Interruption of the aortic arch(Atresia of the aortic arch, atypical coarctation) is the appearance of the result between the visceral and lower aorta. The frequency of wadi becomes 0.02 per 1000 newborns, 0.4% of the average CHD and close to 1% of the average of critical CHD.

So yak aorta arc It can be stored in three segments, which can be more vaguely embarrassing, and I am able to interrupt the aortic arch in small children at a different frequency.

With a diminished number of reasons, Wikidi from the lіvogo shlunochno in the aorta for the increased flow of blood into the Legenev artery (through the abnormal formation of the conotruncus) of the structure of the membranous heart, from which the aortic arch develops, becomes lush in the fetal stagnation and may increase the atresized one. It is especially friendly to be aware of pathology when the DMShP is received with obstructive exit from the lynch.

I will give the classification of vice proponated G. Celoria and R. Patton. With type A (30-41%), interruption of the arch of the distal lymph node artery, with type B (55-69%) - between the lateral sleepy artery and lymph node clavicular artery, with type C (4%) - malignant brachiocephalic artery artery.

For type A it is not easy to get a fibrous cord between the aortic knots ("aortic atresia"). With type B of the right, the clavicular artery can come out of the lower aorta below the interruption.

Isolation to break the aortic arch To develop superficially, no more than 25 types are described in the literature. For the vision of patients with such a pathology, an early development of the straining colaterals is necessary. In 98% of cases of blood flow in the basin of the lower aorta, it is possible to get it through the PDA. The second anomaly, necessary for obtaining blood circulation between oneself, is DMShP (frequency of 90%), through a roof the evil flows to the right. At first, it is necessary to shukati a defect of the aorto-pulmonary septum. On the other hand, there is also a single creeper (11%), an arterial arterial stovbur (10%), anomaly Taussig-Binga (9%).

Obstruction of the vivid tract lіvogo shlunochka (20%) can be pummeled with mucous ringing on the tapering septum, two-stalk aortic valve, hypoplasis of the valve ring.

Interruption of the aortic arch often get along with the deletion of the 22ql 1 chromosome (DiGeorge syndrome).

Hemodynamics in atresia of the aortic arch.

Livy sklunochok will prevent blood circulation in the upper half of the tulub. The right shlunochka viganyaє shelter in the legenevoy stovbur, the stars one її part goes to the legenevia artery, and іnsha through the OAP - to the decayed aorta. Violation of hemodynamics in the most frequent variants of defect (combination of interruption of the aortic arch with PDA and DMSHP) may be caused by people. However, in the world of duct ulceration, hypoperfusion syndrome of the lower half of the tulub, metabolic acidosis, oliguria develop. Metabolic damage to produce tissue damage to the brain, myocardium, and other organs. The progressive decline in the function of the myocardium and the decrease in the heart wikid will end with the death of the sick person.

In children with trivial functions open arterial duct to take advantage of hemodynamics in all types of tulubs. However, there is a strong dynamic competition between systemic and legacy blood flow: the reduction of the remaining blood flow is reduced to a decrease in the peripheral blood flow. From the side, a persistent high blood flow (through blood loss through septal defects) to produce the appearance of congestive heart failure.

fetal EchoKG... Intrauterine diagnostics of interruption of the aortic arch, as it is itself and the isolated coarctation of the aorta, is very difficult. An indirect knowledge of the wadi is the asymmetry of the shlunochkіv with the digestion of the right viddіlіv, as well as the reduction in the speed of blood flow in the viscid aorta.

The intrauterine period has a defect not infused into the blood circulation of the fetus, as it is a good functional PDA.
In the postnatal period the proportion of ailments also occurs through the passage of the duct. So, it’s like a schilny until early close, without an obvious treatment by stretching out the first month of life, go around 60% of children. Up to 6 months live no more than 26%. All of them are quiet, hto vijiv є stiyki dzherela blood loss of the lower aorta (OAP abo colaterali). Delivery is recommended in a special case, which has the capacity for infusion of prostaglandins and other surgical treatment... The vice is recognized up to the 2nd category of gravity.

Clinical symptoms of severing the aortic arch.

So yak viceє a typical ductus-fallow pathology, the first symptoms occur with an ear of a PDA close, start to develop in the early neonatal period. The child's camp, like a spat of looking normal, will start swiftly. The syndrome of a low heart wikidu develops - a shit like a pulse, tachycardia, bad health, coldness, oliguria. About the obviously lack of function of the PDA to reduce the pulse rate on the legs. The ailment will become restless, but then we will be spoiled. In the blood, decompensation of metabolic acidosis begins. The ischemic growth of liver cells to increase the concentration in the blood of transaminases and lactate dehydrogenase, the reduction of nirok - to the reduction of creatine, intestines - to necrotizing enterocolitis.

at more friendly coping with vice symptoms of mensh turn. It is possible that there is a slight cyanosis, which in 10% of ailments has a character of differentiation (more bends on the legs), it is difficult to achieve a physical development. Falsely from the anatomical variant of the defect є less similar to the pulse on the kidneys. With type A, the pulse is on the arms, but not on the legs. In case of type B, there is a decrease in pulse rate on the left hand, and in case of abnormal discharge of the right pidclavicular artery - on all pictures. In the world of development of hypervolemia of a small amount of blood circulation, or of a turn of obstruction to the exit from a lingering slut, symptoms of heart failure develop (tachycardia, zadishka, hepatomegaly). Prognostically unpleasant build-up cyanosis and noise over the heart.

auscultatory picture visualization of the presence of other countries of the heart - DMShP and OAP. Call є a small systolic murmur in the other-third mid-ribbed evil.
Electrocardiography for atresia of the aortic arch... Newborns take the view of the electrical axis of the heart to the right. With a trivial defect, livoshlunochkova or biventricular hypertrophy develops.

radiography breast cancer ... In newborns, the radiological picture may not be changed. Thin heart is slightly expanded, the vascular bundle is often narrow through the opening of the thymus (with DiGeorge syndrome). In the world of lowering the OLS of the Legenevian babies, they can take advantage of the arterial channel; with the development of heart failure, symptoms of venous stasis and a set of legends appear.

Echocardiography with atresia of the aortic arch... In most cases, echocardiography allows for the provision of a secondary diagnosis, which is necessary for the planning of treatment. Before the localization of the interruption, it is necessary to assess the diastasis between the segments of the aorta, the steps of the obstruction of the lingual shlunochka, the diameter of the aortic valve and the viscous aorta. Great value ma vyavlennya suputn_h UPU.

Defect of the slurry partition Spawn spawning, bloodshed off through new straightening of evil to the right or two-sided. Yaksho є OAP, shelter through the new stream from the legacy artery into the decayed aorta.

Lіkuvannya atresia of the aortic arch.

The main method of lіkuvannya neonatal infants with interruption of the aortic arch є infusion of prostaglandins of group E for the management of PDA. One hour later, there was an adequate internal blood volume and anemia.

For a hearty wikidu vikoristovyt inotropic drugs, most often - dopamine (4-6 mcg / kg / xv). Danish drug to repair pre-positive positive infusion on the nirk function, yak is suffering when the aortic arch is interrupted. However, there is a series of high doses of uniqueness, which makes it possible to increase the peripheral support and reduce the amount of blood in the leg.

Do deyak vipadkah be brought go into before the piece ventilation of the legends, it should be carried out in the regimes, which include hyperventilation, and without additional souring. Tsi come in is necessary to prevent the closure of the PDA or excess blood flow. Do not pragmatic until the systemic S02 is increased by 75-85%. It is also necessary to continuously monitor and correct metabolic acidosis.

Have sick people sympathetic syndrome DiGeorge requires periodic conversion of calcium; The transfusion of the precious blood may not be safe.

Hirurgicne involvement vikonuyut the song of stabilization of the sick. Operative vibration є stem of direct anastomosis between segments of the aorta. Interposition of a synthetic prosthesis is possible. With the great shuntuvannya blood through the DMShP surgery on the aorta will add to the sound of the legacy artery; radical correction through the mug of miscellaneous. In the clinics with the great surgical admission, there is a momentary disruption of the aortic arch and the concomitant CHD.

Interruption of the aortic arch (atresia of the aortic arch, atypical coarctation) - the appearance of the result between the viscous and the lower part of the aorta. The frequency of wadi becomes 0.02 per 1000 newborns, 0.4% - the middle of the CHD, and close to 1% - the middle of the critical CHD. G. Celoria and R. Patton (1959) were used to classify the vice.
With type A (30-41%), the break of the arch of the distal left pidclavicular artery.
With type B (55-69%), there is also either an eccentric sleepy or an interclavian artery.
With type C (4%) - between the brachycephalic trunk and the sleepy artery. Isolation to break the aortic arch is suspiciously rare, in the literature no more than 25 types are described. for
The vision of the patient is necessary for the appearance of a cross-cutting blood loss. 98% of the blood flow in the basin of the lower part of the aorta flows through the PDA (from the right to the left). The insha anomaly, which is necessary for obtaining blood circulation between oneself, is DMShP (frequency> 90%), through a kind of roof flowing evil to the right. At first, it is necessary to shukati a defect of the aorto-pulmonary septum. Interruption of the aortic arch is often accompanied by a deletion of chromosome 22qll (D-George syndrome).

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Morphology

Reduction of the aortic arch is established at the same frequency, both distal and distal to the clavicular artery (type A), and distal to the posterior carotid artery (type B). Before it is established to break the distal innominate artery (type C). Mayzhe in all types of abnormalities, most often posterior malignant DMSP, can lead to subaortic obstruction and the associated arterial duct connected with it. Інші forms of children's music schools are developed earlier. Pathology of ventriculoarterial diseases, including discordance, as well as subordinate withdrawal from the pancreas (Taussiga-Bing anomaly) can also be promoted. Evidence of 22q11 delays in shukati in all cases of aortic arch reversal.

Pathophysiology

Most of the time, if you interrupt the arcs and open arterial ducts, the child sees himself good, as long as the duct is ruffled, the lower perfusion in the lower part of the body is not more critical. In most cases, children enter into specialization for the first 2 days of life with a cob, CH, and do not quickly accelerate from shock and acidosis. In rare cases of the arterial duct, there is no evidence of excess blood flow and the development of superfluous blood flow in the world of a decrease in the weight of the leg.

diagnostics

key overrun

The most specific knowledge є the difference in heart rate in the upper half of the body with a weakened heart rate on one or both hands or on one sleepy arteries(The picture may change in response to pharmacological injection into the arterial duct outlet). Auscultation, as a rule, is nonspecific due to the presence of noises associated with congenital heart pathology.

radiography

The heart flutter from the left side with signs of cardiomegaly. Yak, as a rule, you will be satisfied with the Legenevii babies. The appearance of the thymus may be suggestive of the 22q11 deletion.

There is no specific EKG-sign.

EhoKG

With EchoKG, it is necessary to properly describe the aorta, interrupt, and also describe the way the heads of the head and shi are. For the planning of surgical tactics, a real assessment of the intracardiac anatomy on the subject of concomitant pathology is even more important.

Catheterization empty heart

Carrying out for diagnostic purposes is not necessary. Vona widely replaces EchoKG, in addition to MRI or CT.

lykuvannya

During the neonatal period, you want to be carried out outside the renewal of the interrupted aortic arch at once from the closure of the Children's Music School. The results of the operation are due to the nature and severity of the obstruction of the aortic arch and the clinical stun of a child. It is necessary to keep an eye on the aortic arch, so as to prevent excessive or recurrent obstruction of the arch, as in patients undergoing aortic aortic surgery.

John E. Deanfield, Robert Yates, Folkert J. Meijboom and Barbara J.M. Mulder

Vrodzheni wadi hearts for children and grown-ups

The growth of the heart and pathology of the pericardial arteries in the last two ten years are often observed. With such destructions, structural changes in the heart are empty, however, pathology can be seen, in the debris of anatomical anomalies and acceleration, as they are affected.

The most important form of the heart can be inserted into the atresia of the aorta. Qia anomaly is daily visibility enlightenment in the visual part of the aorta.

Atresia to the organ, which can be used for pipes, is most likely to come together with other types of heart defect.

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Such pathologies are referred to:

• hypoplasia of the aorta;
• underperformance of the livny shlunochka;
• atresia or obstruction of the mitral valve;
• pathology, when the arterial duct does not curl up at once;

The atresia of the arterial heart tube can be a viclikan, or we can use a factor that makes it difficult for the blood flow to come out of the empty heart.

Ethiology and pathogenesis

Exciting for that particularity, how the aorta extends into three main directions, during the period of the development of the vibrant movement, which can be young, then the difference in the development of the arc of the arterial court in the early stages of the watch and

In the case of a blood flow from the livny to the aorta, for some reason or not, it is important, for the sake of the movement of the circulatory system into the Legenev's artery because of the blood.

In the end of the structure of the empty heart, the arc of the arterial tube develops, it can be blocked or it can be re-atresized.

The development of such a pathology can be caused by the occurrence of such failures, such as a defect in the mid-hole septum, due to the sound of the exit from the left hole.

Name aortic atresia early stage vaginosti, ale, through those who look like a natural wadi, to be carried to the first category of gravity, likar to gladden to go in before the procedure for recreating vaginosity

hemodynamics

In the process of hemodynamic damage, it becomes difficult to blood flow through the empty heart. All the main navantazhennya take on itself the right lingo, which is responsible for the systemic blood circulation, pumping over the roof. With the year of such damage of hemodynamics, blood circulation changes. internal organs, Zokrema brain and heart itself.

With such a defect in children with such a defect, the decompensation of the right slunker, the wine grows up in size, and the empty one expands. And even the male has seen the control of the legenevo and the systemic blood circulation.

Likewise, it is often the case that the progressive advancement of the Legendary judiciary is made more progressively, as it is tied with the anatomical serpent of the Legendary judges.

With a trivial opening of the arterial duct and its function, for a certain hour, stable hemodynamics is obtained in all types of tulub.

However, when there is a possibility of a dynamic support between the systemic and legenevym blood strum, and if the circulation of the leg blood flow increases, then the peripheral blood circulation of the organs decreases. At the same hour, the circulation of blood flow in the legenevian vessels can be caused to the appearance of congestive symptoms and heart failure.

Groupi Riziku

Such an anomaly, such as atresia of the aorta in newborns, is not easy to develop. As the statistics show, the middle of the neoplasm in the middle of the wads of the heart itself atresia of the arterial tube appears at a frequency of 0.5 to 7.8%, mainly in the boys. Moreover, the pathology is given a serious acceleration, common reasons bend the child.

Before the development of an innate wadi, the hearts of children are to be produced without the help of factors;

• even in the homeland for children with an inborn pathology of the heart;
• as for relatives from the side of the dad є UPU;
• різні decline like at the mother, so at the father.

Talk about maternal factors Riziku Varto okremo.

From the same, to the development of the pathology of the heart in a newborn can lead to:

It is also a good idea to look at the factors that can be used to infuse the diagnosis of pathology during the period of intrauterine development of the fetus:

• extracardiac anomalies;
• a change in the number or the structure of chromosomes;
• distribution of food in the fetus through the lack of masi tila;
• oversized purchase of births from post-office wipes and empty parts of the fetus;
• damage to placental blood flow;
• the vagina is more than one fruit.

Symptoms of aortic atresia

For the diagnosis of aortic atresia in a child, immediately after the people are guilty, the following symptoms appear:

• shkirni pokrivi new-born bloody blooms;
• myazova weakness, as a result of which the child does not show rook activity;
• tachypnoe or a frequent zadishka in a baby;
• vologі wheeze in legends.

If you come to see the child, you may also be able to promote cyanosis, but in some cases of shkirny, twist the blues of the eyes, either on the hands or on the legs. One of the most significant signs, for which it is possible to have a congenital pathology, such as atresia of the aorta is a heart failure.

There may be such symptoms as the swelling of the whole body of the baby, the liver may grow larger.

Frequently in newborns with a heart defect, the pulse on the arms and legs is even weak, at the same time, there may be a reduction in the strength of the heart.

diagnostics

The appearance of the heart of the fetus in the fetus may appear in the uterus during this condition. For the whole vagіtnu periodically send to the SPL. In case of pathology of the aortic arch in a child, immediately because of the disease, all signs of an illness are suspected, but it is not allowed to become confused about the accuracy of the diagnosis set earlier.

For children, carry out additional diagnostic tests, prior to which the following diagnostic methods are used:

Retrograde aortography is also shown.

lykuvannya

Immediately, in order that the diagnosis will be confirmed for additional diagnostic methods, children will recognize ineffectively lіkuvannya atresia of the aorta.

In the first place, the introduction of the drug prostaglandin of group E is shown, it is necessary in order to prevent the expansion of the development of the arterial duct. It is also necessary to suspect all the symptoms and causes, such as the anemic syndrome, for which there is a reconciliation of blood in all the midst of the midst.

For a normal blood count, for a heart-to-heart in the court, indications for taking inotropic drugs. Dopamine is most commonly used in a group of medications, and the drug is positively infused not only with the heart wikid, but with the functionality of nirok, which suffers from such pathology, as I will interrupt the aortic arch.

When Doha is added to the drug, there is a great need for the improvement of peripheral vascular support;

At the turn of an important stage in the development of the heart of the heart of the snake, go to the piece ventilation of the legends in the child, at which there is a hyperventilation. This allows the over-excess circulation of the bloodstream and the closeness of the arterial duct to be closed.

If the child of a troch is in regulation, he / she goes to the operative therapy, as a polyagu, in addition to the surgical engagement of a segment of the aortic artery.

Some people cannot do without the installation of a synthetic prosthesis. With clear signs of a great shuntuvannya to blood flow through the midshlunochkovy septum with a defect in the operation on the aorta, there is a sound of the leg artery.

accelerating

Such a pathology, such as atresia of the aorta, can lead to severe acceleration in the child.

Expect to such problems a decrease in microcirculation in the brain, it is not easy to lead to a breakdown of its development, as well as a decrease in the brain's speech (one of the most common causes of cerebral palsy in children).

Likewise, there is a pathology of the heart and the opening of the aortic arch can go into the last stage, if the new-born Vinnik has a heart failure. At the same time, the anomaly will block the death of the child.

It is also necessary to identify pathology in the fetus at the early terms of vaginosity, and to allow the diagnosis and the importance of the method of effective treatment in advance.

forecasts

The prognosis for atresia of the aorta in the fetus is not optimistic. I will show statistics, for the first time of life of a child with a defect of the heart, in 71% of cases.

Up to six months it is practically all children with a pathological pathology. As soon as an operation was carried out, it gave positive results in lyuvanny, the possibility of a lethal result becomes 20 to 60%.

However, there is a further guarantee that the child’s camp doesn’t go down with rock and don’t get lost, surgical involvement not yesє.

prophylaxis

Come on, the prevention of CHD is ambiguous. On the right, in the fact that the head factor is the growth of the child's heart pathology before the diagnosis of a congenital pathology in the child, the genetic particularity is that it was transmitted from the father.

It’s admittedly, in the homeland there would be three people who would form in a direct homeland, they were born with this anomaly, є it’s great that they have a child from the UPU. In such cases, fakhivtsi women categorically do not recommend a visit or having children.

Those are the very same feeling and quiet vipadkiv, if the woman, and the cholovik, who stay with the girls, roztashovani until the heart of the genie. Є Practically 100% ymovіrnіst, їх їх a child is born with a pathological heart. Besides, in yakosti proactive visits I’m leading a retailer until women’s sleep Infectious diseases(Rubella virus, Epstein-Barr virus, Koksaki virus, etc.)

Even though the woman still has a decline in her heart until she is diagnosed with a pathology of the heart, but she herself does not have a CHD, then with a vaginosity it is also necessary to look for prophylactic visits, such as walking in the offensive:

• continuous control over the overrun of vaginosti;
• vіdhilenya vіdhilenya vіdhilenya fetus;
• continuous control over the camp of the child and retreating to look at it at once for the people;
• The most effective method for the treatment of children from the Air Force (as a rule, it is not necessary to work with them).

It is also necessary to follow up on the preventive measures to accelerate the UPU. Most unsuccessful accelerated heart pathology є infectious endocarditis, It is not easy to develop with a child even in a preschool life.

In general, it is necessary to turn on the use of any antibiotics, as it is not easy to bring a sick person to death with a kind of heart. The blame can become a deprivation of antibiotic-prophylaxis in case of stomatological involvement (the introduction of the drug is clear).