Pulmonary hypertensia

Description:

The Pulmonary Hypertensia (PH) — the group of diseases which is characterized by progressive increase in pulmonary vascular resistance that leads to right ventricular insufficiency and premature death.  Pulmonary hypertensia proceeds hard with the expressed decrease in physical endurance and leads to heart failure. It was for the first time revealed by doctor Ernst von Romberg in 1891.  According to the last classification, distinguish 5 LG types: arterial, venous, hypoxemic, thromboembolic or mixed.

Symptoms of Pulmonary hypertensia:

As symptoms can develop very slowly, patients can not see a doctor for many years. The general symptoms — an asthma, increased fatigue, unproductive cough, stenocardia, faints, peripheral hypostases (standing) and rarely a pneumorrhagia.

Pulmonary venous hypertensia is usually shown by an asthma in a lying state or in a dream (an orthopnea or a paroxysmal night asthma), and at the pulmonary arterial hypertension (PAH), as a rule, it is absent.

The detailed family anamnesis is established for definition of possible heredity of LG. It is important to consider acceptance of drugs, for example cocaine, methamphetamine, and alcohol, leading to cirrhosis, and also the smoking leading to emphysema. Physical examination is conducted for detection of characteristic signs of LG: the loud sound of closing of the pulmonary valve, (pair) of a breast to throw, stretching of jugular veins, hypostases of legs, ascites, a gepato-yugulyarny reflux, nails as hour glasses, etc.

Reasons of Pulmonary hypertensia:

The syndrome has the polyetiological nature:
    * 1. Pulmonary Arterial Hypertension (PAH)
        1. Idiopathic
        2. Hereditary
              1. A mutation of a gene of a receptor of type 2 to a protein of a bone morphogenesis
              2. A mutation of a gene of an aktivinpodobny kinase-1 (with and without hereditary hemorrhagic teleangiectasia)
              3. Unknown mutations
        3. Caused by medicamentous and toxic influences
        4. Associated — connected with:
              1. diseases of connecting fabric
              2. HIV infection
              3. portal hypertensia
              4. inborn heart diseases
              5. schistosomiasis
              6. chronic hemolitic anemia
        5. Persistent pulmonary hypertensia of newborns
    * 1'. Venookklyuzionny pulmonary disease and/or pulmonary capillary гемангиоматоз
    * 2. The pulmonary hypertensia caused by defeat of the left cameras of heart
        1. Systolic dysfunction of a left ventricle
        2. Diastolic dysfunction of a left ventricle
        3. Defeat of valves of the left departments of heart
    * 3. The pulmonary hypertensia caused by pathology of respiratory system and/or a hypoxia
        1. Chronic obstructive pulmonary disease
        2. Intersticial diseases of lungs
        3. Other diseases of lungs with mixed restrictive and obstructive by components
        4. Breath disturbances during sleep
        5. Alveolar hypoventilation
        6. Mountain pulmonary hypertensia
        7. Malformations of respiratory system
    * 4. Chronic thromboembolic pulmonary hypertensia
    * 5. The pulmonary hypertensia caused by not clear multifactorial mechanisms
        1. Blood diseases: myeloproliferative diseases, splenectomy
        2. General diseases: sarcoidosis, Langerkhans's histiocytosis, лимфангиолейомиоматоз, нерофиброматоз, vasculites
        3. Exchange diseases: disease of accumulation of a glycogen, disease to Gosha, diseases of a thyroid gland
        4. Others: tumoral obstruction, a fibroziruyushchy mediastinitis, HPN at the patients who are on a hemodialysis.

Risk factor for LAS — any factor or a state which are potentially contributing or promoting development of a disease. The risk factors and states connected with LAS depending on substantiality level:

    * Drugs and toxins
          o Certain: Aminorex, Fenflyuramin, Deksfenflyuramin, Toxic rape oil
          o Very probable: Amphetamines, L-tryptophane
          o Probable: Meta-amphetamines, Cocaine, Chemotherapeutic drugs
          o Improbable: Antidepressants, Oral contraceptives, Estrogenic drugs, Tobacco smoking
    * Demographic and medical states
          o Certain: floor
          o Possible: Pregnancy, System hypertensia
          o Improbable: Obesity
    * Diseases
          o Certain: HIV infection
          o Very probable: Portal hypertensia / diseases of a liver, the Disease of connecting fabric, Inborn shunts between system and pulmonary vessels
          o Probable: Pathology of a thyroid gland, Hematologic diseases (Aspleniya after a surgical splenectomy, Drepanocytic anemia, a β-thalassemia, Chronic meiloproliferativny diseases), Rare genetic and metabolic disorders (Von Girke's disease, the Disease to Gosha, the Disease of Rendyu-Vebera-Oslera).

Treatment of Pulmonary hypertensia:

For direct lowering of blood pressure in pulmonary arteries, appoint:

    * Prostanoida, such as эпопростенол, treprostinit or илопрост
    * Antagonists of a receptor of endothelin, such, as бозентан and амбрисентан
    * Phosphodiesterase inhibitors, such as sildenafit

In hard cases of pulmonary hypertensia where medicinal therapy is powerless, doctors can recommend:

    * Transplantation of a lung.
    * Atrial septostomiya.

Treat other ways of reduction of symptoms of pulmonary hypertensia:

    * Exercise stresses.
    * Inhalation of oxygen can improve an asthma at some people.
    * Anticoagulative means.
    * Blockers of calcium channels.