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Primary pulmonary hypertension (AERSD syndrome)



It is characterized by myocardial hypertrophy of the right ventricle, expansion of the pulmonary artery trunk. Fibrosis and intima fibroelastosis, fibrinoid-necrotic arteritis of small branches of the pulmonary artery and thrombosis are morphologically detected. It is believed that the direct cause is fibrosis and fibroelastosis of the muscular layer of the pulmonary arterioles, most likely associated with a genetically determined defect in smooth muscle fibers. Treats diseases with an autosomal dominant mode of inheritance. More common in young women and girls.

The clinical picture. Dyspnea, cyanosis, right ventricular hypertrophy, physical changes in the lungs are usually absent, “drumsticks”.

Severe, rapidly progressing primary pulmonary hypertension, occurring with severe cyanosis, shortness of breath, polycythemia, severe right-sided hypertrophy, is defined as AERSA ​​syndrome.

Diagnostics.
At X-ray examination, a sharp expansion of the proximal pulmonary artery is determined with a weakening of the pulmonary pattern in the peripheral parts of the lungs, an increase in the size of the right parts of the heart, expansion of the roots of the lungs and their increased pulsation.

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

ECHO KG: expansion of the pulmonary artery, enlargement of the right ventricle, regurgitation of blood into the right ventricle.

Treatment. Calcium antagonists, a-blockers.

Forecast. Adverse; death from progressive right ventricular failure.

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Primary pulmonary hypertension (AERSD syndrome)

  1. PRIMARY PULMONARY HYPERTENSION
    John Ross, Jr. (John Ross, Jr.) Primary (or idiopathic) pulmonary hypertension is an infrequent disease, the diagnosis of which is based on the exclusion of other known and more common causes of increased pressure in the pulmonary circulation. In most cases, primary pulmonary hypertension is diagnosed in women aged 20–40 years, which, however, does not exclude
  2. Pulmonary hypertension
    580. DECREASE THE HYPERTENSION OF THE SMALL CIRCLE OF CIRCULATION 1. HEPARIN 2. EUFILLIN 3. NITROGLYCERIN 4. PRE-ISOLONE 5. NORADRENALINE 1) only 1,2,4 is true 2) only 2.3 are true 3 3) only 1.4 are true 4) only 3 are true 5) everything is true 121 581. FOR PULMONARY HYPERTENSION OF VENOUS TYPE IS NOT CHARACTERISTIC 1) pulmonary capillary pressure of 10 mm Hg. st. 2) cough, hemoptysis 3)
  3. CLASSIFICATION OF PULMONARY HYPERTENSIONS
    PRIMARY PULMONARY HYPERTENSION Primary pulmonary hypertension (PLH) is a disease of unknown etiology, the diagnostic criteria of which are the following symptoms according to M. Riedel and J. Widimsky (1987): 1. Increased pressure in the pulmonary trunk and normal wedging pressure. 2. The absence of heart and lung diseases. 3. Lack of local anomalies of pulmonary vessels according to
  4. Persistent Pulmonary Hypertension
    Persistent pulmonary hypertension (PLH) 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 persistent fetal circulation syndrome. Etiology. PLG often complicates the course of such
  5. Persistent Pulmonary Hypertension in Newborns
    SYNONYMS Persistent pulmonary hypertension in newborns, persistent fetal circulation. DEFINITION Resistant fetal blood circulation in a newborn (SFKN) is a symptom complex characterized by refractory arterial hypoxemia resulting from persistent high pulmonary vascular resistance and shunting of blood from right to left through the OAP and oval window. CODE
  6. MODERN ASPECTS OF DIAGNOSTICS AND TREATMENT OF PULMONARY HYPERTENSION
    Pulmonary hypertension (LH) is a pathological syndrome caused by an increase in blood pressure in the pulmonary circulation (ICC). The 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 abnormalities in vital organs and systems, especially in the lungs and heart. This causes
  7. PULMONARY HYPERTENSION
    PULMONARY
  8. Primary ciliary dyskinesia (immotile cilia syndrome) and Cartagener syndrome
    It is based on a genetically determined defect in the structure of the ciliated epithelium of the mucous membrane of the respiratory tract. The morphological nature of the defect in its classic version is reduced to the loss of dyneinovyh pens containing ATP, which ensures the movement of cilia. Carthage's syndrome is characterized by the following triad, which includes the reverse arrangement of internal organs, bronchiectasis and
  9. Portal Hypertension Syndrome
    Portal hypertension syndrome is a symptom complex caused by impaired blood circulation in the portal vein system. Leading symptoms: • splenomegaly; • varicose veins of the esophagus and the anterior abdominal wall; • ascites. Etiology. Causes of portal hypertension are blocks of blood circulation in the portal system. Suprahepatic block - thrombosis of the hepatic veins, inferior vena cava; stenosis and obliteration
  10. PRIMARY CARDIO-PULMONARY REANIMATION
    The article "Cardiopulmonary resuscitation in children" is in the section "Emergency conditions in pediatrics", the article "Sudden cardiac death" - in the section "Emergency conditions in diseases of the cardiovascular system." The concept of a “chain of survival” is at the heart of the activities carried out by patients with arrest of blood circulation and respiration. It consists of actions performed sequentially in place.
  11. Primary cardiopulmonary resuscitation of children
    With the development of terminal conditions, the timely and correct conduct of primary cardiopulmonary resuscitation allows, in some cases, to save the lives of children and return the injured to normal activities. Mastery of the elements of emergency diagnostics of terminal states, solid knowledge of the technique of primary cardiopulmonary resuscitation, extremely clear, “automatic” performance of all
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