Pulmonary arterial hypertension


Pulmonary arterial hypertension (PAH) is a Rare Disease that causes an increase in the pulmonary vascular resistance and an increase in blood pressure in the pulmonary circulation. The reduced blood flow through the lungs is accompanied by an insufficient supply of oxygen to the body. As a consequence, patients suffer from lethargy, shortness of breath and a drop in physical performance. If PAH is left untreated for two or three years after first diagnosis has been made, it will lead to life-threatening heart failure. Early diagnosis and commencement of proper treatment are important for the progress of the disease and the prognosis.

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    Cause & Symptoms
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Cause

What happens in the event of pulmonary hypertension (PAH)?


Pulmonary hypertension occurs when the mean arterial blood pressure in the pulmonary artery increases above 25 mmHg during rest (normal pressure: <20 mmHg).

Pulmonary hypertension means that there is an increase in pressure in the pulmonary circulation due to a narrowing of the blood vessels in the lungs. This places extra strain on the heart as it tries to continue pumping a sufficient amount of blood through the narrowed blood vessels of the lungs. These blood vessels will continue to narrow and become increasingly inelastic. If the arteries (the blood vessels that lead away from the right ventricle of the heart) are affected, then the disorder is called pulmonary arterial hypertension. 

As the disease progresses, the right heart muscle mass will continue to increase in size to the point that the heart will finally lose the ability to pump enough blood into the lungs. The supply of oxygen to the body and heart will then deteriorate further. If the disease is left untreated, it will eventually lead to heart failure.

Current classification of pulmonary hypertension

There are four types of pulmonary hypertension. Pulmonary arterial hypertension is one type and can be further divided into:

  • Idiopathic
  • Hereditary
  • Associated forms
  • Drug and toxin-induced

Symptoms

The primary symptom of the disease is overwhelming fatigue and lack of breath to the extent that breathing assistance may be necessary.

Other symptoms that may be present include: 

  • Dizziness
  • Fatigue
  • Swelling around the ankles and feet
  • Fainting
  • Tightening feeling in the chest