PAH Center

What is Primary Pulmonary Hypertension (PPH)?

Primary Pulmonary Hypertension, PPH, is a lung disorder in which the blood pressure in the pulmonary artery rises high above normal levels for no obvious reason. The pulmonary artery is the blood vessel that carries blood from the heart to the lungs so that it can pick up oxygen. Normal pulmonary artery blood pressure is 14 mm Hg at rest. In patients with PPH, it is greater than 25 mm Hg. The abnormally high pressure causes the lung's tiny blood vessels to narrow, resulting in less blood flow. Thus putting a strain on the right side of the heart, forcing it to work harder to move blood to the lungs, potentially resulting in heart failure.

Other Names for PPH

PPH may also be referred to as Unexplained Pulmonary Hypertension, Familial Primary Pulmonary Hypertension or Primary Obliterative Pulmonary Vascular Disease.

What is Pulmonary Arterial Hypertension (PAH)?

Pulmonary Arterial Hypertension often referred to, as PAH, is a serious condition in which the blood pressure in the pulmonary artery stays consistently high. Average blood pressure in the pulmonary artery is 14mm Hg at rest; with PAH it is often greater than 25mm Hg. While there is no cure for PAH, many patients benefit from a variety of treatments.

In PAH, three types of shifts in the arteries can take place:

• Muscles in the artery walls may constrict, therefore narrowing the insides of the arteries.
• Artery walls may thicken as muscle mass increases, forming scar tissue and narrowing the arteries.
• Blood clots may develop in the arteries and create blockages.

With less space for blood to flow, the arteries may harden, and over time become blocked. So the right side of the heart must work harder to pump blood to the lungs. After a while the heart weakens and cannot pump enough blood through the body, resulting in right heart failure.

There are two forms of PAH:

• Primary Pulmonary Arterial Hypertension (PPAH) is hereditary and there is no known cause.
• Secondary Pulmonary Arterial Hypertension (SPAH) is created by or forms because of another disease such as chronic lung or heart disease, blood clots in the lungs, scleroderma or systemic lupus erythematosus.

Other Names for PAH

PAH may also be referred to as Primary Pulmonary Arterial Hypertension (PPAH), Secondary Pulmonary Arterial Hypertension (SPAH), Pulmonary Hypertension, Idiopathic Pulmonary Arterial Hypertension, Sporadic Primary Pulmonary Hypertension or Familial Primary Pulmonary Hypertension.