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Pulmonary Hypertension is Serious. Learn to Spot the Signs.

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

Today, May 5, is World Pulmonary Hypertension Day. Never heard of pulmonary hypertension (PH)? You’re not alone. Many people are unfamiliar with this severe condition that affects the lungs and heart.

Unfortunately, PH is frequently overlooked or misdiagnosed, but it’s often treatable. Here are the warning signs you should look for.

What is Pulmonary Hypertension?

PH is a condition in which high blood pressure occurs in the lungs. Typical symptoms are fatigue and shortness of breath. As the disease progresses it can cause difficulty breathing with activity, a racing pulse, dizziness and even fainting or passing out.

There are 5 distinct types of PH, described as groups:

Group 1: Also known as pulmonary arterial hypertension (PAH), this type of PH causes the walls of the arteries in the lungs to become stiff and tight. Sometimes PAH is what we call “idiopathic” – in other words, the cause isn’t known. However, PAH can be associated with other diseases including connective tissue diseases (such as scleroderma or lupus), congenital heart disease, chronic liver disease and HIV, as well as exposure to certain drugs and toxins. A small number of people inherit a genetic form of PAH.

Group 2: This type of PH affects people with heart problems such as coronary artery disease, high blood pressure or heart valve disease.

Group 3: This group occurs in people with lung diseases that cause low blood oxygen levels, such as chronic obstructive pulmonary disease (COPD) or emphysema, pulmonary fibrosis or severe sleep apnea.

Group 4: This type of PH occurs in people with chronic blood clots in the lung. A one-time clot can increase the risk of chronic blood clot problems as well as PH.

Group 5: This group describes people with various other reasons for PH, such as sarcoidosis or certain blood disorders.

Pulmonary Hypertension Treatments

PH is a serious disease. Over time, the right side of the heart has to work harder to push blood into the lungs against greater resistance. Untreated, it can lead to heart failure on the right side of the heart.

Most types of PH cannot be cured. However, we have a variety of medications that can often help treat the disease, and new drugs continue to be developed. In the 1980s, we had zero drugs approved to treat Group 1, or PAH. Today we have more than a dozen that can allow people with PAH to live for quite a long time with the disease.

In people who have other diseases that go hand-in-hand with PH, we can help manage the lung disease by treating the related disorders. And in severe cases, a patient might be a candidate for lung transplant. Happily, though, the number of lung transplants due to PH has gone down in recent years – evidence that the new drug treatments are effective.

Could I Have PH?

While most types of PH cannot be cured, there is one exception. In people who develop PH after a blood clot, we can sometimes cure the disease with surgery.

Blood clots in the lungs are fairly common. Unfortunately, many people who have had them don’t realize they’re at greater risk of developing PH. If you’ve had a clot in your lung and still feel short of breath a year later, it’s important to follow up with your doctor to be evaluated for PH.

Early signs of PH can be hard to spot, since lack of energy and shortness of breath are common signs of many illnesses. But if you experience the symptoms and have any of the diseases described above, talk to your doctor.

Learn more about the Inova Advanced Lung Disease and Transplant Program.

 

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