What is Pulmonary Hypertension? Learn the Facts to Breathe Easy

Oksana A. Shlobin, MD, is a member of Inova Medical Group board-certified in pulmonary disease. She is the director of education for the Inova Advanced Lung Disease and Transplant Program and director of the Pulmonary Hypertension Program at Inova Fairfax Hospital.

Many people know that high blood pressure is cause for concern. But most people are not aware that high blood pressure can develop in the lungs. Unfortunately, this condition, known as pulmonary hypertension, can have serious consequences.

We answer some common questions about the causes, symptoms and treatments of pulmonary hypertension.

Q: What is pulmonary hypertension?

A: Put simply, it’s a disease of increased resistance and high blood pressure in the vessels of the lungs. When that resistance and pressure are higher than normal, the right side of the heart has to beat harder to push blood through the lungs and to the rest of the body. This can lead to thickening of the right heart muscle, and eventually to right-sided heart failure.

Experts have identified 5 different categories of pulmonary hypertension:

• Group 1, also called Pulmonary arterial hypertension (PAH), is a group of diseases in which the pressure and resistance increase because the arteries in the lungs become stiff and constricted.
• Group 2 pulmonary hypertension is caused by diseases that affect the left side of the heart, such as mitral valve disease or chronic high blood pressure, which result in elevated pressures in the left side of the heart and the lungs.
• Group 3 pulmonary hypertension is caused by various lung diseases, which in turn affect blood vessels.
• Group 4 pulmonary hypertension is caused by chronic blood clots in the lungs.
• Group 5 pulmonary hypertension occurs for reasons that are unclear. Sometimes, it is associated with other diseases, such as sarcoidosis or metabolic disease.

Q: Who is at risk?

A: Every category of pulmonary hypertension has different underlying causes and risk factors.  In some cases, we can’t identify the cause. Yet we do know that certain factors increase the risk of developing the disease:

• Chronic blood clots
• Chronic liver disease
• Congenital heart disease
• Connective tissue diseases such as lupus and scleroderma
• Family history or a genetic form of PAH
• Heart problems such as valvular disease or stiff left ventricle due to high blood pressure, diabetes and/or obesity
• HIV/AIDS
• Illicit drug use or, rarely, some prescription medications
• Lung problems such as emphysema, pulmonary fibrosis and chronic obstructive pulmonary disease

Q: What are the symptoms of pulmonary hypertension?

A: Early symptoms can sometimes be vague. As right-sided heart failure begins, the symptoms become more severe.

Early symptoms:

• Shortness of breath with activity
• Fatigue
• Racing heart
• Chest pain

Later symptoms:

• Shortness of breath with regular activity
• Leg swelling
• Fluid in the belly (which is sometimes misinterpreted as bloating or weight gain)
• Chest pain with exertion
• Dizziness or fainting, especially with activity or exertion
• Racing heart

Q: How is pulmonary hypertension treated?

A: Unfortunately, we can’t cure pulmonary hypertension, but in certain cases we can improve the symptoms. In addition to medications, it’s important for patients to control their fluid levels with diet, diuretic medications and decreased salt intake. It’s also important that people with pulmonary hypertension make exercise a part of their daily routine. We often refer patients for a supervised exercise program.

There are many medications used to manage PAH that dilate blood vessels in the lungs. In fact, more than a dozen drugs have been approved to treat PAH in the last 10 years. Researchers are now testing new drugs that might go a step further and actually reverse changes in the blood vessels of the lungs — and thus prevent right-sided heart dysfunction.

Several studies are also underway to see whether the drugs approved for people with PAH can be used to treat people with other types of pulmonary hypertension.

All in all, for people with pulmonary hypertension, there’s a lot of hope on the horizon.

Q: Is Inova experienced in treating pulmonary hypertension?

A: Absolutely! In fact, the Inova Fairfax Pulmonary Hypertension Program is the only site in the Washington, D.C. area accredited as a Comprehensive Pulmonary Hypertension Care Center by the Pulmonary Hypertension Association.

In addition, we participate in several clinical trials to test new pulmonary hypertension medications.

Learn more about Inova research and clinical trials for lung disease, and read about the expert care we offer by the Inova Pulmonary Hypertension Program.