Understanding your PAH.

Pulmonary arterial hypertension (PAH) means high blood pressure (hypertension) in some blood vessels (arteries) in your lungs (pulmonary). It is complex, but here is an overview that may help you understand PAH and explain it to others.

With PAH, the arteries in your lungs become thinner and stiffer, and may become blocked. These changes make it harder to get enough blood through those vessels into the lungs and then to the heart. When the arteries are “tight,” it’s harder to push blood through them. So, the heart has to work harder and less oxygen gets pumped through the body. Over time, the extra work causes damage to the heart, especially the right side of the heart. When the right side of the heart is damaged, it’s even harder to get oxygen to the lungs.

There are different reasons why the blood vessels become narrow and stiff. But whatever the cause, the symptoms of PAH are the same. These include:

• Trouble breathing. In the early stages of PAH, a person might not notice any shortness of breath (dyspnea) except when exercising. Over time, shortness of breath is there even when resting.
• Feeling tired all the time (fatigue)
• Dizziness or lightheadedness
• Fainting (syncope)
• Chest pain or tightness
• Swelling (edema) in the legs and feet and sometimes in the stomach area (abdomen)
• Lips and skin may start turning a bluish color because not enough oxygen is getting pumped through the body
• Feeling that your heart is fluttering or beating very fast (palpitations)

Because blood vessels are narrow, some people with PAH may get blood clots in their lungs. Some may get an unusual heart rhythm. Both can be life-threatening. Some patients develop a condition called Chronic Thromboembolic Pulmonary Hypertension (CTEPH) and can get blood clots often.

PAH symptoms can often overlap with other heart and lung conditions. To help diagnose PAH, your doctor will run tests to help see how well your heart and lungs are working. These tests usually include a chest x-ray, blood tests, an electrocardiogram (EKG) and echocardiogram. A lung scan (called a pulmonary ventilation/perfusion or VQ scan) may also be done to show how well and where air flows through the lungs. If the doctors think you have PAH, they will do a right-heart catheterization to measure the pressure to make sure it’s PAH and see how severe it is. All this information helps you and your health care providers make a treatment plan.

There is no cure for PAH, but many medications can help manage the symptoms and slow the progression. The medications work in different ways, but all help the blood flow more easily though the arteries in the lung. Then, the heart doesn’t have to work as hard. Some patients also need water pills (diuretics) or oxygen. In some cases, surgery may be needed. Some patients may eventually need to think about a lung transplant.

As PAH progresses, it becomes harder for the patient to breathe. This makes it difficult for the patient to get around and they get tired easily.

For more information, you may find this 4-part YouTube video series helpful: https://www.youtube.com/channel/UCltKGUo-QN_Ij8oVbOfl9iw. Each video is about four minutes long.

The more you understand your PAH, the better you will be able to manage your symptoms. And it’ll be easier to work with your care team to make and adjust treatment plan. Talk to your doctor and your CVS Specialty PAH care team any time you have questions about your symptoms, treatment or supplies. Call 1-87-PAHCARE-8 (1-877-242-2738), anytime.