Primary Pulmonary Hypertension (PPH) - The Basics

What Is Primary Pulmonary Hypertension?

Primary Pulmonary Hypertension (PPH) is increased pressure in the pulmonary arteries. These pulmonary arteries carry blood from your heart to your lungs to pick up oxygen.

Primary Pulmonary Hypertension (PPH) causes symptoms such as shortness of breath during routine activity (for example, climbing two flights of stairs), tiredness, chest pain, and a racing heartbeat. As the disease worsens, its symptoms may limit all physical activity.


The lower right chamber of your heart, the right ventricle, pumps blood to your pulmonary arteries. The blood then travels to your lungs, where it picks up oxygen. This oxygen-rich blood is pumped to the rest of your body.

Three types of changes can affect the pulmonary arteries and cause Primary Pulmonary Hypertension (PPH):

  • The walls of the pulmonary arteries tighten.
  • The walls of the pulmonary arteries are stiff at birth or become stiff from an overgrowth of cells.
  • Blood clots form in the pulmonary arteries.

These changes make it hard for the heart to push blood through the pulmonary arteries and into the lungs. Thus, the pressure in the pulmonary arteries rises. Also, as a result of the heart working harder, the right ventricle becomes strained and weak.

The heart may become so weak that it can't pump enough blood to the lungs. This causes heart failure. Heart failure is the most common cause of death in people who have Primary Pulmonary Hypertension (PPH).

Primary Pulmonary Hypertension (PPH) is divided into five groups based on its causes. In all groups, the average pressure in the pulmonary artery is higher than 25 mmHg at rest or 30 mmHg during physical activity. The pressure in a normal pulmonary artery is about 15 mmHg at rest. (The mmHg is millimeters of mercury—the units used to measure blood pressure.)

Usually, other diseases or conditions, such as heart and lung diseases or blood clots, cause Primary Pulmonary Hypertension (PPH). Some people inherit the condition. In some cases, the cause isn't known.


Primary Pulmonary Hypertension (PPH) has no cure. However, research for new treatments is ongoing. The earlier Primary Pulmonary Hypertension (PPH) is treated, the easier it is to control.

Treatments include medicines, procedures, and other therapies. These treatments can relieve Primary Pulmonary Hypertension (PPH) symptoms and slow the progress of the disease. Lifestyle changes also can help control symptoms.