Double arch of the fetal aorta ultrasound. Anomalies of the aortic arch and brachiocephalic vessels in children

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 crosses 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.

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.

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 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.

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, 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) .

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 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 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 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.

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.

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.

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 revealed elements of ductus arteriosus tissue.

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

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. At IType the left subclavian artery arises as 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.

II Type characterized by mirror compared to normal

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.

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

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.

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:

vascular rings;

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:

mirror arrangement;

retroesophageal left subclavian artery;

with retroesophageal diverticulum;

with left-sided descending aorta.

Right-sided arch with mirror origin of brachiocephalic vessels

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 carotid;

left innominate artery.

Diagnosis

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.

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 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.

Diagnosis

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.

Treatment

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.

Diagnosis

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

Now we can consider the most important anomalies, exciting derivatives of the aortic arches. When the right and left fourth arches and roots of the dorsal aorta are preserved, a defect usually called the aortic annulus occurs. As the upper segments of the aorta shorten, this ring encloses the trachea and esophagus so closely that it interferes with swallowing and often requires surgery to relieve the pressure exerted by the ring.

Usually this is done ligation of one of the arches. If, as often happens, one of the arches turns out to be significantly smaller than the other, the operation does not present any difficulties.

One of the most common clinically important deviations in the structure of the aortic arch there is an anomaly in which the right subclavian artery departs from the aortic arch. In this case, the segment of the right dorsal aortic root located caudal to the origin of the subclavian artery is preserved, and not the fourth aortic arch and part of the dorsal aortic root located cranial to the origin of this artery.

After shifting places origin of the subclavian arteries in a cranial direction resulting from the caudal movement of the heart and aortic roots, this malformed right subclavian artery eventually begins to branch off from the aortic arch. Since its proximal part originates from the dorsal aortic root, it must cross the midline dorsal to the esophagus. However, it can also exert pressure on the esophagus, which interferes with the act of swallowing, although this anomaly usually leads to less severe consequences than in the case of the aortic ring.

In persons with the arch extending in this way aorta by the right subclavian artery The recurrent nerve of the larynx runs almost transversely from the vagus nerve to the region of the larynx. It does not have the usual loop because, along with the portion of the right sixth aortic arch located distal to the origin of the pulmonary artery, the right fourth arch also disappears after the subclavian artery uses the caudal segment of the dorsal aortic root as its proximal part.

Another serious anomaly in aortic arch region is to preserve as the main vessel passing to the dorsal aorta, the right fourth aortic arch and the right root of the dorsal aorta instead of the left ones. By itself, such an arrangement of vessels does not create functional complications similar to those that occur in the cases described above. Nevertheless, the possibility of such a condition should be taken into account when clarifying radiological data and during surgical operations in this area.

Clinical symptoms

Varieties described in the literature

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

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

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

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 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.

Why does vice occur?

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?

What screening shows a defect in pregnant women?

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 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.

Anomalies of the aortic arch and brachiocephalic vessels in children

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

Type II is characterized by mirror compared to normal

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.

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.

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, 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, but in childhood it is easy. 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. 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.

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, which exerts pressure along the right contour of the esophagus or causes its circular narrowing at 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 of various diseases and lesions of the esophagus, accompanied by dysphagia: displacement of the esophagus (indicated by an arrow) with a right-lying aortic arch.

Chapter 22 DOUBLE AORTIC ARCH

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 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 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.

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 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

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.

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 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.