Pulmonary Conditions & Diseases: Pulmonary Arterial Hypertension

Pulmonary hypertension is a disorder of high blood pressure in the lungs, which is different from having high blood pressure based on blood pressure cuff readings. Pulmonary hypertension can be subdivided into five different categories, one being pulmonary arterial hypertension (PAH) caused by narrowing or obstruction of small arteries in your lungs. This makes it more difficult for blood to flow through and increases the pressure need by the heart to pump blood through the lungs. The heart is a muscle that over time can become weakened by the increased pressure needs.

What are the signs and symptoms?

The main symptoms is shortness of breath (dyspnea). This can be present with exertion (activity) initially and progress to being present with even less activity or even when at rest. Other symptoms include:

• Fatigue
• Chest pain or tightness
• Rapid heart beating (palpitations)
• Dizziness, lightheadedness, episodes of passing out
• Swelling in your legs, feet or abdomen

What are the risk factors and triggers?

Pulmonary hypertension is divided into five categories (or groups) based on the cause.

• Group 1: Pulmonary Arterial Hypertension may be from an unknown cause (idiopathic), passed down from parents to child (heritable), congenital heart disease (present at birth), connective tissue disease, liver disease, HIV infection or from certain medications or street drugs.
• Group 2: Pulmonary Hypertension associated with weakness of the left side of the heart or from heart valve dysfunction.
• Group 3: Pulmonary Hypertension associated with lung disease or low oxygen levels (hypoxia). These conditions can include interstitial lung disease, emphysema, chronic obstructive lung disease (COPD) or untreated sleep apnea (periods of stopping breathing during sleep).
• Group 4: Pulmonary Hypertension due to blood clots in the lungs (pulmonary emboli).
• Group 5: Pulmonary Hypertension from other causes such as chronic kidney disease, sickle cell anemia, thyroid disorders, sarcoidosis and other diseases.

How is it diagnosed?

Your healthcare provider will discuss your past medical history, symptoms, and perform a physical exam. If PAH is suspected, further tests may be ordered and can include:

• Chest x-ray (to evaluate the lungs, as well as look at the heart size)
• CT scan (imaging that can look at the lung tissue and pulmonary artery size)
• Pulmonary function tests (breathing tests that measure how much air you can breathe in and out of your lungs)
• 6 minutes walk test (to monitor your oxygen level while you are active)
• Ventilation-perfusion scan [VQ Scan] (to look for blood clots within the blood vessels in the lungs)
• Echocardiogram (ultrasound of the heart evaluate heart function and valve function)
• Right heart catheterization (a procedure to measure directly the amount of pressure the heart is having to generate to pump blood through the lungs)

What is the treatment?

The treatment depends on what is causing the pulmonary hypertension and is directed at managing that condition. There are treatment options aimed at relieving symptoms, slowing the progression of the disease and to enlarge the blood vessels in the lungs. Other medications may be used to help the heart function more effectively and medication to help make the blood thin to prevent the blood from clotting. Pulmonary hypertension in Group 4 due to pulmonary emboli (blood clots) has a potential cure with surgery. In severe cases, heart-lung transplant may be discussed.

The Advanced Lung Disease program at Piedmont Healthcare will take a comprehensive approach to diagnosing, treating and providing specialty care for patients with pulmonary arterial hypertension. We are committed to providing our patients with the highest quality evidence based care, as well as support and educational opportunities, to improve understanding of the disease state and quality of life.