Definition
Pulmonary arterial hypertension (PAH) is a rare and progressive disorder characterized by elevated blood pressure in the pulmonary arteries that supply blood to the lungs. This increased pressure can lead to the narrowing and stiffening of the pulmonary arteries, making it difficult for the heart to pump blood through the lungs.
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 the 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.
Causes
Pulmonary arterial hypertension (PAH) can arise from a variety of causes, but in many cases, the exact cause remains unknown. However, it can also be associated with or mistaken for other serious conditions. Genetics may also play a role in some cases.