Only the right aortic arch has. Anomaly in the structure of the aortic arch

Antipyretics for children are prescribed by a pediatrician. But there are emergency situations for fever when the child needs to be given medicine immediately. Then the parents take responsibility and use antipyretic drugs. What is allowed to be given to infants? How can you lower the temperature in older children? What medications are the safest?

With this anomaly, the ascending aorta goes up and to the right from the trachea and esophagus, spreads over the right bronchus, and goes down either to the right or, passing behind the esophagus, to the left of the spine. The right-sided aorta often manifests itself without pathological symptoms. In these cases, the arterial ligament is located in front of the trachea and is not stretched, and if it passes behind the esophagus, it is long. If the ligamentum arteriosus or patent ductus arteriosus passes from the pulmonary artery to the aorta to the left of the trachea and behind the esophagus, a ring is formed surrounding the esophagus and trachea. The arterial ligament presses on the esophagus and trachea. The left subclavian artery in one case passes in front of the trachea or diverticulum of the residual IV of the left branchial arch. The diverticulum is located at the junction of the right arch with the descending aorta. Diverticula are remnants of the left IV branchial arch with various options for the origin of the subclavian arteries.

Clinical symptoms

In children, a right-sided aortic arch can cause persistent hiccups. In the absence of a constricting ring closed by an arterial ligament, the course of the disease is asymptomatic. In adults with aortic sclerosis, the symptoms of dysphagia intensify. Respiratory disorders worsen after eating.

Varieties described in the literature

Right-sided aortic arch with left-sided arterial ligament according to A. Blalock

The aortic arch extends over the right main bronchus and descends from the right side of the spine as the descending aorta. The left common carotid and left subclavian arteries depart from the innominate artery. The ligamentum arteriosus attaches to the innominate artery.

Right-sided aortic arch with left-sided descending aorta combined with patent ductus arteriosus (Beavan and Fatty)

The right-sided aortic arch is located in the neck, at the level of the thyroid cartilage, on the right side of the larynx. The aortic arch is formed in this case from the third pair of the right branchial arch. The patent ductus arteriosus enters the descending aorta opposite the left subclavian artery. The left common carotid artery arises from the ascending aorta and ascends anteriorly and to the left of the trachea. The ductus arteriosus is involved in a vascular ring that compresses the trachea and esophagus.

X-ray diagnostics

X-ray data. When inhaling - insufficient aeration of the lungs, when exhaling - hyperaeration. Signs of infection in the lungs. The protrusion of the aorta is visible on the right side of the mediastinal shadow, and on the left the normal shadow of the aortic arch is absent. On the left side there is often a shadow image of a diverticulum located where the aortic bulge would normally be. The descending aorta is sometimes displaced towards the pulmonary fields. In the first oblique position, the trachea is shifted forward, and the shadow of the diverticulum is detected at the level of the arch between the trachea and the spine. In the left oblique position, the descending aorta bends. Lateral radiographs show a trachea filled with air in the upper normal part and clearly narrowed in the lower part.
Examination of the esophagus. A barium swallow reveals a sharp narrowing of the esophagus and compression of its left lateral and posterior surface, if there is a diverticulum or an arterial ligament in a closed ring. Above the notch on the posterior surface of the esophagus, a separate defect running obliquely upward and to the left is determined. It is caused by compression of the left subclavian artery, which passes behind the esophagus to the left clavicle. The shadow of the left subclavian artery, passing behind the esophagus, is located above the shadow of the right aortic arch. A pulsating left aortic diverticulum is seen posterior to the esophagus. The esophagus is displaced anteriorly.
Examination of the trachea with lipidol. If there are symptoms of tracheal compression, a contrast study of it shows the localization of the aortic ring. The introduction of lipoidol into the trachea reveals an elongated notch along the right wall of the trachea, caused by the adjacent aortic arch, a notch on the anterior wall of the trachea from compression by the pulmonary artery, and a depression on the left wall of the trachea - from the ligament arteriosus. If there is no compression of the trachea, then there is no point in examining it with lipidol.
Angiocardiography. It is produced when a right-sided aortic arch is combined with other congenital heart defects.

Differential diagnosis

A right-sided aortic arch may produce a pattern similar to that seen with a double aortic arch. In the anterior image, the right-sided aortic arch in children in the presence of an enlarged shadow of the thymus gland is not clearly visible. However, the gland does not move the esophagus forward. Tumors in the posterior portion of the superior mediastinal shadow may simulate the right aortic arch, but they do not pulsate. The normal prominence of the aortic arch on the left is preserved. With aneurysms of the innominate artery or left descending aorta, a shadow of the descending aorta is always detected.

Right-sided position of the aortic arch

Gorky region publishing house, 1942

Given with abbreviations

The fourth right gill arch turns into art. Anonyma and the beginning of art. Subclavia dex.

In the case of the described anomaly, the opposite occurs: the aortic arch develops from the fourth right embryonic arch, and the fourth left embryonic arch develops into art. Anonyma sin. et art. Subclavia sin.

Anatomically, the right-sided position of the aortic arch is that instead of the usual position of the aortic arch to the left of the trachea and the arch crossing the left bronchus, the aortic arch is located to the right of the trachea, crossing the right bronchus.

Subsequently, the descending part of the thoracic aorta can take a different direction: either it moves to the left side at different heights, continuing to the left of the spine, or until the diaphragm it (the descending part) goes to the right of the spine. X-ray - in typical cases of the described anomaly, the left aortic protrusion (aortic arch and the beginning of the descending part) is visible on the right side under the sternoclavicular joint.

In special positions, the right nipple position (first oblique) and the left nipple position (second oblique), it is possible to see a course unusual for the aorta above the right bronchus, and the shadow of the aorta in both oblique positions represents a mirror image of one another; in the 1st oblique position, instead of the usually visible aortic bell, the overlapping shadows of the ascending part and the initial descending part are visible separately: the ascending part (to the right of the observer), the aortic arch and the descending part (to the left of the observer). On the contrary - in the 2nd oblique position. With this anomaly, the deviation in the course of the esophagus deserves special attention. An X-ray examination of the latter with a contrast mass reveals (in the dorsoventral position) a pronounced deviation of the esophagus to the left at the height of the aortic arch (instead of the usual slight deviation to the right). In oblique positions, in addition, a significant forward deviation of the esophagus is visible (instead of the usual slight posterior deviation) and a semicircular depression on the posterior contour. Based on the literature data, verified by autopsy, such a change in the position and configuration of the esophagus should be explained by the right-sided position of the aortic arch, the more medial position of the ascending aorta, the intersection of the right bronchus by the aortic arch and the intersection of the esophagus posteriorly by the left subclavian artery, which often forms an extension with this anomaly .

At present, when the kymography method is deservedly being introduced more and more into cardiological practice, the use of this method to study and identify the described anomaly should be mandatory.

A cardioaorthokymogram will undoubtedly help in those difficult cases (in pediatric practice, with a small medial heart and narrow aorta, etc.) when individual segments of the aorta are poorly differentiated during normal examination.

Situs inversus arc. Aortae can occur as an independent, isolated anomaly and often in combination with other malformations of the cardiovascular bundle: with dextrocardia with and without inversion of the heart chambers, with Ductus apertus Botalli, with Roget's defect, etc.

Right aortic arch: what is it, causes, development options, diagnosis, treatment, when is it dangerous?

The right aortic arch in the fetus is a congenital heart defect, which can occur alone or be combined with other, sometimes severe, defects. In any case, during the formation of the right arch, disturbances in the normal development of the fetal heart occur.

The aorta is the largest vessel in the human body, the function of which is to move blood from the heart to other arterial trunks, up to the arteries and capillaries of the whole body.

Phylogenetically, the development of the aorta undergoes complex changes during evolution. Thus, the formation of the aorta as an integral vessel occurs only in vertebrates, in particular in fish (two-chamber heart), amphibians (two-chamber heart with an incomplete septum), reptiles (three-chamber heart), birds and mammals (four-chamber heart). However, all vertebrates have an aorta, into which arterial blood mixed with venous, or entirely arterial, flows.

During the process of individual development of the embryo (ontogenesis), the formation of the aorta undergoes changes as complex as the heart itself. Starting from the first two weeks of embryo development, there is an increased convergence of the arterial trunk and the venous sinus, located in the cervical part of the embryo, which subsequently migrated more medially, towards the future thoracic cavity. The arterial trunk gives rise not only to two ventricles subsequently, but also to six branchial (arterial) arches (six on each side), which, as they develop, within 3-4 weeks, are formed as follows:

the first and second aortic arches are reduced,
the third arch gives rise to the internal carotid arteries that supply the brain,
the fourth arch gives rise to the aortic arch and the so-called “right” part,
the fifth arc is reduced,
the sixth arch gives rise to the pulmonary trunk and the arterial (Botallov) duct.

The heart becomes completely four-chambered, with a clear division of the cardiac vessels into the aorta and pulmonary trunk, by the sixth week of development. A 6-week embryo has a fully formed, beating heart with large vessels.

After the formation of the aorta and other internal organs, the topography of the vessel is as follows. Normally, the left aortic arch begins from the aortic bulb in its ascending part, which, in turn, originates from the left ventricle. That is, the ascending part of the aorta passes into the arch approximately at the level of the second rib on the left, and the arch bends around the left main bronchus, heading posteriorly and to the left. The uppermost part of the aortic arch projects onto the jugular notch just above the upper part of the sternum. The aortic arch goes down to the fourth rib, located to the left of the spine, and then passes into the descending part of the aorta.

In the case when the aortic arch “turns” not to the left, but to the right, due to a failure in the formation of human vessels from the branchial arches of the embryo, they speak of a right-sided aortic arch. In this case, the aortic arch extends through the right main bronchus, and not through the left, as it should normally be.

Why does vice occur?

Any malformation is formed in the fetus if a woman is influenced by negative environmental factors during pregnancy - smoking, alcoholism, drug addiction, ecology and unfavorable background radiation. However, an important role in the development of a child’s heart is played by genetic (hereditary) factors, as well as existing chronic diseases from the mother or transferred infectious diseases, especially on early stages pregnancy (flu, herpes infection, chickenpox, rubella, measles, toxoplasmosis and many others).

But, in any case, when any of these factors influence a woman in the early stages of pregnancy, the normal processes of ontogenesis (individual development) of the heart and aorta, formed during evolution, are disrupted.

So, in particular, the period of pregnancy of approximately 2-6 weeks is especially vulnerable to the fetal heart, since it is at this time that the aorta is formed.

Classification of the right-sided aortic arch

variant of the right aortic arch with the formation of a vascular ring

Depending on the anatomy of the duct anomaly, there are:

The right aortic arch without the formation of a vascular ring, when the arterial ligament (overgrown arterial, or Botallov, duct, as it should be normally after childbirth) is located behind the esophagus and trachea,
The right arch of the aorta with the formation of a vascular ring, code arterial ligament, or patent ductus arteriosus, is located on the left of the trachea and esophagus, as if surrounding them.
Also, a double aortic arch is distinguished as a separate similar form - in this case, the vascular ring is formed not by a connective ligament, but by a tributary of the vessel.

Figure: a variety of options for the atypical structure of the aortic arch

Depending on whether any other structures of the heart were damaged during its formation, the following types of defect are distinguished:

An isolated type of malformation, without other developmental anomalies (in this case, if the right-sided aorta is not combined with the DiGeorge syndrome characteristic of it in some cases, the prognosis is as favorable as possible);
In combination with dextrapposition (mirror, right location of the heart and great vessels, including the aorta), (which is also usually not dangerous),
In combination with a more serious heart defect - in particular tetralogy of Fallot (dextraposition of the aorta, ventricular septal defect, pulmonary stenosis, right ventricular hypertrophy).

Tetralogy of Fallot combined with the right arch is an unfavorable development option

How to recognize a vice?

Diagnosis of the defect is not difficult even during pregnancy. This is especially true in cases where the right aortic arch is combined with other, more severe anomalies of heart development. However, to confirm the diagnosis, a pregnant woman is repeatedly examined, including with expert-class ultrasound machines, and a council of geneticists, cardiologists and cardiac surgeons is assembled to make a decision on the prognosis and the possibility of delivery in a specialized perinatal center. This is due to the fact that with some types of defects combined with the right aortic arch, the newborn baby may require heart surgery immediately after delivery.

Regarding the clinical manifestations of the right aortic arch, it should be mentioned that an isolated defect may not manifest itself at all, only sometimes accompanied by frequent obsessive hiccups in a child. In the case of combination with tetralogy of Fallot, which accompanies the defect in some cases, clinical manifestations are clearly expressed and appear in the first days after birth, such as increasing pulmonary heart failure with severe cyanosis (blue skin) in the baby. That is why tetralogy of Fallot is classified as a “blue” heart defect.

What screening shows a defect in pregnant women?

To further clarify the lack of connection between the formation of the right-sided aorta and severe genetic mutations Fetal DNA analysis may be possible. In this case, chorionic villus material or amniotic fluid is usually collected through a puncture. First of all, DiGeorge syndrome is excluded.

Treatment

In the event that the right aortic arch is isolated and is not accompanied by any clinical manifestations after the birth of the child, the defect does not require surgical treatment. All you need is a monthly examination by a pediatric cardiologist with regular (every six months - once a year) ultrasound of the heart.

When combined with other heart defects, the type of surgical intervention is selected based on the type of defect. Thus, with tetralogy of Fallot, surgery is indicated in the first year of a child’s life, carried out in stages. At the first stage, palliative (auxiliary) shunts are applied between the aorta and the pulmonary trunk to improve blood flow into the pulmonary circulation. At the second stage, open heart surgery is performed using a cardiopulmonary bypass machine (ACB) to eliminate pulmonary stenosis.

In addition to surgery, cardiotropic drugs that can slow the progression of chronic heart failure (ACE inhibitors, diuretics, etc.) are prescribed for auxiliary purposes.

Forecast

The prognosis for an isolated right-sided aortic arch is favorable, since in most cases surgical intervention is not even required. So, in general, we can say that an isolated right aortic arch is not life-threatening for the child.

With combined types, the situation is much more complicated, since the prognosis is determined by the type of concomitant heart defect. For example, with tetralogy of Fallot, the prognosis without treatment is extremely unfavorable; unoperated children with this disease usually die in the first year of life. After surgery, the duration and quality of life increase, and the prognosis becomes more favorable.

Anomalies of the aortic arch. Causes. Treatment options. Consequences.

Congenital malformations of the aortic arch have been known at least since the anatomical publications of Hunauld's anomalous right subclavian artery in 1735, Hommel's double aortic arch in 1937, Fioratti and Aglietti's right-sided aortic arch in 1763, and Steidele's interrupted aortic arch in 1788. The clinical and pathological relationship of swallowing disorders with an anomaly of the right subclavian artery was described by Bayford in 1789, but only in the 1930s, with the help of barium esophagography, some defects of the aortic arch were diagnosed during life. Since then, clinical interest in this pathology has grown in parallel with the expansion of the possibilities of surgery. The first transection of the vascular ring was performed by Gross in 1945, and the first successful repair of a ruptured aortic arch was performed by Merrill and coworkers in 1957. Developments in echodiagnosis of these malformations since the 1990s have been the impetus for early non-invasive recognition and timely surgical treatment.

Anatomical classification

Defects of the aortic arch in isolated form or in combination are presented:

anomalies in the branching of brachiocephalic vessels;

anomalies in the location of the arch, including the right-sided aortic arch and cervical aortic arch;

increasing the number of arcs;

interruption of the aortic arch;

abnormal origin of a branch of the pulmonary artery from the ascending aorta or from the opposite branch of the pulmonary artery.

Individual anomalies are better understood in terms of their embryonic origin.

Embryology

The embryology of the aortic arch is better described as the successive appearance, persistence, or resorption of the six pairs of vessels connecting the truncoaortic sac of the embryonic heart tube to the dorsal dorsal aorta, which coalesce to form the descending aorta. Each arc corresponds to the branchial sac formed from the embryonic germ.

The normal left-sided aortic arch originates from the aortic part of the fetal arterial trunk, the left branch of the truncoaortic sac, the left IV aortic arch, the left dorsal aorta between the IV and VI embryonic arches, and the left dorsal aorta distal to the VI arch. The three brachiocephalic branches of the arch originate from different sources. The innominate artery is from the right branch of the trancoaortic sac, the right common carotid artery is from the right III embryonic arch, and the right subclavian artery is from the right VI arch and the right dorsal aorta in the proximal part and the right VII intersegmental artery in the distal part. The left carotid artery originates from the left III aortic arch, the left subclavian artery - from the left VII intersegmental artery. Although the appearance and disappearance of vessels such as arches or parts of brachiocephalic vessels occur sequentially, Edwards proposed the concept of a "hypothetical double aortic arch" that potentially contributes to almost all embryonic arches and components of the final aortic arch system.

Clinical classification

In addition to the anatomical classification, it is possible to subdivide the anomalies of the arch according to clinical signs:

compression of the trachea, bronchi and esophagus by vessels that do not form a ring;

anomalies of the arch that do not create compression of the mediastinal organs;

ductus-dependent arch anomalies, including interruption of the aortic arch;

isolated subclavian, carotid or innominate arteries.

Determination of the left and right aortic arch

The left and right aortic arches are determined by the main feature - which bronchus the arch crosses, regardless of which side of the midline the ascending aorta is located. This is especially important to remember when studying angiographic images. Usually, the position of the aortic arch is determined indirectly by echocardiography or angiography by the nature of the branching of the brachiocephalic vessels. In all cases, except for isolated or retroesophageal innominate or carotid arteries, the first vessel - the carotid artery - is located on the opposite side of the aortic arch. MRI directly shows the relationship of the arch, trachea and bronchi, eliminating the uncertainty with atypical branching of the vessels.

Right aortic arch

The right-sided aortic arch crosses the right main bronchus from above and passes to the right of the trachea. There are four main types of right-sided arch:

retroesophageal left subclavian artery;

with retroesophageal diverticulum;

with left-sided descending aorta.

There are also several rare variants. The right-sided aortic arch in Fallot's tetrad occurs with a frequency of 13-34%, in OSA - more often than in Fallot's tetrad, with simple transposition - 8%, complex transposition - 16%.

Right-sided arch with mirror origin of brachiocephalic vessels

With a mirror right-sided arch, the first branch is the left innominate artery, which divides into the left carotid and left subclavian arteries, the second is the right carotid and the third is the right subclavian artery. However, this symmetry is not complete, since the arterial duct is usually located on the left side and departs from the base of the innominate artery, and not from the aortic arch. Therefore, the typical right-sided mirror arrangement of the arch with a left-sided duct or ligament does not form a vascular ring. This variant in frequency accounts for 27% of aortic arch anomalies. It is almost always associated with congenital heart disease, most often with tetralogy of Fallot, less often with OSA and other conotruncus anomalies, including transposition of the main arteries, departure of both large vessels from the right ventricle, anatomically corrected transposition, and other defects. The mirror location of the arc also accompanies defects that are not related to the group of conotruncus anomalies, such as pulmonary atresia with an intact interventricular septum, VSD with abnormal muscle bundles in the right ventricle, isolated VSD, coarctation of the aorta.

A rare variant of the mirror right aortic arch has a left-sided ductus arteriosus or ligament arising from the right descending aorta behind the esophageal diverticulum. This variant forms a vascular ring and is not accompanied by other congenital defects. Since this type of right-sided arch does not cause compression of the esophagus and does not form a vascular ring, it does not manifest itself clinically, and therefore is diagnosed during examination for concomitant congenital heart disease.

By itself, the right-sided arc does not require intervention. However, in certain circumstances it is useful for the surgeon to know the location of the aortic arch. It is better to perform systemic-pulmonary anastomoses according to Blalock-Taussig or a modified anastomosis from the side of the innominate artery. In the classical operation, the more horizontal origin of the subclavian artery makes it less likely to kink if the severed end is sutured to the pulmonary artery than if the subclavian artery arises directly from the arch. Even when using a Gore-Tex vascular graft, the innominate artery is more convenient for proximal anastomosis because it is wider.

Another situation in which it is useful to know the location of the aortic arch is the correction of esophageal atresia and tracheoesophageal fistula, since access to the esophagus is more convenient from the side opposite to the location of the aortic arch.

Right-sided arc with isolation of opposite vessels of the arc

The term "isolation" means that this vessel departs exclusively from the pulmonary artery through the ductus arteriosus and is not associated with the aorta. Three forms of this anomaly are known:

isolation of the left subclavian artery;

left innominate artery.

Isolation of the left subclavian artery is much more common than the other two. This pathology in half of the cases is combined with CHD, and in 2/3 of them - with Fallot's tetrad. In the literature, there are single reports of an isolated left carotid artery in combination with Fallot's tetrad and an isolated innominate artery without concomitant defects.

Patients with this pathology of the vessels of the arc have a weakened pulse and lower pressure in the corresponding artery. When the subclavian and vertebral arteries are isolated, a "steal" syndrome develops, in which blood from the vertebral artery is directed downward into the subclavian artery, especially when the arm is loaded. In 25% of patients, the pathology is manifested by cerebral insufficiency or ischemia of the left hand. With a functioning ductus arteriosus, blood from the vertebral artery flows through the ductus arteriosus into the pulmonary artery, which has low resistance. In patients with a right-sided arch and decreased pulse amplitude or decreased pressure in the left arm, this defect should be suspected.

A contrast agent injected into the aortic arch demonstrates late filling of the subclavian artery through the vertebral and various collateral arteries. Doppler echocardiography allows you to register reverse blood flow through the vertebral artery, which confirms the diagnosis.

During surgery for congenital heart disease, the ductus arteriosus is closed to eliminate pulmonary steal. If cerebral symptoms or developmental delays in the left arm are present, surgical ligation or occlusion of the ductus botellus using catheter technology, as well as reimplantation of the subclavian artery into the aorta, may be required.

Cervical aortic arch

A cervical aortic arch is a rare anomaly in which the arch is located above the level of the clavicles. There are two types of cervical arch:

with abnormal subclavian artery and descending aorta opposite to the arch;

with virtually normal branching and a unilateral descending aorta.

The first type is characterized by a right aortic arch that descends on the right to the level of the T4 vertebra, where it crosses the esophagus posteriorly and goes to the left, giving rise to the left subclavian artery and sometimes the ductus arteriosus. This type, in turn, is divided into a subtype, in which the internal and external carotid arteries arise from the arch separately, and a subtype, in which there is a bicarotid trunk, when both common carotid arteries arise from one vessel, and both subclavian arteries arise separately from the distal arcs. In each of these subtypes, the vertebral arteries arise separately from the arch. While most patients with the contralateral descending aorta have a vascular ring formed by the aortic arch on the right, the retroesophageal segment of the aorta posteriorly, the ligamentum arteriosus on the left, and the pulmonary artery anteriorly, only half of them exhibit Clinical signs rings.

When the bicarotid trunk accompanies the descending aorta opposite the cervical arch, compression of the trachea or esophagus at the bifurcation between the bicarotid trunk and the retroesophageal aorta may occur without formation of a complete vascular ring.

The second type is characterized by a left-sided aortic arch. Narrowing caused by the aortic arch due to the long, tortuous, hypoplastic retroesophageal segment is rare.

In patients with both types of arch - with the opposite and unilateral descending arch - discrete coarctation of the aorta occurs. For unclear reasons, stenosis or atresia of the left subclavian artery ostium sometimes occurs in both types.

The cervical aortic arch manifests itself as a pulsating formation in the supraclavicular fossa or on the neck. In infants, before the appearance of pulsation, signs characteristic of a vascular ring are detected:

recurring respiratory infections.

Adults usually complain of dysphagia. In patients with stenosis or atresia of the left subclavian artery and the origin of a unilateral vertebral artery distal to the obstruction, blood may leak from the cerebral arterial system with neurological symptoms.

If there is a pulsating formation in the neck, the presumptive diagnosis can be made by the disappearance of the pulse in femoral artery with short-term pressing of a pulsating formation.

A cervical aortic arch should be differentiated from a carotid or subclavian aneurysm to avoid inadvertent ligation of an aortic arch mistaken for a carotid aneurysm. The diagnosis can be suspected on a plain radiograph by the widened superior mediastinum and the absence of a round shadow of the arch. Anterior displacement of the trachea supports the diagnosis.

Angiography has been the standard diagnostic modality in the past and will remain so in the presence of intracardiac abnormalities. However, without concomitant pathology, the diagnosis of the cervical aortic arch can be established using echocardiography, CT and MRI.

Surgical intervention is necessary for hypoplasia of the cervical arch, clinically manifested vascular ring or arch aneurysm. The nature of the operation depends on the specific complication. In case of a right-sided cervical arch and a tortuous, hypoplastic retroesophageal segment, a left-sided anastomosis is performed between the ascending and descending aorta or a tubular vascular prosthesis is implanted.

Persistent V aortic arch

Persistent V aortic arch was first described in humans by R. Van Praagh and S. Van Praagh in 1969 as a double-lumen aortic arch, in which both arches are on the same side of the trachea, as opposed to a double aortic arch, in which the arches are located on opposite sides of the trachea . Since the first publication, three types of this rare pathology have been identified:

double-lumen aortic arch with passable both lumens;

atresia or interruption of the upper arch with a passable lower arch, accompanied by the departure of all brachiocephalic vessels with a common mouth from the ascending aorta;

a systemic pulmonary junction located proximal to the first brachiocephalic artery.

A double-lumen aortic arch, in which the lower vessel is below the normal aortic arch, is the most common of the three types. This inferior arch extends from the innominate artery to the origin of the left subclavian artery proximal to the ductus arteriosus or ligament. It is often associated with CHD and is an incidental finding with no clinical significance. Atresia or interruption of the superior arch with a common truncus arteriosus giving rise to all four brachiocephalic arteries is sometimes accompanied by coarctation of the aorta, which is the reason for hospitalization.

A persistent V arch connecting to the pulmonary artery occurs only with pulmonary atresia. The rudiment of the V arch, as the first branch of the ascending aorta, is connected to the pulmonary trunk or one of its branches. In this subgroup, the persistent V arch can be located both on the side of the main aortic arch and on the opposite side. The main aortic arch is usually left-sided, with a right innominate artery, although a left-sided arch with a retroesophageal right subclavian artery and a right-sided aortic arch with a left innominate artery have been described.

Coarctation of the aorta occurs in all three subgroups, including in combination with pulmonary atresia.

The double-lumen arch was diagnosed at angiography and at autopsy as a channel located below the normal aorta. It can also be diagnosed with an MRI. Atresia or interruption of the superior arch is recognized by the presence of a common brachiocephalic trunk from which all four vessels of the arch arise, including the left subclavian artery. This feature of the origin of the brachiocephalic arteries is the main sign of a persistent V arch, since the rudiment of the atretic dorsal IV arch is not visualized. However, during surgery for coarctation of the aorta distal to the fifth arch, an obliterated strip can be found connecting the left subclavian artery to the descending aorta.

Without concomitant coarctation of the aorta, a double-lumen arch has no physiological significance.

With the V persistent arch, which has an anatomical connection with the pulmonary artery, EchoCG, angiography and MRI can detect a vessel arising from the ascending aorta proximal to the I brachiocephalic branch, which ends in the pulmonary artery. In one case histological examination found elements of the arterial duct tissue.

Anomalies of the aortic arch and brachiocephalic vessels in children

Anomalies of the aortic arch and brachiocephalic (brachycephalic) vessels occur in isolated form and in combination with congenital heart defects. Some anomalies are not clinically manifested and are variants of the norm, others, on the contrary, lead to compression of the trachea and esophagus, are characterized by a certain clinical picture, and therefore should be classified as pathological conditions.

Anomalies of the aortic arch are very diverse. Thus, in the classification proposed by J. Stewart et al. (1964), 25 variants were identified. This section will discuss the main, most common anomalies (Fig. 21).

Rice. 21. Types of anomalies of the aortic arch (diagram).

A - aberrant right subclavian artery with a left-sided aortic arch; b - right-sided aortic arch with aberrant left subclavian artery; c - right-sided aortic arch - mirror type; d - double aortic arch. BA - ascending aorta; DA - descending aorta; RA - right subclavian artery; PS - right carotid argeria; LS - left carotid artery; LA - left subcutaneous artery.

Aberrant right subclavian artery (a. Iusoria) - the origin of the right subclavian artery as the last trunk in the case of a left-sided aortic arch. In such cases, the artery is located retroesophageally; More often, the anomaly is asymptomatic, but can lead to transient dysphagia. On radiographs taken with contrast of the esophagus, in the anteroposterior projection at the Tm - Tiv level, a filling defect of a linear shape is determined, located obliquely from left to bottom to right to top. In the left anterior oblique and lateral projections at the same level, a depression is revealed on the dorsal wall of the esophagus (Fig. 22).

Aortography allows one to establish the origin of the right subclavian artery distal to all brachiocephalic vessels. This anomaly becomes important in infants with congenital heart defects when conducting intracardiac studies, including left ventricular catheterization. If it is performed using access through the left axillary artery, which is often used in practice, then a. Iusoria does not allow catheter placement into the ascending aorta and left ventricular catheterization and left ventriculography.

A right-sided aortic arch is an anomaly in which it extends over the right main bronchus; the thoracic aorta is located to the right of the spine. W. Shuford et al. (1970) distinguish three types of right-sided aortic arch depending on the location of the brachiocephalic vessels. With Type I, the left subclavian artery leaves the last trunk, i.e. there is a. Iusoria in right-sided aortic arch. In these cases, the artery often arises from the aortic diverticulum, and the patent ductus arteriosus or ligamentum arteriosus connects the left subclavian and left pulmonary arteries, forming a vascular ring.

Type II is characterized by mirror compared to normal

Rice. 22. X-ray in the left anterior oblique projection of a 3-year-old child. Aberrant right subclavian artery, A depression on the dorsal wall of the esophagus formed by an abnormally arising artery.

The location of the brachiocephalic vessels, when the first trunk leaves the innominate artery, dividing into the left carotid and left subclavian arteries. This type is the most common.

Type III - isolated left subclavian artery - differs from type I. that it does not communicate with the aorta and is supplied collaterally.

X-ray of the right-sided aortic arch is diagnosed in the anteroposterior projection by the deviation of the contrasted esophagus to the left at the level of the aortic arch (Fig. 23). If the contrasted esophagus in lateral and oblique projections is deviated anteriorly, this indicates the presence of an aberrant left subclavian artery. If the deviation is significant, it can be assumed that the aberrant left subclavian artery arises from the aortic diverticulum.

Angiography can usually determine the order of origin of the brachiocephalic vessels and, therefore, determine the type of anomaly. In type I, the left common carotid artery, which arises as the first trunk, is contrasted first, and lastly, the left subclavian artery, often arising from a diverticulum located at the junction of the aortic arch and its descending section. In the mirror type, the innominate artery is the first to be contrasted, dividing into the left common carotid and left subclavian arteries.

Rice. 23. X-ray in direct projection of a 12-year-old child. Tetralogy of Fallot. The right-sided aortic arch deviates the contrast-enhanced esophagus to the left.

Double aortic arch is a very rare anomaly. With it, the right and left aortic arches existing in the embryonic period are preserved, and the trachea and esophagus are located inside the vascular ring formed by them. This usually results in dysphagia and stridor. With this aiomaly, as a rule, the right brachiocephalic vessels arise from the right, and the left from the left aortic arch. Usually the right arch is better developed; The descending aorta can be located either to the right or to the left of the spine. Superexposed radiographs in the anteroposterior projection may reveal depressions on the lateral walls of the trachea immediately before its bifurcation. When contrasting the esophagus in this projection at the Tm - Tiv level, filling defects are usually visible. In the lateral projection, anterior bending of the esophagus or a filling defect on its dorsal wall is determined.

Diagnosis of a double aortic arch is difficult even with high-quality aortography. It is necessary to accurately establish the patency of both aortic arches, the order of origin of all brachiocephalic vessels, and, in the presence of a patent ductus arteriosus, its localization.

Anomalies and variants

The following types of aortic arch deformation are distinguished:I. By topographic-anatomical type

1) Right-sided aortic arch;

Right-sided aortic arch with left-sided descending aorta;

Right-sided aortic arch with right-sided descending aorta and aortic diverticulum;

2) Double aortic arch. II. By type of deformation: 1) lengthening (cervical aortic arch); 2) tortuosity (kinking) of the aorta; - loop and ring formation; - inflection;

3) Hypoplasia of the aortic arch: narrowed aorta (aorta angusta);

4) Absence of the aortic arch.

III. Variants of branching of the aorta.

1) The brachiocephalic trunk is absent;

2) Left brachiocephalic trunk, with the absence of the right one;

3) Right and left brachiocephalic trunk.

4) The right and left common carotid arteries arise from one trunk.

A right-sided aortic arch is an anomaly in which it extends over the right main bronchus; The thoracic aorta is located to the right of the spine.

The aortic arch turns to the right, and above the right main bronchus turns back behind the heart. Or it runs all the way along the right side of the spine and only at the level of the diaphragm passes to the left side, or at the higher thoracic segment it crosses the spine.

This developmental anomaly occurs in such a way that the artery of the left IV branchial arch, from which the aortic arch arises during normal development, atrophies, and instead the aortic arch is formed by the artery of the right IV branchial arch. The vessels extending from it originate in the reverse order compared to the norm. In approximately 25% of cases, this developmental anomaly is associated with Fallot's tetralogy. By itself, it does not affect blood circulation and does not cause clinical symptoms. The diagnosis is important from the point of view of surgery for combined developmental anomalies. In infancy, this developmental anomaly is more difficult to determine by X-ray examination, and in childhood easily. Using angiocardiography, the position of the aortic arch and descending aorta can be clearly identified.

Right-sided aortic arch with left-sided descending aorta.

The aortic arch is formed from the artery of the right IV branchial arch, but the Botallian duct or subclavian artery arising from the artery of the left VI branchial arch, arising from the descending aorta, in front of the spine between the esophagus and trachea, with a sharp bend, pulls the vessel to the left side. The aortic arch bends behind the esophagus to the left side, expands the median shadow and forms a deep depression behind the esophagus, clearly visible in both oblique positions.

Right-sided aortic arch with right-sided descending aorta and aortic diverticulum.

Along with the right-sided aortic arch and the descending aorta, a rudimentary left-sided aortic root is preserved, from which the subclavian artery arises. The diverticulum is located behind the esophagus and forms a deep depression on its posterior surface. If it extends beyond the esophagus, then on sagittal examination it appears in the form of a mediastinal shadow with a border convex to the right.

Double aortic arch in a child

What is a double aortic arch in a child?

Double aortic arch is one of the types of cardiac vascular defects. In a healthy heart, blood flows from the body into the right atrium and then into the right ventricle. Further, blood is flowing into the lungs through the pulmonary valve, where it is saturated with oxygen. Then the blood returns to the left atrium and enters the left ventricle, after which it is distributed throughout the body through the aorta.

If there is a double arch of the aorta, it branches into right and left parts. A bifurcation of the aorta forms a vascular ring and can cause compression of the airway and/or esophagus.

Causes of double aortic arch

A double aortic arch in a child is a congenital defect. This means that the abnormality develops while the baby is in the womb and the baby is born with the condition. It is not yet known exactly why some babies develop abnormalities in heart development.

Risk factors for double aortic arch

Risk factors influencing the appearance of a double aortic arch are still unknown.

Symptoms of a double aortic arch in a child

Symptoms of a double aortic arch may include:

Labored breathing;
Lung infections;
Poor appetite, including attacks of vomiting and choking;
Problems with swallowing, including attacks of choking;
Vomit;
Heartburn.

Diagnosis of double aortic arch

Most often, this disease is discovered in infancy; it is often found later.

The doctor will ask about your child's symptoms and medical history and perform a physical examination. It may be necessary to take pictures and examine the structure of internal organs. For these purposes the following are used:

An ECG is prescribed to study the functioning of the heart.

Treatment of double aortic arch in a child

Treatment options for double aortic arch include:

Surgery to treat double aortic arch

If the child has symptoms that negatively affect their health, such as difficulty breathing, surgery will be performed. The purpose of the operation is to close or separate one of the arches. After this, go straight away and after a while there should be an improvement.

Monitoring the child's condition

Your child should have regular checkups with a cardiologist, a specialist in heart disease.

Prevention of the appearance of a double aortic arch in a child

There are currently no methods to prevent the occurrence of a double aortic arch in a child. Despite this, it is very important to receive appropriate prenatal care.

Right-sided aortic arch?

Is it possible to write here the right aortic arch?

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In the first image it even seemed to me (or not) that the aorta was deviating the trachea to the left.

Probably possible. It’s better to wait for our experts. What if we give barium and look at the position of the esophagus?

Next time I'll definitely give it to you. And it’s already late, they left (medical examination at the clinic). In the first case, I wrote “More data for the rightness of the aortic arch.” In the second: “More data for the rightness of the aortic arch; CT-OGP is recommended to exclude an aortic aneurysm.” Before this, they wrote the norm. And I read about contrasting the esophagus only after that.

A very interesting “community”.

Anything is possible, but I think blood vessels. Yes, and the styling has changed compared to 2008.

Anything is possible, but I think blood vessels.

Is this a certainty or an assumption?

There is never any certainty in our business.

What do you think about the right-sided arch?

Isn’t it about the “arc” marked with an arrow?

Yes, with a rim of sclerosis (sclerosis of the aortic arch). At first I thought it was the sternum, but it stuck out too much on the sternal end of the clavicle.

I also wrote an aneurysm under?, because along the left contour there is still a noticeable shadow similar to the aortic arch, and on the right there is a possible aneurysm. Or is it all a fantasy?

But in the first case, I didn’t find any arc on the left at all. And the trachea at the level of the “arch?” on the right is deviated to the left

In this not so rare variant of development (one case per 2000 people), local expansion of the mediastinal shadow is detected along the right contour of the median shadow at the level of the aortic arch, which often causes diagnostic difficulties.

diseases of the respiratory system and mediastinum",

Right aortic arch

The right-sided aortic arch can be combined with anomalies of other large vessels and the heart, as well as organs of other systems. This anomaly in adults may be asymptomatic, and in some cases the symptoms may be quite pronounced. The most characteristic and dominant symptom in the clinic is dysphagia, which usually appears at the age of 40-60 years, which is explained, as with other vascular anomalies, by age-related changes in the aortic arch and esophagus. Dysphagia is unstable, more often manifested when swallowing solid food, aggravated by physical and nervous stress, sometimes combined with pain behind the sternum and in the epigastrium.

S. A. Reinberg et al. rightly noted that an elementary x-ray examination is the main method for recognizing this anomaly. Already with a survey X-ray examination of the chest, the absence of the aortic arch on the left, a typical turn of the arch, the aortic window in the right lateral and right oblique positions, and the pulsation of the aortic arch on the right are revealed.

The pathognomonic sign of the right-sided aortic arch is the displacement of the contrasted esophagus at the level of the arch anteriorly and more often to the left. At the same time, in a direct projection, a filling defect of a semicircular shape is determined along the right contour of the esophagus at the level of the aortic arch, and in an oblique view, along the posterior one.

X-ray of the esophagus with a right-lying aortic arch in a direct projection. A filling defect of a semicircular shape along the right contour of the esophagus at the level of the aortic arch.

A rare anomaly is a double aorta that exerts pressure on the right esophageal contour or causes it to become circumferentially narrowed. different levels according to the location of the aortic arches. In this case, a circular narrowing of the trachea is also noted.

X-ray of the esophagus with a right-lying aortic arch in the lateral projection. A filling defect of a semicircular shape along the posterior contour of the esophagus at the level of the aortic arch.

All changes in the esophagus, caused by abnormally located aorta and large vessels, are of great practical importance in the differential diagnosis of diseases of the esophagus.

“X-ray diagnosis of gastrointestinal diseases”, V.B. Antonovich

X-ray picture in various diseases and lesions of the esophagus, accompanied by dysphagia

Rice. G). X-ray picture at various diseases and lesions of the esophagus, accompanied by dysphagia: displacement of the esophagus (indicated by the arrow) with the right-lying aortic arch.

Chapter 22 DOUBLE AORTIC ARCH

The posterior right arch crosses over the right pulmonary artery and the right bronchus, and the anterior right arch crosses the left pulmonary artery and the left bronchus. Both arches join behind the esophagus with the upper part of the descending aorta, which descends further to the left or right of the spine. In this case, the common carotid and subclavian arteries depart from each arch. The posterior right arch is wider and is located slightly higher than the anterior left one. Most often, both arcs are not obliterated, atresia of one of them (usually anterior) is less common. The ductus arteriosus can be located on both sides, but, as a rule, occurs on the left.

Other variants of abnormal formation of vascular rings are also possible:

with a right-lying aortic arch and a left-sided PDA or ligament arteriosus. The trachea and esophagus are also enclosed in a ring, where the right and posterior contours of the ring are formed by the right-sided aortic arch, the anterior contour is formed by the bifurcation of the pulmonary artery, and the left contour is formed by the arterial duct connecting the lower surface of the aortic arch with the left branch of the pulmonary artery;

the same combination, but with the presence of an abnormal origin from the right aortic arch of the left brachiocephalic trunk, which goes from right to left in front of the trachea and esophagus, forming the anterior contour of the ring, and its right-posterior and left contours are formed, respectively, by the right aorta and PDA or arterial ligament (Burakovsky V.I. et al., 1996).

Anomalies in the development of the aortic arch are associated with disturbances in the process of embryogenesis. The fetus initially has two aorta - ventral and dorsal, connected by 8 pairs of vascular arches. With the final formation of the vessels, the aortic arch, trunk and pulmonary arteries are formed, the remaining arches regress, atrophy and disappear. Violation of the process of normal regression of the remaining arches, probably, becomes the cause of the formation of abnormal arches and branches of the aorta (Bankl G., 1980;

Anomalies of the aortic arch and its branches are, according to clinical data,

7-1%, and according to sectional studies - 3-3.8% of all congenital heart and vascular defects (Nosla8 A., Ru1er O., 1972; Bankle G., 1980). In 20% of cases, this anomaly is combined with other congenital heart defects, most often with TF, VSD, ASD, CoA, TMS, EZhS. The right-sided aortic arch is usually combined with other congenital heart defects. The true incidence of aortic arch anomalies is difficult to determine, since this defect, without accompanying congenital heart disease, occurs without hemodynamic disturbances, asymptomatically, and in cases of clinical symptoms, children are observed and treated for a long time in general pediatric departments, often as patients with respiratory or gastroenterological pathologies.

Symptoms of compression of the trachea and esophagus are more often observed with a double aortic arch (37% of cases), less often with a right-lying aortic arch and left-sided ductus arteriosus (26% of cases), or various types of vascular anomalies of the aortic arch (37% of cases) (Ogozz K. 1964 ). TniMa et al. (1986) when examining children of the first year of life with stridor, a vascular ring was detected in 35% of cases.

Clinical picture. Clinical manifestations of the defect depend on the degree of compression of the esophagus and (or) trachea. Compression of the esophagus manifests itself already in the first months of life, difficulties with feeding, frequent regurgitation and vomiting, poor weight gain and developmental delays, which are usually regarded as signs of pyloric spasm or pyloric stenosis. After 1 year of age, difficulty swallowing solid food and the child’s desire to swallow it slowly or with liquid are observed. Regurgitation and vomiting are observed with large meals. Older children may complain of a dull pain in the chest when breathing deeply or swallowing.

Compression by the vascular ring of the trachea is accompanied in infants by stridorous, noisy, “snoring” breathing. Unlike stridor caused by laryngomalacia, birth trauma, previous intubation or other reasons and usually significantly decreasing or disappearing after 3-12 months, stridor breathing with a vascular ring progresses with age, accompanied by difficulty breathing and shortness of breath. Sometimes attacks of stridor breathing and shortness of breath are syncopal in nature, accompanied by coughing, suffocation, apnea and even cyanosis. Such attacks can occur during physical exertion, psycho-emotional arousal, or heavy food intake. Some relief is caused by the patient accepting a forced position with his head thrown back, in which the patency of the trachea improves and breathing becomes freer (\Vetberg R.M., 1995).

Children are prone to recurrent bronchitis and bronchopneumonia, occurring with minor acute inflammatory manifestations and an abundance of physical findings. In addition to compression of the trachea, frequent aspiration of food appears to contribute to the recurrence of respiratory diseases. In some cases, respiratory failure increases significantly and becomes permanent. Children are usually treated in general somatic or pulmonology hospitals as patients with respiratory allergosis, recurrent bronchitis and even COPD.

If there are no concomitant congenital heart defects, then during physical examination of cardio-vascular system no pathology is detected. Pulse and arterial pressure not changed, the boundaries of the heart are within the age norm, heart sounds are clear, no murmurs are heard. On auscultation, dry and moist coarse and medium bubble rales can be heard in the lungs.

Electrocardiogram and phonocardiogram - without pronounced signs of pathology.

Radiography. In direct projection pathological changes No. In the lateral projection, as the contrast agent passes through the esophagus, depressions caused by an external pulsating formation are revealed on its anterior and posterior contours. In addition, in the lateral projection, narrowing of the trachea at the level of the aortic arch can be detected.

With direct bronchoscopy, it is possible to determine the degree of narrowing of the tracheal lumen and its pulsation in the area of the suprabifurcation segment.

Two-dimensional echocardiography and aortography make it possible to definitively diagnose the presence of a double aortic arch, as well as an anomaly of the bracheocephalic vessels.

Differential diagnosis is carried out with a tumor of the mediastinum, tracheoesophageal fistula, anomaly of the tracheobronchial tree.

Natural history and prognosis. In the absence of clinical symptoms of compression of the trachea and esophagus, the anomaly of the aortic arch and its branches does not manifest itself and may become an accidental finding. In children with symptoms of compression (depending on its degree), the clinical manifestations of the anomaly occur early, progressively increase and sometimes underlie developmental delay, dystrophy, persistent dysphagia, retrosternal pain, neurosis, recurrent bronchitis and bronchopneumonia, bronchiectasis and other COPD, and life-threatening respiratory failure. Therefore, all children with progressive stridor and dysphagia that do not respond to therapy, recurrent respiratory diseases, it is necessary to conduct a targeted examination to identify anomalies of the aortic arch and its branches. In the presence of concomitant congenital heart disease clinical picture The disease is determined by the specifics of these defects.

Patient Denis P., 11 years old, was in the pulmonology department of Children's hospital No. 19 in January 2003.

Main diagnosis: connective tissue dysplasia syndrome:

congenital heart disease, double aortic arch; mitral valve prolapse of the 1st degree with regurgitation of the 1st degree, regurgitation on the pulmonary valve of the 1st degree. False chord of the left ventricle of the heart, heart failure I FC;

anomaly of the large intestine (incomplete intestinal rotation, megadol ihosi gm a).

Complications: compression stenosis of the middle third of the trachea II degree. Secondary recurrent bronchitis. Chronic constipation.

Concomitant diagnosis: residual encephalopathy. Logoneurosis. Daytime enuresis

The child was admitted for a bronchological examination due to recurrent obstructive bronchitis, noisy breathing during exercise and while eating.

Anamnesis. Perinatal history without features. In the first months after birth, manifestations of connective tissue dysplasia such as umbilical hernia and dysplasia were discovered hip joints. The cry from birth was hoarse, breathing physical activity and was noisy during meals, which was associated with congenital stridor. From the second half of life, the child suffers from prolonged recurrent bronchitis, which occurs with an obstructive component or stridor breathing, respiratory failure,

degree II, which even necessitated hospitalization in the intensive care unit for intubation and sanitation of the tracheobronchial tree. After 5-7 years, he began to get sick less often (4-5 times a year), suffered from whooping cough, which was severe. Parents categorically refused bronchoscopic examination. From 8 months. The child has increased constipation with stool retention for up to 7-14 days. He was examined gastroenterologically, on the basis of which chronic colitis and dolichosigma were diagnosed.

Heredity. On the mother's side there are cases of stomach, lung, and breast cancer; the grandmother's brother and sisters have leukemia, the mother herself has dolichosigma, chronic constipation for up to 7-10 days. On the paternal side - the grandmother has pulmonary tuberculosis, the father has tuberculosis. peptic ulcer stomach, logoneurosis Physical examination. State of moderate severity Satisfactory nutrition The chest is not deformed, but the vascular network on the chest is pronounced Phenotypic signs of connective tissue dysplasia are expressed: flaccid posture, muscle hypoplasia, flat feet, joint hypermobility, “sandal-shaped” gap between the first and second toes, long toes Pulse is symmetrical, satisfactory filling in the arms and legs, blood pressure in the arms 100/60 mm Hg, in the legs 115/80 mm Hg Apex impulse of normal strength, heart boundaries are normal Distinct, rhythmic tones A 3rd degree systolic murmur is heard along the left edge of the sternum intensity, soft, occupying '/3-'/ systole, weakening in a standing position Tympanitis is pronounced over the lungs percussion, breathing is hard (after exercise - noisy), but there is no wheezing. The abdomen is swollen, painless, the liver is not enlarged. Stool after 2-3 days, only after an enema and laxatives Enough urination, no edema

In clinical and biochemical analyzes blood and urine pathology was not detected

Electrocardiography. Sinus tachycardia, heart rate 97 beats/min Normogram Slowing of intra-atrial conduction Phenomenon of ventricular pre-excitation Disturbance of metabolic processes in the ventricular myocardium (Fig. 22 2) Echocardiography. The heart is formed correctly, the chambers are of normal size, the septa are intact, there is no myocardial hypertrophy. Systolic deflection of the anterior leaflet of the mitral valve of the 1st degree, regurgitation on the mitral and pulmonary valves of the 1st degree. Additional chord in the cavity of the left ventricle. Systolic and diastolic functions of the myocardium are normal. EF 68%, FU 37 % Aortic diameter 22 mm, opening 22 mm, pulmonary artery diameter 22 mm Suspicion of a right aortic arch

Chest X-ray. The pulmonary fields are swollen The chest is barrel-shaped The pulmonary pattern in the medial sections is strengthened, unclear, in the lateral sections it is depleted The roots are non-structural, probably due to hyperplasia of the lymph nodes The diaphragm is clear, the sinuses are free The heart is not expanded in diameter The arch of the aorta and its descending section are not differentiated (Fig. 22 3)

Computed tomography of the chest. A study with intravenous contrast revealed an anomaly thoracic of the aorta in the area of the arch, where the aorta forms a ring that compresses the trachea from the sides. Lymph nodes are not enlarged (Fig. 22 4)

Fiberglass bronchoscopy. The size of the trachea corresponds to age. In the middle third of the trachea, along the 12-14th cartilaginous rings, a decrease is determined

Irrigography. With the introduction of two portions of a large amount of liquid barium suspension (about 2 liters), it was possible to partially fill the large intestine, up to the transverse section. All loops of the colon are located in the left half abdominal cavity, sharply expanded, but with preserved haustration. The ampulla of the rectum is sharply dilated. The cecum and ascending colon are located normally. Conclusion: intestinal malformation - incomplete rotation, megadolichosigma.

Diagnosis: double aortic arch. The chest is barrel-shaped, the lung fields are swollen. The pulmonary pattern in the medial sections is enhanced and unclear, in the lateral sections it is depleted. The roots are unstructured, the diaphragm is clear, the sinuses are free. The heart is not expanded in diameter, but the aorta and its descending section are not contoured.

Diagnosis: double aortic arch. A study with intravenous contrast reveals an anomaly of the thoracic aorta in the area of the arch, where the aorta forms a ring that compresses the trachea from the sides. Lymph nodes are not enlarged.

The peculiarity of the case is the presence in a child with extremely unfavorable heredity of multiple defects and developmental anomalies associated with generalized dysplasia connective tissue. However, the defect that determined the severity of the condition was a double aortic arch, complicated by severe compression tracheal stenosis, recurrent tracheobronchitis and respiratory failure, requiring further examination in a specialized surgical hospital and urgent correction of the defect.

Thus, in the EG, at the end of the experiment, positive dynamics of indicators of static and general endurance, flexibility were revealed, and the duration of exercises for the strength of the extensor muscles of the spine, rectus and oblique abdominal muscles increased.

CONCLUSION

Conducting training sessions using the developed methodology made it possible to identify positive dynamics in almost all studied indicators of the physical condition of female students in the experimental group within one year.

The results of the experiment confirm the need to introduce into the educational process in physical education with students of a special medical group the developed author’s methodology, based on the use of therapeutic physical culture, traditional and auxiliary non-traditional health remedies and methods.

LITERATURE

1. Ilyinich V.I. Physical culture and student life. - M.: Gardariki, 2008. - 366 p.

2. Kochukova I.V. Clinical polymorphism of vegetative syndromes in young people // Journal of Theoretical and Practical Medicine. - 2008. - No. 2. - P. 143-147.

3. Kuramshin Yu. F. Physical culture and healthy image life of student youth. - M.: Soviet sport, 2010. - 464 p.

4. Sapozhnikova Yu. I., Karpov V. Yu. Design of the content and methodology of educational classes in physical education of a correctional orientation with students of special medical groups // Uchen. zap. University named after P. F. Lesgaft. - 2008. - No. 8. - P. 41-44.

Contact Information

Elena Valentinovna Pivovarova - senior lecturer of the Department of Physical Culture and Health, Volgograd State University medical University, e-mail: [email protected]

PRENATAL ULTRASONIC DIAGNOSIS OF DOUBLE AORTIC ARCH

N. A. Altynnik, Yu. V. Shatokha

Institute for Advanced Studies of the Federal Medical and Biological Agency of the Russian Federation, Department of Ultrasound and Prenatal Diagnostics, Moscow

An analysis of six cases of prenatal diagnosis of double aortic arch (DAA), diagnosed at 20-33 weeks of pregnancy, was carried out. In fetuses with DDA, ultrasound examination revealed a characteristic vascular ring formed by the left and right aortic arches when examining a section through three vessels and the trachea. In this case, the trachea was located between these vessels. In four cases, the diagnosis of DDA was made in the second trimester of pregnancy. No heart changes were noted in all fetuses; in one case, a combined defect was recorded - a single umbilical cord artery. Only in two cases (33.3%) was an abnormal location of the cross section of the thoracic aorta and in one observation (16.7%) an abnormal location of the cardiac axis. Thus, DDA can be diagnosed prenatally using a three-vessel and tracheal section during screening ultrasound in the second half of pregnancy.

Key words: fetus, congenital heart defects, double aortic arch, prenatal diagnosis.

PRENATAL ULTRASOUND DIAGNOSIS OF DOUBLE AORTIC ARCH

N. A. Altynnik, Y. V Shatokha

And double aortic arch (DAA) was identified in 6 fetuses at 20-33 weeks of gestation. All fetuses with DAA during ultrasound examination revealed a vascular ring across three vessels and trachea. The trachea was located between the vessels. In 4 cases the diagnosis of DAA was made in the second trimester of gestation. All fetuses had normal intracardiac anatomy and 1 was associated with single umbilical artery. In 2 (33.3%) cases the abnormal location of cross section of the thoracic aorta was revealed and anomalous values of the axis of the heart were reported only in 1 (16.7%) of the 6 fetuses. Fetal DAA can be prenatally diagnosed during the second screening examination using the three-vessel and trachea view.

Key words: fetus, congenital heart defects, double aortic arch, prenatal diagnosis.

Double aortic arch (DAA) is a rare aortic arch anomaly in which the ascending aorta becomes two arches, a right and a left one. In this case, the right aortic arch is located to the right of the trachea and esophagus and spreads through the right pulmonary artery and the right main bronchus. Left aortic arch, dis-

placed to the left of the esophagus and trachea, makes a similar path, passing through the left pulmonary artery and left bronchus. The right aortic arch is usually wider and located higher than the left one. The vascular ring, often of small diameter, decreases with age and causes compression of the trachea immediately after birth.

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Behind the esophagus, both arches merge to form the descending thoracic aorta, which can be located either to the right or to the left of the spine.

Brachiocephalic vessels in DDA arise from four separate trunks: the right common carotid and subclavian arteries - from the right arch, the left ones - from the left. Other options for the origin of brachiocephalic vessels in DDA are also possible.

With DDA, an anatomically complete vascular ring is formed—the trachea and the esophagus are surrounded by vascular structures on all sides. DDA usually exists as an isolated anomaly, but can be associated with tetralogy of Fallot, right descending aorta, atrial and ventricular septal defects, coarctation of the aorta, and transposition of the great vessels. The main clinical manifestations of DDA are: shortness of breath, wheezing, cough, asthma attacks, recurrent bronchopneumonia, regurgitation and vomiting, dysphagia and weight loss. The severity of the children's condition is aggravated by the frequent addition of respiratory infections and bronchopneumonia. Therefore, diagnosis of DDA should be as early as possible and preferably in the prenatal period.

Publications dedicated to prenatal ultrasound diagnostics DDA, mainly began to appear only in last years thanks to the introduction of assessment of the section through three vessels and the trachea into the screening ultrasound protocol in the second trimester of pregnancy. However, most published works describe 1 to 3 cases of prenatal diagnosis of DDA. In our country, the first observation of prenatal ultrasound diagnosis of DDA was published by M. V. Medvedev in 2006. Therefore, it is relevant to analyze new cases of prenatal diagnosis of DDA based on more factual material.

GOAL OF THE WORK

To study the echographic manifestations of DDA during screening ultrasound examination in the second and third trimesters of pregnancy.

RESEARCH METHODOLOGY

We analyzed 6 cases of prenatal ultrasound diagnosis of DDA at 20-33 weeks of pregnancy. The age of the pregnant patients varied from 22 to 32 years, there were three primiparous patients, and three multiparous patients. The weight of newborns varies

ranged from 3100 to 3400 g. The gender ratio (F: M) was 1:1.

For each ultrasound examination, a four-chamber section of the fetal heart was assessed during a transverse scan of its chest and a section through three vessels and the trachea, also obtained through a transverse scan at the level of the ventricular outflow tracts.

The study of a four-chamber section of the fetal heart and the assessment of a section through three vessels and the trachea were carried out according to the scheme proposed by M. V. Medvedev.

The location of the axis of the fetal heart was carried out in relation to the sagittal plane when studying a four-chamber section of the heart. The standard values of the angle between the axis of the fetal heart passing through the interventricular septum and the sagittal direction in the second half of pregnancy were considered to be from 30 to 60° (on average 45°). The location of the fetal thoracic descending aorta was assessed by studying a four-chamber section of the heart. Normally, the cross-section of the fetal thoracic aorta is located to the left of the sagittal plane.

RESULTS OF THE STUDY

AND THEIR DISCUSSION

In the course of our studies, we found that in the majority of fetuses with DDA (5/6, 83.3%), an ultrasound examination in a section through three vessels revealed the presence of a characteristic vascular ring formed by the left and right aortic arches. In this case, the trachea was located between these vessels. This was most clearly recorded when using the color Doppler mapping mode.

In only one case, the DDA was characterized by the presence of a parallel course of arcs, between which the trachea was visualized, which initially caused difficulties in accurately identifying the DDA when using traditional gray scale echography. Therefore, in this case, volumetric echography based on STIC technology was additionally used, which made it possible to establish the final diagnosis of DDA.

Analysis of prenatally diagnosed cases of DDA showed that in five (83.3°%) of six cases, the right aortic arch was dominant and had a larger diameter compared to the left aortic arch. In these cases, the brachiocephalic vessels originated from the right dominant aortic arch. In only one fetus, the aortic arches were of equal size, and two brachiocephalic vessels each branched off from them (Table).

Summary data of the results of prenatal ultrasound examination of fetuses with DDA

No. Age, years Diagnostic period, weeks Dominant aortic arch Location of the heart axis (angle), degrees Location of the thoracic aorta

1 K., 33 24 right 45 left

2 L., 25 33 right 45 left

3 M., 28 32 right 42 left

4 B., 30 20/4 right 95 center

5 S., 28 24 right 48 center

6 K., 22 23/4 equal arcs 40 on the left

Issue 4 (52). 2014

Thus, the main prenatal diagnostic criterion DDA during ultrasound examination of the fetus in our studies was a characteristic vascular ring formed by the left and right aortic arches when studying a section through three vessels and the trachea.

In four cases (66.7%), the diagnosis of DDA was established in the second trimester of pregnancy and in two cases (33.3%) - after 26 weeks of pregnancy. It should be noted that the prenatal diagnosis of DDA before 20 weeks of pregnancy was not established in any observation. The period of detection of DDA in the fetus in our study averaged 26.1 weeks of pregnancy. Apparently, this can be explained by the still insufficient experience of domestic specialists in prenatal ultrasound diagnosis of DDA in the fetus. Although, in general, the prenatal ultrasound diagnosis of DDA was established before 25 weeks of pregnancy in 66.7% of cases, of which before 22 weeks of pregnancy - in 16.7% of cases.

It should be noted that in two cases (33.3%) of establishing a prenatal echographic diagnosis of DDA in the fetus during a screening ultrasound examination in the third trimester of pregnancy (32-33 weeks), this anomaly was not correctly identified during a screening ultrasound examination of the fetus in the second trimester (18 -21 weeks) of pregnancy.

When studying the location of the thoracic descending aorta and the cardiac axis when studying a four-chamber section of the fetal heart, which, according to the results of previous studies, often change with conotruncal anomalies, including DDA, the following results were obtained.

When assessing the location of the thoracic descending aorta at the level of the four-chamber section of the fetal heart, it was found that the normal location of the thoracic aorta (to the left of the sagittal scanning plane) when assessing the four-chamber section of the heart in cases of its double arch was registered in four (66.7%) out of six fruits Only in two cases (33.3%) was an abnormal (central) location of the cross section of the thoracic aorta identified.

The location of the heart axis in cases of DDA, when assessing a four-chamber section of the heart, varied in a fairly wide range - from 40 to 95°, but at the same time, abnormal values of the location of the heart axis were recorded in only one (16.7%) of six fetuses.

Isolated DDA was noted in five (83.3%) of six cases. In only one case, a single umbilical cord artery was additionally recorded.

CONCLUSION

Thus, our research and published results convincingly demonstrate

Rumor has it that screening ultrasonography in the second and third trimesters of pregnancy, studying a section through three vessels and the trachea is highly informative for identifying fetuses with DDA. Evaluation of a four-chamber section of the heart is not effective for identifying fetuses with DDA, since the abnormal location of the cross section of the thoracic descending aorta and the abnormal position of the cardiac axis were recorded by us only in 33.3 and 16.7% of cases, respectively.

LITERATURE

1. Bockeria L. A., Arakelyan V. S., Gidaspov N. A. Congenital anomalies of the aortic arch. Diagnostics, treatment tactics // Thoracic and cardiovascular surgery. - 2012. - No. 4. - P. 14-19.

2. Burakovsky V.I., Bukharin V.A., Podzolkov V.P. et al. Congenital heart defects // Cardiovascular surgery / Ed. V. I. Burakovsky, L. A. Bockeria. - M.: Medicine, 1996. - 768 p.

3. Medvedev M. V. Prenatal echography. Differential diagnosis and prognosis. - M.: Real Time, 2012. - 3rd ed. - pp. 245-247.

4. Medvedev M. V. Prenatal echocardiography: what are we doing and what should we do? // Prenat. diag. - 2006. - T. 5 (1). - pp. 14-17.

5. Sharykin A. S. Congenital heart defects. Guide for pediatricians, cardiologists, neonatologists. - M.: BINOM, 2009.

6. Achiron R, Simand S., Hegesh J, Lipitz S., Zalel Y, Rotstein Z. Fetal aortic arch measurements between 14 and 38 weeks" gestation: in-utero ultrasonographic study // Ultrasound Obstet. Gynecol. - 2000. - Vol. 15. - P 226-230.

7. Berg C, BenderF, Soukup M., GeipelA, Axt-FliednerR., Breuer J., Herberg U., Gembruch U. Right aortic arch detected in fetal life // Ultrasound Obstet. Gynecol. - 2006. - Vol. 28. - P 882-889.

8. Budziszewska P., Kuka D., Sodowski M., Sodowski K., Jeanty P., Skrzypulec V. Double aortic arch: prenatal case report // Ginekol. Paul - 2009. - Vol. 80 (9). - P 712-715.

9. Galindo A., Nieto O., Nieto M. T., Rodriguez-Martin M.

O., Herraiz I., Escribano D., Granados M. A. Prenatal diagnosis of right aortic arch: associated findings, pregnancy outcome, and clinical significance of vascular rings // Prenat. Diagn. - 2009. - Vol. 29(10). - P 975-981.

10. Miranda J. O., Callaghan N., Miller O., Simpson J., Sharland G. Right aortic arch diagnosed antenatally: associations and outcome in 98 fetuses // Heart. - 2014. - Vol. 100(1). - P 54-59.

11. Seo H. K., Je H. G., Kang I. S., Lim K. A. Prenatal double aortic arch presenting with a right aortic arch and an anomalous artery arising from the ascending aorta // Int. J. Cardiovasc. Imaging. - 2010. - Vol. 26. - P 165-168.

Contact Information

Altynnik Natalya Anatolyevna - Doctor of Medical Sciences,

Professor of the Department of Ultrasound and Prenatal Diagnostics, Institute for Advanced Studies of the Federal Medical and Biological Agency of the Russian Federation, Moscow, e-mail: [email protected]

There is a slight narrowing in front of the arch called the isthmus of the aorta. This isthmus is located between the aortic arch and the descending aorta.

The aortic arch is directed from the second cartilage of the rib to the left 3-4 thoracic vertebrae. In some cases, the branches of the aortic arch reach the brachiocephalic trunk and the right carotid artery, and there may also be development options in which the branches of the aortic arch and two brachiocephalic trunks - right and left - are connected.

The aortic arch connects with three large vessels - the common carotid artery, the subclavian artery and the brachiocephalic trunk. The largest vessel, 4 cm long, is the brachiocephalic trunk. It arises from the aortic arch upward at the level of the sternoclavicular joint and divides into two branches - the right carotid artery and the right subclavian artery. With certain features of anatomical development in humans, the inferior thyroid artery may branch off from the brachiocephalic vessel.

Congenital deformity of the aortic arch

In some cases of abnormal development of the aortic arch, its congenital tortuosity, which is called deformity, may appear. This developmental anomaly occurs in 0.4-0.6% of patients with cardiovascular diseases and 3% of patients with coarctation of the aorta.

Congenital tortuosity of the aortic arch is expressed in its lengthening, bending and pathological thinning of the walls of blood vessels. In some cases, the aortic arch is thickened and has symptoms of stenosis (narrowing).

The causes of the deformity have not been identified, but medical studies have shown that this anomaly begins during intrauterine development under the influence of a number of factors and hereditary predisposition.

There are two main types of deformation:

Congenital deformity with lengthening and bending of the artery;
A congenital deformity in which the aortic arch is compacted and the walls of the vessels are narrowed.

In the future, the deformation can develop into several defects:

A defect with an inflection between the carotid and innominate arteries;
Defect with an inflection of the left carotid and subclavian arteries;
Deformity with a kink of the aorta at the branching of the subclavian artery.

In the overwhelming majority of cases, when the aortic arch is deformed, no circulatory disturbances are observed, but the load on the vessel walls increases and an aortic aneurysm can form.

With severe deformation of the aortic arch, compression of the esophagus, trachea and nerve trunks may occur. To treat deformation of the aortic arch, a special course of medication is prescribed followed by surgical intervention.

Aortic arch diseases

The main diseases of the aortic arch are aneurysm and hypoplasia.

An aortic arch aneurysm is an atherosclerotic or traumatic vascular lesion. Symptoms of an aortic arch aneurysm include damage to the blood vessels of the heart and brain, headache, pain syndrome in the chest, shortness of breath, strong pulsation in the chest, paresis of the recurrent nerve.

To diagnose an aneurysm, X-ray examination and aortography are performed, which allows one to determine the degree of change in the aortic wall. Aneurysm of the aortic arch is treated with surgical replacement of the aorta and its branches.

Hypoplasia of the aortic arch is a hypotrophy of the middle elements of the aortic vessels and degenerative changes in the plastic membrane, which lead to a break in the aortic isthmus.

The causes of hypoplasia of the aortic arch are hormonal disorders, congenital pathologies, hereditary predisposition, and innervation disorders. Hypoplasia of the aortic arch can affect other arteries, including the functioning of the renal artery.

For serious disorders caused by aortic hypoplasia, surgery is performed. After the operation, partial correction of heart defects and other cardiovascular diseases is carried out. Then drug treatment with glycosides and diuretics is prescribed.

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74-year-old Australian resident James Harrison has donated blood about 1,000 times. Him rare group blood, whose antibodies help newborns with severe anemia survive. Thus, the Australian saved about two million children.

Dentists appeared relatively recently. Back in the 19th century, pulling out diseased teeth was the responsibility of an ordinary hairdresser.

When lovers kiss, each of them loses 6.4 calories per minute, but at the same time they exchange almost 300 types of different bacteria.

Human bones are four times stronger than concrete.

Even if a person's heart does not beat, he can still live for a long period of time, as the Norwegian fisherman Jan Revsdal demonstrated to us. His “engine” stopped for 4 hours after a fisherman got lost and fell asleep in the snow.

A job that a person doesn’t like is much more harmful to his psyche than no job at all.

The human brain weighs about 2% of the total body weight, but it consumes about 20% of the oxygen entering the blood. This fact makes the human brain extremely susceptible to damage caused by a lack of oxygen.

The average life expectancy of left-handers is shorter than that of right-handers.

In 5% of patients, the antidepressant Clomipramine causes orgasm.

The first vibrator was invented in the 19th century. It was powered by a steam engine and was intended to treat female hysteria.

The liver is the heaviest organ in our body. Its average weight is 1.5 kg.

The most heat The body was recorded in Willie Jones (USA), who was admitted to the hospital with a temperature of 46.5°C.

If your liver stopped working, death would occur within a day.

Human blood “runs” through the vessels under enormous pressure and, if their integrity is violated, it can shoot at a distance of up to 10 meters.

Most women are able to derive more pleasure from contemplating their beautiful body in the mirror than from sex. So, women, strive to be slim.

This issue worries many men: after all, according to statistics in economically developed countries, chronic inflammation of the prostate gland occurs in 80–90% of men.

Anatomy and topography of the aortic arch: norm, pathology

The aortic arch is the middle part of the largest blood vessel in the human body.

Almost all organs and systems depend on its normal functioning.

In case of pathology of this blood vessel, serious therapeutic measures are often required.

About anatomy and topography

The aorta is the main trunk of arteries in the systemic circulation. It originates in the cavity of the left ventricle of the heart. Consists of 3 parts:

The aortic arch is the middle part. It is a derivative of the 4th left arterial arch. Topographically located between the manubrium of the sternum and the IV thoracic vertebra. The arc moves backward and to the left. Then it spreads over the apex of the left bronchus, where the descending part of the aorta begins.

Conventionally, the structure consists of 2 parts:

From the concave side of the aortic arch, blood vessels depart from the bronchi and thymus gland. 3 trunks originate from the convex part, located from right to left:

Brachiocephalic (brachiocephalic).
General carotid (carotid) left.
Left subclavian.

The branches of the aortic arch extend upward from its middle part. All of these arteries supply the upper half of the body, including the brain.

Anomalies, defects and diseases

Blood vessel pathology can be divided into 2 large groups:

In the first case, disturbances occur at the stage of embryogenesis. This depends on hereditary predisposition and the action of aggressive factors in the early stages of pregnancy. Changes may be found in other parts of the aorta. If such a situation arises, then they talk about combined and combined defects.

With acquired pathology, the aortic arch initially does not have anatomical defects or abnormalities. The lesion is a consequence of the underlying disease.

Congenital defects and anomalies include:

Hypoplasia.
Atresia.
Pathological tortuosity (Kinking syndrome).
Coarctation.
Defects of the middle section system, among which are:

complete double aortic arch;
malformations of the right and left arches;
anomalies in length, size, continuity;
anomalies of the pulmonary trunk and arteries.

Of the acquired diseases, the middle part is affected by:

atherosclerosis;
gunshot and knife wounds;
aortoarteritis Takayasu;
aneurysms.

Such a variety of possible lesions of this part of the circulatory system ensures that doctors are interested in early diagnosis and timely treatment.

Brief characteristics of individual species

Hypoplasia is a uniform tubular narrowing. This limitation in the diameter of the blood vessel prevents the full outflow of blood from the left ventricle. Get involved in pathological process in this case, not only the arch itself, but also the descending aorta and the ascending section can.

In most cases it is combined with other defects. Most of these patients die at an early age. Treatment is only surgical.

Atresia or break is called Steidel's anomaly. In this case, one of the segments of the vessel is completely absent. The consequence of this is that the descending aorta does not communicate with the ascending aorta.

They are isolated from each other. Blood supply is carried out due to the open ductus arteriosus. Children with this defect die in the first month of life without surgical intervention.

Pathological tortuosity is called Kinking's syndrome. Its essence is that the aortic arch at its distal end has an abnormal length and curvature. Patients with such a defect do not present complaints.

When Kinking syndrome is detected in children, doctors choose a wait-and-see approach. As the child grows, the defect may go away on its own.

Coarctation of the aorta

This anomaly is more often diagnosed in women. It is a narrowing of any part of a blood vessel. When the branches of the aortic arch are affected, there are several options:

Stenosis or atresia of the left subclavian artery.
Stenosis of the right subclavian artery.
Anomalous origin of the right subclavian artery:

distal;
proximal.

Coarctation in combination with a double aortic arch.

The narrowing can be localized, but usually consists of a pathological process spread over several cm. Often combined with others congenital anomalies. Included in tetralogy of Fallot, Turner syndrome. The defect is detected from birth.

With adequate drug support and a small degree of severity of the anomaly, patients have a favorable prognosis. Early surgical correction can significantly increase life expectancy (up to 35–40 years) and its quality.

Malformations of the aortic arch system

This group includes anomalies of the position, size, shape, course, relationship and continuity of arterial vessels. Such defects are most often asymptomatic.

Complaints appear when there are pronounced changes and the anomaly spreads to the proximal part of the descending section. Dysphagia or respiratory phenomena may occur due to close pathological contact of the aortic arch and its branches with the trachea and esophagus.

In this case, surgical intervention is necessary to prevent the development of severe complications.

The most common type is a complete double aortic arch. Distinctive feature such a defect is the presence of both arches (right and left), from which branches also extend. They all then merge with the descending artery behind the esophagus.

The prognosis for life in such patients is extremely favorable. In most cases, they do not require medication support.

Acquired vices

The most important secondary lesions of the blood vessel are:

In the first case, the lumen narrows due to the forming “fatty” plaques. It is easily diagnosed due to the compacted, emphasized contour of the vessel during an ultrasound of the heart or an x-ray of the lungs.

Compliance with the principles proper nutrition And rational pharmacotherapy will help slow down the process and prevent complications.

An aneurysm is an area of dilation of a blood vessel. The consequence of this is a narrowing of the lumen of its branches at the place of their origin. The cause of this situation is most often injury or atherosclerotic changes.

For a long time, the pathology may not reveal itself. If the ascending or descending aorta is involved in the process, big size aneurysm the first symptoms appear.

The main method of treatment is surgical. The treatment regimen before surgery must include medications that lower blood pressure to prevent dissection or rupture of the aneurysm.

Top main symptoms

Despite the variety of pathology options in the aortic arch system and its branches, most patients note the following complaints:

shortness of breath;
cough;
hoarseness of voice;
swallowing disorders;
headache;
dizziness;
temporary paralysis of the limbs;
swelling of the face.

The listed complaints are due to the involvement of the main branches of the middle part of the aorta in the pathological process. Only a doctor can determine what kind of disease or defect occurs.

To do this, a whole range of various instrumental examinations is performed. Treatment regimens are selected individually, taking into account the type of pathology.

The aortic arch occupies an important place in the process of blood supply to organs and systems. The presence of a defect or disease in its system can lead to serious consequences, death.

Therefore, it is important to undergo medical examination, contact a specialist in a timely manner, and follow all his recommendations.

Right aortic arch: what is it, causes, development options, diagnosis, treatment, when is it dangerous?

The aorta is the largest vessel in the human body, the function of which is to move blood from the heart to other arterial trunks, up to the arteries and capillaries of the whole body.

Phylogenetically, the development of the aorta undergoes complex changes during evolution. Thus, the formation of the aorta as an integral vessel occurs only in vertebrates, in particular in fish (two-chamber heart), amphibians (two-chamber heart with an incomplete septum), reptiles (three-chamber heart), birds and mammals (four-chamber heart). However, all vertebrates have an aorta, into which arterial blood mixed with venous, or entirely arterial, flows.

the first and second aortic arches are reduced,
the third arch gives rise to the internal carotid arteries that supply the brain,
the fourth arch gives rise to the aortic arch and the so-called “right” part,
the fifth arc is reduced,
the sixth arch gives rise to the pulmonary trunk and the arterial (Botallov) duct.

The heart becomes completely four-chambered, with a clear division of the cardiac vessels into the aorta and pulmonary trunk, by the sixth week of development. A 6-week embryo has a fully formed, beating heart with large vessels.

After the formation of the aorta and other internal organs, the topography of the vessel looks like this. Normally, the left aortic arch begins from the aortic bulb in its ascending part, which, in turn, originates from the left ventricle. That is, the ascending part of the aorta passes into the arch approximately at the level of the second rib on the left, and the arch bends around the left main bronchus, heading posteriorly and to the left. The uppermost part of the aortic arch projects onto the jugular notch just above the upper part of the sternum. The aortic arch goes down to the fourth rib, located to the left of the spine, and then passes into the descending part of the aorta.

Why does vice occur?

Any malformation is formed in the fetus if a woman is influenced by negative environmental factors during pregnancy - smoking, alcoholism, drug addiction, ecology and unfavorable background radiation. However, genetic (hereditary) factors play an important role in the development of the child’s heart, as well as existing chronic diseases in the mother or past infectious diseases, especially in the early stages of pregnancy (influenza, herpes infection, chickenpox, rubella, measles, toxoplasmosis and many others) .

So, in particular, the period of pregnancy of approximately 2-6 weeks is especially vulnerable to the fetal heart, since it is at this time that the aorta is formed.

Classification of the right-sided aortic arch

variant of the right aortic arch with the formation of a vascular ring

Depending on the anatomy of the duct anomaly, there are:

The right aortic arch without the formation of a vascular ring, when the arterial ligament (overgrown arterial, or Botallov, duct, as it should be normally after childbirth) is located behind the esophagus and trachea,
The right arch of the aorta with the formation of a vascular ring, code arterial ligament, or patent ductus arteriosus, is located on the left of the trachea and esophagus, as if surrounding them.
Also, a double aortic arch is distinguished as a separate similar form - in this case, the vascular ring is formed not by a connective ligament, but by a tributary of the vessel.

Figure: a variety of options for the atypical structure of the aortic arch

Depending on whether any other structures of the heart were damaged during its formation, the following types of defect are distinguished:

An isolated type of malformation, without other developmental anomalies (in this case, if the right-sided aorta is not combined with the DiGeorge syndrome characteristic of it in some cases, the prognosis is as favorable as possible);
In combination with dextrapposition (mirror, right location of the heart and great vessels, including the aorta), (which is also usually not dangerous),
In combination with a more serious heart defect - in particular tetralogy of Fallot (dextraposition of the aorta, ventricular septal defect, pulmonary stenosis, right ventricular hypertrophy).

Tetralogy of Fallot combined with the right arch is an unfavorable development option

How to recognize a vice?

Regarding the clinical manifestations of the right aortic arch, it should be mentioned that an isolated defect may not manifest itself at all, only sometimes accompanied by frequent obsessive hiccups in a child. In the case of a combination with tetralogy of Fallot, which accompanies the defect in some cases, the clinical manifestations are pronounced and appear in the first days after birth, such as increasing pulmonary heart failure with severe cyanosis (blue discoloration of the skin) in the baby. That is why tetralogy of Fallot is classified as a “blue” heart defect.

What screening shows a defect in pregnant women?

An analysis of fetal DNA can further clarify the absence of a connection between the formation of a right-sided aorta and severe genetic mutations. In this case, chorionic villus material or amniotic fluid is usually collected through a puncture. First of all, DiGeorge syndrome is excluded.

Treatment

In addition to surgery, cardiotropic drugs that can slow the progression of chronic heart failure (ACE inhibitors, diuretics, etc.) are prescribed for auxiliary purposes.

Forecast

Anomalies of the aortic arch. Causes. Treatment options. Consequences.

Anatomical classification

Defects of the aortic arch in isolated form or in combination are presented:

anomalies in the branching of brachiocephalic vessels;

anomalies in the location of the arch, including the right-sided aortic arch and cervical aortic arch;

increasing the number of arcs;

interruption of the aortic arch;

abnormal origin of a branch of the pulmonary artery from the ascending aorta or from the opposite branch of the pulmonary artery.

Individual anomalies are better understood in terms of their embryonic origin.

Embryology

Clinical classification

In addition to the anatomical classification, it is possible to subdivide the anomalies of the arch according to clinical signs:

compression of the trachea, bronchi and esophagus by vessels that do not form a ring;

anomalies of the arch that do not create compression of the mediastinal organs;

ductus-dependent arch anomalies, including interruption of the aortic arch;

isolated subclavian, carotid or innominate arteries.

Determination of the left and right aortic arch

Right aortic arch

The right-sided aortic arch crosses the right main bronchus from above and passes to the right of the trachea. There are four main types of right-sided arch:

retroesophageal left subclavian artery;

with retroesophageal diverticulum;

with left-sided descending aorta.

Right-sided arch with mirror origin of brachiocephalic vessels

Another situation in which it is useful to know the location of the aortic arch is the correction of esophageal atresia and tracheoesophageal fistula, since access to the esophagus is more convenient from the side opposite to the location of the aortic arch.

Right-sided arc with isolation of opposite vessels of the arc

The term "isolation" means that this vessel departs exclusively from the pulmonary artery through the ductus arteriosus and is not associated with the aorta. Three forms of this anomaly are known:

isolation of the left subclavian artery;

left innominate artery.

Cervical aortic arch

A cervical aortic arch is a rare anomaly in which the arch is located above the level of the clavicles. There are two types of cervical arch:

with abnormal subclavian artery and descending aorta opposite to the arch;

with virtually normal branching and a unilateral descending aorta.

The first type is characterized by a right aortic arch that descends on the right to the level of the T4 vertebra, where it crosses the esophagus posteriorly and goes to the left, giving rise to the left subclavian artery and sometimes the ductus arteriosus. This type, in turn, is divided into a subtype, in which the internal and external carotid arteries arise from the arch separately, and a subtype, in which there is a bicarotid trunk, when both common carotid arteries arise from one vessel, and both subclavian arteries arise separately from the distal arcs. In each of these subtypes, the vertebral arteries arise separately from the arch. While most patients with contralateral descending aorta have a vascular ring formed by the aortic arch on the right, the retroesophageal segment of the aorta posteriorly, the ligamentum arteriosus on the left, and the pulmonary artery anteriorly, only half of them exhibit clinical signs of a ring.

The second type is characterized by a left-sided aortic arch. Narrowing caused by the aortic arch due to the long, tortuous, hypoplastic retroesophageal segment is rare.

recurring respiratory infections.

In the presence of a pulsating formation in the neck, the presumptive diagnosis can be made by the disappearance of the pulse in the femoral artery when the pulsating formation is briefly pressed.

Persistent V aortic arch

double-lumen aortic arch with passable both lumens;

atresia or interruption of the upper arch with a passable lower arch, accompanied by the departure of all brachiocephalic vessels with a common mouth from the ascending aorta;

a systemic pulmonary junction located proximal to the first brachiocephalic artery.

Coarctation of the aorta occurs in all three subgroups, including in combination with pulmonary atresia.

Without concomitant coarctation of the aorta, a double-lumen arch has no physiological significance.

With the V persistent arch, which has an anatomical connection with the pulmonary artery, EchoCG, angiography and MRI can detect a vessel arising from the ascending aorta proximal to the I brachiocephalic branch, which ends in the pulmonary artery. In one case, histological examination revealed elements of ductus arteriosus tissue.

The aortic arch is the middle part of the largest blood vessel in the human body.

Almost all organs and systems depend on its normal functioning.

In case of pathology of this blood vessel, serious therapeutic measures are often required.

About anatomy and topography

The aorta is the main trunk of arteries in the systemic circulation. It originates in the cavity of the left ventricle of the heart. Consists of 3 parts:

ascending;
average;
descending.

Conventionally, the structure consists of 2 parts:

concave;
convex.

From the concave side of the aortic arch depart blood vessels that feed the bronchi and thymus gland. 3 trunks originate from the convex part, located from right to left:

Brachiocephalic (brachiocephalic).
General carotid (carotid) left.
Left subclavian.

The branches of the aortic arch extend upward from its middle part. All of these arteries supply the upper half of the body, including the brain.

Anomalies, defects and diseases

Blood vessel pathology can be divided into 2 large groups:

Congenital.
Acquired.

With acquired pathology, the aortic arch initially does not have anatomical defects or abnormalities. The lesion is a consequence of the underlying disease.

Congenital defects and anomalies include:

Hypoplasia.
Atresia.
Pathological tortuosity (Kinking syndrome).
Coarctation.
Defects of the middle section system, among which are:

complete double aortic arch;
malformations of the right and left arches;
anomalies in length, size, continuity;
anomalies of the pulmonary trunk and arteries.

AND Acquired diseases affect the middle part:

atherosclerosis;
gunshot and knife wounds;
aortoarteritis Takayasu;
aneurysms.

Such a variety of possible lesions of this part of the circulatory system ensures that doctors are interested in early diagnosis and timely treatment.

Brief characteristics of individual species

Hypoplasia is a uniform tubular narrowing. This limitation in the diameter of the blood vessel prevents the full outflow of blood from the left ventricle. In this case, not only the arch itself, but also the descending aorta and the ascending section can be involved in the pathological process.

In most cases it is combined with other defects. Most of these patients die at an early age. Treatment is only surgical.

They are isolated from each other. Blood supply is carried out due to the open ductus arteriosus. Children with this defect die in the first month of life without surgical intervention.

When Kinking syndrome is detected in children, doctors choose a wait-and-see approach. As the child grows, the defect may go away on its own.

This anomaly is more often diagnosed in women. It is a narrowing of any part of a blood vessel. When the branches of the aortic arch are affected, there are several options:

Stenosis or atresia of the left subclavian artery.
Stenosis of the right subclavian artery.
Anomalous origin of the right subclavian artery:

distal;
proximal.

The narrowing can be localized, but usually consists of a pathological process spread over several cm. Often combined with other congenital anomalies. Included in tetralogy of Fallot, Turner syndrome. The defect is detected from birth.

Malformations of the aortic arch system

This group includes anomalies of the position, size, shape, course, relationship and continuity of arterial vessels. Such defects are most often asymptomatic.

In this case, surgical intervention is necessary to prevent the development of severe complications.

The most common type is a complete double aortic arch. A distinctive feature of such a defect is the presence of both arcs (right and left), from which branches also depart. They all then merge with the descending artery behind the esophagus.

The prognosis for life in such patients is extremely favorable. In most cases, they do not require medication support.

Acquired vices

The most important secondary lesions of the blood vessel are:

atherosclerosis;
aneurysm.

Compliance with the principles of proper nutrition and rational pharmacotherapy will help slow down the process and prevent complications.

For a long time, the pathology may not reveal itself. When the ascending or descending aorta is involved in the process, the first symptoms appear when the aneurysm is large.

The main method of treatment is surgical. The treatment regimen before surgery must include medications that lower blood pressure to prevent dissection or rupture of the aneurysm.

Top main symptoms

Despite the variety of pathology options in the aortic arch system and its branches, most patients note the following complaints:

shortness of breath;
cough;
hoarseness of voice;
swallowing disorders;
headache;
dizziness;
temporary paralysis of the limbs;
swelling of the face.

The listed complaints are due to the involvement of the main branches of the middle part of the aorta in the pathological process. Only a doctor can determine what kind of disease or defect occurs.

To do this, a whole range of various instrumental examinations is performed. Treatment regimens are selected individually, taking into account the type of pathology.

The aortic arch occupies an important place in the process of blood supply to organs and systems. The presence of a defect or disease in its system can lead to serious consequences, death.

Therefore, it is important to undergo medical examination, contact a specialist in a timely manner, and follow all his recommendations.

Double Aortic Arch-Child

What is a double aortic arch in a child?

Double aortic arch is one of the types of cardiac vascular defects. In a healthy heart, blood flows from the body into the right atrium and then into the right ventricle. Next, the blood goes to the lungs through the pulmonary valve, where it is saturated with oxygen. Then the blood returns to the left atrium and enters the left ventricle, after which it is distributed throughout the body through the aorta.

If there is a double arch of the aorta, it branches into right and left parts. A bifurcation of the aorta forms a vascular ring and can cause compression of the airway and/or esophagus.

Causes of double aortic arch

A double aortic arch in a child is a congenital defect. This means that the abnormality develops while the baby is in the womb and the baby is born with the condition. It is not yet known exactly why some babies develop abnormalities in heart development.

Risk factors for double aortic arch

Risk factors influencing the appearance of a double aortic arch are still unknown.

Symptoms of a double aortic arch in a child

Symptoms of a double aortic arch may include:

Labored breathing;
Lung infections;
Poor appetite, including attacks of vomiting and choking;
Problems with swallowing, including attacks of choking;
Vomit;
Heartburn.

Diagnosis of double aortic arch

Most often, this disease is discovered in infancy; it is often found later.

Examination of the upper gastrointestinal tract;

An ECG is prescribed to study the functioning of the heart.

Treatment of double aortic arch in a child

Treatment options for double aortic arch include:

Surgery to treat double aortic arch

Monitoring the child's condition

Your child should have regular checkups with a cardiologist, a specialist in heart disease.

Prevention of the appearance of a double aortic arch in a child

There are currently no methods to prevent the occurrence of a double aortic arch in a child. Despite this, it is very important to receive appropriate prenatal care.

Vascular rings are a pathology of large vessels that surround and compress the esophagus and/or trachea. They can be formed by the aorta, its branches and the pulmonary artery. The anomaly can act as an isolated defect or accompany other congenital heart defects.

The first description of a vascular ring in the form of a retroesophageal subclavian artery belongs to Hunauld and dates back to 1735. Then Hommel in 1737 described a double aortic arch. Fifty-seven years later, in 1794, Bayford associated the clinical picture of esophageal compression with the aberrant retroesophageal subclavian artery, which he called a trick of nature and used the term dysphagia lusoria to describe the symptoms. For two centuries there was no mention of this anomaly, and only in 1936 Kommerell made a diagnosis of this pathology during life, using barium esophagography. The ability to diagnose the defect contributed to the beginning of the era of surgical treatment. The first double aortic arch correction was first performed by Gross in 1945 in a one-year-old boy with chronic stridor. The pulmonary arterial loop was described by Glaevecke and Doehle in 1897. Potts in 1954 was the first to correct the defect by dividing and reimplanting the pulmonary artery.

Terminology

The term sling has no analogue in Russian and means “outweighing” or “formation of a knot.” Typically this term is used when the left pulmonary artery forms a loop around the trachea and right bronchus. This designation may also be used in the presence of a variety of incomplete vascular loops formed by the esophageal branch of the subclavian artery and abnormalities of the brachiocephalic trunk. The Latin term arteria lusoria corresponds to the English term "vagus subclavian artery". The name “aberrant artery” is used literally in Russian literature. This term refers to the retroesophageal location of both the right and left subclavian arteries. The term dysphagia lusoria means "difficulty in swallowing" caused by vascular rings.

Vascular rings, both aortic and pulmonary, can cover the esophagus and trachea completely or partially. In both cases, identical symptoms of compression may appear, regardless of whether the ring is closed or open.

Embryogenesis

Understanding embryonic development is probably more important in the treatment of aortic arch anomalies than any other congenital malformations of the cardiovascular system. The most common is the Edwards classification, based on the mechanism of progression of a double arch into a single arch. At an early stage of development, 6 pairs of aortic arches connect the ventral and dorsal aortas, although not all of them exist at the same time. These arches undergo degradation, fusion, and remodeling to form the typical left-sided aorta and its major branches. Inappropriate persistence or resorption of archwires can lead to the development of a vascular ring. The first, II and V arches regress. The third pair gives rise to the carotid arteries, the VI arches become the pulmonary arteries, and the VII intersegmental arteries become the subclavian arteries. The ventral part of the left IV arch becomes the ductus arteriosus, usually on the left side, as the right IV arch undergoes involution. The location of the aortic arch is determined by which of the fourth arches persists. The double arch is formed as a result of the bilateral persistence of the fourth arches.

Anatomy

In a complete vascular ring, abnormal vascular structures form a closed circle around the trachea and esophagus. The annulus is formed by the double aortic arch or the right aortic arch with the left arterial ligament. An incomplete vascular ring is an open circle. Despite this, it compresses the trachea and esophagus. An open ring is formed with the participation of an abnormal innominate artery, an aberrant right subclavian artery, and a pulmonary vascular loop.

Double aortic arch

Double aortic arch is the most common type of vascular ring. It is formed as a result of a violation of the process of degradation of the right or left IV brachial arches, which leads to the persistence of the right and left aortic arches. The anomaly comes in several variants:

both arches are widely passable;

one of the arches, usually the left, is hypoplastic or atretic.

The defect may be accompanied by a PDA or ligament. The right arch is usually located higher than the left. Both arcs completely surround and compress the trachea and esophagus, which is manifested by difficulty in breathing and feeding in early infancy. Two vessels depart from the right arch - the right common carotid and right subclavian arteries, and the left common carotid and left subclavian arteries depart from the left arch. The right aortic arch is usually larger than the left. The right arch is dominant in 75% of cases, the left arch is dominant in 15%, and in 10% the arches are of the same size. The smaller of the two arches may have a segment of hypoplasia or atresia anywhere along the arch, but usually at the junction with the descending aorta.

Despite the fact that all variants of the double arch form a closed vascular ring around the trachea and esophagus, the branching pattern of the brachiocephalic vessels is determined by the patency of the various components of the arch and the side of the descending aorta. With two patent arches, the four main brachiocephalic arteries arise relatively symmetrically from both arches. A double arch with atresia of the left arch distal to the origin of the left subclavian artery is characterized by a distribution of vessels originating from it, similar to the mirror right aortic arch: the left innominate artery, the right carotid and right subclavian arteries arise successively in combination with the left descending aorta. In fact, this type of vascular distribution, together with signs of tracheal compression, is indistinguishable from the rare anomaly of the right aortic arch with the left descending aorta; the differences are visible only during surgery.

A double arch with left arch atresia between the left carotid and left subclavian arteries may mimic a right aortic arch with a Kommerell retroesophageal diverticulum. Atresia of the right arch is quite rare. Among patients operated on for a vascular ring, 77% have a double aortic arch, in 85% of them both arches are patent, in 15% the left arch is atretic.

Double aortic arch is rarely combined with congenital defects, mainly with tetralogy of Fallot, and even more rarely with TMA. Occasionally, a double arch is combined with coarctation of the left or both arches, cervical left aortic arch.

A double aortic arch is usually an isolated anomaly, but can sometimes be accompanied by various congenital heart defects:

tetralogy of Fallot;

coarctation of the aorta.

Left-sided aortic arch with aberrant right subclavian artery

The most common anomaly of the arch, occurring in 0.5% of people in the general population and in 20% of patients with corresponding symptoms. The anomaly is formed as a result of the disappearance of the right IV aortic arch. The distal right dorsal aorta becomes the proximal right subclavian artery, forming its retroesophageal portion. In most cases, the right VI arch undergoes involution.

With this anomaly, the three vessels of the arch arise normally. The right subclavian artery departs independently from the posteromedial wall of the descending aorta, passes behind the esophagus, pressing down on its posterior wall and creating moderate swallowing disorders. Most patients have no symptoms. The esophagus is compressed more if the right subclavian artery branches off from the aortic diverticulum.

The anomaly may be associated with tetralogy of Fallot, coarctation of the aorta, interruption of the aortic arch. Very often it occurs in patients with Down syndrome combined with congenital heart disease. In most cases, the anomaly is not clinically manifested and is discovered during examination undertaken for another reason or at autopsy. It is the second most common anomaly after the right-sided aortic arch.

Left-sided aortic arch with right-sided descending aorta and right-sided ductus arteriosus

This is a rare arch anomaly with the same branching as in a left-sided arch with the right subclavian artery passing behind the esophagus. However, the arch itself is located behind the esophagus. Consequently, the right subclavian artery, which arises from the descending aorta as the fourth vessel of the arch, does not pass behind the esophagus.

The descending aorta is connected to the right pulmonary artery by the ductus botallus or ligament, forming a vascular ring.

Compression of the trachea by an anomalous innominate artery

A variant of an incomplete vascular ring, which occurs in 10% of patients with anomalies of the arch vessels. Compression of the anterior wall of the trachea by an anomalous innominate artery extending unusually far from the aortic arch to the left and behind is a not entirely understood pathology. A common explanation for this phenomenon is a more distal origin of the innominate artery from the aortic arch, but this mechanism is not obvious. The presumptive cause is tracheomalacia adjacent to the innominate artery, idiopathic or caused by a tracheoesophageal fistula. The anomaly is combined with other congenital heart defects, such as VSD.

Right aortic arch with retroesophageal Kommerell diverticulum or left ligamentum arteriosus

This is the second most common variant of the vascular ring. The true frequency of this anomaly is unknown. According to some data, it accounts for 6% of all arch anomalies and 9% of cases of right-sided arch. It is a consequence of the persistence of the right IV brachial arch. The aorta passes to the right of the trachea and esophagus, further behind and to the left of them, continuing into the left-sided descending aorta. In combination with the left ligament arteriosus or PDA, the right arch forms a tight ring that compresses the trachea and esophagus. The duct may attach to the left subclavian artery or behind the esophagus to the diverticulum of the descending aorta. The aortic arch and the vessels extending from it often occupy a mirror position, the descending aorta passes to the right of the trachea and the ligament arteriosus does not close the ring. In a right-sided arch with Kommerell's diverticulum, the first branch is the left carotid artery, the second is the right carotid artery, the third is the right subclavian artery, and the last is the retroesophageal vessel, from which the left subclavian artery arises and with which the left duct or ligament of the botallus is connected.

The anomaly is combined with various heart defects. Among patients operated on for a vascular ring, 19% had a right-sided arch and retroesophageal diverticulum. Most of them had no additional defects. This arch anomaly in many cases does not manifest itself clinically and is discovered accidentally.

If the ductus arteriosus is patent, the vascular ring is formed by the aortic arch on the right, the retroesophageal vessel supplying the subclavian artery posteriorly, the ductus arteriosus on the left, and the pulmonary artery anteriorly. The Kommerell diverticulum is a significantly larger vessel than the subclavian artery. Typically, the orifice of the diverticulum is equal to the diameter of the descending aorta and narrows to the size of the subclavian artery at the junction with the left botallic ligament.

This variant of the vascular ring is almost always combined with other congenital heart defects, especially often with tetralogy of Fallot.

Right-sided arch with aberrant left subclavian artery

The aberrant artery arises from the right-sided arch and goes to the left behind the esophagus. It is pulled anteriorly by the left-sided arterial ligament and can compress it. The arc passes to the right of the trachea. The brachiocephalic arteries arise in the following order: left carotid, right carotid, right subclavian, and retroesophageal left subclavian arteries. This option differs in that the length of the proximal left subclavian artery is not greater than the length of its distal part.

The anomaly occurs in tetralogy of Fallot, OSA, and right ventricular aorta with pulmonary atresia.

Right-sided arch with retroesophageal innominate artery

In contrast to the general pattern, which consists in the fact that the first vessel of the arch is the carotid artery opposite to the aortic arch, with this anomaly the sequence of brachiocephalic vessels is different: the right carotid, right subclavian, retroesophageal left innominate artery. The ductus arteriosus or ligament completes the ring as it connects the left pulmonary artery to the base of the innominate artery.

Right aortic arch with left descending aorta and left ductus arteriosus

The right-sided arch, left descending aorta, also called the right aortic arch, with a retroesophageal segment enveloping the retroesophageal right aortic arch, is similar to the right-sided arch with a retroesophageal diverticulum, but less common. Unlike a retroesophageal diverticulum, with this anomaly, the arch crosses the midline to the left at the level of the IV or V thoracic vertebrae, where the left arterial duct departs. The first branch of the arch is the left carotid artery, followed by the right carotid artery, the right subclavian artery, and the last left subclavian artery. An alternative order of the brachiocephalic arteries is: first - the left innominate, then the carotid and right subclavian arteries.

Loop pulmonary artery

The loop is formed due to the departure of the left pulmonary artery from the proximal part of the right. Heading towards the left lung, the anomalous artery passes over the proximal part of the right main bronchus behind the trachea and anterior to the esophagus to the root of the left lung. Sometimes the ligamentum arteriosus completes the ring around the trachea. The anomaly is a rare defect in which the lower part of the trachea is partially surrounded by vascular structures. This is the only situation where a large vessel passes between the trachea and the esophagus. The pulmonary loop sometimes participates in the formation of a complete cartilaginous ring around the distal trachea, leading to tracheal stenosis. The anomaly occurs alone or in combination with other heart defects, including tetralogy of Fallot.

The distal pulmonary arteries normally arise from the corresponding lung primordia and separately merge with the pulmonary arterial part of the truncoaortic sac. If the two distal arteries fuse together through potential vascular islands from the splanchnic bed before entering the truncoaortic sac, there is a possibility that the left pulmonary artery will be posterior to the trachea before the union. This leads to the formation of a loop of the pulmonary artery. If it passed in front of the trachea, the anatomy will be indistinguishable from the norm. In half of the patients, the pulmonary loop is accompanied by a PDA, VSD, ASD, AVSD, single ventricle, and aortic arch anomalies.

Clinic, diagnostics

The clinical picture of vascular rings is characterized by stridor or pneumonia and bronchitis. Inspiratory stridor and feeding problems of varying severity are observed in at different ages. Infants may experience postural swelling of the neck, and less commonly, a reflexive holding of breath during feeding. A common sign of the disease in 1-3 month old infants is noisy breathing from birth and subsequent difficulty breathing in combination with intercurrent respiratory tract infection. Sometimes noted characteristic feature– Babies stretch their neck to relieve tracheal compression. Older children and adolescents complain of difficulty swallowing and a feeling of being full of food. Detailed questioning of parents sometimes reveals stridor in infancy and recurrent bronchitis. In patients with concomitant intracardiac malformations respiratory symptoms mistakenly associated with heart disease, but in fact they are completely caused by the vascular ring. Sometimes in asymptomatic patients, pathology is discovered during examination for other causes. The diagnosis can be suspected based on the history and plain x-ray. If symptoms are present, the patient should be carefully examined.

With incomplete forms of the vascular ring, clinical manifestations are less pronounced than with a complete vascular ring. The anamnesis is dominated by a tendency to pneumonia. Physical studies are uninformative if the vascular ring is not accompanied by other CHD. Bronchial rales may be noted on auscultation.

On anteroposterior and lateral radiographs, compression of the trachea can be seen, sometimes signs of aspiration pneumonia or atelectasis. Angiocardiography for vascular pathology is the most comprehensive diagnostic method. However, in some cases, in severe and even critical condition of patients, it poses a certain risk, so the combined use of esophagography with barium and echocardiography may be sufficient to make the most approximate diagnosis.

The arsenal of diagnostic tools also includes digital subtraction angiography, computed tomography scanning, and MRI.

Tracheography and bronchoscopy are uninformative and dangerous for patients. However, these studies may be useful in detailing the anatomy of tracheobronchial abnormalities in patients with double aortic arches or pulmonary loops.

Clinical manifestations of a double aortic arch depend on the size of the annulus. They were described in detail in 1939. With two passable arcs, the ring is usually narrow. Stridor is noted in the first weeks of life. Symptoms occur already in the neonatal period or in early infancy and are most pronounced during feeding. With left arch atresia, the ring is usually wider and symptoms appear at 3-6 months of age or later. Less commonly, complaints first appear in adults in the form of difficulty swallowing or respiratory symptoms.

The diagnosis of a double arch with both patent arches can be made quite reliably on the basis of a plain radiograph: the air contour of the trachea is located higher than the right-sided arch, and the left-sided arch is even lower. In lateral view, the right arch can be seen behind the trachea. These features are more obvious on esophagography: two large filling defects are noted on both sides in the anteroposterior projection and a posterior filling defect in the lateral projection.

To clarify the anatomy, it is advisable to use echocardiography, angiography or MRI, since the arches may not be of the same caliber. It is necessary to identify the hypoplastic segment that can be sacrificed by sectioning the ring. In addition, during the operation, the surgeon cannot identify appearance diaphragmatic coarctation in one of the arches.

The supraclavicular echocardiographic approach makes it possible to clearly identify the two arches, while subcostal and parasternal approaches rely on inference. The left arch is more often hypoplastic than the right one, however, the presence of exceptions to this rule requires a detailed assessment in each case.

Angiography has historically been the standard examination method, but its use does not guarantee the exclusion of error due to superimposed structures. More accurate information can be obtained using retrograde arteriography, which details the pattern of branch origin. Magnetic resonance imaging allows you to obtain a real image of the spatial relationship of the vessels, trachea and esophagus and better plan surgical intervention.

A left aortic arch with an aberrant right subclavian artery is characterized by the absence of the innominate artery. The first and second branches, i.e. the right and left carotid arteries have the same dimensions as the left and right subclavian arteries. A specific diagnostic method is esophagography, which makes it possible to detect a fixed filling defect inclined upward and to the right. It is better visible with fluoroscopy. The filling defect is relatively small compared with that of arch anomalies or diverticula pushing back the esophagus.

Angiography in the anteroposterior view can be misleading because the right subclavian artery may be mistaken for the right carotid artery. However, time-lapse analysis demonstrates earlier filling of the right carotid artery when contrast material is injected into the ascending aorta or earlier filling of the right subclavian artery when contrast material is injected into the descending aorta.

This anomaly is also recognized during echocardiography by the absence of bifurcation of the first branch and the presence of the fourth branch, which goes to the right and can disappear behind the trachea. The course of the subclavian artery behind the esophagus can be seen on a transverse section during MRI. There is a slight depression in the oblique projection. It can be severe if caused by an aortic diverticulum.

A left-sided aortic arch with a right-sided descending aorta and a right-sided ductus arteriosus can be suspected if a patient with symptoms of a vascular ring has a left-sided aortic arch without signs of a descending aorta. A plain radiograph may show the left-sided arch and the right-sided superior descending aorta. Esophagography reveals a large depression in the posterior wall of the esophagus created by the aorta passing behind it. However, the course of this vessel does not differ from the more common right-sided arch with a posterior diverticulum. In both cases, the upper part of the descending aorta is located on the right.

The aortic arch is usually on the left side on x-ray, but this is not always obvious, especially in infants with a large thymus.

Angiography reveals an abnormal course of the aorta from the left arch to the retroesophageal segment of the descending aorta. The right subclavian artery can be seen arising from the descending aorta at the point where it changes from transverse to vertical.

MRI shows the position of the aorta relative to the trachea. Although most vascular rings can be divided through a left-sided approach, with this type of ring the ligament is accessible from a right-sided or midline approach.

An anomalous innominate artery may be suspected by the presence of severe inspiratory and expiratory stridor at 2–6 months of age, coupled with anterior deviation of the tracheal air column on a lateral radiograph. It is necessary to exclude a vascular ring based on esophagography and MRI. The artery does not compress the esophagus and therefore is not detected during a contrast study.

In the case of a right aortic arch with a left arterial ligament, a right-sided aortic arch is determined on a plain radiograph. Esophagography reveals a large filling defect along the posterior contour of the esophagus, in contrast to a smaller defect in the retroesophageal subclavian artery. However, occasionally, vascular rings, such as a left-sided aortic arch with a right-sided descending aorta and right botallic ligament, can give a similar picture on esophagoscopy.

Since the surgical treatment is different, this sign cannot be considered sufficient to make a decision. Although the ligamentum arteriosus is not visualized by any of the existing methods, its presence is confirmed by the characteristic appearance of a tapering diverticulum. Echocardiography shows the left carotid artery arising independently as the first vessel of the arch. However, the final diagnosis is made when a diverticulum narrows to the size of the subclavian artery. It usually cannot be identified because the trachea and adjacent lungs may prevent the area from being seen.

Angiography reveals the characteristic branching of the arch vessels and, more importantly, demonstrates a sharp decrease in caliber from the diverticulum to the subclavian artery. In the direct anteroposterior view, the posterior subclavian artery may overlap the anterior left carotid artery, creating the appearance of a left innominate artery, as in a mirror-like right aortic arch. However, frame-by-frame study of cine angiography makes it possible to identify individual vessels. The introduction of a contrast agent into the more distal part of the arch reveals a wide diverticulum of the aorta, which continues into the narrow subclavian artery.

Angiography is not advisable in infants, so MRI is an ideal non-invasive method for diagnosing arch abnormalities and also showing Airways and their relative position. The aortic arch is located to the right of the trachea, the diverticulum is behind it.

Right-sided arch with aberrant left subclavian artery.

The diagnosis can be suggested by esophagoscopy. A small linear depression is detected along the posterior semicircle of the esophagus, going upward and to the left. Due to the absence of a vascular ring, the trachea is intact, with the exception of a slight deviation to the left, which is present in all variants of the right-sided arch.

On echocardiography, as noted above, it is possible to identify the first branch of the aorta - the left carotid artery, since it does not have a bifurcation like the innominate artery. Its diameter is the same as that of the second branch - the right carotid artery. However, it is difficult to identify the retroesophageal vessel, the left subclavian artery. MRI and angiography provide clear information about the anatomy of the left subclavian artery and distinguish it from a retroesophageal diverticulum.

Due to the absence of a vascular ring, there is no need to perform correction. It is indicated in the presence of associated anomalies.

Right-sided arch with retroesophageal innominate artery. As a rule, tracheal compression occurs, although the severity of symptoms varies significantly. An important anatomical feature is the presence of a single carotid artery arising from the proximal aorta. This sign is present in other anomalies: a break in the aortic arch between the two carotid arteries and an isolated left carotid or innominate artery. However, in the pathology under consideration, there is a normal sized right-sided arch and a distal origin of the carotid artery from this arch.

Right-sided aortic arch with left-sided descending aorta and left-sided ductus arteriosus.

The X-ray picture and esophagography data are identical to those with a right-sided arch with a diverticulum. The difference is that the depression of the posterior wall of the esophagus is oriented down and to the left, and not up and to the left. In some cases, the descending aorta can be found to the left of the spine rather than to the right.

Echocardiography from a suprasternal approach reveals an abnormal branching of the arch vessels and a right-sided arch. Along with the left-sided passage of the descending aorta, its passage behind the esophagus can be determined. With concomitant hypoplasia of the arch, this anomaly can be mistaken for a break in the aortic arch. Under these conditions, it is difficult to determine which of the carotid arteries arises first, but careful examination of the origin of other brachiocephalic vessels by echocardiography or angiography allows these anomalies to be differentiated.

On angiography, it may be unclear whether the aorta passes in front of the trachea, as is the case with a right ascending and left descending aorta, with a normal left-sided arch, or behind the trachea, as with a right-sided aortic arch with a left-sided descending one. The key to a correct assessment of the anatomy is to determine the order of origin of the brachiocephalic vessels. In a right-sided arch with a left descending aorta, the first vessel is the left carotid artery. In a normal left-sided arch, the first is the right carotid artery.

MRI provides images of the entire aorta, not only the normal right-sided ascending aorta and left-sided descending segment, but also the relationship to the trachea.

The pulmonary artery loop is characterized by the presence of atelectasis, emphysema, and pneumonia in the right lung. Patients typically suffer from both severe respiratory failure and swallowing problems. However, clinical manifestations may be in an unexpressed form. A pulmonary arterial loop is accidentally discovered during angiography performed for another defect.

Esophagography reveals an anterior filling defect of the esophagus in the lateral projection. This is the only variant of the vascular ring in which the anterior wall of the esophagus is depressed. There is also a right-sided filling defect in the anteroposterior projection. The right lung may have increased transparency, with signs of atelectasis or inflammatory infiltration.

Echocardiography allows one to exclude intracardiac defects and make a diagnosis of a vascular ring. To do this, it is necessary to conduct a sequential segment-by-segment study of the aortic arch and its branches, although detailed anatomical information is difficult to obtain.

On angiocardiography, the origin and path of the anomalous left pulmonary artery are best seen when a contrast agent is injected into the pulmonary trunk with the patient in a semi-sitting position.

During surgery, bronchoscopy is useful because of its frequent association with a complete cartilaginous ring.

Double aortic arch (DAA), or vascular ring, is an anomaly in the development of the aortic arch and its branches, as a result of which their normal location in the mediastinum is disturbed, which can lead to compression of the trachea and esophagus. The ascending aorta, located in the anterior mediastinum in front of the trachea and esophagus, is divided into two arches: postero-right and anterior-left, which cover the trachea and esophagus on all sides in the form of a ring (Fig. 22.1).
The posterior right arch crosses over the right pulmonary artery and the right bronchus, and the anterior right arch crosses the left pulmonary artery and the left bronchus. Both arches join behind the esophagus with the upper part of the descending aorta, which descends further to the left or right of the spine. In this case, the common carotid and subclavian arteries depart from each arch. The posterior right arch is wider and is located slightly higher than the anterior left one. Most often, both arcs are not obliterated, atresia of one of them (usually anterior) is less common. The ductus arteriosus can be located on both sides, but, as a rule, occurs on the left.

Rice. 22.1. Schematic representation of the heart and double aortic arch.
Other variants of abnormal formation of vascular rings are also possible:
with a right-lying aortic arch and a left-sided PDA or ligament arteriosus. The trachea and esophagus are also enclosed in a ring, where the right and posterior contours of the ring are formed by the right-sided aortic arch, the anterior contour is formed by the bifurcation of the pulmonary artery, and the left contour is formed by the arterial duct connecting the lower surface of the aortic arch with the left branch of the pulmonary artery;
the same combination, but with the presence of an abnormal origin from the right aortic arch of the left brachiocephalic trunk, which goes from right to left in front of the trachea and esophagus, forming the anterior contour of the ring, and its right-posterior and left contours are formed, respectively, by the right aorta and PDA or arterial ligament (Burakovsky V.I. et al., 1996).
Anomalies in the development of the aortic arch are associated with disturbances in the process of embryogenesis. The fetus initially has two aorta - ventral and dorsal, connected by 8 pairs of vascular arches. With the final formation of the vessels, the aortic arch, trunk and pulmonary arteries are formed, the remaining arches regress, atrophy and disappear. Violation of the process of normal regression of the remaining arches, probably, becomes the cause of the formation of abnormal arches and branches of the aorta (Bankl G., 1980;
1995).
Anomalies of the aortic arch and its branches are, according to clinical data,
7-1%, and according to sectional studies - 3-3.8% of all congenital heart and vascular defects (Nosla8 A., Ru1er O., 1972; Bankle G., 1980). In 20% of cases, this anomaly is combined with other congenital heart defects, most often with TF, VSD, ASD, CoA, TMS, EZhS. The right-sided aortic arch is usually combined with other congenital heart defects. The true incidence of aortic arch anomalies is difficult to determine, since this defect, without accompanying congenital heart disease, occurs without hemodynamic disturbances, asymptomatically, and in cases of clinical symptoms, children are observed and treated for a long time in general pediatric departments, often as patients with respiratory or gastroenterological pathologies.
Symptoms of compression of the trachea and esophagus are more often observed with a double aortic arch (37% of cases), less often with a right-lying aortic arch and left-sided ductus arteriosus (26% of cases), or various types of vascular anomalies of the aortic arch (37% of cases) (Ogozz K.. , 1964). TniMa et al. (1986) when examining children of the first year of life with stridor, a vascular ring was detected in 35% of cases.
Clinical picture. Clinical manifestations of the defect depend on the degree of compression of the esophagus and (or) trachea. Compression of the esophagus manifests itself already in the first months of life, difficulties with feeding, frequent regurgitation and vomiting, poor weight gain and developmental delays, which are usually regarded as signs of pyloric spasm or pyloric stenosis. After 1 year of age, difficulty swallowing solid food and the child’s desire to swallow it slowly or with liquid are observed. Regurgitation and vomiting are observed with large meals. Older children may complain of a dull pain in the chest when breathing deeply or swallowing.
Compression by the vascular ring of the trachea is accompanied in infants by stridorous, noisy, “snoring” breathing. Unlike stridor caused by laryngomalacia, birth trauma, previous intubation or other reasons and usually significantly decreasing or disappearing after 3-12 months, stridor breathing with a vascular ring progresses with age, accompanied by difficulty breathing and shortness of breath. Sometimes attacks of stridor breathing and shortness of breath are syncopal in nature, accompanied by coughing, suffocation, apnea and even cyanosis. Such attacks can occur during physical exertion, psycho-emotional arousal, or heavy food intake. Some relief is caused by the patient accepting a forced position with his head thrown back, in which the patency of the trachea improves and breathing becomes freer (\Vetberg R.M., 1995).
Children are prone to recurrent bronchitis and bronchopneumonia, occurring with minor acute inflammatory manifestations and an abundance of physical findings. In addition to compression of the trachea, frequent aspiration of food appears to contribute to the recurrence of respiratory diseases. In some cases, respiratory failure increases significantly and becomes permanent. Children are usually treated in general somatic or pulmonology hospitals as patients with respiratory allergosis, recurrent bronchitis and even COPD.
If there are no concomitant congenital heart defects, then a physical examination of the cardiovascular system will not reveal any pathology. Pulse and blood pressure are not changed, the boundaries of the heart are within the age norm, heart sounds are clear, no murmurs are heard. On auscultation, dry and moist coarse and medium bubble rales can be heard in the lungs.
Electrocardiogram and phonocardiogram - without pronounced signs of pathology.
Radiography. There are no pathological changes in the direct projection. In the lateral projection, as the contrast agent passes through the esophagus, depressions caused by an external pulsating formation are revealed on its anterior and posterior contours. In addition, in the lateral projection, narrowing of the trachea at the level of the aortic arch can be detected.
With direct bronchoscopy, it is possible to determine the degree of narrowing of the tracheal lumen and its pulsation in the area of the suprabifurcation segment.
Two-dimensional echocardiography and aortography make it possible to definitively diagnose the presence of a double aortic arch, as well as an anomaly of the bracheocephalic vessels.
Differential diagnosis is carried out with a tumor of the mediastinum, tracheoesophageal fistula, anomaly of the tracheobronchial tree.
Natural history and prognosis. In the absence of clinical symptoms of compression of the trachea and esophagus, the anomaly of the aortic arch and its branches does not manifest itself and may become an accidental finding. In children with symptoms of compression (depending on its degree), the clinical manifestations of the anomaly occur early, progressively increase and sometimes underlie developmental delay, dystrophy, persistent dysphagia, retrosternal pain, neurosis, recurrent bronchitis and bronchopneumonia, bronchiectasis and other COPD, and life-threatening respiratory failure. Therefore, all children with progressive stridor and dysphagia, unresponsive to therapy, and recurrent respiratory diseases require a targeted examination to identify abnormalities of the aortic arch and its branches. In the presence of concomitant congenital heart defects, the clinical picture of the disease is determined by the specifics of these defects.
Treatment. Absolute reading To surgical treatment- presence and progression of symptoms of compression of the esophagus and trachea. Delay in the intervention leads to irreversible changes in the walls of the trachea and the operation does not work. During the operation, the PDA or arterial ligament is crossed, the left aortic arch, which is smaller in diameter, is bandaged or sutured and fixed with the help of the arterial duct stump to the sternum, thereby releasing the trachea and esophagus as much as possible (Krivchenya D.Yu., 1985; Belokon N. A., Podzolkov V.P., 1991). The immediate results of the operation are good, mortality is 3.8-7.1%, and long-term results are excellent (Burakovsky VI et al., 1996; Scharskop I., 1981).
Clinical observation
Patient Denis P., 11 years old, was in the pulmonology department of Children's hospital No. 19 in January 2003.
Main diagnosis: connective tissue dysplasia syndrome:
congenital heart disease, double aortic arch; mitral valve prolapse of the 1st degree with regurgitation of the 1st degree, regurgitation on the pulmonary valve of the 1st degree. False chord of the left ventricle of the heart, heart failure I FC;
anomaly of the large intestine (incomplete intestinal rotation, megadol ihosi gm a).
Complications: compression stenosis of the middle third of the trachea II degree. Secondary recurrent bronchitis. Chronic constipation.
Concomitant diagnosis: residual encephalopathy. Logoneurosis. Daytime enuresis
The child was admitted for a bronchological examination due to recurrent obstructive bronchitis, noisy breathing during exercise and while eating.
Anamnesis. Perinatal history without features. In the first months after birth, such manifestations of connective tissue dysplasia as umbilical hernia and hip dysplasia were found. The cry from birth was hoarse, breathing during exercise and during meals was noisy, which was associated with congenital stridor. From the second half of life, the child suffers from prolonged recurrent bronchitis, which occurs with an obstructive component or stridor breathing, respiratory failure,
degree II, which even necessitated hospitalization in the intensive care unit for intubation and sanitation of the tracheobronchial tree. After 5-7 years, he began to get sick less often (4-5 times a year), suffered from whooping cough, which was severe. Parents categorically refused bronchoscopic examination. From 8 months. The child has increased constipation with stool retention for up to 7-14 days. He was examined gastroenterologically, on the basis of which chronic colitis and dolichosigma were diagnosed.
Heredity. On the mother's side there are cases of stomach, lung, and breast cancer; the grandmother's brother and sisters have leukemia, the mother herself has dolichosigma, chronic constipation for up to 7-10 days. On the paternal side, the grandmother has pulmonary tuberculosis, the father has gastric ulcer , logoneurosis Physical examination. State of moderate severity Satisfactory nutrition The chest is not deformed, but the vascular network on the chest is pronounced Phenotypic signs of connective tissue dysplasia are expressed: flaccid posture, muscle hypoplasia, flat feet, joint hypermobility, “sandal-shaped” gap between the first and second toes, long toes Pulse is symmetrical, satisfactory filling in the arms and legs, blood pressure in the arms 100/60 mm Hg, in the legs 115/80 mm Hg Apex impulse of normal strength, heart boundaries are normal Distinct, rhythmic tones A 3rd degree systolic murmur is heard along the left edge of the sternum intensity, soft, occupying "/3-"/ systole, weakening in a standing position Tympanitis is pronounced above the lungs percussion, breathing is hard (after exercise - noisy), but there is no wheezing. The abdomen is distended, painless, the liver is not enlarged. Stool after 2-3 days, only after an enema and laxatives. Urinates enough, no swelling
No pathology was detected in clinical and biochemical tests of blood and urine
Electrocardiography. Sinus tachycardia, heart rate 97 beats/min Normogram Slowing of intra-atrial conduction Phenomenon of ventricular pre-excitation Disturbance of metabolic processes in the ventricular myocardium (Fig. 22 2) Echocardiography. The heart is formed correctly, the chambers are of normal size, the septa are intact, there is no myocardial hypertrophy. Systolic deflection of the anterior leaflet of the mitral valve of the 1st degree, regurgitation on the mitral and pulmonary valves of the 1st degree. Additional chord in the cavity of the left ventricle. Systolic and diastolic functions of the myocardium are normal. EF 68%, FU 37 % Aortic diameter 22 mm, opening 22 mm, pulmonary artery diameter 22 mm Suspicion of a right aortic arch
Chest X-ray. The pulmonary fields are swollen The chest is barrel-shaped The pulmonary pattern in the medial sections is strengthened, unclear, in the lateral sections it is depleted The roots are non-structural, probably due to hyperplasia of the lymph nodes The diaphragm is clear, the sinuses are free The heart is not expanded in diameter The arch of the aorta and its descending section are not differentiated (Fig. 22 3)
Computed tomography of the chest. A study with intravenous contrast revealed an anomaly of the thoracic aorta in the area of the arch, where the aorta forms a ring that compresses the trachea from the sides. The lymph nodes are not enlarged (Fig. 22 4)
Fiberglass bronchoscopy. The size of the trachea corresponds to age. In the middle third of the trachea, along the 12-14th cartilaginous rings, a decrease is determined

lumen by 2/, semicircular in shape, due to compression from the right anterolateral wall. In the area of prolapse, vascular pulsation is noted. Behind the compression zone in the lower third of the trachea there is a moderate prolapse of the membranous part. The cartilaginous pattern is embossed, the walls are of normal tone, without signs of dystonia. Conclusion: compression stenosis of the second degree, pulsating formation, of the middle third of the trachea.
Irrigography. With the introduction of two portions of a large amount of liquid barium suspension (about 2 liters), it was possible to partially fill the large intestine, up to the transverse section. All loops of the colon are located in the left half of the abdominal cavity, sharply dilated, but with preserved haustration. The ampulla of the rectum is sharply dilated. The cecum and ascending colon are located normally. Conclusion: intestinal malformation - incomplete rotation, megadolichosigma.

Rice. 22.3. Chest X-ray of Denis P., 11 years old.
Diagnosis: double aortic arch. The chest is barrel-shaped, the lung fields are swollen. The pulmonary pattern in the medial sections is enhanced and unclear, in the lateral sections it is depleted. The roots are unstructured, the diaphragm is clear, the sinuses are free. The heart is not expanded in diameter, but the aorta and its descending section are not contoured.

Rice. 22.4. Computer tomogram of Denis P., 11 years old.
Diagnosis: double aortic arch. A study with intravenous contrast reveals an anomaly of the thoracic aorta in the area of the arch, where the aorta forms a ring that compresses the trachea from the sides. Lymph nodes are not enlarged.
The child was consulted by a cardiac surgeon and further examination (angiography) and planned surgery to correct the defect were recommended. In the future, examination and treatment are planned in the proctology department.
The peculiarity of the case is the presence in a child with extremely unfavorable heredity of multiple defects and developmental anomalies associated with generalized connective tissue dysplasia. However, the defect that determined the severity of the condition was a double aortic arch, complicated by severe compression tracheal stenosis, recurrent tracheobronchitis and respiratory failure, requiring further examination in a specialized surgical hospital and urgent correction of the defect.

Double aortic arch (DAA) is one of the anomalies that forms a vascular ring and causes compression of the trachea and esophagus. The defect was first described by W. Hommel in 1773.
DDA represents the simultaneous existence of the right and left aortic arches (Fig. 47). The ascending aorta is located in front of the trachea, it divides into two arches, which then pass on either side of the trachea and esophagus. The common carotid and subclavian arteries depart from each arch; behind the esophagus, the arches connect to the descending aorta, which descends into the chest to the right or left of the midline. More often, the right (posterior) arch passes behind the trachea and esophagus, and the left (anterior) arch passes in front. Most often, with DDA, both arches are not closed, the right arch is wider and is located above the left, the open ductus arteriosus (or ligamentum arteriosus) and the descending aorta are located on the left (less often on the right); another option: one arch is not healed, the second (left) is atretic.
DDA usually exists as an isolated anomaly, but can be combined with tetralogy of Fallot, right-sided descending aorta, VSD, ASD, coarctation of the aorta, transposition of the great vessels.
The clinical manifestations of the defect are varied. With slight pressure on the esophagus and trachea, the only manifestation of the defect may be dull pain behind the sternum, some difficulty in breathing and swallowing solid food, in connection with which a tumor of the esophagus is suspected. In infants, the main manifestation of the vascular ring is stridor breathing from the moment of birth or shortly after it. Other causes of wheezing include laryngomalacia, previous intubation, choanal atresia, infection, and birth trauma. Usually, in children without DDA, stridor breathing goes away after 2-3 months, and in the presence of DDA, its clinical manifestations persist and progress. At the same time, shortness of breath, cough, attacks of suffocation, apnea, cyanosis, recurrent bronchopneumonia (due to aspiration of food), regurgitation, vomiting, dysphagia, and weight loss are observed. Children take a forced position on their side with their head thrown back (tracheal patency improves). Respiratory failure progresses over time and threatens the patient’s life [Krivchenya D. Yu., 1985]. During an objective examination, there are no changes in the heart, the sounds are loud, there is no murmur, except in cases with concomitant congenital heart disease.
DDA cannot be determined by a conventional radiograph, but it can sometimes be suspected by additional education, resembling a right-sided aortic arch. In the lateral projection, a narrowing of the trachea at the level of the aortic arch is visible.
D. Yu. Krivchenya (1985), observing and successfully operating on such children, considers the most highly informative diagnostic
ke

P - esophagus; Tr - trachea.

esophagography with the introduction of a water-soluble contrast agent (15-20 ml) into the esophagus through a probe. The use of fatty and barium-containing solutions has some danger due to the possibility of their aspiration and the development of asphyxia. On esophagograms in the lateral projection, compression deformations of the shadow of the esophagus, typical for the defect, are determined. Bronchoscopy reveals narrowing of the lumen and pulsation of the suprabifurcation segment of the trachea. Tracheography with a water-soluble contrast agent in the lateral projection reveals deformation and narrowing of the suprabifurcation part of the trachea. After the examination, the contrast agent must be carefully sucked out.
Aortography, as an examination method, remains in reserve, since it is not always informative, for example, if the vessels overlap each other or one of the arches is atretic.
Currently, two-dimensional echocardiography is used to identify the vascular ring. J. Huhta et al. (1986) examined 22 children of the first year of life with stridor and in 35% the cause of stridor was a vascular ring.
The authors believe that two-dimensional echocardiography should be the first method of evaluating children with stridor and respiratory problems.
Surgical treatment is indicated only in cases where there are clinical manifestations of compression of the esophagus or trachea. The purpose of the operation is to restore the normal function of these organs. During the operation, the ligamentum arteriosus or patent ductus arteriosus is isolated and transected, and a long stump is left on its left arch. The smaller diameter (left) arch is ligated, stitched and fixed with the stump of the ductus botallus to the sternum, thereby causing decompression of the trachea and esophagus [Krivchenya D. Yu., 1985].

Congenital aneurysm of the sinus of Valsalva (SVA) is a saccular or finger-like expansion or protrusion of the aortic wall of the sinus, often accompanied by a breakthrough into the presenting cavities of the heart. The first description of this pathology belongs to J. Nora (1839), and in the domestic literature - to F. M. Openhovsky (1894).
The frequency of congenital ASV is 0.1-3.5% of all congenital heart defects [Neklasov Yu. F. et al., 1975; Korolev B. A. et al., 1979; Meyer J. et al., 1975]. If by the end of 1975 the literature contained information about 220 such observations, by the end of 1985 about 350 had already been described.
Anatomy. A distinctive feature of congenital ASV is their saccular or finger-like shape and protrusion into adjacent parts of the heart. Most often, the aneurysm ends with one or more perforations leading to communication of the aortic root with the corresponding chamber of the heart. The dimensions of the aneurysmal sac range from 0.8 to 3 cm, the diameter of the perforation is from 0.2 to 1.8 cm.
The aneurysm may originate from any sinus of Valsalva, but most commonly (75-95%) from the right coronary sinus. The incidence of aneurysm localization in the non-coronary sinus is 5-25%. Aneurysms originating from the left coronary sinus are exceptionally rare.
There is a pattern of aneurysms rupturing in certain cavities of the heart. Aneurysms arising from the left or central part of the right coronary sinus usually rupture into the outflow tract of the right ventricle. If the aneurysm is located in the right part of the right coronary sinus, then in "/* cases it ruptures into the inflow tract of the right ventricle and is visible immediately below the septal cusp of the tricuspid valve, and in 3/4 of cases the rupture occurs in the cavity of the right atrium. Aneurysm arising from non-coronary sinus, as a rule, ruptures into the cavity of the right atrium and only sometimes into the cavity of the right ventricle.Aneurysms of the left coronary sinus, which are extremely rare, can rupture in the cavity of the right atrium, right ventricle and extracardially.
Among concomitant congenital heart disease, VSD should be noted, which usually occurs with aneurysms of the right coronary sinus; the defect is located above the supraventricular crest and below the pulmonary valve. The incidence of VSD with congenital ASV is 40-50%. Less common are ASD, PDA, coarctation of the aorta, aortic insufficiency, and pulmonary stenosis.
Hemodynamics. Unruptured ASV proceeds without hemodynamic disturbances. However, in a number of cases, bulging into the outflow tract of the right ventricle, it can create obstacles

obstructing the flow of blood into the trunk of the pulmonary artery and, extremely rarely, squeezing the conduction pathways of the heart, causing various types of rhythm disturbances.
Hemodynamic disturbances during aneurysm rupture are caused by the discharge of blood from the aorta into one or another chamber of the heart, but most often into the right ventricle or right atrium. Blood flows from the aorta constantly throughout the entire cardiac cycle, since the systolic and diastolic pressure in it is much higher than in the right parts of the heart. The volume of blood discharge depends on both the diameter of the perforation and pulmonary vascular resistance.
The discharge of blood from left to right leads to an increase not only in pulmonary blood flow, but also in the return of blood to the left atrium and left ventricle, therefore, volume overload occurs in both the right and left parts of the heart. The pressure in the pulmonary circulation is usually less than 70% of the systemic one. If high pulmonary hypertension and rapidly progressing heart failure develop soon after the aneurysm ruptures, then delay in surgery can be fatal.
Clinic, diagnostics. The majority (70-80%) of patients with congenital ASV are males. Aneurysm rupture most often occurs at the age of 25-40 years, but is also possible in children early age. Most often, aneurysm rupture occurs during sudden physical stress, although it is also possible at rest. Predisposing factors include bacterial endocarditis.
ASV without a fistula between the aorta and the heart cavity usually does not manifest itself in any way and is discovered accidentally during angiography or during surgery for some other concomitant heart defect or at autopsy.
If, after the rupture of an aneurysm, the communication between the aorta and the right parts of the heart is large, then heart failure develops and rapidly progresses; with small hole sizes it is absent or stopped drug treatment. According to the nature of the clinical course, all patients can be divided into two groups: a) with a gradual deterioration of the condition (when ASV is combined with other congenital heart defects, when hemodynamic disturbances have occurred since birth, but deterioration occurs when the ASV ruptures); b) with a sudden deterioration of the condition (usually occurs with an isolated form of the defect against the background of a rupture) [Burakovsky V.I. et al., 1987]. When an aneurysm ruptures, pain in the heart area, shortness of breath and palpitations appear. Often, patients can note not only the day, but also the hour of onset of the disease; less often, the onset is asymptomatic. Shortness of breath is caused by a sudden increase in pulmonary blood flow. and tachycardia and pain in the heart area - a decrease in coronary blood flow and cardiac output great circle blood circulation due to the discharge of blood into the right side of the heart.

Simultaneously with the appearance of shortness of breath and pain in the heart area, a long, machine-like systolic-diastolic murmur is heard over the heart area for the first time. Unlike PDA, with ASV, the noise is localized in the second and fourth intercostal spaces, on the left near the sternum - with a breakthrough into the right ventricle and above the center or to the right of the sternum in the third and fourth intercostal spaces with a breakthrough into the right atrium; the noise is more superficial and is often accompanied by trembling above chest determined by palpation. There is a moderate decrease in diastolic blood pressure (on average up to 40 mm Hg) and an increase in pulse pressure (on average up to 80 mm Hg).
The ECG does not have specific changes; signs of myocardial hypertrophy of both ventricles (more than the left) and the left atrium are determined. Sometimes manifestations of complete or incomplete atrioventricular block, junctional rhythm, blockade of the right bundle branch are found as a result of mechanical compression of these parts of the conduction system of the heart. Cases of ventricular and supraventricular tachycardia and syncope due to them have been described.
On FCG, a high-amplitude systolic-diastolic murmur is recorded, less often - only diastolic. On X-ray examination, the pulmonary pattern is enhanced due to the arterial bed, pronounced cardiomegaly is determined, caused by an enlargement of the right and left parts of the heart.
Echocardiography is of great importance in the examination of patients with DIA rupture. One-dimensional echocardiography does not reveal specific changes; there is volume overload syndrome of the left heart, an increase in the cavity of the right ventricle, and a tendency to expand the base of the aorta. With two-dimensional echocardiography (Fig. 48), you can directly see the aneurysm and the site of its breakthrough - chaotic dense echo signals are visible in the cavity of the right ventricle, a break in the echo signal in the area of the fistula. A protrusion of the right coronary sinus of Valsalva into the cavity of the pancreas is determined, at the apex of the aneurysm - the site of the breakthrough (indicated by the arrow in Fig. 48, a). In the diastolic phase, closed aortic valve leaflets are visible; The aneurysmically dilated right coronary sinus of Valsalva bulges into the cavity of the right ventricle, at its apex a hole is visible (indicated by the arrow in Fig. 48.6). In the systole phase, open cusps of the aortic valve, thickening of the right coronary cusp, and aneurysmal protrusion of the right coronary sinus are determined (Fig. 48, c). In the outflow tract of the pancreas, directly under the pulmonary valve leaflets, the upper part of the aneurysm is visible (Fig. 48, d).
Doppler cardiography determines turbulent systolic and diastolic blood flow in the chamber into which the aneurysm ruptured.
During catheterization of the cavities of the heart, a moderate degree is usually detected. pulmonary hypertension, high saturation rates

Rice. 48. Breakthrough of the aneurysm of the sinus of Valsalva into the right ventricle. Echocard diogram.
a - parasternal projection of the long axis of the left ventricle; b, c - parasternal projection of the short axis at the level of the aortic root; d - parasternal projection of the long axis of the outflow tract of the right ventricle.

supply of blood with oxygen in the right ventricle or right atrium, i.e. in the cavity where the aneurysm ruptured. When an aneurysm bulges into the outflow tract of the right ventricle, a systolic pressure gradient between the right ventricle and the pulmonary artery trunk is determined to be up to 10-40 mm Hg. Art.
Among various methods For angiocardiographic studies, preference is given to retrograde aortography. When a contrast agent is injected into the aortic root, a sac-like aneurysmal protrusion is contrasted, protruding into the outflow tract of the right ventricle or right atrium, through which the contrast agent enters the corresponding part of the heart. In a number of patients, the aortic valve leaflets sag, which causes the flow of contrast agent into the cavity of the left ventricle; this indicates concomitant aortic insufficiency.
Differential diagnosis is carried out with a PDA, VSD with aortic insufficiency, aortopulmonary septal defect, coronary arteriovenous fistula.
Course, treatment. As a rule, patients with ASV need surgical treatment, since the average life expectancy after a explosion is 1-2 years. Cases of sudden death due to rupture and cases where patients lived after rupture for up to 17 years have been described [Korolev B. A. et al., 1979]. Indications for surgery are hemodynamic disturbances caused by left-to-right shunting of blood. In case of rapidly progressing heart failure, urgent treatment should be sought. surgical intervention without waiting for the effect of drug treatment.
The first operations for ASV were performed by S. W. Lillehei (1957), in our country - by V. I. Burakovsky (1963).
Operations to eliminate ASV are performed under conditions of artificial circulation and moderate hypothermia. The basic principles of surgical correction of the defect: resection of the aneurysmal sac and elimination of the resulting hole, connection of the aortic media with the fibrous annulus of the aortic valve, correction of concomitant congenital heart disease. Approaches can be through the cavity into which the aneurysm ruptured, or transaortically. Using access through the right ventricle or right atrium, resection of the aneurysmal sac is performed, followed by suturing the resulting hole with separate sutures reinforced with pads, or closing it with a patch. With access through the aorta, depending on the diameter of the hole leading into the aneurysm, it is closed with separate sutures or a patch. The aortic valve is inspected and, if necessary, reconstructive surgery (leaf plication) or prosthetic replacement is performed.
Mortality after correction of the defect is 2.2-11.7%. Lethal outcomes are usually associated with the serious condition of patients undergoing surgery
against the background of rapidly progressing heart failure, the development of bacterial endocarditis, less often they occur during aortic valve replacement in children. At the Institute of Cardiovascular Surgery named after. A. N. Bakulev of the USSR Academy of Medical Sciences operated on 25 patients on May 1, 1985, of which two were fatal [Burakovsky V. I. et al., 1986].
Long-term results are usually favorable. In some cases, the development or exacerbation of bacterial endocarditis can lead to aortic insufficiency, requiring replacement of the aortic valve with a prosthesis.

Double aortic arch (DAA) is one of the anomalies that forms a vascular ring and causes compression of the trachea and esophagus. The defect was first described by W. Hommel in 1773.
DDA represents the simultaneous existence of the right and left aortic arches (Fig. 47). The ascending aorta is located in front of the trachea, it divides into two arches, which then pass on either side of the trachea and esophagus. The common carotid and subclavian arteries depart from each arch; behind the esophagus, the arches connect to the descending aorta, which descends into the chest to the right or left of the midline. More often, the right (posterior) arch passes behind the trachea and esophagus, and the left (anterior) arch passes in front. Most often, with DDA, both arches are not closed, the right arch is wider and is located above the left, the open ductus arteriosus (or ligamentum arteriosus) and the descending aorta are located on the left (less often on the right); another option: one arch is not healed, the second (left) is atretic.
DDA usually exists as an isolated anomaly, but can be combined with tetralogy of Fallot, right-sided descending aorta, VSD, ASD, coarctation of the aorta, transposition of the great vessels.
The clinical manifestations of the defect are varied. With slight pressure on the esophagus and trachea, the only manifestation of the defect may be dull pain in the chest, some difficulty breathing and swallowing solid food, which raises suspicion of a tumor of the esophagus. In infants, the main manifestation of the vascular ring is stridor breathing from the moment of birth or shortly after it. Other causes of wheezing include laryngomalacia, previous intubation, choanal atresia, infection, and birth trauma. Usually, in children without DDA, stridor breathing goes away after 2-3 months, and in the presence of DDA, its clinical manifestations persist and progress. At the same time, shortness of breath, cough, attacks of suffocation, apnea, cyanosis, recurrent bronchopneumonia (due to aspiration of food), regurgitation, vomiting, dysphagia, and weight loss are observed. Children take a forced position on their side with their head thrown back (tracheal patency improves). Respiratory failure progresses over time and threatens the patient’s life [Krivchenya D. Yu., 1985]. During an objective examination, there are no changes in the heart, the sounds are loud, there is no murmur, except in cases with concomitant congenital heart disease.
It is impossible to determine DDA from a conventional x-ray, but it can sometimes be suspected by an additional formation resembling a right-sided aortic arch. In the lateral projection, a narrowing of the trachea at the level of the aortic arch is visible.
D. Yu. Krivchenya (1985), observing and successfully operating on such children, considers the most highly informative diagnostic
ke

P - esophagus; Tr - trachea.

obstructing the flow of blood into the trunk of the pulmonary artery and, extremely rarely, squeezing the conduction pathways of the heart, causing various types of rhythm disturbances.
Hemodynamic disturbances during aneurysm rupture are caused by the discharge of blood from the aorta into one or another chamber of the heart, but most often into the right ventricle or right atrium. Blood flows from the aorta constantly throughout the entire cardiac cycle, since the systolic and diastolic pressure in it is much higher than in the right side of the heart. The volume of blood discharge depends on both the diameter of the perforation and pulmonary vascular resistance.
The discharge of blood from left to right leads to an increase not only in pulmonary blood flow, but also in the return of blood to the left atrium and left ventricle, therefore, volume overload occurs in both the right and left parts of the heart. The pressure in the pulmonary circulation is usually less than 70% of the systemic one. If high pulmonary hypertension and rapidly progressing heart failure develop soon after the aneurysm ruptures, then delay in surgery can be fatal.
Clinic, diagnostics. The majority (70-80%) of patients with congenital ASV are males. Aneurysm rupture most often occurs at the age of 25-40 years, but is also possible in young children. Most often, aneurysm rupture occurs during sudden physical stress, although it is also possible at rest. Predisposing factors include bacterial endocarditis.
ASV without a fistula between the aorta and the heart cavity usually does not manifest itself in any way and is discovered accidentally during angiography or during surgery for some other concomitant heart defect or at autopsy.
If, after the rupture of an aneurysm, the communication between the aorta and the right parts of the heart is large, then heart failure develops and rapidly progresses; if the hole is small, it is absent or can be treated with medication. According to the nature of the clinical course, all patients can be divided into two groups: a) with a gradual deterioration of the condition (when ASV is combined with other congenital heart defects, when hemodynamic disturbances have occurred since birth, but deterioration occurs when the ASV ruptures); b) with a sudden deterioration of the condition (usually occurs with an isolated form of the defect against the background of a rupture) [Burakovsky V.I. et al., 1987]. When an aneurysm ruptures, pain in the heart area, shortness of breath and palpitations appear. Often, patients can note not only the day, but also the hour of onset of the disease; less often, the onset is asymptomatic. Shortness of breath is caused by a sudden increase in pulmonary blood flow. and tachycardia and pain in the heart - by a decrease in coronary blood flow and minute volume of the systemic circulation due to the discharge of blood into the right side of the heart.

Simultaneously with the appearance of shortness of breath and pain in the heart area, a long, machine-like systolic-diastolic murmur is heard over the heart area for the first time. In contrast to PDA, with ASV, the noise is localized in the second and fourth intercostal spaces, on the left near the sternum - with a breakthrough into the right ventricle and above the center or to the right of the sternum in the third and fourth intercostal spaces with a breakthrough into the right atrium; the noise is more superficial and is often accompanied by tremors over the chest, determined by palpation. There is a moderate decrease in diastolic blood pressure (on average up to 40 mm Hg) and an increase in pulse pressure (on average up to 80 mm Hg).
The ECG does not have specific changes; signs of myocardial hypertrophy of both ventricles (more than the left) and the left atrium are determined. Sometimes manifestations of complete or incomplete atrioventricular block, junctional rhythm, blockade of the right bundle branch are found as a result of mechanical compression of these parts of the conduction system of the heart. Cases of ventricular and supraventricular tachycardia and syncope due to them have been described.
On FCG, a high-amplitude systolic-diastolic murmur is recorded, less often - only diastolic. On X-ray examination, the pulmonary pattern is enhanced due to the arterial bed, pronounced cardiomegaly is determined, caused by an enlargement of the right and left parts of the heart.
Echocardiography is of great importance in the examination of patients with DIA rupture. One-dimensional echocardiography does not reveal specific changes; there is volume overload syndrome of the left heart, an increase in the cavity of the right ventricle, and a tendency to expand the base of the aorta. With two-dimensional echocardiography (Fig. 48), you can directly see the aneurysm and the site of its breakthrough - chaotic dense echo signals are visible in the cavity of the right ventricle, a break in the echo signal in the area of the fistula. A protrusion of the right coronary sinus of Valsalva into the cavity of the pancreas is determined, at the apex of the aneurysm - the site of the breakthrough (indicated by the arrow in Fig. 48, a). In the diastolic phase, closed aortic valve leaflets are visible; The aneurysmically dilated right coronary sinus of Valsalva bulges into the cavity of the right ventricle, at its apex a hole is visible (indicated by the arrow in Fig. 48.6). In the systole phase, open cusps of the aortic valve, thickening of the right coronary cusp, and aneurysmal protrusion of the right coronary sinus are determined (Fig. 48, c). In the outflow tract of the pancreas, directly under the pulmonary valve leaflets, the upper part of the aneurysm is visible (Fig. 48, d).
Doppler cardiography determines turbulent systolic and diastolic blood flow in the chamber into which the aneurysm ruptured.
Catheterization of the cardiac cavities usually reveals a moderate degree of pulmonary hypertension, high saturation levels,

supply of blood with oxygen in the right ventricle or right atrium, i.e. in the cavity where the aneurysm ruptured. When an aneurysm bulges into the outflow tract of the right ventricle, a systolic pressure gradient between the right ventricle and the pulmonary artery trunk is determined to be up to 10-40 mm Hg. Art.
Among various methods of angiocardiographic research, preference is given to retrograde aortography. When a contrast agent is injected into the aortic root, a sac-like aneurysmal protrusion is contrasted, protruding into the outflow tract of the right ventricle or right atrium, through which the contrast agent enters the corresponding part of the heart. In a number of patients, the aortic valve leaflets sag, which causes the flow of contrast agent into the cavity of the left ventricle; this indicates concomitant aortic insufficiency.
Differential diagnosis is carried out with a PDA, VSD with aortic insufficiency, aortopulmonary septal defect, coronary arteriovenous fistula.
Course, treatment. As a rule, patients with ASV require surgical treatment, since the average life expectancy after a rupture is 1-2 years. Cases of sudden death due to rupture and cases where patients lived after rupture for up to 17 years have been described [B. A. Korolev et al., 1979]. Indications for surgery are hemodynamic disturbances caused by left-to-right shunting of blood. In case of rapidly progressing heart failure, urgent surgical intervention should be resorted to, without waiting for the effect of drug treatment.
The first operations for ASV were performed by S. W. Lillehei (1957), in our country - by V. I. Burakovsky (1963).
Operations to eliminate ASV are performed under conditions of artificial circulation and moderate hypothermia. The basic principles of surgical correction of the defect: resection of the aneurysmal sac and elimination of the resulting hole, connection of the aortic media with the fibrous annulus of the aortic valve, correction of concomitant congenital heart disease. Approaches can be through the cavity into which the aneurysm ruptured, or transaortically. Using access through the right ventricle or right atrium, resection of the aneurysmal sac is performed, followed by suturing the resulting hole with separate sutures reinforced with pads, or closing it with a patch. With access through the aorta, depending on the diameter of the hole leading into the aneurysm, it is closed with separate sutures or a patch. The aortic valve is inspected and, if necessary, reconstructive surgery (leaf plication) or prosthetic replacement is performed.
Mortality after correction of the defect is 2.2-11.7%. Lethal outcomes are usually associated with the serious condition of patients undergoing surgery
against the background of rapidly progressing heart failure, the development of bacterial endocarditis, less often they occur during aortic valve replacement in children. At the Institute of Cardiovascular Surgery named after. A. N. Bakulev of the USSR Academy of Medical Sciences operated on 25 patients on May 1, 1985, of which two were fatal [Burakovsky V. I. et al., 1986].
Long-term results are usually favorable. In some cases, the development or exacerbation of bacterial endocarditis can lead to aortic insufficiency, requiring replacement of the aortic valve with a prosthesis.