Understanding PPH

What is Primary Pulmonary Hypertension (PPH)?

Primary pulmonary hypertension (PPH) is increased pressure in the pulmonary arteries, and its cause is unknown. These arteries carry blood from your heart to your lungs to pick up oxygen.

What causes PPH and how does it Affect your Body?

Your right ventricle, the lower right chamber of your heart, pumps blood to your pulmonary arteries. The blood then travels to your lungs, where it picks up oxygen and is pumped throughout of your body.

Pulmonary hypertension (PH) is caused by changes in the cells that line your lungs' arteries. This process includes three types of changes that affect the pulmonary arteries:

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

Any or all of these changes make it hard for the heart to push blood through the arteries and into the lungs. As a result, the blood pressure in the arteries rises and PPH occurs.

Eventually, your heart may become so weak that it can't pump enough blood to the lungs. This leads to heart failure.

In some types of pulmonary hypertension, other diseases (such as heart and lung diseases or blood clots) cause pulmonary hypertension. In PPH, the cause is unknown.

Who gets PPH?

Anyone can develop PPH. It usually develops between the ages of 20 and 60, but it can occur at any age. It is two to three times as common among women as men.

How Serious is PPH?

There is no cure for PPH. It worsens over time and makes everyday tasks more difficult.

There can be a wide range among how well patients do and how long they survive with PPH. People diagnosed with PPH after age 40 and people without heart failure generally survive longer than others.

Many patients make lifestyle changes that allow them to go about many of their daily tasks.

Doctors identify how severe a patient's PPH is and decide on treatment based on that person's "functional status". Functional status is a person's ability to perform everyday tasks and is ranked from Class I (no limitations) to Class IV (unable to perform any physical activity).

Specifically:

  • Class I: No limitation of usual physical activity; ordinary physical activity does not cause increased breathing problems, fatigue, chest pain or lightheadedness.
  • Class II: Mild limitation of physical activity; there is no discomfort when the person is at rest, but normal physical activity causes increased breathing problems, fatigue, chest pain or lightheadedness.
  • Class III: Marked limitation of physical activity; there is no discomfort when the person is at rest, but less than ordinary physical activity causes increased breathing problems, fatigue, chest pain or lightheadedness.
  • Class IV: Unable to perform any physical activity and possible signs of heart failure in the right ventricle (the chamber of the heart that pumps blood to the lungs); at rest, patients have difficulty breathing and/or fatigue, and symptoms get worse by almost any physical activity.