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      • Diverse pulmonary arterial hypertension

      Diverse pulmonary arterial hypertension

      Diverse pulmonary arterial hypertension

      Pulmonary hypertension – a syndrome of various diseases with a main significant feature – elevated arterial pressure in pulmonary artery (greater than 25 mmHg at rest).

      Elevated blood pressure causes great tonicity of heart which leads to arrest of the right side of the heart.

      Pulmonary arterial hypertension (PAH) is a very serious illness and, unfortunately, incurable. But today there are a lot of therapies to help people with this disease, to prolong the life and improve its quality.

      What are pulmonary arteries?

      Pulmonary arteries are blood vessels which transfer blood with low oxygen levels from lower right side of the heart (right ventricle) to small arteries and capillaries in lungs.

      What happens in case of Pulmonary arterial hypertension  (PAH)?

      Blood in capillaries is saturated with oxygen and flows to left side of heart from where it goes to all over the body and takes oxygen to all body cells. Oxygen deficiency provokes hypoxia which leads to pathological changes in the body. These changes lead to PAH progression and development of a vicious circle:

      • Narrowing of blood vessels resulting from contraction of the muscular wall of the vessels – vasoconstriction
      • Change of shape and thickness of blood vessels due to muscle mass increase and swelling of blood vessels walls caused by inflammation.
      • Development of small blood clots (thrombus) in blood vessels

      Who can have a pulmonary arterial hypertension?

      Pulmonary arterial hypertension may affect any person regardless of age, sex or race. But women of 30-50 years old are more affected by this disease.


      What are the symptoms of pulmonary arterial hypertension?

      As PAH treatment should be started on time, it is very important to detect it earlier.

      But it is very difficult to diagnose PAH initially, as its symptoms are non specific and similar to those of cardiac and pulmonary diseases. It can be said that PAH diagnosis is the way to exclude other diseases.

      Dyspnea, especially exertional one, is the first and main symptom. It seems you are unable to breath enough. The following symptoms can appear during deterioration: fatigue, tiredness, dizziness, tachycardia (rapid heartbeat), constricting pain behind the breastbone, leg edema, cyanosis (blueing) of extremities, ascites (liquid in the abdominal cavity).

      What are the types of pulmonary arterial hypertension?

      1. Idiopathic pulmonary hypertension – pulmonary hypertension of unknown etiology.
      2. Heritable PAH – the result of genetic disease in your family. It was detected that some genes can influence PAH and the treatment can be improved during genetic studying. The most important genetic mutations connected with PAH are BMPR2 gene mutation with a protein code on the cells which are inside of pulmonary arteries.
      3. Associated pulmonary arterial hypertension is developed in patients with other diseases. They are: congenital heart disease (CHD), connective tissue disease (scleroderma, systemic lupus erythematosus (SLE), HIV-infection and others).

      Taking diet pills, cocaine and amphetamines raise the risk of pulmonary arterial hypertension.

      How to diagnose pulmonary arterial hypertension?

      A number of analyses should be provided in order to diagnose pulmonary arterial hypertension, some of which can be used to exclude diseases with the symptoms alike.

      Additionally, pulmonary functional test, exercise and blood tests can be used.

      ECG (electrocardiogram) measures heartbeat and can show whether you have any problems with your heart.

      If you have PAH, ECG will detect problems in the right side of your heart.

      Thorax x-ray can show if the right side of the heart or pulmonary arteries are enlarged. This also helps to exclude some other pulmonary and cardiac diseases.

      Echocardiography (Cardiac ultrasound): allows to detect heart sizes and to measure pressure in pulmonary arteries non-directly.

      Right heart catheterization:

      This is a standard method to diagnose PAH.

      Right heart blood pressure and pulmonary arteries pressure are measured with exact accuracy. Besides a doctor sees how good the right side of the heart pumps blood. Mean blood pressure in pulmonary arteries at rest – 14 mmHg. But in case of PAH it is greater than 25 mmHg.

      The only place to use this method is specialized departments of cardiac surgical centers.

      How to treat pulmonary arterial hypertension?

      Though PAH is incurable, for the last decade a significant progress has been achieved in studying of this disease. This has led to significant improvements in its treatment.

      Modern treatment can help you to bear a greater strain, to live a longer life and improve its quality.

      Most of drug treatments are directed to stop or reduce processes` intensity which lead to pulmonary arteries blockage and elevated pressure.

      Different people demand different treatment. New types of drugs are developed and tested. Physicians prescribe several drugs of specific therapy to stabilize diseases.

      Life with pulmonary arterial hypertension

      Pulmonary arterial hypertension diagnosis should not complicate your life until pain or other problems appear. But the more severe symptoms you have, the less your possibilities are.

      You can do a lot to release the symptoms without drugs:

      Try:

      1. To cook with less salt
      2. To control liquid you drink
      3. To eat three times a day
      4. To sit while eating and eat slowly
      5. To brush teeth regularly in order to prevent infection
      6. To take breaks during a day to rest
      7. To prepare and systemize your drugs
      8. To keep a diary to take drugs on time
      9. To ensure that you have an additional stock of drugs: you never know when you will need them
      10. To collect information about new methods of treatment
      11. To know about possible side effects of new drugs
      12. Always have contact details of your physicians by you for emergent cases
      13. To prepare all necessary things in advance, if you are far from home.
      14. To stay positive
      15. To get a psychological help from a specialist
      16. To get a vaccine to prevent infection

      To avoid:

      1. To travel to a higher altitudes
      2. Ready-to-cook food
      3. Smoking and alcohol
      4. Pregnancy as it leads to extra burden on the heart
      5. Overcrowded places
      6. Contact with infected people (virus)
      7. Intense exercises 
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