Primary Pulmonary Hypertension (PPHP) is a condition involving increased blood pressure in the pulmonary arteries due to an unknown cause. (It is identified as “primary” because the cause is unknown, unlike Pulmonary Arterial Hypertension where a cause has been identified involving an underlying condition of the heart or lungs.) The pulmonary arteries bring blood from the heart to the lungs to gather oxygen to deliver to the rest of the body.
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.
