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What is Pulmonary Hypertension?

Pulmonary Hypertension IllustrationPulmonary hypertension is a type of high blood pressure that affects the arteries in the lungs and the right side of your heart. Pulmonary hypertension begins when tiny arteries in your lungs, called pulmonary arteries, and capillaries become narrowed, blocked or destroyed. This makes it harder for blood to flow through your lungs, and raises pressure within your lungs’ arteries. As the pressure builds, your heart’s lower right chamber (right ventricle) must work harder to pump blood through your lungs, eventually causing your heart muscle to weaken and eventually fail. Pulmonary hypertension is a serious illness that becomes progressively worse and is sometimes fatal. Although pulmonary hypertension isn’t curable, treatments are available that can help lessen symptoms and improve your quality of life.

Pulmonary Hypertension (PH) also known as Pulmonary Arterial Hypertension (PAH) is high blood pressure in the lungs that can lead to right sided heart failure. You have probably heard of “hypertension” which is high blood pressure throughout the body. Pulmonary Hypertension is different. PH is when the pressures in the pulmonary arteries are elevated. To understand this it is necessary to understand some basic anatomy. Your heart has two sides, the right and the left. They are separated by an inner wall called the septum. Each side of your heart has an upper and lower chamber. The lower right chamber of your heart is known as your right ventricle. The right ventricle pumps blood to your pulmonary arteries. The blood then goes to your lungs where it picks up oxygen. The upper left chamber of your heart, the left atrium receives this oxygen rich blood and the blood is then pumped into the lower left chamber of your heart, the left ventricle. From there the blood is pumped to the rest of your body through an artery called the aorta.

Normal pulmonary artery pressure is 8-20 mmHg at rest (mmHg stands for millimeters of mercury which is the units used to measure blood pressure). PH is diagnosed when the pulmonary artery pressure is greater than 25 mmHg at rest or 30 mmHg when exercising.

Symptoms of PH can include:

  • chest pain
  • shortness of breath
  • cyanosis or blue lips/ blueness around the mouth
  • syncope or fainting
  • dizziness
  • a heart murmur or irregular heartbeat

PH begins with inflammation and changes in the cells that line your pulmonary arteries. There are other factors that can affect the pulmonary arteries and cause PH. For example, the condition may develop if:

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

These changes cause narrowing of the arteries and make it hard for your heart to push blood through the pulmonary arteries and into the lungs. As a result, the pressure in the arteries rises. This causes the right ventricle to work harder and it becomes strained and weak. The heart can then become so weak that it can’t pump enough blood to your lungs which leads to heart failure. Approximately 75% of people with PH die of heart failure. Other diseases or conditions, such as heart and lung diseases or blood clots can also cause PH. For some people, PH is genetic, meaning it is inherited from their parents. In many cases, the cause of PH isn’t known and this is what is known as Idiopathic Primary Pulmonary Arterial Hypertension.

Diagnosing Pulmonary Arterial Hypertension

Several tests can be used to diagnosis PAH including blood tests, chest x-rays, electrocardiogram (ECG), echocardiogram, Pulmonary Function Tests, Exercise Tolerance Tests, Nuclear Scans and a right heart cardiac catheterization also known as a “cardiac cath”. A cardiac cath is considered the gold standard for diagnosis as it is the only way to measure the actual pressures within the pulmonary arteries. During this test a thin catheter is passed through a vein either in the neck or groin and the blood pressure inside the right heart and lungs is measured. All PH patients should have at least one cardiac cath to confirm the diagnosis.

Treatment

Currently there is no cure for PH. There are several treatments available such as oral, IV or subcutaneous (through the skin) medications. There are new treatments on the horizon and the PH community refers to the phrase “Phenomenal Hope” in that there is always hope for a cure. A heart and/or lung transplant is also sometimes a treatment option.

If you’d like to learn more about Pulmonary Hypertension, the Pulmonary Hypertension Association’s website contains many valuable resources.