Abstract

Pulmonary arterial hypertension (PAH) is a serious disease of pulmonary arteries. When Pulmonary Arterial Hypertension (PAH) develops, blood flow through the pulmonary arteries is restricted and the right side of the heart is put under increasing strain to pump blood through to the lungs. The different changes in the blood vessels raise the pressure and increase the resistance to blood flow through the lungs, making it harder for the heart to pump blood through the lungs. Over time, due to the additional strain, the heart begins to work less effectively and eventually, the right heart can fail (this is known as 'right ventricular failure'). Pulmonary arterial hypertension (PAH) is relatively rare. It is estimated that there are about 30-50 people with PAH for every million of the population. While Pulmonary Arterial Hypertension (PAH) can occur at any age, it is more common in women in their childbearing years and the average age of diagnosis is 36. The exact cause of pulmonary arterial hypertension (PAH) remains unknown. In recent years research has focused particularly on the role of three specific substances that are produced in these cells: prostacyclin, nitric oxide and endothelin. These three agents work together to help the blood to flow smoothly through the heart and lungs and it is thought that an imbalance in levels of one or more of these substances contributes to the development of conditions such as Pulmonary Arterial Hypertension (PAH). This leads to the progressive changes in the vessels and the subsequent rise in pressure. This area of research has led to the development of specific treatments for Pulmonary Arterial Hypertension (PAH).

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