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PULMONARY ARTERIAL HYPERTENSION: Types. Signs, Causes and Treatment!

PULMONARY ARTERIAL HYPERTENSION: Types. Signs, Causes and Treatment!

The “right heart”, circulates the oxygen-poor blood within the pulmonary arteries into the lungs, where oxygen is incorporated. The oxygen-enriched blood is then delivered to the left heart via the pulmonary veins. The pulmonary arteries and pulmonary veins make up the pulmonary circulation.


Pulmonary arterial hypertension Definition: Pulmonary arterial hypertension is a disease of the pulmonary arteries, that is, the vessels that transport the blood from the right heart to the lung. In this disease, the vessels are severely narrowed and there is increased pressure in the pulmonary arteries. The right heart must pump against this resistance and do much more, which ultimately lead to a weakening of the right heart. As a result of the constriction of the pulmonary vessels, the oxygen intake into the blood and thus the oxygen supply to the organs is made more difficult. This lack of oxygen and the increasing weakness of the right half of the heart are responsible for the symptoms of PAH, such as respiratory distress or rapid fatigue.

One differentiates an idiopathic (congenital, not easily explainable) from a family (inherited) pulmonary arterial hypertension. The forms that are associated with other diseases such as connective tissue diseases or congenital heart defects or which are also associated with the use of certain drugs can be distinguished from this.


Within the WHO classification group “pulmonary arterial hypertension” an additional subdivision is made which comprises on the different causes of the disease:

  • The idiopathic Pulmonary arterial hypertension (IPAH), in which no cause of the disease can be detected.
  • The familial Pulmonary arterial hypertension (FPAH), with a genetic predisposition
  • The associated Pulmonary arterial hypertension (APAH) with different causes

In addition to the diagnostic classification, a classification of the patients according to the severity of the disease (functional class) is carried out for prognostic reasons and for therapeutic planning.


Pulmonary Arterial Hypertension Causes: The Pulmonary arterial hypertension can be produced by a number of causes which might include:

  • Connective tissue diseases (eg scleroderma)
  • Congenital “short circuits” between arterial and venous system (congenital vaticides)
  • Hypertension of the liver vein (portal hypertension)
  • HIV infection – drugs (eg certain appetite suppressants) and toxins
  • Other forms – PAH in combination with significant venous and/or capillary involvement
  • Pulmonary venous disease (PVOD), ie the occlusion of pulmonary veins
  • Pulmonary capillary hem angiomatosis (PCH), is an extensive new formation of pulmonary capillaries
  • Existing pulmonary hypertension in the newborn


Pulmonary Arterial Hypertension Symptoms: The symptoms of pulmonary arterial hypertension are, unfortunately, very uncharacteristic. Even in the more advanced stages, the disease symptoms and signs can be confused with other lung or heart disease. Therefore, a great deal of time can pass from the first symptoms to the definitive diagnosis. Some patients with pulmonary hypertension only look for the doctor when they can no longer master their daily life.

The main symptoms of pulmonary arterial  hypertension, or the sequelae of a permanent load on the right heart, are as follows:

  • General fatigue, occasionally even depression
  • Dyspnoea, which is often misinterpreted as “no longer in shape”
  • Dizziness and dizziness
  • Fast pulse (tachycardia)
  • Impotence attacks (syncope)
  • Coughing sensation during exertion and chronic hoarseness
  • Swelling of the ankles or legs (oedema) and also in the abdomen (ascites)
  • Blue discolouration of the lips and skin (cyanosis)
  • Pain in the chest (angina pectoris)


Pulmonary Arterial HypertensionTreatment: The treatment of pulmonary arterial hypertension has been the subject of intensive research in recent years. Thus the prognosis of the PAH and the quality of life could be significantly improved. The primary goal of the therapeutic measures is to improve the circulation in the small circulation. This relieves the right heart, increases oxygen intake and increases physical performance. Patients suffering from PAH should be treated in a specialized centre.

PULMONARY ARTERIAL HYPERTENSION: Types. Signs, Causes and Treatment!


Pulmonary Arterial Hypertension Medication:

The basis of the treatment are vasodilators such as:

  • Prostacyclin preparations (intravenous: epoprostenol, subcutaneous and intravenous: treprostinil, inhaled: iloprost)
    Treprostinil is usually administered subcutaneously by means of a small pump. An implantable pump is also available for the administration of Treprostinil.
  • Endothelin receptor antagonists, selective phosphodiesterase 5 inhibitors and Rio-cig ate a stimulator of soluble guanylate cyclase (sGC) are orally administrable drugs.

In addition, anticoagulant drugs, cardiac glycosides and dehydrating preparations are used.

Scientifically Proven: PULMONARY HYPERTENSION: Causes, Signs and Treatment

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