Defect of an aortolegochny partition – the congenital warm anomaly consisting available messages between the ascending aorta and the pulmonary artery adjoining to it. Defect of an aortolegochny partition is shown at early age by short wind, lag in physical development, repeated pneumonia, formation of "a warm hump", cyanosis. Detection of defect of an aortolegochny partition is carried out on the basis of these EhoKG, a kateterization of heart, an aortografiya, the ECG, FKG, a X-ray analysis, an auskultation. At this defect cardiac treatment - closing of an aortolegochny window by an ushivaniye or plasticity of defect is shown.
Defect of an aortolegochny partition
Defect of an aortolegochny partition (aortolegochny window, aortopulmonalny septalny defect, aortolegochny fistula or fenestration) is caused by existence of an opening by means of which the aorta and a trunk of a pulmonary artery are reported among themselves. Defect of an aortolegochny partition – the rare anomaly making 0,2−0,3% of cases of all congenital heart diseases in cardiology.
Localization of the pathological message can be various: defect of an aortolegochny partition can settle down on any site between adjacent departments of the ascending aorta and a trunk of a pulmonary artery. Defects of an aortolegochny partition differ in a form and size and can reach diameter from 2 to 30 mm. Almost in half of cases defect of an aortolegochny partition is combined with other congenital anomalies of heart – defect of an interventricular partition, an open arterial channel, Fallo's tetrad, a koarktatsiy aorta, a subvalvate stenosis of an aorta, steyonozy a pulmonary artery, etc.
Reasons of defect of an aortolegochny partition
The Aotrolegochny window is a consequence of violation of an embryogenesis as a result of which the full-fledged aortolegochny partition is not formed. Division of an embryonic arterial trunk into an aorta and a pulmonary trunk happens between 6-7 weeks of pre-natal development of a fruit. Violation of this process leads to emergence of defect of an aortolegochny partition.
The reasons of development of defect of an aortolegochny partition are similar to that when forming other congenital heart diseases. As the factors breaking formation of heart of a fruit at early stages of pre-natal development infections of the pregnant woman (a rubella, chicken pox, herpes, flu, syphilis, etc.), alcohol intake and toxic medicines, somatic diseases of mother (diabetes), the pathological course of pregnancy (threat of spontaneous interruption, severe toxicoses), heredity, ionizing radiation and so forth can act.
Features of haemo dynamics at defect of an aortolegochny partition
Morphological criteria of defect of an aortolegochny partition are: the pathological message between related departments of the ascending aorta and a pulmonary trunk, existence of the isolated fibrous rings of aortal and pulmonary valves and the created output departments of both ventricles. The last sign distinguishes this defect from the general arterial trunk. Defect in an aortolegochny partition represents an oval or roundish opening with an intra pericardiac arrangement between the left wall of the ascending aorta and the right wall of a pulmonary trunk.
Expressiveness of violation of haemo dynamics at defect of an aortolegochny partition depends on the size of the message, the place of its arrangement, a ratio of all-pulmonary and peripheral vascular resistance.
At defect of an aortolegochny partition blood under big pressure comes from the ascending aorta to a pulmonary artery. The expressed arteriovenozny (left-right) dumping early leads to development of pulmonary hypertensia which is present at most of patients with defect of an aortolegochny partition. When resistance in a small and big circle of blood circulation is leveled, there is a cross, and then and venoarterialny (right-left) dumping of blood that is followed by development of cyanosis.
Classification of defect of an aortolegochny partition
Taking into account criteria of localization, a form of an opening and its relation to pulmonary arteries distinguish the following anatomic types of defect of an aortolegochny partition (V. I. Burakovsky and ., NTsSSH of A. N. Bakulev):
The I type – defect has the channel form with well expressed poles; it is located in the middle of an aortolegochny partition from Valsalva's sine at a distance. The left and right pulmonary arteries depart distalny aortolegochny defect.
The II type – defect has the window form; it is located in a proximal (lower) part of an aortolegochny partition over Valsalva's sine. The Aortolegochny partition partially a sformirvoana in the disteel part; branches of a pulmonary artery depart normally, distalny defect.
The III type - defect has the window form; it is located in a disteel (top) part of an aortolegochny partition from Valsalva's sine at a distance. The Aortolegochny partition partially a sformirvoana in the proximal part. Branches of a pulmonary artery depart in a defect top zone.
The IV type – defect has the window form; it is located in a disteel (top) part of an aortolegochny partition, at the level of an otkhozhdeniye of the right branch of a pulmonary trunk. At this type of defect of an aortolegochny partition the aorta is reported both with the right pulmonary artery, and with the general pulmonary trunk.
The V type – total absence of an aortolegochny partition, however valves of an aorta and pulmonary artery are created and separated. Pulmonary arteries depart from the general main vessel originating from the heart basis.
Symptoms of defect of an aortolegochny partition
At children of the first months of life as an early symptom of defect of an aortolegochny partition serves short wind on type . Lag in physical development, bystry fatigue, frequent SARS, repeated pneumonia is characteristic.
At a stage of primary arteriovenozny dumping integuments of physiological coloring or a little pale. Small cyanosis can develop at shout, crying, the phenomena of heart and respiratory failure. At development of cross and venoarterialny dumping of blood cyanosis of skin and visible mucous becomes expressed and to constants. Deformation of a thorax as "a warm hump" which formation is connected with increase in the right ventricle working in the hyperfunction mode is found in 70% of patients with defect of an aortolegochny partition.
At defect of an aortolegochny partition of small diameter and at children the long time remains satisfactory condition. At big defects pulmonary hypertensia early develops. Life expectancy of patients with defect of an aortolegochny partition depends on the sizes of an opening and makes from several months to 30-40 years (on average - 14 years).
Diagnostics of defect of an aortolegochny partition
At survey of patients with defect of an aortolegochny partition lag in the weight and growth, protrusion of a thorax in the form of a warm hump is noted. Perkutorno comes to light expansion of limits of warm dullness in both parties; at an auskultation systolic noise with a diastolic component, change of warm tones is listened; palpatorno systolic trembling in the II-III mezhreberye is defined.
By means of a phonocardiography the auskultativny picture stated above will be recorded. The electrocardiography reveals signs of an overload of the right departments of heart, a hypertrophy of both ventricles, EOS deviation to the right. The radiological picture at defect of an aortolegochny partition is typical: increase in a shadow of heart, expansion of a shadow of an aorta and pulmonary artery, strengthening of the pulmonary drawing during the early period of a disease and its impoverishment at development of pulmonary hypertensia. By means of an echocardiography defect in an aortolegochny partition is found, the window arrangement, its sizes is established, the accompanying heart diseases are excluded.
By sounding of cavities of heart expressiveness of pulmonary hypertensia, blood dumping size is defined, extent of saturation of blood by oxygen in a pulmonary artery is investigated. A diagnostic sign of defect of an aortolegochny partition is the possibility of carrying out a catheter from a pulmonary artery through an opening in initial departments of the ascending aorta. When performing an aortografiya hit of contrast substance from the ascending aorta in a trunk of a pulmonary artery is visible. Differential diagnostics at defect of an aortolegochny partition is carried out with DMZhP, the general arterial trunk opened by an arterial channel.
Treatment of defect of an aortolegochny partition
As spontaneous closing of defect of an aortolegochny partition is impossible, the only way of treatment of congenital heart disease is cardiac operation. At big defect of an aortolegochny partition operation is carried out in early terms - till 1 year.
Closing of an aortolegochny window is made by an ushivaniye or plasticity of defect by means of a patch by accesses through a gleam of an aorta or a pulmonary artery, or on both sides. Operation is carried out in the conditions of IK, a hypothermia and a pharmakokholodovy kardioplegiya.
At defect of an aortolegochny partition of the I type bandaging of a channel is made. The impossibility of performance of radical operation at pulmonary hypertensia dictates need of performance of two-stage intervention: 1 - narrowing of a pulmonary trunk or pulmonary artery; 2 - removal of a suyozhivayushchy manzhetka and radical operation.
The intraoperative lethality makes 3%; postoperative reaches 15-20%.
The forecast at defect of an aortolegochny partition
The big extent of defect of an aortolegochny partition causes the malignant course of defect: 25-30% of patients perish within the first half a year of life. As causes of death of patients with defect of an aortolegochny pereyogorodka usually serve heart failure, a rupture of aneurism of a pulmonary artery in a cavity of a pericardium, tamponad of heart (haemo pericardium), a thrombembolia, a bakteriyoalny endocarditis.
At the survived patients lack of surgical treatment leads to development of irreversible changes in the lungs making impossible surgical treatment in later terms. In literature single instructions on the patients with defect of an aortolegochny partition who lived up to 35−40 years meet.