Depending on the length of the narrowing of the vessel, experts distinguish two varieties of coarctations:
- infantile – a segment of stenosis is observed in a significant area of the aortic walls and is accompanied by an open arterial duct;
- adults – a small portion of the aorta undergoes stenosis, but the ductus arteriosus is closed.
The severity of stenosis with this defect is different, and the severity of the coarctation course depends on this factor. The narrowing is determined by the results of imaging and invasive diagnostic techniques, these data are an important criterion for assessing the appropriateness of cardiac surgery.
Coarctation of the aorta in children in its progression goes through three stages:
- stage of latent arterial hypertension. Blood pressure in the vessels of the upper half of the body rises only after crying, feeding, changing clothes or physical activity;
- stage of transient disturbances. On the ribs of the baby there are patterns. These irregularities are the result of arising tortuosity and expansion of the costal arteries. When measuring blood pressure, hypertension is detected, which is observed after physical exertion, and at rest;
- stage of sclerosis of the aorta. Due to constant increased pressure, the walls of the artery are stretched, compacted and undergo connective tissue proliferation. Vascular sclerosis processes lead to the progression of circulatory disorders in the chambers of the heart and left ventricular hypertrophy.
The stage of sclerotherapy smoothly goes into complications. Particularly high risk in newborns. Stably progressive circulatory disorders lead to a deterioration in the supply of all systems and organs.
Like tetralogy of Fallot, coarctation can occur secretly or almost compensate for newborns, but with age it always leads to serious and life-threatening consequences, therefore it always needs modern and proper treatment. Even after performing a cardiac surgery correction consisting of two operations, aortic stenosis may recur in the future. Children with such an anomaly should be on a dispensary account all their lives to prevent complications.
Depending on the relationship with the arterial duct (see) Bonnet (L. M. Bonnet, 1903) identified two main types of K. a.
1. Children’s, or infantile, type, at Krom an arterial duct is open. A variant when the functioning arterial duct flows distal to the place of narrowing is called preductal K. a., And when the functioning arterial duct falls above the place of narrowing – postductal K. and.
2. Adult type To. And., At Krom an arterial duct is closed.
Fig. 1. Schematic representation of some options for coarctation of the aorta: a – postductal coarctation (arterial duct above the site of narrowing): 1 – aorta; 2 – brachiocephalic trunk; 3 – left common carotid artery; 4 – the left subclavian artery; the arrow indicates a narrowing of the aorta; b – preductal coarctation (arterial duct below the site of narrowing); c – postductal coarctation with a partially narrowed aorta; g – preductal coarctation with partial narrowing of the aorta; d – the arterial duct departs from the site of narrowing of the aorta; e – hypoplasia of the descending aorta.
The degree of aortic narrowing in the area of its isthmus varies widely: from atresia and complete closure of the aortic lumen to a small degree of narrowing (Fig. 1). Often, the aortic lumen, with its relatively normal outer diameter, can be closed by a membrane with an eccentrically located small hole or narrowed due to a sharply thickened cushion of the inner membrane.
The variety of forms K. and. and combinations with other congenital malformations led to the creation of numerous classifications. The most common classification developed by A. V. Pokrovsky (1966) at the Institute of Cardiovascular Surgery. A.N. Bakuleva: a) isolated K. a .; b) K. a. in combination with an open arterial duct; c) K. a. in combination with other congenital heart defects.
According to Wollmar (J. Vollmar, 1975), a combination of K. and. with other congenital heart defects (valvular defects, septal defects, open ductus arteriosus, etc.) occurs in 10% of cases.
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Pathogenetic mechanisms of circulatory disorders
A mechanical obstruction (gateway) to the blood flow in the aorta leads to the development of two modes of blood circulation. Above the obstacle Blood pressure is increased, the vascular bed is dilated, the left ventricle is hypertrophied. Below obstacles, blood pressure is reduced, blood flow compensation is due to the development of numerous ways of collateral blood supply. Depending on the degree of narrowing of the aorta, its extent, type of coarctation, the nature of hemodynamic disturbances and their severity vary widely.
In the adult type with an obstructed ductus arteriosus, the main hemodynamic disorders are associated with arterial hypertension in the vessels of the upper half of the body (see Hypertension arterial), and both systolic and diastolic blood pressure are increased. Arterial hypertension is accompanied by an increase in the mass of circulating blood and cardiac output, the work of the left ventricle is sharply increased.
In the vessels of the lower half of the body Blood pressure is reduced, especially the pulse pressure. The decrease in pulse pressure, in accordance with the ideas of Goldblatt (N. Goldblatt), leads to the inclusion of the renal mechanism of arterial hypertension and the mechanisms of secondary neuroendocrine hypertension (secondary aldosteronism).
The presence of a renal factor and subsequent neuroendocrine disorders allows us to consider the genesis of arterial hypertension during coarctation as quite complex, in addition to a mechanical obstruction of blood flow through the aorta, other mechanisms are also involved.
In the childhood type, arterial hypertension of the big circle may not be expressed, collateral circulation is poorly developed. At postductal K. and. there is a discharge of blood from left to right with hypertension of the pulmonary circulation. The preductal variant of coarctation is characterized by a discharge from right to left – from the pulmonary trunk through the open arterial duct into the descending aorta.
K. a. child type leads to impaired pulmonary circulation and in the vast majority of cases is complicated in early childhood by heart failure.
What complications does it lead to?
Coarctation of the aorta in children with severe course and in the absence of adequate cardiac surgery correction can cause the following dangerous consequences:
- arterial hypertension;
- acute circulatory disorders (strokes, heart attacks of other organs);
- cardiac asthma;
- pulmonary edema;
- bacterial or septic endocarditis;
- aneurysms on the walls of the aorta itself and its branches;
- kidney failure;
- calcification of the walls of the aorta;
- heart failure.
The result of all of the above complications may be the death of the child.
Clinical periods of aortic coarctation
on the hands Blood pressure is increased (systolic approx. 200 mmHg), on the legs it is reduced or not determined. Accordingly, the pulsation of the vessels of the neck is sharply enhanced and the pulsation of the arteries of the lower extremities is weakened or not determined. When examining patients 10-12 years of age and older, well-developed chest, shoulders and neck draw attention to themselves, while the lower extremities are relatively behind in development. The pulsation of collateral vessels in the intercostal space is visible to the eyes of some patients.
Palpation in patients older than 15-16 years of age is easily determined by the increased pulsation of arteries along the intercostal space, starting from III – V, especially in the lateral parts of the chest and from the back. The cardiac impulse is sharply amplified, systolic trembling in the II – III intercostal space is often determined. The borders of the heart are expanded due to the left departments.
During auscultation, systolic murmur above the apex, even rougher murmur above the aorta, amplification and emphasis of II tone above the aorta are determined. Rough systolic murmur is determined above the brachiocephalic vessels and from the back in the interscapular space. The presence of systolic-diastolic or “machine” noise indicates an open ductus arteriosus.
Wedge, picture K. a. in infants and in early childhood, they differ sharply. Initial symptoms occur shortly after birth or during the first month of life. Shortness of breath, cough, anxiety, pronounced general cyanosis or mainly on the legs, hypotrophy develops, symptoms of circulatory failure (enlargement of the liver, ascites, swelling on the legs) progress.
G. M. Soloviev and Yu. A. Khrimlyan (1968) distinguish four stages of the disease: 1) the stage of latent hypertension; in this period, hypertension manifests itself only with physical. load 2) the stage of transient changes, characterized by the initial manifestations of the defect in the form of a transient increase. Blood pressure both at rest and in Ch. arr. at physical load
during this period, the patient’s characteristic appearance is formed with K. a., rib ribs appear; 3) stage of sclerosis – persistently high blood pressure on the hands, a significant increase in the left ventricle, compaction and aortic sclerosis; 4) stage of complications; this is a late stage of the disease, when various complications from the cardiovascular system appear.
Complications at K.a. can be divided into three groups. The first group – complications associated with arterial hypertension: hemorrhages in the brain or spinal cord (paresis, paralysis); heart failure as a result of constant overload of the left and right heart; early atherosclerosis of cerebral vessels, coronary arteries of the heart.
The second group – complications associated with changes in the aorta and collaterals: the formation of aneurysms of the pre- or post-stenotic aorta; formation of an ascending aortic aneurysm and relative aortic insufficiency; the formation of aneurysm of the intercostal arteries; ruptures of aneurysms of the listed localizations.
The third group – complications caused by the joined infectious-allergic and dystrophic processes: endocarditis; endo-aortitis; calcification of the aortic wall.
In the clinical course of the disease, the following periods are usually distinguished:
- Critical. This period is limited to the first year of the child’s life, with the detection of a severe form of the disease in the clinical picture, manifestations of heart failure are observed along with frequent pneumonia. To save the life of a child requires immediate intervention – a surgical operation of coarctation of the aorta. With a mild course of the defect, systolic murmur in the heart of the newborn and the difference in blood pressure on the lower and upper limbs are noted. In this case, the operation is usually postponed until the child is 6-14 years old, due to the risk of early surgical intervention of recarctation at the correction site;
- Fixtures. The period lasts from 1 year to 5 years, in most cases there are minor manifestations of anomalies. With coarctation of the aorta in children at this time, headache, fatigue, shortness of breath can be detected, but in most patients, the disease often does not manifest itself;
- Compensation. In this period, approximately it lasts up to 14 years, children do not make any complaints about the disease, for the most part they normally develop and do not lag behind their peers in growth;
- Relative decompensation. The period covers the age of 15 to 20 years, and is marked by the appearance of complaints in patients about well-being, which is caused by a gradual increase in aortic narrowing, the development of arterial hypertension and myocardial stress. With a disease not previously diagnosed, patients in this period are extremely late, often seek medical attention for the first time about aortic coarctation. Many complaints are vague and do not always allow you to immediately determine the disease;
- Decompensation. The period begins with 20 years, with untimely access to doctors and without surgery for coarctation of the aorta, complications of arterial hypertension are noted. During this period, pathologies in the form of aneurysms, ruptures of the aorta and arteries of the brain and spinal cord, which can cause death at the age of 30-40, are possible.
The boundaries of the periods are extremely vague and can be adjusted.
To identify coarctation of the aorta and other congenital malformations in newborns, clinical recommendations suggest the following studies:
- chest x-ray;
- electro and phonocardiography;
- magnetic resonance imaging of the heart;
- aortography with contrast;
- heart sounding.
Analysis of the obtained data allows us to formulate a further strategy for the observation and treatment of the patient. Examination results in newborns may indicate the need for urgent intervention by surgeons – only such treatment saves the baby. Guided by diagnostic information, cardiac surgeons can choose the tactics of further correction necessary to save the life of the child.
The diagnosis is made on the basis of a set of wedges, manifestations of K. a .: hypertension syndrome with a pronounced gradient Blood pressure on the vessels of the upper and lower extremities and symptoms of developed collateral circulation (pulsation of collateral vessels, rib ribs according to x-ray data). In doubtful cases for the diagnosis it is necessary to carry out aortography (see). Other research methods are also used.
Electrocardiography (see) reveals large changes depending on the level of arterial hypertension, the age of the patient, the presence of concomitant defects. The electrical axis of the heart varies from the rightogram in infants to the levogram in adults, there are signs of left ventricular hypertrophy, conduction disturbances and coronary insufficiency.
Phonocardiography (see) reveals a systolic murmur of a fusiform shape and a high amplitude of II tone above the aorta. Noises can be fixed from the back and above the collaterals.
Volumetric sphygmography, like other methods of recording blood flow and pressure in the upper and lower extremities, reveals increased blood flow and increased numbers. Blood pressure in the vessels of the upper extremities, and in the vessels of the lower extremities, a decrease in Blood pressure (often not in absolute terms, but compared to Blood pressure of the vessels of the upper extremities) and volumetric blood flow.
X-ray examination reveals the aortic configuration of the heart with the expansion of the ascending aorta and a decrease or absence of a shadow of the aortic arch. At the level of Th3-5, there is a change in the contour of the aortic shadow in the form of a notch or “notch” resembling the number 3. This deformation corresponds to the site of narrowing of the aorta and is formed by two arches with a retraction between them.
The upper arch is formed by the enlarged left subclavian artery, the lower – by the post-stenotic site of the dilated aorta. When the esophagus is contrasted with a barium suspension, two impressions are noted on it due to the enlarged sections of the aorta above and below the site of narrowing. On radiographs in a direct projection, the patches of the lower edges of the posterior segments of the ribs, arising due to the pressure of the dilated intercostal arteries, are revealed.
Fig. 2. Aortogram of a patient with aortic coarctation (right oblique projection): a complete break in the shadow of the contrasted aorta in the isthmus (indicated by an arrow).
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