home→Diagnostics→ Right-sided aortic arch descending treatment. Right aortic arch: what is it, causes, development options, diagnosis, treatment, when is it dangerous? Anomalies and variants

Right-sided aortic arch descending treatment. Right aortic arch: what is it, causes, development options, diagnosis, treatment, when is it dangerous? Anomalies and variants

Thus, in the EG, at the end of the experiment, positive dynamics of static and general endurance, flexibility was 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 according to the developed methodology made it possible to identify positive dynamics in almost all studied indicators of the physical condition of female students of the experimental group within one year.

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

LITERATURE

1. Ilyinich V. I. Physical culture and life of a student. - 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. - S. 143-147.

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

4. Sapozhnikova Yu. I., Karpov V. Yu. Designing the content and methods of training sessions in physical education of correctional orientation with students of special medical groups // Uchen. app. un-ta im. P. F. Lesgaft. - 2008. - No. 8. - S. 41-44.

Contact Information

Pivovarova Elena Valentinovna - Senior Lecturer, Department of Physical Culture and Health, Volgograd State Medical University, e-mail: [email protected]

PRENATAL ULTRASOUND DIAGNOSIS OF DOUBLE AORTA 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

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

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

PRENATAL ULTRASOUND DIAGNOSIS OF DOUBLE AORTIC ARCH

N. A. Altynnik, Y. V Shatokha

A 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 a 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 anomaly of the aortic arch in which the ascending aorta passes into two arches - right and left. 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 higher than the left. The vascular ring, often small in 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 left of the spine.

The brachiocephalic vessels in DDA depart in separate four trunks: the right common carotid and subclavian arteries - from the right arch, the left ones - from the left. There are also other options for the discharge of brachiocephalic vessels in DDA.

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

Publications devoted to prenatal ultrasound diagnosis of DDA mainly began to appear only in last years thanks to the introduction of a slice assessment through three vessels and the trachea into the protocol for screening ultrasound in the second trimester of pregnancy. However, most of the published works describe from 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.

PURPOSE OF THE WORK

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

RESEARCH METHOD

An analysis of 6 cases of prenatal ultrasound diagnosis of DDA at 20-33 weeks of pregnancy was carried out. The age of pregnant patients ranged from 22 to 32 years, there were three primiparous patients, and three multiparous. The weight of newborns varies

she was from 3100 to 3400 g. The sex ratio (W: M) was 1: 1.

At each ultrasound examination, a four-chamber section of the fetal heart was assessed by transverse scanning of its chest and a section through three vessels and the trachea, also obtained by transverse scanning at the level of the output tracts of the ventricles.

The study of a four-chamber section of the fetal heart and the assessment of the 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 normative 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° (45° on average). The location of the thoracic descending aorta of the fetus 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, it was found that in the majority of fetuses with DDA (5/6, 83.3%), 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.

Only in one case, DDA was characterized by the presence of a parallel course of arches between which the trachea was visualized, which initially caused difficulties in accurately identifying DDA using traditional grayscale 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.

An 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 departed from the right dominant aortic arch. Only in one fetus, the aortic arches were equal, and two brachiocephalic vessels departed from them (Table).

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

No. Age, years Diagnosis time, 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 equivalent arcs 40 on the left

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

In four cases (66.7%), DDA was diagnosed 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 gestation 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, prenatal ultrasound diagnosis of DDA was established before 25 weeks of pregnancy in 66.7% of cases, of which up to 22 weeks of pregnancy - in 16.7% of cases.

It should be noted that in two cases (33.3%) of prenatal echographic diagnosis of DDA in the fetus during screening ultrasound in the third trimester of pregnancy (32-33 weeks), this anomaly was not correctly identified during 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 axis of the heart 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) in assessing the four-chamber section of the heart in cases of its double arch was registered in four (66.7%) of six fruits. Only in two cases (33.3%) was an abnormal (central) location of the cross section of the thoracic aorta.

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 95o, but at the same time, abnormal values of the location of the heart axis were recorded only in one (16.7%) of the six fetuses.

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

CONCLUSION

Thus, our studies and published results convincingly demonstrate

opine that screening ultrasound in the second and third trimesters of pregnancy with the study of the cut through three vessels and the trachea is highly informative for the identification of fetuses with DDA. Evaluation of the 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 heart axis were registered 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. Diagnosis, treatment tactics // Thoracic and cardiovascular surgery. - 2012. - No. 4. - S. 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. Bokeria. - M.: Medicine, 1996. - 768 p.

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

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

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

6. Achiron R, SimandS., 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 developmental options in which the branches of the aortic arch and two brachiocephalic trunks, the right and left, are connected.

The aortic arch connects to 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 departs from the aortic arch upward at the level of the sternoclavicular joint and is divided into two branches - the right carotid artery and the right subclavian artery. With some features of the anatomical development in humans, the inferior thyroid artery may depart 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 aortic coarctation.

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

The causes of the deformation have not been identified, but medical studies have shown that this anomaly begins during fetal 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;
Congenital deformity in which the aortic arch is sealed 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 vast majority of cases, with deformation of the aortic arch, circulatory disorders are not observed, but the load on the walls of the vessels increases and an aortic aneurysm may form.

With a serious deformation of the aortic arch, compression of the esophagus, trachea and nerve trunks can occur. For the treatment of deformity of the aortic arch, a special medication course is prescribed, followed by surgical intervention.

Diseases of the aortic arch

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 aneurysm of the aortic arch are damage to the vessels of the heart and brain, headache, pain syndrome in chest, shortness of breath, strong pulsation in the chest, paresis of the recurrent nerve.

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

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

The causes of aortic arch hypoplasia 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.

In case of serious disorders caused by aortic hypoplasia, a surgical operation is performed. After the operation, a partial correction of heart disease and other cardiovascular diseases is carried out. Then appointed drug treatment glycosides and diuretics.

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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.

With the pathology of this blood vessel, serious therapeutic measures are often required.

About anatomy and topography

The aorta is the main trunk of the arteries in big circle circulation. It begins 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 handle of the sternum and the fourth thoracic vertebra. The stroke of the arc in this case is back and to the left. Then it spreads through the top of the left bronchus, where the already descending part of the aorta begins.

Conventionally, 2 parts are distinguished in the structure:

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

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

Branches of the aortic arch depart from its middle part upwards. All of these arteries supply the upper half of the body, including the brain.

Anomalies, defects and diseases

The pathology of the blood vessel can be divided into 2 large groups:

In the first case, violations occur at the stage of embryogenesis. It depends on hereditary predisposition, the action of aggressive factors in the early stages of pregnancy. Changes can be found in other parts of the aorta. If such a situation arose, then they speak of combined and combined defects.

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

Congenital defects and anomalies include:

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

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

And from the acquired diseases, the middle part is affected by:

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

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

Brief characteristics of individual species

Hypoplasia is a uniform tubular narrowing. This limitation of 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 vices. Most of these patients die at an early age. Treatment is surgical only.

Atresia or interruption 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 such a defect without surgical intervention die in the first month of life.

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

When Kinking syndrome is detected in children, doctors choose expectant tactics. 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.
Abnormal origin of the right subclavian artery:

distal;
proximal.

Coarctation with double aortic arch.

The narrowing may be localized, but usually consists of a pathological process spread over several cm. Often associated with other congenital anomalies. Included in the tetrad of Fallot, Turner's syndrome. The defect is revealed from birth.

With adequate medical 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 in the position, size, shape, course, ratio and continuity of arterial vessels. Such defects are most often asymptomatic.

Complaints appear with pronounced changes and the spread of the anomaly to the proximal part of the descending section. Perhaps the appearance of dysphagia or respiratory phenomena due to the close pathological contact of the aortic arch and its branches with the trachea, esophagus.

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

The most common 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 then all merge with the descending artery behind the esophagus.

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

Acquired vices

Of the secondary lesions of the blood vessel, the most important are:

In the first case, the lumen narrows due to the formation of "fatty" plaques. It is easily diagnosed due to the compacted underlined contour of the vessel during ultrasound of the heart, radiographs of the lungs.

Adherence to the principles of proper nutrition and rational pharmacotherapy help slow down the process, prevent complications.

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

For a long time, pathology may not show itself. When involved in the process of the ascending or descending aorta, big size aneurysms appear first symptoms.

The main method of treatment is surgical. The treatment regimen before surgery necessarily includes drugs that reduce arterial pressure to prevent dissection or rupture of the aneurysm.

Top main symptoms

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

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

These complaints are due to the involvement in the pathological process of the main branches of the middle part of the aorta. What kind of disease or defect takes place, only a doctor can establish.

For 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 of its system can lead to serious consequences, death.

Therefore, it is important to undergo a 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 right aortic arch in the fetus is a congenital heart disease that can occur in isolation or in combination 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 in the course of evolution. Thus, the formation of the aorta as an integral vessel occurs only in vertebrate animals, 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 there is an outflow of arterial blood mixed with venous, or completely arterial blood.

In the process of individual development of the embryo (ontogenesis), the formation of the aorta undergoes as complex changes as the heart itself. Starting from the first two weeks of embryo development, there is an increased convergence of the arterial trunk and venous sinus located in the cervical part of the embryo, which subsequently migrated more medially towards the future chest 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 arc gives rise to the internal carotid arteries that feed the brain,
the fourth arch gives rise to the aortic arch and the so-called "right" part,
the fifth arc is reduced,
the sixth arc gives rise to the pulmonary trunk and the arterial (Botallov) duct.

Fully four-chamber, with a clear division of the cardiac vessels into the aorta and the pulmonary trunk, the heart becomes by the sixth week of development. A 6-week-old 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 originates from the aortic bulb in its ascending part, which, in turn, originates from the left ventricle. That is, the ascending aorta passes into the arch approximately at the level of the second rib on the left, and the arch goes around the left main bronchus, heading backwards and to the left. The uppermost part of the aortic arch projects onto the jugular notch just above the top 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 laying of human vessels from the gill arches of the embryo, they speak of a right-sided aortic arch. In this case, the aortic arch is thrown through the right main bronchus, and not through the left, as it should be normal.

Why does vice occur?

Any malformation is formed in the fetus if a woman during pregnancy is influenced by negative environmental factors - smoking, alcoholism, drug addiction, ecology and adverse radiation background. However, an important role in the development of the heart in a child is played by genetic (hereditary) factors, as well as existing chronic diseases in the mother or past infectious diseases, especially in early 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 gestational age of approximately 2-6 weeks is especially vulnerable to the fetal heart, since it is at this time that the formation of the aorta occurs.

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 anomaly of the duct, 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 normal after childbirth) is located behind the esophagus and trachea,
The right aortic arch with the formation of a vascular ring, code arterial ligament, or open 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 the connective ligament, but by the inflow of the vessel.

Figure: a variety of options for atypical aortic arch structure

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 defect, without other developmental anomalies (in this case, if the right-sided aorta is not combined with the DiGeorge syndrome, which is 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 disease - in particular Fallot's tetrad (dextraposition of the aorta, ventricular septal defect, pulmonary stenosis, right ventricular hypertrophy).

Tetralogy of Fallot, combined with the right arch - an unfavorable variant of development

How to recognize vice?

Diagnosis of the defect is not difficult even during the period of gestation. This is especially true in cases where the right aortic arch is combined with other, more severe anomalies in the development of the heart. Nevertheless, to confirm the diagnosis, a pregnant woman is repeatedly examined, including on expert class ultrasound devices, a council of geneticists, cardiologists and cardiac surgeons is assembled to decide on the prognosis and the possibility of delivery in a specialized perinatal center. This is due to the fact that for some types of defects, combined with the right aortic arch, a newborn child may need 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 in any way, only sometimes accompanied by frequent obsessive hiccups in a child. In the case of combination with Fallot's tetrad, which accompanies the defect in some cases, the clinical manifestations are pronounced and appear on the first day after birth, such as increasing pulmonary heart failure with severe cyanosis (blue skin) in an infant. That is why Fallot's tetrad is referred to as "blue" heart defects.

What screening shows a defect in pregnant women?

Additionally, the analysis of fetal DNA can clarify the absence of a connection between the formation of the right-sided aorta and severe genetic mutations. In this case, chorionic villus material or amniotic fluid is usually taken 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 a child, the defect does not require surgical treatment. It is enough just a monthly examination by a pediatric cardiologist with regular (once every six months - once a year) ultrasound of the heart.

When combined with other malformations of the heart, the type of surgical intervention is selected based on the type of defects. So, with Fallot's tetrad, an operation is shown in the first year of a child's life, which is carried out in stages. At the first stage, palliative (auxiliary) shunting is performed between the aorta and the pulmonary trunk to improve blood flow to the pulmonary circulation. At the second stage, open heart surgery is performed using a heart-lung machine (AIC) in order to eliminate stenosis of the pulmonary trunk.

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

Forecast

The prognosis for an isolated right-sided aortic arch is favorable, since in most cases surgery is not even required. So, in general, we can say that the 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 disease. 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. The reasons. Treatment options. Effects.

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 increased 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 an isolated form or in combination are presented:

branching anomalies of the brachiocephalic vessels;

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

an increase in 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 arc come 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 rings;

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

ductus-dependent anomalies of the arch, 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 examining 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 arc:

retroesophageal left subclavian artery;

with retroesophageal diverticulum;

with left 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 specular 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, a typical right-sided mirror arrangement of the arch with a left-sided duct or ligament does not form a vascular ring. This variant accounts for 27% of aortic arch anomalies in frequency. 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 a mirror-image right-sided aortic arch has a left-sided ductus arteriosus or ligament extending from the right-sided descending aorta behind the esophageal diverticulum. This variant forms a vascular ring and is not accompanied by other congenital malformations. 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, therefore it is diagnosed during examination for concomitant CHD.

By itself, the right-sided arc does not require intervention. However, under 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 classical surgery, 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 originates directly from the arch. Even with a Gore-Tex vascular prosthesis, 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 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 reduced pulse amplitude or reduced pressure on 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 CHD surgery, the ductus arteriosus is closed to eliminate pulmonary steal. Surgical ligation or occlusion of the ductus arteriosus using catheter technology, as well as reimplantation of the subclavian artery into the aorta, may be required in the presence of brain symptoms or retarded development of the left arm.

Cervical aortic arch

The cervical aortic arch is a rare anomaly in which the arch is 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 behind the esophagus and heads to the left, giving rise to the left subclavian artery and sometimes the ductus arteriosus. This type, in turn, is subdivided into a subtype, in which the internal and external carotid arteries depart from the arch separately, and a subtype, in which there is a two-carotid trunk, when both common carotid arteries depart from one vessel, and both subclavian arteries depart separately from the distal arcs. In each of these subtypes, the vertebral arteries arise separately from the arch. While most patients with the opposite descending aorta have a vascular ring formed by the aortic arch on the right, the retroesophageal segment of the aorta posteriorly, the arterial ligament on the left, and the pulmonary artery anteriorly, only half of them manifest Clinical signs rings.

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

The second type is characterized by a left-sided aortic arch. Narrowing created 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 orifice of the left subclavian artery sometimes occurs in both types.

The cervical aortic arch is manifested by a pulsating formation in the supraclavicular fossa or on the neck. In infants, before the appearance of pulsation, signs characteristic of the vascular ring are found:

recurring respiratory infections.

Adults usually complain of dysphagia. Patients with stenosis or atresia of the left subclavian artery and a branching of a unilateral vertebral artery distal to the obstruction may leak blood from arterial system brain with neurological symptoms.

In the presence of a pulsating formation on the neck, the presumptive diagnosis can be made by the disappearance of the pulse on femoral artery with short-term pressing of a pulsating formation.

The cervical aortic arch must be differentiated from a carotid or subclavian aneurysm to avoid inadvertently ligating an aortic arch mistaken for a carotid aneurysm. Diagnosis can be suspected on a plain radiograph showing an enlarged superior mediastinum and the absence of a round shadow of the arch. Anterior displacement of the trachea supports the diagnosis.

In the past, angiography was standard method diagnosis and in the presence of intracardiac anomalies will remain so. However, without comorbidity, the diagnosis of cervical aortic arch can be established by echocardiography, CT, and MRI.

Surgical intervention is necessary for cervical arch hypoplasia, a clinically manifested vascular ring, or an aneurysm of the arch. The nature of the operation depends on the specific complication. With 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 combined with the CHD and is an accidental finding that does not have clinical significance. Atresia or rupture 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 cause of hospitalization.

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 to 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 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.

A 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 depart, including the left subclavian artery. This feature of the origin of the brachiocephalic arteries is the main sign of the 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 a V persistent arch that has an anatomical connection with the pulmonary artery, echocardiography, angiography and MRI can detect a vessel extending 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.

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.

With the pathology of this blood vessel, serious therapeutic measures are often required.

About anatomy and topography

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

ascending;
middle;
descending.

Conventionally, 2 parts are distinguished in the structure:

concave;
convex.

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

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

Branches of the aortic arch depart from its middle part upwards. All of these arteries supply the upper half of the body, including the brain.

Anomalies, defects and diseases

The pathology of the blood vessel can be divided into 2 large groups:

Congenital.
Acquired.

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

Congenital defects and anomalies include:

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

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

And From acquired diseases, the middle part is affected by:

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

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

Brief characteristics of individual species

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

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

When Kinking syndrome is detected in children, doctors choose expectant tactics. 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.
Abnormal origin of the right subclavian artery:

distal;
proximal.

Malformations of the aortic arch system

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

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

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

Acquired vices

Of the secondary lesions of the blood vessel, the most important 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, pathology may not show 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 necessarily includes drugs that lower blood pressure to prevent delamination or rupture of the aneurysm.

Top main symptoms

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

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

These complaints are due to the involvement in the pathological process of the main branches of the middle part of the aorta. What kind of disease or defect takes place, only a doctor can establish.

For 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 of its system can lead to serious consequences, death.

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

Anatomical classification

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

branching anomalies of the brachiocephalic vessels;

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

an increase in 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 rings;

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

ductus-dependent anomalies of the arch, 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 arc:

mirror arrangement;

retroesophageal left subclavian artery;

with retroesophageal diverticulum;

with left descending aorta.

Right-sided arch with specular 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

Cervical aortic arch

The cervical aortic arch is a rare anomaly in which the arch is 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 behind the esophagus and heads to the left, giving rise to the left subclavian artery and sometimes the ductus arteriosus. This type, in turn, is subdivided into a subtype, in which the internal and external carotid arteries depart from the arch separately, and a subtype, in which there is a two-carotid trunk, when both common carotid arteries depart from one vessel, and both subclavian arteries depart 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 annulus formed by the aortic arch on the right, the retroesophageal aorta posteriorly, the ligamentum arteriosus posteriorly, and the pulmonary artery anteriorly, only half of them show clinical signs of an annulus.

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

Diagnosis

recurring respiratory infections.

Adults usually complain of dysphagia. Patients with stenosis or atresia of the left subclavian artery and a branching of the unilateral vertebral artery distal to the obstruction may experience blood leakage from the arterial system of the brain with neurological symptoms.

In the presence of a pulsating formation on the neck, a presumptive diagnosis can be made by the disappearance of the pulse on the femoral artery with a short-term pressing of the pulsating formation.

In the past, angiography was the standard diagnostic modality and will remain so in the presence of intracardiac abnormalities. However, without comorbidity, the diagnosis of cervical aortic arch can be established by echocardiography, CT, and MRI.

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.

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 rupture 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 cause of hospitalization.

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.

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

Most congenital heart defects (CHDs) are diseases that endanger the health or life of a newborn. The experience of Russian and foreign colleagues shows that the frequency of congenital heart defects ranges from 10 to 13 cases per 1000 newborns, of which 4:1000 are complex congenital heart diseases. In our country, the average incidence of CHD ranges from 8-9 to 11-13 cases per 1000 live births. Up to 50% of newborns die from cardiac pathology in early infancy.

The distribution of CHD diagnostics depending on the nosological form varies significantly. Certain congenital heart diseases can be detected by prenatal diagnosis in 90% of cases. Statistics figures inexorably show a low percentage of CHD detection (30%) based on the results of a screening study. In specialized institutions, the frequency of detection of CHD is higher and reaches 54%.

The accuracy of ultrasound diagnostics of fetal pathology in recent years has been repeatedly criticized by obstetrician-gynecologists and, to a greater extent, pediatricians. Sometimes clinical signs are of interest to specialists much less than the data of an antenatal ultrasound examination of the fetus.

This paper presents an analysis of the possibilities of ultrasound prenatal examination in aortic coarctation. The work takes into account the data available in the publications of domestic and foreign authors, as well as the material of the department functional diagnostics Center.

The Latin term "coarctatius" means "narrowed, constricted". It was first described by J.F. Meckel in 1750. Coarctation of the aorta is a pathological narrowing of its lumen that can occur anywhere along its entire length. The frequency of the defect is 6.3%, according to clinical observations from a sample of 2000 cases, and 8.4% according to the results of pathological material.

In children of the first year of life, aortic coarctation ranks fourth among CHD. As a rule, it develops distal to the branch of the left subclavian artery near the arterial duct (ductus arteriosus). Depending on the localization of the narrowing of the aorta relative to the arterial duct, two classical types of coarctation are distinguished: preductal and postductal.

If there is narrowing of the aorta clinical picture may increase like a flurry after the closure of the ductus arteriosus. During the first year, 56% of patients die. Most death is caused by a combination of coarctation with other cardiac anomalies. The isolated form in the first weeks and days of life is also characterized by a high mortality rate (34%). If children survive this period, then life expectancy is on average 30-50 years. The cause of death in newborns is cardiopulmonary failure. With high arterial hypertension, as an exception, there may be a cerebral hemorrhage.

In FGBU " Science Center obstetrics and perinatology IN AND. Kulakov "Ministry of Social Development of the Russian Federation for a two-year period (2010-2011) 27 children were born with aortic anomalies, from a slight narrowing to a complete break. In 15 observations in the department of functional diagnostics, the diagnosis was made antenatally. These were pregnant women who applied at a later date from 35 to 39 week of gestation. In all cases, aortic pathology was combined with left ventricular hypoplasia. The diagnosis was confirmed in all cases. Isolated aortic coarctation was diagnosed in 6 cases during this period. In 3 of them, the diagnosis was made antenatally, in 2 - narrowing was detected in newborns on the 3-5th day after the closure of the ductus arteriosus.In one case, aortic kinking was diagnosed after birth.Thus, our data indicate the complexity and low percentage of detection of isolated aortic coarctation.Only in those cases where there are pronounced hemodynamic disorders leading to significant anatomical changes in the form of hypoplasia and left ventricle, disproportion of the ventricles of the heart, an increase in the diameter of the pulmonary artery, the diagnosis can be made antenatally with great certainty.

The first work on the diagnosis of coarctation of the aorta in the fetus dates back to 1984. According to the literature, transvaginal access at 12-15 weeks of gestation makes it possible to diagnose aortic coarctation in 21.4% of cases. With transabdominal access, the frequency of detection of aortic coarctation increases: at 16-30 weeks it is 43%, after 30 weeks, as hemodynamic disorders progress, it does not exceed 54%.

According to the results of a screening ultrasound examination of 20,248 fetuses at 19-22 weeks of gestation in a large region of Germany (1990-1994), the prenatal diagnosis of aortic coarctation was not established in any of the 7 cases. Similar data were obtained in Norway, even in a specialized center for prenatal diagnosis. According to a multicenter analysis conducted in 12 European countries, an accurate prenatal diagnosis of isolated aortic coarctation in the late 1990s was established only in 9 (15.8%) of 57 cases. The average time to detect isolated coarctation was 22 weeks, with 7 of 9 cases detected before 24 weeks. When coarctation of the aorta was combined with extracardiac defects and chromosomal abnormalities, the accuracy of its prenatal diagnosis was significantly higher due to the severity of hemodynamic disorders and extended echocardiographic examination of the fetus and amounted to 52%.

According to the Institute of Cardiovascular Surgery. A.N. Bakulev, the diagnostic accuracy does not exceed 27%. As a rule, pathology is most often noted when aortic coarctation is combined with other cardiac anomalies.

Thus, summarizing the statistical data of the Center's material, publications of leading Russian experts and foreign colleagues, one can note a very low percentage of aortic coarctation diagnosis in the fetus.

What is the reason for such a low percentage of detection of coarctation of the aorta antenatally?

There are several factors that make it difficult to diagnose aortic coarctation antenatally.

The first cause of undiagnosed coarctation of the aorta is the morphogenesis of the pathology and the theory of the primary developmental disorder of the aortic arch. The theory was proposed in 1828. The author believes that this pathology is associated with insufficient connection of the 4th and 6th aortic arch with its descending aorta. Therefore, the isthmus is the place where the narrowing site is most often localized, since both sections are formed from different embryonic rudiments. Normally, the area of the isthmus of the aorta is equal to 2/3 of the diameter of the ascending aorta (Fig. 1).

According to the "ductal tissue theory", coarctation of the aorta occurs as a result of the migration of smooth muscle cells of the duct into the preductal aorta, followed by constriction and narrowing of the aortic lumen. During the formation of coarctation of the aorta in the isthmus, a narrowing of various lengths and shapes persists (Fig. 2, 3). Most often, this manifests itself in the form of a local constriction, above or below which the aortic diameter remains normal.

Rice. 2. Ultrasound procedure ascending aorta, arch and descending aorta. The arrow indicates the location of the narrowing.

Rice. 3. Three-dimensional image of the heart and aorta of the fetus at 16 weeks of gestation. The arrow indicates the site of narrowing of the aorta. AO DESC - descending aorta, COR - heart.

Therefore, the assessment of narrowing of the aorta in a typical place as a sign of developing pathology is not always possible in the early stages of pregnancy. Since we are talking about preductal coarctation of the aorta (narrowing of the aorta is localized proximal to the duct), it is obvious that during intrauterine life, at a later date, there is a decrease in blood flow through the left heart and isthmus of the aorta and hypoplasia of the left ventricle and aorta develops. This contributes to the later manifestation of the pathology and the possibility of its ultrasound diagnosis.

The second reason that makes it difficult to diagnose aortic coarctation is the theory of postnatal development. In the fetus, the aortic isthmus is narrow and after birth and closure of the ductus arteriosus, it should normally dilate to provide adequate blood flow to the descending aorta. Thus, the existing physiological narrowing of the aortic isthmus does not suggest the formation of aortic coarctation after birth.

Postductal coarctation develops after birth and is almost always an isolated defect. This anomaly is most likely the result of an overgrowth of the muscular tissue of the ductus arteriosus into the fetal aorta. When the duct narrows after birth, the ectopic tissue inside the aorta also contracts, blocking, like a tampon, the lumen of the vessel along its entire circumference. Unlike preductal coarctation, aortic hypoplasia does not develop in this case. Closure of the arterial duct in some children leads to the presence of narrowing of the aorta and the development of coarctation 2-3 weeks after birth.

The third reason for undiagnosed aortic coarctation is the peculiarities of intracardiac hemodynamics in the fetus. The blood circulation of the fetus, as well as that of an adult, is subject to a number of basic laws of physical and biological effects. The flow of blood moves from the area high pressure into an area of low pressure. With a pronounced flow, the dimensions increase, with a decrease in the volume of the flow, they decrease. So, hypoplasia of the left ventricle and coarctation of the aorta often accompany defects with reduced blood ejection into the aorta (aortic stenosis, ventricular septal defect) and practically do not occur in defects with a large volume of flow through the aorta (with tetralogy of Fallot).

Based on the characteristics of fetal intracardiac hemodynamics, indirect signs of pathology in the antenatal period were proposed for the diagnosis of aortic coarctation: dilatation of the right ventricle and its hypertrophy (see Fig. 3), dilatation of the pulmonary artery. The authors believe that these are reliable signs and they occur quite often (in 18 out of 24 cases of verified diagnoses of aortic coarctation). Therefore, an assessment of the indices of the ratio of the cavity of the right ventricle to the left ventricle (in the norm - 1.1) and the pulmonary artery to the aorta (in healthy people - 1.2) was proposed. Based on the increase in these parameters, one can assume the presence of obstructed ejection into the aorta or hypoplasia of the aortic arch. However, according to the authors who proposed these indirect signs, such changes are detected only in 30% of observations (Fig. 4).

Rice. four. Disproportion of the size of the ventricles of the heart in coarctation of the aorta. RV - right ventricle; LV - left ventricle; AO DESC - cross section of the descending aorta.

In the second half of the 90s. Several papers have been published on the diagnostic value of right ventricular enlargement and left ventricular reduction in aortic coarctation. The authors who published these results believe that the earlier the study is carried out (from 14-16 to 25 weeks of gestation), the more informative it is for diagnosing aortic coarctation. The data obtained by D. Brown et al. can serve as evidence. . In the presented studies, coarctation of the aorta was noted in 8 (62%) of 13 fetuses with a disproportion in the size of the ventricles up to 34 weeks and only in 6 (21%) of 29 fetuses after 34 weeks of pregnancy. Despite the fact that more than half of cases of coarctation of the aorta is accompanied by a disproportion in the size of the ventricles, which is quite easily detected when studying a four-chamber section of the fetal heart, it was not diagnosed in many studies in Norway, even with a mandatory screening study of heart size before 18 weeks of gestation.

Such contradictory results can be explained by the hemodynamic theory. In utero, 50% of the total blood ejection enters the ascending aorta, 65% enters the descending aorta, and only 25% through the aortic isthmus. Based on the reduced volume of blood flow, its physiological narrowing occurs, which is aggravated only after birth.

Dopplercardiography, which allows assessing blood flow, is informative only with severe narrowing of the aorta. In this case, an inverse relationship arises between the maximum blood flow velocity in the ascending and descending aorta. Normally, the speed in the ascending section slightly prevails over the speed of the descending section.

When the aortic arch is interrupted, there is a retrograde blood flow in the filling of the aortic arch area after the interruption through the open ductus arteriosus and the absence of direct blood flow at the level of the interruption.

And finally, the last reason for undiagnosed aortic coarctation is the frequency of association with other cardiac anomalies. Isolated coarctation of the aorta is only 15-18%. According to M. Campell and P. Polani, the percentage of combination of aortic coarctation with cardiac anomalies ranges from 13 to 18%. Most often (85%), coarctation of the aorta is combined with bicuspid aortic valve, often combined with endomyocardial fibroelastosis, extending to the output tract of the left ventricle and to the interventricular septum. Perhaps, with coarctation of the aorta, an abnormal origin of the right subclavian artery or both subclavian arteries from the descending aorta, distal to the coarctation (5%).

The presence of concomitant cardiac pathology can neutralize the hemodynamic changes in the fetal heart typical for coarctation, which the specialist must pay attention to during ultrasound examination.

There is also the concept of pseudocoarctation, or "kinking" of the aorta - deformity of the aorta, similar to classical coarctation, but the obstruction to blood flow is insignificant, since there is a simple elongation and tortuosity of the aorta (Fig. 5).

Rice. 5. Kinking of the aorta in a fetus at 34 weeks of gestation. Research in the energy mode.

So, it becomes obvious that practically none of the ultrasound signs in the antenatal period has high degree diagnostic accuracy.

It is known that the diagnosis of this heart disease is based on a direct sign - visualization of the site of narrowing of the aorta, and, possibly, expansion of the proximal aorta. However, it is quite difficult to clearly visualize the area of narrowing of the aorta in the fetus and it is possible only in single observations. The defect can be seen only when there is a decrease in the diameter of the isthmus of the aorta by more than 1/3 compared with the norm for each period of pregnancy (see Fig. 2, 3).

The key to prenatal diagnosis of coarctation of the aorta is a comprehensive accounting of data obtained both in the study of a four-chamber section of the heart (dilatation of the right ventricle, hypoplasia of the left ventricle), and in the assessment of the main arteries themselves. In the presence of coarctation, the diameter of the aorta is on average 2 times smaller than the diameter of the dilated pulmonary artery. Therefore, for the diagnosis of coarctation of the aorta, visualization of the aorta itself should be used, which is most appropriate for screening assessment of the cut through three vessels. Diagnosis can be aided by measuring the diameter of the ductus arteriosus, which is wide in coarctation of the aorta.

The final diagnosis of coarctation of the aorta is established when using a cut through the aortic arch, since most often the narrowing of the aorta is noted in the area of its isthmus - at the level of the confluence of the arterial duct. It should be noted that the narrowing can cover a large area of the aorta up to tubular hypoplasia. The normal transition of the aortic arch to the ductus arteriosus is normally smooth and smooth. In addition, when examining the aorta along the long axis, hypoplasia of the transverse part of the aortic arch should be considered the most reliable sign. As one of the indirect signs of coarctation of the aorta, one can use the tortuosity of the aortic arch when studying it along the long axis.

An important additional value is Doppler echocardiography, which, in case of coarctation of the orta, makes it possible to reveal the acceleration and turbulent nature of the blood flow in the aorta. However, in the antenatal period, this symptom may not appear. It is more reliable to identify a left-right shunt through the foramen ovale. It is assumed that this shunt is a compensatory mechanism that reduces blood flow to the left ventricle and aorta. G. Sharland et al. noted a left-right shunt through the foramen ovale in 7 of 12 fetuses with aortic coarctation. The remaining 5 fetuses showed both left-right and right-left directions of blood flow through the foramen ovale.

In cases of pronounced anatomical changes in the aorta and left ventricular function with increased afterload, heart failure may occur. Hemodynamic disorders and heart failure are characterized prenatally by the presence of effusion in the pericardium, a decrease in cardiac output with a decrease in the size of the left ventricle.

After the birth and closure of the ductus arteriosus, the entire flow of blood enters the ascending aorta and the isthmus expands to a normal level. After birth, when the duct is blocked, coarctation of the aorta leads to serious clinical manifestations.

So, an analysis of the possibilities and errors of ultrasound diagnosis of aortic coarctation in the prenatal period shows how problematic it is to make a diagnosis in all cases of this heart disease in the fetus. Unfortunately, all echocardiographic antenatal signs of pathology do not provide a high percentage of diagnosis.

We would like to hope that the development of new technologies and the accumulation of positive and negative experience will allow in the future to increase the percentage of diagnosis of such a complex heart disease with high neonatal mortality.

Literature

Bronshtein M., Zimmer E.Z. Sonographic diagnosis of fetal coarctation of aorta at 14-16 weeks of gestation // Ultrasound Obstetric Gynecologie. 1998. N 11. R. 254-257.
Allan L.D., Cook A.C., Huggon I.C. Fetal echocardiography. A practical guide. Cambridge University Press. 2009. P. 258.
Frederiksen T. Coarctation of the aorta. A genetic study // Acta genet.1963. V. 13. R. 263.
Buncle G. Congenital heart disease and large vessels. M.: Medicine, 1980.
Oztunc F., Eroglu A.G., Aksoy F. et al. Antenatal diagnosis of posductal coarctation of the aorta report // Turk journal Pediatr. 2001. V. 43. N 1. P. 67-69.
Allan L.D., Crawford D.C., Tynan M. Evolution of coarctation of the aorta in intrauterine life // Br. Heart J. 1984. V. 52. P. 471.
Allan L.D., Crawford D.C., Anderson R.H. et al. Echocardiography and anatomical correlations in fetal congenital heart disease // Br. Heart J. 1984. V. 52. P. 542.
Franklin O., Burch M., Manning N. et al. Prenatal diagnosis of coarctation of the aorta improves survival and reduces morbility // Heart. 2002. V. 87. N 11. P. 67-69.
Norbenger L.K., Sahn D.J., Kleimann C.S. et al. Antenatal diagnosis of coarctation of the aorta: a multicenter experience // J. Am. Coll. cardiology. 1994. V. 23. P. 417-423.
Bespalova U.D., Suratova O.G., Tyumeneva A.I., Gasanova R.M. Ultrasound diagnostics congenital heart defects in the fetus / Ed. Bokeria L.A. M., 2009.
Rosenberg H. Coarctation of the aorta; Morphology and pathogenesis considerations. In: Perspectives in Pediatric Pathology. V. 1. Chicago, Year Book, 1974.
Patten B.M. The changes in circulation after birth // Am. Heart J. 1930. V. 6. P. 192.
Burakovsky V.I., Bokeria L.A. Cardiovascular surgery. M.: Medicine, 1989.
Allan L.D., Chita S.K., Anderson R.H., Fagg N., Crawford D.C., Tynan M.J. Coarctation of the aorta in prenatal life: and echocardiographic, anatomical and functional study // Brit. Heart J. 1988. V. 59. P. 356.
Brown D.L., Durfee S.M., Hornberger L.K. Ventriculal disgrepancy as a sonographic sign of coarctation of the aorta: how reliable is it? // J. Ulytrasound Med. 1997. V. 16. N 2. P. 95-99.
Rudolf A., Heyman M.A., Spitznas U. Hemodynamic considerations in the development of narrowing of the aorta // Am. J. Cardiolog. 1972. V. 30. P. 514.
Palmer N.S., Berman M.A. Postnatal development of obstruction in coarctation of the aorta; Role of the ductus arteriosus // Pediatrics. 1975. V. 56. P. 462.
Sharland G.K., Chan K.Y., Allan L.D. Coarctation of the aorta:difficulties in prenatal diagnosis // Br. Heart J. 1994. V. 71. P. 70-75.
Campell M., Polani P.E. Etiology of coarctation of the aorta // Lancet. 1961. V. 1. P. 473.
Edwards J.E., Burchell H.B. The pathologic anatomy of deficiency betwen the aortic rood and the heart including aortic sinus aneuvrysms // Thorax 1957. V. 12. P. 125.
Rudolph A.M. Congenital Diseases of the Heart: Clinical-Physiologic Considerations in Diagnosis and Management. Chicago Year Book. 1974.
Kleimman C.S., Donnerstein R.L., De Vore G.R. et al. Fetal echocardiography for evaluation of in utero congestive heart failure: A technique for study of nonimmune fetal hydrops. N. Engl. J. Med. 1982.