What is Pulmonary Arterial Hypertension?



Pulmonary Arterial Hypertension (PAH) is a disease affecting the pulmonary arteries, the arteries that carry blood from the heart to the lungs. The right ventricle of the heart pumps blood into the lungs to gather oxygen for delivery to the rest of the body. In this condition, the blood pressure within those arteries is too high. With the difficulty pushing through the pulmonary arteries, the right ventricle of the heart strains under the pressure.

PAH is a life-threatening condition affecting 1 in 100,000 to 1 in 1,000,000. There are three forms of PAH. Familial PAH is linked to a genetic defect that is inherited. Idiopathic PAH is PAH with an unknown cause. Associated PAH is the most common and is connected to a specific underlying cause such as HIV, stimulant drug use, thyroid disorders, congenital lung disease or heart disease, collagen vascular disease and others.

The basic explanation for PAH is an issue with the pulmonary arteries themselves. Typically, due to any number of related factors, these arteries thicken, tighten, or are clogged with blood clots. Sometimes the arteries tighten due to muscle contractions. The arteries may be thickening with overgrowth of the cells and blood clots similarly narrow the passageway.

Because the heart must work extra hard to get blood through the pulmonary arteries for oxygenating the body organs and tissues, it begins to weaken over time. This begins to reduce oxygen levels. In some cases, heart failure manifests itself through swelling of the heart. This can cause fatigue, fainting, shortness of breath, chest pain, and dizziness.

PAH is more common among women than men. In newborns, it is called persistent pulmonary hypertension of the newborn (PPHN). It is more prolific among those with family histories of pulmonary hypertension or sudden death. And for pregnant women who have it, it can cause death during childbirth. PAH is a very serious condition, taking the lives of half of those diagnosed within five years of diagnosis. Those who are untreated tend to live only three years on average. Treatments can slow the progression of the disease and ensure a higher quality of life for patients with PAH.

 

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