"Blue" congenital heart disease and methods of its correction: tetralogy of Fallot in newborns. Etiology and pathogenesis

Tetralogy of Fallot (TF) - CHD, characterized by underdevelopment of the outlet section of the right ventricle and displacement of the embryonic conical septum anteriorly and to the left, resulting in four defects: subaortic nonrestrictive VSD, stenosis of the outlet section of the right ventricle (usually with impaired development of the fibrous ring) of the pulmonary valve , right ventricular hypertrophy and aortic dextroposition. Statistical data ... 9% of all CHD diagnosed in infancy. 10-15% of all UPUs. 50% of the defects of the blue type (with blood discharge from right to left).

Code for the international classification of diseases ICD-10:

Causes

Etiology ... Violation of heart formation at 2-8 weeks of gestation under the influence of viral infection, industrial hazards, some drugs, hereditary factors. Often combined with Cornelia de Lange syndrome, multiple hereditary anomalies, "clown face", small feet and palms, polyphalangia, vertebral anomalies, oligophrenia, low birth weight.

Pathogenesis ... Due to the fact that the aorta largely departs not only from the left, but also from the right ventricle (the location of the aorta "on top" on the interventricular septum), with severe stenosis and large defect sizes, blood from the ventricles enters the aorta and into the smaller degree into the pulmonary artery. Therefore, with this defect, right ventricular failure does not develop. With moderate stenosis, when the resistance to the release of blood into the lungs is lower than into the aorta, a discharge from left to right occurs, which is clinically manifested by the acianotic form of TF. As the severity of stenosis increases, a cross discharge occurs, and then a discharge from right to left with a transition to the cyanotic form of the defect.

Symptoms (signs)

Clinical picture

There are five forms and, accordingly, five periods of disease manifestation. Early cyanotic form (cyanosis appears from the first months of life, but more often by the first year). The classic form (cyanosis appears when the child begins to walk and run). Severe form (with dyspnea - cyanotic attacks). Late cyanotic form (the appearance of cyanosis at 6-10 years). Pale (acianotic) form.

. Complaints .. Developmental delay .. Central cyanosis .. Shortness of breath .. Dyspnea - cyanotic attacks associated with paroxysmal spasm of the outlet of the right ventricle - sudden anxiety, shortness of breath and cyanosis with possible subsequent apnea, loss of consciousness and seizures.

. Objectively .. Pallor or cyanosis .. Symptoms of "drumsticks" and "watch glasses" .. Squatting after exercise (provides an increase in OPSS and a decrease in blood discharge from right to left) .. Systolic tremor along the left edge of the sternum .. Strengthening I tone. Rough systolic ejection murmur with a maximum in the second - third intercostal spaces to the left of the sternum. Above the pulmonary artery II, the tone is weakened. With pronounced collateral blood circulation on the back over the pulmonary fields, systolic or systolic-diastolic murmur is heard.

Diagnostics

Instrumental diagnostics

. ECG: signs of hypertrophy and overload of the right sections, AV - blockade, various variants of sick sinus syndrome.

. X-ray of organs chest .. Depletion of the pulmonary pattern. In older children and adults, it can be enhanced due to the developed collateral circulation .. For children, a small heart in the form of a "shoe" is characteristic .. Bulging of the right ventricular arch .. Bulging of the left ventricular arch when combined with an open arterial duct.

. Echocardiography .. Evaluation of myocardial hypertrophy and the size of the cavity of the right ventricle .. Diagnostics of the degree of narrowing of the outlet section of the right ventricle and its anatomical type (embryological, hypertrophic, tubular or multicomponent) .. Dextraposition of the aorta .. VSD .. It is obligatory to study the aortic valve for its stenosis, since undiagnosed stenosis aortic valve after radical correction of the defect, it leads to rapid decompensation and pulmonary edema. The diameter of the fibrous rings of all valves is determined and the structure of their valves is examined.

. Cardiac catheterization .. High pressure in the right ventricle .. Measurement of the pressure gradient between the right ventricle and the pulmonary artery .. Low oxygenation of blood in the aorta.

. Right and left ventriculography and atriography, angiopulmonography, coronary angiography: determination of the anatomical type of defect, identification of accompanying anomalies of valves, great vessels and coronary arteries.

Drug therapy ... Relief of dyspnea - cyanotic attacks - inhalation of humidified oxygen. 1% p - p trimeperidine i / m - 0.05 ml per year of life. Niketamide - 0.1 ml per year of life. With no effect - intravenous administration crystalloids. With acidosis - 4% p - p sodium bicarbonate, 5% p - p glucose with insulin, rheopolyglucin, aminophylline. If there is no effect - an emergency aorto-pulmonary anastomosis. For newborns with a combination of TF with atresia of the pulmonary artery valve, when pulmonary blood flow depends on the state of the ductus arteriosus, infusion of PgE 1 (alprostadil) 0.05-0.1 mg / kg / min to maintain blood flow in it before the aorto-pulmonary anastomosis is applied.

Treatment

Surgery

Indications: all patients with TF.

Contraindications: irreversible pulmonary hypertension.

Surgical treatment methods ... Palliative interventions - creation of intersystem anastomoses (Blalock-Taussig operation - subclavian - pulmonary anastomosis, Waterstone-Cooley operation - intrapericardial anastomosis between the ascending aorta and the right pulmonary artery) and infundibuloplasty (plastic of the right arterial cone) - open extension the outlet section of the right ventricle. Palliative interventions are carried out on an emergency basis for children weighing less than 3 kg and concomitant defects that aggravate the condition. Radical surgery - VSD plastic with elimination of stenosis of the right ventricular outlet. Anatomical types of defect and options for their radical correction are presented below.

Type I - embryological .. Characteristics ... Obstruction of the right ventricular outlet is caused by anterolateral displacement and / or low penetration of the conical septum ... Maximum narrowing is usually localized at the level of the dividing muscle ring ... The pulmonary valve ring is moderately hypoplastic or almost normal .. Method of surgical correction: economical resection of the parietal pedicle of the arterial cone, aimed at eliminating stenosis caused by a change in its normal orientation and lengthening.

Type II - hypertrophic .. Characteristics ... The obstruction is caused by antero-left displacement and / or low penetration of the tapered septum, which more often has normal sizes or lengthened, with pronounced hypertrophy of its proximal segment ... The maximum narrowing is localized at the level of the entrance to the outlet section of the right ventricle. the inflow and outflow parts of the right ventricle and in the area of \u200b\u200bthe opening leading to the right arterial cone.

Type III - tubular .. Characteristics ... Obstruction is associated with a pronounced uneven division of the arterial trunk, as a result of which the cone is sharply hypoplastic ... With this type of defect, as a rule, there is no hypertrophy of the septum of the arterial cone .. Methods of surgical correction ... Primary radical Correction is contraindicated ... Palliative plasty of the right arterial cone prevents the aggravation of structural changes in the right ventricle and allows one to count on the growth of both the central and peripheral parts of the pulmonary arterial tree, as well as on the appropriate preparation of the left ventricle for radical surgery. Therefore, palliative plastic has advantages over inter-arterial anastomoses.

Type IV - multicomponent .. Characteristics: the narrowing is due to a significant lengthening of the conical septum or a high discharge of the moderator cord - the septal-marginal trabecula .. Methods of surgical correction ... With a high discharge of the moderator cord, it is optimal to bypass the outlet of the right ventricle with conduits ... In some variants of this type of defect, the implementation of radical correction is problematic.

Specific postoperative complications ... Hypofunction and thrombosis of the anastomosis. Residual VSD. Pulmonary hypertension. Pulmonary artery rupture on the side of the anastomosis. Right ventricular aneurysm. Ventricular arrhythmias. AV - blockade. Infective endocarditis.

Forecast ... 25% of children die during the first year of life, the majority during the newborn period. The average life expectancy of non-operated patients is 12 years, in rare cases, patients live up to 75 years. Dyspnea - cyanotic attacks often disappear after 3 years. Postoperative mortality with radical correction is 5-16%. Physical performance in early dates after Fontaine's operation (see. Atresia of the tricuspid valve) corresponds to 30-42% of the age norm, after 1 year - 80%.

Synonym: Fallot tetralogy.

Abbreviations... TF - Fallot's tetrad.

ICD-10. Q21.3 Fallot's tetrad.

Applications . Fallot Triad - VPS, consisting of three components: 1 ) stenosis of the pulmonary artery, 2 ) DMPP, 3 ) compensatory hypertrophy of the right ventricle. Hemodynamics, symptoms, diagnostics consist of manifestations of pulmonary artery stenosis and ASD. Pentad of Fallot - CHD, consisting of five components: four signs of Fallot's tetrad and ASD. Hemodynamics, clinical picture, diagnosis and treatment are similar to those for Fallot's tetrad and ASD. ICD-10... Q21 Congenital malformations of cardiac septum

Tetralogy of Fallot (TF) - CHD, characterized by underdevelopment of the outlet section of the right ventricle and displacement of the embryonic conical septum anteriorly and to the left, resulting in four defects: subaortic nonrestrictive VSD, stenosis of the outlet section of the right ventricle (usually with impaired development of the fibrous ring) of the pulmonary valve , right ventricular hypertrophy and aortic dextroposition. Statistical data . 9% of all CHD diagnosed in infancy . 10-15% of all UPU . 50% of the defects of the blue type (with blood discharge from right to left).

Etiology . Violation of the formation of the heart at 2-8 weeks of gestation under the influence of a viral infection, industrial hazards, some drugs, hereditary factors . Often combined with Cornelia de Lange syndrome, multiple hereditary anomalies, "clown face", small feet and palms, polyphalangia, vertebral anomalies, oligophrenia, low birth weight.

Pathogenesis . Due to the fact that the aorta largely departs not only from the left, but also from the right ventricle (the location of the aorta "on top" on the interventricular septum), with severe stenosis and large defect sizes, blood from the ventricles enters the aorta and into the smaller degree into the pulmonary artery. Therefore, with this defect, right ventricular failure does not develop. . With moderate stenosis, when the resistance to the release of blood into the lungs is lower than in the aorta, a discharge from left to right occurs, which is clinically manifested by the acianotic form of TF . As the severity of stenosis increases, a cross discharge occurs, and then a discharge from right to left with a transition to the cyanotic form of the defect.

There are five forms and, accordingly, five periods of disease manifestation . Early cyanotic form (cyanosis appears from the first months of life, but more often by the first year) . Classic form (cyanosis appears when a child starts walking and running) . Severe form (with dyspnea-cyanotic attacks) . Late cyanotic form (onset of cyanosis at 6-10 years of age) . Pale (acianotic) form.

. Complaints .. Delayed development .. Central cyanosis .. Dyspnea .. Dyspnea-cyanotic attacks associated with paroxysmal spasm of the outlet of the right ventricle - sudden anxiety, shortness of breath and cyanosis with possible subsequent apnea, loss of consciousness and convulsions.

. Objectively .. Pallor or cyanosis .. Symptoms of "drum sticks" and "watch glasses" .. Squatting position after load (provides an increase in TPVR and a decrease in blood flow from right to left) .. Systolic tremor along the left edge of the sternum .. Strengthening the I tone. Rough systolic ejection murmur with a maximum in the second or third intercostal spaces to the left of the sternum. Above the pulmonary artery II, the tone is weakened. With pronounced collateral blood circulation on the back over the pulmonary fields, systolic or systolic-diastolic murmur is heard.

. ECG... signs of hypertrophy and overload of the right sections, AV block, various variants of sick sinus syndrome.

. X-ray organs chest cells .. Depletion of the pulmonary pattern. In older children and adults, it can be enhanced due to the developed collateral circulation .. Small hearts in the shape of a "shoe" are typical for children. .. Bulging of the right ventricular arch .. Bulging of the left ventricular arch when combined with an open arterial duct.

. Echocardiography .. Assessment of myocardial hypertrophy and the size of the right ventricular cavity .. Diagnostics of the degree of narrowing of the outlet of the right ventricle and its anatomical type (embryological, hypertrophic, tubular or multicomponent) .. Dextraposition of the aorta .. DMZhP .. It is imperative to study the aortic valve for its stenosis, since undiagnosed aortic valve stenosis after radical correction of the defect leads to rapid decompensation and pulmonary edema .. The diameter of the fibrous rings of all valves is determined and the structure of their cusps is examined.

. Catheterization cavities hearts .. High pressure in the right ventricle .. Measurement of the pressure gradient between the right ventricle and the pulmonary artery .. Low oxygenation of the blood in the aorta.

. Rights and left ventriculography and atriography. angiopulmonography. coronary angiography... determination of the anatomical type of defect, identification of concomitant anomalies of valves, great vessels and coronary arteries.

Medication therapy . Relief of dyspnea-cyanotic attacks - inhalation of humidified oxygen. 1% solution of trimeperidine i / m - 0.05 ml per year of life. Niketamide - 0.1 ml per year of life. If there is no effect, intravenous crystalloid administration . With acidosis - 4% sodium solution bicarbonate, 5% solution of glucose with insulin, rheopolyglucin, aminophylline. In the absence of effect - emergency aorto-pulmonary anastomosis . For newborns with a combination of TF with atresia of the pulmonary artery valve, when pulmonary blood flow depends on the state of the ductus arteriosus, infusion of PgE 1 (alprostadil) 0.05-0.1 mg / kg / min to maintain blood flow in it before the aorto-pulmonary anastomosis is applied.

Indications... all patients with TF.

Contraindications... irreversible pulmonary hypertension.

Methods surgical treatment . Palliative interventions - creation of intersystem anastomoses (Blaloc-Taussig operation - subclavian-pulmonary anastomosis, Waterstone-Cooley operation - intrapericardial anastomosis between the ascending aorta and the right pulmonary artery) and infundibuloplasty (plastic of the right arterial cone) - part of the right ventricle expansion of the outlet. Palliative interventions are carried out on an emergency basis for children weighing less than 3 kg and concomitant defects that aggravate the condition . Radical surgical treatment - VSD plasty with elimination of stenosis of the outlet of the right ventricle . Anatomical types of defect and options for their radical correction are presented below.

. Type I - embryological. . Characteristic ... Outflow obstruction of the right ventricle due to anterolateral displacement and / or low penetration of the tapered septum ... The maximum narrowing, as a rule, is localized at the level of the dividing muscle ring ... The pulmonary valve ring is moderately hypoplastic or nearly normal. . Surgical correction method: economical resection of the parietal pedicle of the arterial cone, aimed at eliminating stenosis caused by a change in its normal orientation and lengthening.

. Type II is hypertrophic. . Characteristic ... Obstruction is caused by antero-left displacement and / or low penetration of the tapered septum, which is often normal or elongated, with pronounced hypertrophy of its proximal segment ... The maximum narrowing is localized at the level of the entrance to the outlet of the right ventricle. . Surgical correction method: massive resection of the parietal leg of the conical septum, since the nature of the changes in this case determines two levels of narrowing - at the border between the inflow and outflow parts of the right ventricle and in the area of \u200b\u200bthe opening leading to the right arterial cone.

. Type III is tubular. . Characteristic ... Obstruction is associated with pronounced uneven division of the arterial trunk, as a result of which the cone is sharply hypoplastic ... With this type of defect, as a rule, there is no hypertrophy of the arterial cone septum. . ... Primary radical correction is contraindicated ... Palliative plasty of the right arterial cone prevents the aggravation of structural changes in the right ventricle and allows one to count on the growth of both the central and peripheral parts of the pulmonary arterial tree, as well as on the appropriate preparation of the left ventricle for radical surgery. Therefore, palliative plastic has advantages over inter-arterial anastomoses.

. Type IV - multicomponent. . Characteristic: the narrowing is due to a significant lengthening of the conical septum or a high discharge of the moderator cord - the septal-marginal trabecula. . Surgical correction methods ... With a high discharge of the moderator cord, the optimal right ventricular outflow bypass with conduits ... In some variants of this type of defect, the implementation of radical correction is problematic.

Specific postoperative complications . Hypofunction and thrombosis of the anastomosis . Residual VSD . Pulmonary hypertension . Pulmonary artery rupture on the side of the anastomosis . Right ventricular aneurysm . Ventricular arrhythmias . AV block . Infective endocarditis.

Forecast . 25% of children die during the first year of life, the majority during the newborn period . The average life expectancy of non-operated patients is 12 years, in rare cases, patients live up to 75 years . Dyspnea-cyanotic seizures often subside after 3 years . Postoperative mortality with radical correction - 5-16% . Physical performance in the early stages after Fontaine's operation (see. Tricuspid atresia) corresponds to 30-42% of the age norm, after 1 year - 80%.

Synonym... Fallot tetralogy.

Abbreviations... TF - Fallot's tetrad.

Applications . Triad Fallot - VPS, consisting of three components: 1 ) stenosis of the pulmonary artery, 2 ) DMPP, 3 ) compensatory hypertrophy of the right ventricle. Hemodynamics, symptoms, diagnostics consist of manifestations of pulmonary artery stenosis and ASD. Pentad Fallot - CHD, consisting of five components: four signs of Fallot's tetrad and ASD. Hemodynamics, clinical picture, diagnosis and treatment are similar to those in Fallot's tetrad and ASD. ICD-10... Q21 Congenital malformations of cardiac septum

Atrial septal defect

Atrial septal defect (ASD) - CHD with a message between the atria. Statistical data... 7.8% of all CHD detected in infancy and 30% in adults; VSD type ostium secundum - 70%, ostium primum - 15%, sinus venosus - 15%; Lutembashe syndrome accounts for 0.4% of all ASD cases, the combination of ASD with prolapse mitral valve - 10-20%; the predominant sex is female (2: 1-3: 1).

Etiology

factors that form CHD (see Tetralogy of Fallot).

Pathogenesis

ASD options

Clinical picture

Complaints

shortness of breath, palpitations, rapid fatigability during physical exertion, delay in physical development, frequent infections, paradoxical embolism.

Objectively

Pallor skin... Harrison sulcus - displacement of areas of the chest as a result of chronic shortness of breath. Splitting of the I tone with a pronounced tricuspid valve component. Pronounced fixed splitting of the II tone (pronounced - due to the lengthening of the time of blood ejection from the right ventricle; fixed - due to the fact that the dependence of venous return on the phases of breathing is leveled by the discharge from the left atrium). Ejection click and soft systolic murmur of the relative stenosis of the pulmonary artery in the second intercostal space to the left of the sternum. Due to the increase in blood flow through the tricuspid valve, a low-frequency diastolic murmur sometimes occurs over the xiphoid process of the sternum.

Instrumental diagnostics

ECG... Signs of hypertrophy and overload of the left sections, and with pulmonary hypertension - and right. In ostium primum - a sharp deviation of the EOS to the left due to the forward displacement of the hypoplastic branch of the left leg of the His bundle. Various variants of sick sinus syndrome, AV block. With a sinus venosus-type defect, the lower atrial rhythm or the AV-junction rhythm.

Jugular phlebography... the same amplitude of waves A and V.

X-ray examination organs of the chest. Strengthening the pulmonary pattern. Expansion and nonstructurality of the roots of the lungs, bulging of the right atrial arch and upward displacement of the right cardiovasal angle. With fluoroscopy - increased pulsation of the roots of the lungs (a rather specific sign). Symptom of the "Turkish saber" with concomitant abnormal drainage of the right pulmonary veins into the superior vena cava.

Echocardiography... Hypertrophy and dilatation of the left sections, and with pulmonary hypertension - and right. DMPP visualization in Doppler and Vmode. Differentiation from an open oval window (anatomical closure of the latter occurs no later than 2 years of life) - inconsistent visualization of the flush in the color Doppler mapping mode and the presence of a leaflet in the left atrial cavity. Diagnosis of concomitant anomalies (abnormal drainage of pulmonary veins, valvular defects, etc.). Determine the degree of discharge and the ratio of pulmonary minute to systemic blood flow (Qp / Qs). Adults undergo transesophageal echocardiography. With intravenous contrasting of the right heart - the effect of negative contrasting (displacement contrast agent a stream of blood from the left atrium).

Radionuclide angiocardiography (by the method of first pass or equilibrium): registration of pathological discharge and its quantitative assessment, diagnosis of concomitant abnormal drainage of pulmonary veins and dysfunctions of the ventricles.

Probing heart cavities... It is indicated for suspected pulmonary hypertension, before surgery open heart and with conflicting clinical data. If the catheter can be passed from the right atrium to the left, then in itself this cannot serve as a sign of an atrial septal defect: sometimes the catheter can also be passed through the open oval window. Tests with aminophylline and oxygen inhalation are performed to determine the prognosis for the reversibility of pulmonary hypertension. Calculate the ratio of pulmonary minute to systemic blood flow (Qp / Qs) - a reference indicator of the amount of discharge.

Right atriography, angiopulmonography... the flow of contrast from the right atrium to the left; identification of concomitant abnormal drainage of the pulmonary veins.

Drug therapy

In uncomplicated ASDs of the ostium secundum type, prophylaxis of infective endocarditis is usually not carried out. For ostium primum ASD, large sinus venosus defects, and ASD with mitral valve defects, antibiotics are prescribed before and for 6 months after uncomplicated surgical correction... With right ventricular failure, diuretics are prescribed.

Surgery

Indications... the Qp / Qs ratio is 1.5 or more, defects of the ostium primum type, large defects of the ostium secundum type, concomitant hemodynamically significant anomalies (abnormal drainage of the pulmonary veins, mitral stenosis, etc.).

Contraindications... severe concomitant pathology that threatens the patient's life; terminal stage of circulatory failure, irreversible pulmonary hypertension, the ratio of total pulmonary vascular resistance to systemic vascular resistance is 0.9 or more.

Methods surgical treatment ... Endovascular correction with a button or two-patch Sideris device or the Amplatz device is feasible for central defects no more than 2 cm in size. In the absence of experience in endovascular treatment, small defects are sutured in conditions of artificial circulation. In other cases, ASD plasty with a synthetic or autopericardial patch is recommended under conditions of artificial circulation.

Specific postoperative complications

Sick sinus syndrome (after correction of sinus venosus defects). AV block (after correction of ostium primum defects). With preoperative mitral regurgitation, symptoms may worsen after ASD correction. Atrial fibrillation that occurs before surgery usually persists after surgery.

Forecast

In the early childhood the course is benign. In rare cases, severe circulatory disorders can lead to death in the first months of life. Spontaneous closure of the defect is possible up to 5 years of age. The average life expectancy without treatment is 40 years. 5-15% of patients die before the age of 30. 10-year survival rate - 90%, 20-year-old - 88%, 30-year - 67%; 40 years old - 44%, 50 years old - 25%, 60 years old - 13%, 70 years old - 7%. More than 75% of patients with large defects die from other causes. In case of uncomplicated defects of the ostium secundum type, perioperative mortality is less than 1%, it is slightly higher with defects of the ostium primum type, with the latter, prosthetics or plastic of the mitral valve is also necessary.

Fallot's tetrad

Fallot's tetrad (TF) – combined congenital anomaly of the heart, which is characterized by stenosis of the excretory tract of the right ventricle, defect of the interventricular septum, dextroposition of the aorta and hypertrophy of the right ventricular myocardium. TF is one of the most common blue (accompanied by cyanosis) congenital defects hearts. Its frequency is 10-15% among all congenital heart defects.

Causes of occurrence

Perhaps the defect is hereditary.

Symptoms

The most common complaints and manifestations are: developmental delay; dyspnea; severe attacks shortness of breath with manifestations of cyanosis; central cyanosis; loss of consciousness; convulsions.

During the examination, the following symptoms are noted:

• pallor of the skin or cyanotic manifestations
• symptom of "drumsticks" or "watch glasses"
• the position of the patient squatting after light loads
• strengthening the first heart sound
• weakening the second tone over pulmonary artery
• the presence of systolic murmur or systolic diastolic.

Diagnostics

To confirm the diagnosis of TF, a complete medical history is taken, an ECG and a chest X-ray are taken.

Disease types

There are four main forms of the disease:

• embryological
• hypertrophic
• tubular
• multicomponent.

The embryological form is characterized by the displacement of the conical septum anteriorly and to the left. The zone of maximum development of stenosis corresponds to the muscle ring. In this case, the fibrinous ring is not deformed.

The hypertrophic form is characterized by the same displacement of the septum as in the embryological form, but, in addition, its pronounced hypertrophic changes in the proximal segment are noted. In this case, the zone of maximum development of stenosis is at the level of the outlet section of the right ventricle and the boundary muscle layer.

Tubular form. Obstruction in the third type of TF is caused by an unevenly formed division of the common arterial trunk. As a result, the pulmonary cone becomes shortened, narrowed, and hypoplastic. Also, this form is accompanied by hypoplasia of the fibrinous ring and stenosis of the pulmonary artery.

Multi-component form. The reason for the development of obstruction in this form of TF is the lengthening of the conical septum. The severity of hemodynamic disturbances is manifested in the form of obstruction of the right ventricular outflow duct. With severe stenosis, blood flows from both ventricles mainly into the aorta and in a very small volume into the pulmonary artery, and this condition is accompanied by arterial hypoxia. With moderately severe stenosis, peripheral resistance is greater in the excretory tract, therefore, left-right ejection of blood from the ventricles develops, which subsequently leads to the acyanotic form of the disease.

Treatment

The indications for surgery are absolute. In our country, most surgeons adhere to the milestone surgical method treatment of TF.

Up to 3 years of age, palliative operations are performed, which greatly facilitate the life of patients. The purpose of performing palliative operations is to increase blood flow into the pulmonary circulation.

Complications

The main complications of TF are infective endocarditis, paradoxical thromboembolism, erythrocytosis, hemostasis disorders, strokes, brain abscesses.

In non-operated patients, bacterial endocarditis joins, but it is more common in children who have undergone palliative bypass surgery in infancy. In children infancy, suffering from pulmonary atresia against the background of large collateral blood flow, congestive heart failure may develop.

Prevention

All patients with TF require regular follow-up in specialized centers for timely detection of complications, most of them annually. Echocardiography is performed during each visit, MRI is performed according to individual indications.

In asymptomatic patients without hemodynamic impairment, limitation physical exercise not. Patients at high risk of developing arrhythmias and PCC, with dysfunction of both ventricles, its progression, and patients with obvious pathology of the ascending aorta should avoid isometric exercises and physical activity moderate to significant intensity.

This disease is one of the most common congenital (intrauterine) heart defects. Congenital Tetralogy of Fallot externally manifests itself as cyanosis, therefore this disease is also called "blue" heart disease. The doctor, having looked at the child, will immediately diagnose the Tetrad of Fallot according to microbial data 10, if he has cyanotic skin, places near the ears and lips, the fingertips are also blue.

Description

For the first time, the doctor Etienne-Louis Fallot described this disease in detail. It got its name due to the presence of 4 heart disorders at once:

• Absence or malfunction of the interventricular septum.
• Unnatural position of the aorta. It is usually located over the left ventricle, but it shifts to the right, and the aorta moves directly over the defective interventricular septum (dextroposition).
• Stenosis () of the mouth of the pulmonary artery or some part of the right ventricle, from where venous blood flows into the artery.
• Increase in the tissue of the muscle of the right ventricle several times (hyperotrophy).

If the first three signs are an anomaly of the heart, then the last is a consequence of the presence of this anomaly. In the diagnosis and prognosis of the disease in the future, it will be extremely important how narrowed the pulmonary artery and what is the septal defect. All signs in each case of the disease have a very different form of expression.

The more pronounced the narrowing of the pulmonary artery, the more work falls on the right ventricle, and the sooner it begins to hypertrophy under such a load.

Due to the narrowing, only a small amount of venous blood enters the lungs, and the rest is directed through the deforming septum into the aorta. As a result, blood overflow occurs large circle blood circulation and its lack in small, as a result of this begins organs.

Diseases such as Fallot's triad (malfunction of the interatrial septum, enlargement of the ventricle, narrowing of the pulmonary artery), Pentad of Fallot (tetralogy of Fallot and atrial septal defect) are close to the tetrad of Fallot's tetrad in children.

Often the Tetralogy of Fallot disease is confused with the following diseases:

• Pulmonary artery infection.
• Common arterial trunk.
• The presence of only one ventricle.
• Double discharge of the main vessels from the right ventricle.

Often, along with the tetrad, the following pathologies appear:

• Di Giorgi syndrome (absence or underdevelopment of the parathyroid and thymus glands).
• Right location of the aortic arch.
• Incomplete drainage of the pulmonary veins.
• Narrowing of the pulmonary artery.
• Violation of the coronary arteries.
• Excess left superior vena cava.

Causes

Disorders occur during fetal development. Fallot's tetralogy develops up to the 9th week of pregnancy, during the formation of the heart of the unborn child.

Causes of Tetralogy of Fallot in newborn babies:

• Mother-borne diseases at the initial stage such as measles, rubella, scarlet fever.
• Use alcoholic beverages and narcotic drugs.
• The use of sedative, hormonal, sleeping pills.
• The impact of bad factors.
• Ionizing radiation.
• Heredity, genetics (if someone from close relatives also had such a disease)

Another reason may be chronic illness mothers, bad habits, improper poor nutrition.

Very often, the pathology of heart defect occurs in children with Amsterdam dwarfism syndrome, which also includes foot syndactyly, oligophrenia, gothic palate, lack of fingers, strabismus, ear deformities, disruption of work internal organs and much more.

In the first days or weeks, nothing causes concern, since the heart is still able to perform its function and disturbances do not affect the blood flow in any way. However, after some time, newborn babies' lips turn blue when they cry, their bodies turn blue when they are fed, that is, it happens, which only intensifies over time.

Cyanosis of the nasolabial triangle is the main symptom of Fallot's tetrad

Then shortness of breath is added, by the age of 2-3 years the child is no longer active, but often breathes deeply, sometimes faints.

External signs and symptoms of Tetralogy of Fallot in a child:

• A slight hump is a bulge in the heart.
• The fingers look like drumsticks.
• The nails take on a convex shape and are rounded.
• Development lag.
• Frequent infection with infectious and colds (acute respiratory, sinusitis, pneumonia, in older adults - pulmonary tuberculosis).

There are 5 types of the course of Fallot's tetrad in the fetus:

• Earlycyanotic form (manifestation of symptoms in the first month).
• Classic (manifestation at 2-3 years of age).
• Heavy (constant bouts of shortness of breath, symptoms from 3-4 months of life).
• Late cyanotic (identifying symptoms at 6-10 years old).
• Acianotic (pale).

Typically, blue heart disease can be seen on electrocardiography, which indicates the existence of right ventricular hyperotrophy, a phonocardiogram, where a murmur can be heard over the pulmonary artery, and an x-ray, where the heart looks like a shoe and the heart shadow is enlarged.

Pathology can be detected thanks to the following diagnostics:

• Ultrasound - (level of displacement of the aorta, level of pulmonary constriction, malfunction of the interventricular septum).
• Catheterization and examination of cardiac cavities (detect high blood pressure in the ventricles, oxygen starvation of the aortas).
• Aortography and pulmonary arteriography (existence of collateral blood flow and pulmonary artery disease).
• MRI of the heart.
• Left ventriculography.
• Multispiral.

Also, the diagnosis can often be identified with the help of certain studies and analyzes, and geneticists can help with this, since the disease is based on a violation of all genes in the body, and therefore a violation of the embryo.

A gynecologist can also diagnose Tetralogy of Fallot, since the causes are often:

• Dropsy of the fetus.
• Maternal phenylketonuria syndrome.
• Fetal alcohol syndrome.

Treatment

If a disease is detected, there is only one way out - an operation performed on time. The more complex the form of the disease and more often, the sooner the intervention of specialist doctors is necessary. The most common cases of death in children are in the first 2 years of life, so this age is especially dangerous with such a disease.

A very small proportion of children survive to adulthood without treatment. During the operations themselves, about 10% of children die, but after its successful completion 80% of patients live a normal, albeit very limited life. The cause of death may be asphyxiation, brain abscess or.

Operations with Tetralogy of Fallot in the fetus are usually two: palliative and radical.

Palliative means "incomplete", that is, this treatment is not complete. This operation is performed on children under 3 years of age in order to alleviate the patient's condition somewhat. The aim of this intervention is to reduce dyspnea and cyanosis by placing additional vessels between the arteries so that blood flows through the anastomoses to the lungs and left atrium.

A radical operation is performed at the age of 3-4 years, when the organs of children are large enough. During this surgical intervention the heart is stopped and the child is connected to a heart-lung machine, then the narrowing of the artery is removed and the septal defect is corrected. There are some contraindications to such procedures - endocarditis, various disorders nervous systems, acute course of any disease. After such an operation, some patients begin to develop arrhythmias of varying degrees in older age.

After a pathology detected on time and a successful operation, the chance to live is very great full lifetherefore, this disease is not a fatal diagnosis.