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Pulmonary Atresia

Typically, there is a lack of normal communication between the ventricles of the heart and the pulmonary artery. The literature data indicate significant variability of the defect among newborns - from 0.0065 to 0.02%. Among all CHD, the proportion of ALA ranges from 1.1 to 3.3%, increasing among critical CHD to 6.3%.

This pathology is determined in two main options:

• atresia of the pulmonary artery with DMS;

• atresia of the pulmonary artery with an intact interventricular septum (described in the next section). In addition, pulmonary atresia can be one of the components of other complex CHD (a single ventricle, Ebstein's anomaly, the full form of the atrioventricular canal, tricuspid atresia, corrected TMA, etc.).

Atresia of the pulmonary artery in combination with DMS

The frequency is about 0.07 per 1000 newborns, 1% among all CHD and about 3.5% among critical CHD. Characteristically, there is no connection of the right ventricle with the pulmonary artery, and the excretory section of the right ventricle ends blindly. The vice is also inherent in large DIC, a single aortic valve, aortic dextroposition of varying degrees. Blood in the vessels of the lungs comes from the aorta through a functioning OAI or large aorto-pulmonary collateral arteries. The best known classification of the defect is based on the level of atresia of the pulmonary artery.

Atresia of the pulmonary valve.

Atresia of the pulmonary valve and pulmonary trunk.

Atresia of the pulmonary valve, pulmonary trunk and one of the pulmonary arteries.

Atresia of the pulmonary valve, trunk and both pulmonary arteries (the lungs are supplied with blood only due to the collateral arteries).

Hemodynamics. With this pathology, all of the blood from the right (via DMS) and the left ventricle enters the ascending aorta. As a result, arterial hypoxemia occurs, the degree of which is inversely proportional to the value of pulmonary blood flow. In turn, pulmonary blood flow is determined by the diameter of the OAA or large aorto-pulmonary collateral arteries. In most cases, the diameter of these vessels is small and hypoxemia quickly reaches a critical degree.

The natural course. The intrauterine development of the fetus usually does not undergo significant changes, since the oxygen content in the systemic and coronary channels does not depend on blood flow through the lungs. Arterial hypoxemia develops after birth with the separation of blood circulation circles. The lethal outcome is usually associated with severe hypoxia, which is the result of the closure of the OAP or the progressive narrowing of the collateral arteries. Up to 6 months, about 62% of patients survive.
The total mortality among the operated and non-operated patients, even with the current level of cardiac surgery, is high and reaches 25% within 1 year of life.

Clinical symptoms. The main symptom of the defect is central cyanosis. Atresia of the pulmonary artery is a typical ductus-dependent pathology, and a sharp increase in cyanosis, anxiety or lethargy of the child, as well as loss of consciousness indicate the closure of OAP. Dyspnea-cyanotic seizures, as with tetralogy of Fallot, does not occur. Since there is no blood flow through the pulmonary valve, there may be a “noiseless” picture of the defect. With a functioning OAP there is a soft blowing noise. With large collaterals, you can listen to systolic or systolodiastolic murmur, localized to the left or right of the sternum and conducted on the back.

Instrumental research methods

• The electrocardiogram is normal with the usual deviation of the electrical axis of the heart to the right.

• Chest x-ray: pulmonary pattern is often depleted; with large OAP or collaterals, it can be normal or even strengthened, often asymmetric. The size of the heart is moderately increased, the arc of the pulmonary artery sinks.

• Echocardiography: reveal a large DMS, the only way out of the right ventricle; there is no blood flow through the pulmonary valve. The ascending aorta is expanded. Depending on the type of atresia, the presence or absence of a pulmonary trunk and branches is determined. In most cases, OAP can be detected as a source of blood supply to the pulmonary artery.

• Cardiac catheterization and angiocardiography are more informative, due to the possibility of a detailed analysis of the sources of blood supply to the lungs, the size and confluence of the pulmonary arteries, etc.

Treatment. Basic therapy aims to reduce the body's oxygen requirements and correct metabolic disorders. It is possible to achieve an increase in pulmonary blood flow by means of an infusion of group E prostaglandins that support the patency of OAP. It should be remembered that oxygen inhalation is contraindicated, as it can provoke the closure of OAP.

With progressive cyanosis and small sizes of the pulmonary arteries, a palliative operation is performed aimed at increasing pulmonary blood flow and creating the prerequisites for the growth of pulmonary vessels. The most common operation is a modified subclavian-pulmonary anastomosis created using a synthetic prosthesis. Radical correction of the defect is performed at an older age and in most cases a multi-stage approach is used.

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Pulmonary Atresia

  1. Atresia of the pulmonary artery with intact ventricular septum
    The pathology frequency is about 0.06-0.07 per 1000 newborns, 1-3% among all CHD, 3-5% among critical CHD. For this variant of the defect, normally formed atria and concordant atrioventricular connections are characteristic; interventricular septum intact. There is no exit from the right ventricle: approximately 75% of cases are due to the complete fusion of the valves
  2. Abnormal discharge of the left coronary artery from the pulmonary artery
    SYNONYMS Bland-White-Garland Syndrome (ALCAPA in English). DEFINITION An abnormal discharge of the left coronary artery from the pulmonary artery is a congenital pathology of the heart, in which the trunk of the left coronary artery begins from the pulmonary artery system. The disease is accompanied by severe myocardial dysfunction and proceeds with the clinical picture of DCMP. EPIDEMIOLOGY Frequency of discharge
  3. Pulmonary Embolism
    General information Pulmonary embolism is caused by embolism from the pulmonary circulation veins entering the pulmonary artery. Emboli can be blood clots (blood clots), fat, tumor cells, air, amniotic fluid, and foreign particles. The most common cause of embolism is blood clots from veins of the lower extremities (almost always they come from veins located above the knee), pelvic veins
  4. Pulmonary catheterization
    Indications Indications for pulmonary catheterization are expanding as more and more anesthetists master this technique (Table 6-2), and the American Society of Anesthetists has developed guidelines and protocols for pulmonary artery catheterization. Although in many groups of surgical patients, the effectiveness of monitoring by a pulmonary catheter is
  5. Pulmonary embolism
    Pulmonary embolism (pulmonary embolism) is a severe complication that is often not diagnosed during life. The probability of its occurrence in certain groups of patients is very high. Predisposing factors: prolonged immobilization (especially in the elderly and senile age), heart disease, circulatory failure, shock, ARF, burns, injuries (most often a hip fracture). To contributing factors
    Pulmonary embolism (pulmonary embolism) is a severe complication that is often not diagnosed during life. The probability of its occurrence in certain groups of patients is very high. Predisposing factors: prolonged immobilization (especially in the elderly and senile age), heart disease, circulatory failure, shock, ARF, burns, injuries (most often a hip fracture). To contributing factors
  7. Pulmonary embolism
    Pulmonary thromboembolism (pulmonary embolism) - occlusion of the lumen of the main trunk or branches of the pulmonary artery with an embolus (thrombus), leading to a sharp decrease in blood flow in the lungs. ETIOLOGY AND PATHOGENESIS Emboli from the venous system of the pulmonary circulation are carried by a blood stream to the arteries of the pulmonary circle, causing them to become clogged, which will lead to an increase in pressure in the pulmonary artery pool (up to
  8. Critical pulmonary stenosis
    A pathology that creates obstruction of exit from the right ventricle. The frequency of isolated pulmonary stenosis is 0.08-0.36 per 1000 newborns, 4-6% among children with CHD, 1.3% among children with critical CHD. In newborns, it manifests itself as critical valve stenosis with an opening on the verge of atresia and moderate hypoplasia of the right ventricle. Hemodynamics Obstacle exit right
  9. Pulmonary embolism
    Pulmonary embolism (pulmonary embolism) is a blockage of the main or middle trunk, small vascular trunks of the pulmonary artery, leading to increased pressure in the pulmonary circulation, right ventricular failure. Predisposing factors Diseases of the cardiovascular system - atherosclerosis, veins of the lower extremities, pathology of organs and vessels of the small pelvis. Postoperative pulmonary embolism in
    Increased cardiac impulse, systolic trembling at the site of attachment of the II - III ribs to the sternum on the left; gross systolic murmur at the site of attachment of II - III ribs to the sternum on the left; electrocardiographic signs of right ventricular hypertrophy; phonocardiographic - intense rhomboid systolic murmur in the II intercostal space to the left of the sternum; radiological - depletion of the vascular network of a small
    Kenneth M. Moser (Kenneth M. Moser) According to epidemiological studies in the United States, more than 50,000 people die directly from pulmonary embolism (pulmonary embolism) every year. However, it is estimated that only about 10% of all cases of pulmonary embolism are fatal. In this regard, the total number of pulmonary thromboembolism (both fatal and non-fatal) diagnosed during
  12. Isolated pulmonary stenosis.
    Undivided valve leaflets lead to a narrowing of the valve opening, a decrease in blood flow into the pulmonary circulation and overload of the right ventricle. Less commonly observed narrowing of the output section of the right ventricle. Compensation of hemodynamic disturbances occurs due to the lengthening of the phase of expulsion of the right ventricle. CLINIC AND DIAGNOSTICS. The patient is troubled by shortness of breath. Cyanosis only appears
  13. Pulmonary embolism
    With pulmonary embolism (pulmonary embolism), the ECG displays dramatically changed conditions of intracardiac hemodynamics, specifically, overload of the right heart, which is manifested by several electrocardiographic options: 1. The first ECG option is SI-QIII-TIII syndrome. 2. The second ECG option - acute hypertrophy of the right heart. 3. The third ECG option - acute
  14. Pulmonary thromboembolism (code 126)
    Definition Thromboembolism of the pulmonary artery and its branches (pulmonary embolism) is caused by an embolism, the source of which is blood clots from the veins of the pulmonary circulation or the right heart. Statistics. Pathological damage accounts for 2-3% of the population. These figures can be underestimated, since intravital and even morphological diagnosis of pulmonary embolism in small branches of the pulmonary artery is difficult. IN
  15. Pulmonary embolism
    Pulmonary embolism (pulmonary embolism) is a blockage of the arterial bed of a lung with a thrombus formed in the venous system, right atrium, or right ventricle. Pulmonary embolism is considered to be one of the most severe and catastrophic acute vascular diseases accompanied by high mortality. In the vast majority of patients (more than 90%), pulmonary embolism is the source of pulmonary embolism
  16. Pulmonary embolism
    DIAGNOSTICS Massive pulmonary embolism is manifested by sudden cardiac arrest (electromechanical dissociation) or shock with severe shortness of breath, tachycardia, pallor of the skin or sharp cyanosis of the upper half of the body, swelling of the cervical veins, angina-like pain, ECG signs of an acute pulmonary heart. Non-massive PE is manifested by shortness of breath, tachycardia, arterial hypotension, signs
  17. Pulmonary embolism
    Embolism is a blockage of a blood vessel. Pathophysiology • Pulmonary embolism occurs when a blood clot partially or completely clogs an artery, which entails a decrease in lung ventilation, hypoxemia. Factors contributing to the development of Pulmonary embolism Patients are at greatest risk of development: • with cancer; • traumatic injuries; • insufficiency
  18. Pulmonary stenosis and tetralogy of Fallot
    Pulmonary stenosis can be isolated or combined with other abnormalities, in particular with septal defects. Often isolated stenosis of the pulmonary artery is valvular, sometimes there is also instillation of isolated stenosis or stenosis due to valvular hypoplasia. Possible dysplasia of the pulmonary valve cusps, which are thickened, rigid and may have deposits
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