includes: a) the development of polycythemia with an increase in the number of red blood cells to 8 10 12 / l and hemoglobin to 250 g / l, helps to increase the oxygen content in the blood b) the formation of an anastomosis between the bronchial arteries and the pulmonary artery system. As a result, blood from the aorta additionally enters the pulmonary circulation and is oxygenated.

Violation of compensation is manifested by insufficiency of the hypertrophied right ventricle and dysfunction of organs, which is caused by hypoxia and thrombosis as a result of polycythemia.

It should be noted that the hemodynamic state of each patient with tetralogy of Fallot is quite dynamic and changes depending on the magnitude of the peripheral vascular resistance. Thus, with an increase in AT, the systolic pressure in the right ventricle also increases and pulmonary blood flow increases. This is also typical for persistent concomitant arterial hypertension in adult patients - right ventricular failure develops due to an increase in the afterload of this ventricle. On the contrary, a decrease in peripheral vascular resistance, including during physical activity, leads to an increase in the right-to-left shunt. In patients with subvalvular muscular stenosis, increased contractions of the right ventricular myocardium or a sudden decrease in its filling with a decrease in venous inflow causes an even greater narrowing of the outflow canal and an increase in the pressure gradient in it, and, consequently, an increase in the release of venous blood into the aorta. There is an opinion that this mechanism, as well as a decrease in peripheral vascular resistance, is the basis for the posterior cyanotic attacks characteristic of patients with tetralogy of Fallot, and confirms the effectiveness of their treatment with 3-blockers.

Clinical picture. Manifestations of the disease depend on the severity of pulmonary artery stenosis and the direction of blood ejection. The main complaint of patients is shortness of breath during physical activity due to arterial hypoxemia. The “blue” notebook of Fallot is very characterized by hypoxic, or cyanotic, attacks with severe shortness of breath, increased cyanosis, sometimes loss of consciousness and convulsions, which can be fatal. These attacks can be prevented by adopting a squatting or knee-elbow position. At the same time, pulmonary blood flow increases due to an increase in peripheral vascular resistance due to compression of the femoral arteries and an increase in systemic venous turn. There is a history of cyanosis from early childhood (from 3-6 months), less often, in the case of a large defect and progressive subvalvular muscular stenosis, cyanosis occurs later.

On examination, attention is paid to a noticeable lag in physical development, diffuse cyanosis, up to “inky”, and fingers resembling drumsticks. The time of appearance and severity of these signs caused by arterial hypoxemia depend on the severity of pulmonary artery stenosis. At the left edge of the sternum, the pulsation of the hypertrophied right ventricle is palpated, and in some patients there is also systolic trembling in the second or third intercostal spaces. Signs of right ventricular failure are very rare in childhood; they begin to appear in adults through the development of pulmonary valve insufficiency and systemic arterial hypertension.

The main auscultatory signs of tetralogy of Fallot are:

1) a relatively short systolic ejection murmur with an epicenter in the second or third intercostal spaces to the left of the sternum

2) a sharp weakening and delay of P 2. often inaudible. The less pronounced the stenosis, the greater the pulmonary blood flow, the louder and longer the systolic murmur. With a relatively small stenosis, it is accompanied by systolic tremors. During cyano-political attacks, the noise weakens or disappears. In patients with significant VSDs with tetralogy of Fallot, no murmur is heard. In adults, a protodiastolic murmur of pulmonary valve insufficiency (after P2) may also be detected due to their calcification. In some patients, the murmur of aortic regurgitation is heard (after A ^ into the right ventricle. It leads to the development of right ventricular failure, dilatation of the right ventricle and the appearance of a murmur of relative tricuspid valve insufficiency. Identification signs of the “white” and “blue” notebook of Fallot are presented in Table 20.

Table 20

Identification features of clinical manifestations and data from additional methods of studying patients with “blue” and “white” notebooks Fallot

Diagnostics. The ECG shows signs of hypertrophy of the right ventricle and atrium. During an X-ray examination, the heart, as a rule, is not enlarged and has a characteristic aortic configuration in the form of a clog (wooden shoe) with an elevated apex due to an enlargement of the right ventricle and a concavity in the area of the pulmonary trunk. Since subvalvular muscular stenosis of the pulmonary artery predominates, post-stenosis non-expansion is rare. With pronounced stenosis, the aortic arch is more noticeable. The pulmonary vascular pattern is weakened or unchanged.

During two-dimensional echocardiography with Doppler study, all anatomical signs of deficiencies and enlargement of the right ventricle with slightly changed sizes of the right atrium are clearly visible.

The diagnosis can be confirmed by cardiac catheterization, during which the following signs are detected: 1) systolic pressure gradient between the right ventricle and the pulmonary artery, 2) systolic pressure in the right ventricle is equal to the pressure in the left ventricle and aorta, 3) pressure in the pulmonary artery is moderately reduced or not changed; 4) signs of blood shunting at the level of the ventricles. Right-sided ventriculography helps to clarify the morphology of the outflow canal of the right ventricle, the presence of a VSD and the direction of blood ejection through it.

During laboratory testing, polycythemia is detected and a decrease in the saturation of hemoglobin in arterial blood with oxygen, as well as an increase in the level of uric acid in the blood that accompanies polycythemia.

Differential diagnosis in adult patients with “blue” tetralogy of Fallot is carried out mainly with Eisenmenger syndrome and chronic cor pulmonale, with “white” tetralogy - with isolated pulmonary artery stenosis and VSD (see above).

The diagnosis of tetralogy of Fallot is made based on the characteristics characteristic of the tetralogy:

1) central cyanosis

2) systolic ejection murmur over the pulmonary artery

3) II tone, represented by one A

4) right ventricular hypertrophy on the ECG.

It is verified using two-dimensional Doppler echocardiography and cardiac catheterization.

Main complications and causes of death:

1. Chronic right ventricular failure due to resistance overload and left ventricular failure due to volume overload, which are facilitated by hypoxemia, myocardial fibrosis, and atrial rhythm disturbances. Appear relatively late.

2. Complications associated with arterial hypoxemia, primarily damage to the cerebral cortex, up to fatal consequences during cyanotic attacks.

3. Complications associated with polycythemia and increased blood density. Frequent and severe among them are thrombosis of the cerebral veins and stroke due to paradoxical thromboembolism of the cerebral arteries. As with Eisenmenger syndrome, there are also brain abscesses, for which one should be alert in case of complaints of headache and fever. Brain complications are observed in 5% of patients with tetralogy of Fallot.

4. Infective endocarditis.

The course of the disease and prognosis depend on the severity of pulmonary artery stenosis. Mostly they are unfavorable. Tetralogy of Fallot is the most common blue PVS in adults. Although some patients may have a life expectancy of more than 40 years, only 25% of children survive to 10 years of age. With “white” forms the prognosis is better. With age, muscular (infundibular) stenosis can progress due to increased hypertrophy, which leads to a decrease in blood flow in the lungs. At the same time, the right-left shunt increases, leading to the appearance of cyanosis, the symptom of drumsticks and polycythemia.

Treatment. Radical surgical correction of tetralogy of Fallot under artificial circulation is ideal. Operational treatment is indicated for almost all patients with this defect. In severe forms (significant limitation of physical activity, frequent squatting positions and cyanotic attacks, hemoglobin 200 g/l or more), especially under the age of 5 years, a palliative operation of anastomosis between the aorta and the pulmonary artery is first performed. There are several options for such operations. Creating a semblance of the Botallo Strait allows increasing pulmonary blood flow, leading to a decrease in hypoxia, cyanosis and an improvement in the physical condition of patients. In some cases, with complex anatomical variants of the defect, this operation becomes the final stage of surgical treatment.

Only radical correction is indicated for patients, because the very fact of reaching such an age indicates the relative “ease” of the deficiencies. Mortality with radical correction in adults is lower than in children and is currently about 10%.

Drug therapy for retrocyanotic attacks includes placing patients in the knee-elbow position, oxygen inhalation, and administration of morphine and p-blockers. To prevent attacks, beta-blockers are prescribed with caution to avoid arterial hypotension. In case of severe polycythemia (hematocrit 70%), bloodletting is performed with replacement of the bcc with plasma substitutes or erythrocytopheresis. Prevention of infective endocarditis is indicated.

Long-term consequences of surgical treatment. Even palliative surgery produces striking clinical improvement. Despite the disappearance of complaints, systolic murmur often persists through residual infundibular or valvular stenosis in the outflow tract of the right ventricle. Signs of mild pulmonary valve or aortic insufficiency may appear. The later surgical correction is performed, the worse its functional consequences.

What is Tetralogy of Fallot? Prognosis and treatment.

Tetralogy of Fallot– complex congenital heart defect of the cyanotic (blue) type. In cardiological practice, of all congenital heart defects, it occurs in 7-10% of cases and accounts for 50% of all “blue” type defects.

The anomaly combines four congenital heart defects:

narrowing of the outlet of the right ventricle;
extensive ventricular septal defect;
aortic displacement;
hypertrophy of the wall of the right ventricle.

Causes of Tetralogy of Fallot

The pathology develops as a result of disruption of the process of cardiogenesis.

It can lead to:

infectious diseases suffered in the early stages of pregnancy (rubella, measles, scarlet fever);
maternal diabetes mellitus;
taking medications, vitamin A in large quantities, alcohol, drugs;
influence of harmful environmental factors;
The formation of anomalies is also influenced by heredity.

Symptoms of Tetralogy of Fallot

The main symptom is cyanosis, which has varying degrees of severity. In children with tetralogy of Fallot, only severe forms of the defect are diagnosed at birth. Typically, cyanosis develops gradually, by three months to a year, and has different shades: from pale bluish to cast-iron blue.

Some children with mild cyanosis are stable, while others have severe symptoms and disruption of normal development. Attacks of sudden increase in cyanosis occur during crying, feeding, straining, and emotional stress. Any physical activity causes an increase in shortness of breath. development of dizziness, tachycardia, weakness.

The extremely severe manifestation of the disease is dyspnea-cyanotic attacks, which occur suddenly, with increased cyanosis and shortness of breath, weakness, tachycardia, and loss of consciousness. Apnea and hypoxic coma may develop, resulting in death.

Treatment of tetralogy of Fallot

If the manifestations of tetralogy of Fallot allow the child to survive, the pathology is corrected through surgery, the indication for which is absolute. If for some reason the operation is not performed, three out of four children die by the age of one year.

For dyspnea-cyanotic attacks, drug therapy is indicated. If it is ineffective, an aortopulmonary anastomosis is urgently performed.

Surgical correction of the pathology depends on the severity of the defect and the age of the patients. For young children with a severe form of the disease, the operation is performed in stages. At the first stage, palliative operations are performed to make the child’s life easier. Their goal is to increase blood flow into the pulmonary circulation and reduce the risk of complications during radical correction.

Radical intervention involves eliminating the narrowing of the outlet of the right ventricle and performing plastic surgery of the ventricular septal defect. The operation is usually performed after 6 months and up to 3 years. Long-term results when performing surgery at an older age (especially after 20 years) are much worse.

Prognosis of tetralogy of Fallot

The prognosis of the disease depends on the degree of pulmonary stenosis. A quarter of children with tetralogy of Fallot die in the first year of life, most in the first month. Without surgery, 40% die by 3 years, 70% by 10, and 95% by 40. The usual cause of death is cerebral thrombosis or brain abscess.

Successful surgery gives a good prognosis for long life.

Tetralogy of Fallot (the so-called “cyanotic” disease) is one of the most common severe pathologies of heart development in children.

The patient's body experiences an acute oxygen deficiency, since congenital anatomical defects of underdevelopment of the cardiac septum lead to inevitable mixing of arterial and venous blood. Children with tetralogy of Fallot require medical attention.

The Svyatoslav Fedorov Medical Center provides comprehensive services in the field of pediatric cardiac surgery. Modern diagnostic methods with a complete examination of the child’s body make it possible to identify congenital heart defects (CHD) at an early stage.

What is tetralogy of fallot in children?

Children's congenital heart disease is characterized by the following anomalies: underdevelopment of the right ventricle of the heart with simultaneous displacement of the arterial conical septum to the side, as well as a ventricular septal defect. Against the background of obstructed blood outflow from the right ventricle of the heart, secondary hypertrophy develops.

Such a displacement of the conus septum is caused, first of all, by stenosis (atresia) of the right ventricle, underdevelopment of the pulmonary trunk and the entire valvular apparatus of the heart.

Diagnosis of tetralogy of Fallot

The Svyatoslav Fedorov Children's Center provides comprehensive diagnostics of the cardiovascular system.

As part of the examination for subsequent treatment of tetralogy of Fallot, it is necessary to obtain the following results:

Laboratory examination of blood sample analysis (general);
Electrocardiography (ECG);
Echocardiography (ECHOCG);
Ultrasound examination of the heart (ultrasound);
X-ray examination of the heart and great vessels (chest x-ray).

Indications for surgery

In general, the clinical picture of this disease and the choice of treatment tactics entirely depend on the presence/absence of concomitant pathologies. Along with this, the indications for surgery for Tetralogy of Fallot are absolute, therefore, even with an asymptomatic course, children with this diagnosis are indicated for early surgical treatment.

At our medical center, doctors and surgeons adhere to a step-by-step method of treating congenital pathologies of heart development in children:

Conservative treatment - carried out with inotropic support drugs (cardiotrophics, sympathomimetics, cardiac glycosides, diuretics);
Heart surgery - palliative and radical correction.

It should be noted that palliative operations and radical correction of the defect with the elimination of stenosis and plastic surgery of the VSD are performed on children at least 3 years old.

The essence of the operation for tetralogy of Fallot

Of the palliative operations currently performed at the Svyatoslav Fedorov Medical Center, preference is given to the technique of subclavian-pulmonary anastomosis (Blelock-Taussig shunt).

Radical operation- one-stage, performed with artificial circulation.

The procedure for Tetralogy of Fallot is as follows: the wall of the right ventricle is opened above the outflow tract of the right ventricle, and the muscles that narrow the outlet ducts from the ventricle into the pulmonary artery are cut out with a scalpel. The site of the ventricular septal defect is closed with a felt Teflon patch. To prevent possible narrowing of the ducts of the outflow tract, a similar patch is sewn into the incision in the wall of the right ventricle.

With great success at the MC named after. Svyatoslav Fedorov uses a step-by-step method of radical correction. In particular, at the 1st stage, one of the bypass anastomoses is created, and after 2-3 years a radical intervention is performed to ligate the previously created anastomosis.

Prognosis after surgery

Of course, it is extremely difficult to judge any stability of results after such operations, since information about this is only being accumulated at the present time. Simple radical correction for tetralogy of Fallot is marked by a relatively high percentage of mortality in the immediate postoperative period, while with staged radical correction the risk of postoperative mortality is reduced to 7%.

Does tetralogy of Fallot occur after surgery?

Along with favorable results of radical surgery, there are also cases of structural changes in biological valves that were implanted in the area of the pulmonary valve. Ultimately, this led to pulmonary stenosis.

How long do they live after such an operation?

In general, the prognosis for tetralogy of Fallot is unfavorable, since the overall life expectancy depends entirely on the degree of oxygen starvation (brain hypoxia) of the patient.

There are quite a few pathologies that can develop in a child even before he is born. Such disorders are most often classified by doctors as congenital defects. They can be caused by a variety of factors: genetic disorders, the pathogenic influence of drugs, the environment, bad habits of parents, etc. Fortunately, a significant number of such defects do not affect the survival of infants and are quite amenable to correction. And one of these disorders is Tetralogy of Fallot in a newborn. Let’s discuss the treatment of such a disease, and consider what prognosis doctors give after surgery for this anomaly.

Tetralogy of Fallot in newborns is a combined congenital anomaly of the heart - it consists of four different defects of this organ, which are observed simultaneously in one newborn. With this disorder, the child is diagnosed with stenosis of the outflow tract of the right ventricle and a ventricular septal defect in combination with dextroposition of the aorta, as well as with hypertrophy of the right ventricular myocardium. Thus, venous blood in tetralogy of Fallot is mixed with arterial blood, which impairs the functioning of the lungs and heart, causing a lack of oxygen in the body and oxygen starvation of the brain.

Why does tetralogy of Fallot occur, what are the reasons for its occurrence?

Tetralogy of Fallot in children occurs due to disturbances in the process of cardiogenesis around the second to eighth week of embryonic development. It is believed that tetralogy of Fallot can be triggered by infectious diseases suffered by the expectant mother in the very early stages of pregnancy. Such ailments include rubella, measles, as well as scarlet fever, etc. In addition, this defect can be caused by the consumption of medications at this stage of pregnancy. Sleeping pills, sedatives, hormonal and other medications are especially dangerous. The use of alcohol and drugs, as well as the influence of aggressive production, can also play the role of a provoking factor.

Among other things, some scientists argue that the tendency to congenital heart defects can be inherited.

Tetralogy of Fallot is often combined with the so-called Cornelia de Lagne syndrome.

How does tetralogy of Fallot manifest, what are its symptoms?

Manifestations of tetralogy of Fallot depend on the severity of this disorder. Thus, in newborn babies, a severe form of the disease is manifested by cyanosis and shortness of breath during feeding. Hypoxemic attacks are possible; they are caused by physical activity, manifested by frequent and deep breaths, anxiety, prolonged screaming, increasing cyanosis and a decrease in the intensity of heart murmurs. A severe attack can cause lethargy, seizures, and sometimes death.

How is tetralogy of Fallot detected, what is its diagnosis?

The diagnosis of “tetralogy of Fallot” can be made by a doctor after collecting anamnesis and conducting a series of studies. Most often, young patients undergo chest X-ray, ECG, and two-dimensional echocardiography in combination with color Doppler.

How is tetralogy of Fallot corrected, what treatment is effective?

The only possible method of eliminating tetralogy of Fallot is surgery. Before surgical correction is carried out, conservative methods of influence will come to the rescue. Thus, in order to eliminate severe cyanosis due to closure of the ductus arteriosus, prostaglandin is administered to newborn children, which allows the ductus arteriosus to reopen.

If a hypoxemic attack occurs, you need to give the baby a position with his knees pressed to his chest. Also, during an attack, small patients are given morphine in the amount of 0.1-0.2 mg/kg, it is administered intramuscularly. To increase the volume of circulating blood, fluid is administered intravenously. If such measures do not stop the attack, phenylephrine or ketamine effectively increases systemic blood pressure. The use of Propranol helps avoid relapses.

Radical surgical intervention is performed on children aged from six months to three years. Doctors close the ventricular septal defect with a patch and also widen the outlet of the right ventricle.

Newborn babies who have been diagnosed with a particularly severe form of tetralogy of Fallot undergo palliative surgery. It allows you to reduce the likelihood of complications during subsequent radical correction of the duct.

Palliative surgeries are also called bypass surgeries. They can be performed according to different principles, which are determined by the attending physician - a cardiologist and a cardiac surgeon.

Surgery and tetralogy of Fallot - prognosis after surgery

The prognosis for patients who undergo surgery to correct tetralogy of Fallot is good. Patients retain full working capacity and social activity, they can satisfactorily tolerate physical activity.

It is worth noting that the earlier the surgical intervention was performed, the better its long-term results will be.

All patients suffering from tetralogy of Fallot must be systematically monitored by a cardiologist and cardiac surgeon. They also need to receive antibiotic prophylaxis for endocarditis before dental or surgical procedures, which are particularly dangerous in the potential development of bacteremia.

Folk recipes

Not a single traditional medicine recipe will help cope with tetralogy of Fallot or prevent its development. However, some herbs will benefit children who have undergone radical surgery. So, traditional medicine recipes are great for strengthening the body’s immune system.

For kindergarten-age children, you can prepare medicine using the aloe vera plant. Wash them thoroughly and dry. Combine three tablespoons of crushed aloe leaves with the same amount of high-quality honey, lemon juice and chopped walnuts. Pour the prepared raw materials with one hundred milliliters of homemade milk. Infuse the mixture for two days, then give the baby a teaspoon two or three times a day.

Fortunately, modern developments in medicine make it possible to successfully cope with tetralogy of Fallot in children through radical surgery.