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Primary pulmonary hypertension (AERCA syndrome)



It is characterized by myocardial hypertrophy of the right ventricle, extension of the pulmonary artery trunk. Morphologically revealed fibrosis and intestinal fibroelastosis, fibrinoid-necrotic arteritis of small branches of the pulmonary artery and thrombosis. It is believed that the immediate cause is fibrosis and fibroelastosis of the muscle layer of pulmonary arterioles, most likely associated with a genetically conditioned defect of smooth muscle fibers. It refers to diseases with an autosomal dominant type of inheritance. It is more common in young women and girls.

Clinical picture. Shortness of breath, cyanosis, right ventricular hypertrophy, physical changes in the lungs are usually absent, "drum sticks."

Severe, rapidly progressive primary pulmonary hypertension, occurring with a sharp cyanosis, dyspnea, polycythemia, severe right heart hypertrophy is defined as AERSA ​​syndrome.

Diagnostics.
Radiographic examination determines a sharp expansion of the proximal pulmonary artery with a weakening of the pulmonary pattern in the peripheral parts of the lungs, an increase in the size of the right heart, an expansion of the roots of the lungs, and their enhanced pulsation.

On the ECG: signs of a sharp overload of the right ventricle and its hypertrophy.

ECHO KG: an expansion of the pulmonary artery, an increase in the right ventricle, regurgitation of blood in the right ventricle.

Treatment. Calcium antagonists, a-adrenoblockers.

Forecast. Adverse; death from progressive right ventricular failure.

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Primary pulmonary hypertension (AERCA syndrome)

  1. PRIMARY PULMONARY HYPERTENSION
    John Ross, Jr. Primary (or idiopathic) pulmonary hypertension is a rare disease, the diagnosis of which is based on the exclusion of other known and more common causes of increased pressure in the small circulation. In most cases, primary pulmonary hypertension is diagnosed in women aged 20-40 years, which, however, does not exclude
  2. Pulmonary hypertension
    580. REDUCE THE HYPERTENSION OF A SMALL CIRCLE OF CIRCULATION 1. HEPARIN 2. EVUFILLIN 3. NITROGLYCERINE 4. PREDNISOLONE 5. NORADRENALIN 1) only 1,2,4 is true 2) it is only 2,3) 3) it is true only 1,4 4) only 3 5) All are true 121 581. FOR PULMONARY HYPERTENSION OF THE VENOUS TYPE IS NOT CHARACTERISTIC 1) pulmonary-capillary pressure 10 mm Hg. Art. 2) cough, hemoptysis 3)
  3. CLASSIFICATION OF PULMONARY HYPERTENSIONS
    PRIMARY PULMONARY HYPERTENSION Primary pulmonary hypertension (PLG) is a disease of unclear etiology, the criteria for diagnosis of which are the following features according to M. Riedel and J. Widimsky (1987): 1. Increased pressure in the pulmonary trunk and normal seizure pressure. 2. Lack of heart and lung diseases. 3. Absence of local anomalies of pulmonary vessels according to data
  4. Persistent pulmonary hypertension
    Persistent pulmonary hypertension (PLG) in newborns is a disease characterized by severe arterial hypoxemia, resulting from an increase in pulmonary vascular resistance with shunting of blood from the pulmonary arteries into the systemic circulation. This pathology is also known as the syndrome of persistent fetal circulation. Etiology. PLA often complicates the flow of such
  5. Perspirational pulmonary hypertension of newborns
    SYNONYMS Persistent pulmonary hypertension of newborns, persistent fetal circulation. DEFINITION Persistent fetal circulation in the newborn is a symptomatic complex characterized by refractory arterial hypoxemia resulting from persisting high pulmonary vascular resistance and shunting the blood from right to left through the OAA and oval window. CODE
  6. Modern aspects of diagnosis and treatment of pulmonary hypertension
    Pulmonary hypertension (LH) is a pathological syndrome caused by increased blood pressure in the small circulatory system (ICC). Pressure in the ICC is considered elevated if it exceeds normal values: systolic - 26-30 mm Hg, diastolic 13-19 mm Hg. LH causes severe disturbances in vital organs and systems, primarily in the lungs and heart. This causes
  7. PULMONARY HYPERTENSION
    PULMONARY
  8. Primary ciliary dyskinesia (syndrome of immobile cilia) and Kartagener's syndrome
    The basis is a genetically determined defect in the structure of the ciliated epithelium of the mucosa of the respiratory tract. The morphological essence of the defect in its classical version is reduced to the loss of the dineine handles containing ATP, which ensures the movement of cilia. The syndrome of Cartagena is characterized by the following triad, which includes the reverse location of internal organs, bronchiectasis and
  9. Syndrome of portal hypertension
    Syndrome of portal hypertension is a symptomatic complex caused by a circulatory disturbance in the portal vein system. Leading symptoms: • splenomegaly; • widening of the esophagus and anterior abdominal wall; • ascites. Etiology. The causes of portal hypertension are circulatory blocks in the portal system. Superhepatic block - thrombosis of the hepatic veins, inferior vena cava; stenosis and obliteration
  10. Primary cardiopulmonary resorption
    The article "Cardiopulmonary resuscitation in children" is in the section "Urgent conditions in pediatrics", the article "Sudden cardiac death" - in the section "Urgent conditions in diseases of the cardiovascular system". At the heart of the activities carried out by patients with a stoppage of blood circulation and breathing is the concept of a "chain of survival". It consists of actions that are consistently performed on site
  11. Primary cardiopulmonary resuscitation
    With the development of terminal conditions, timely and correct primary cardiopulmonary resuscitation allows in some cases to save the life of children and return the victims to normal life. Mastering the elements of emergency diagnosis of terminal conditions, a solid knowledge of the technique of primary cardiopulmonary resuscitation, extremely clear, "automatic" implementation of all
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