About Pulmonary Arterial Hypertension

Disclaimer: I am in no way a medical professional. Please do not take the information here to self-diagnose you or someone you know. Please visit a doctor to receive help if you need it. Additionally, this is in no way a comprehensive article about pulmonary arterial hypertension. I have included what was relevant to my own personal case of PAH. Please continue to research for yourself and/or speak to your doctor if needed.

Words to Know:

Chronic Illness: An illness that lasts longer than 1 year and requires ongoing treatment, and impacts daily life

Congestive Heart Failure (or Heart Failure): A chronic condition where your heart muscle has trouble pumping blood like it should

Edema: Swelling of the limbs

Idiopathic: The cause of the disease is unknown

Pulmonary Hypertension: The general term for high blood pressure in the lungs, also called PH

Pulmonary Arterial Hypertension: A specific type of PH where the blood vessels in the lungs have become too narrow to push blood through effectively. Also called PAH.

WHO: World Health Organization – an agency related to the United Nations that is responsible for international public health

About Pulmonary Arterial Hypertension (PAH)

PAH is a chronic, progressive lung disease that hardens the arteries in your lungs, and affects the cardiac vessels and right side of your heart.

Due to the hardening of your blood vessels and arteries, high blood pressure manifests in your lungs. Your heart works overtime to pump the blood through your body, enlarging the right side of your heart, causing congestive heart failure.

Symptoms can include:

-Swelling of your body/limbs

-Shortness of breath, especially with exertion

-Dizziness

-Fast heart rate

-Palpitations

-Fainting

-Death

Research has said that typically, people who have Pulmonary Arterial Hypertension will die within 5 years if they receive no diagnosis or treatment.

Causes

There are some diseases that can cause PH/PAH, like Sarcoidosis, pulmonary embolisms, HIV, sleep apnea, and others.

My personal case of PH is classified as WHO Group 1 (Pulmonary Arterial Hypertension) and idiopathic – meaning the cause of my PAH is unknown. Research is being done to see how genetics play a role in PAH and that is interesting to me because I have a family member that also has PAH. However, more research needs to be done to definitively say that genetics has any effect on PAH.

Treatments

While there are several treatments available, there is no cure. Treatments serve to slow down the progression of the disease and in some cases, repair damage that was done to your heart.

Treatments range from oral medications, inhalers, subcutaneous infusions and IV infusions. Some patients also need oxygen during the day or night, or all the time. It is very common for PAH patients to need lung and heart transplants.

In some cases, medications are prescribed that help the symptoms of PAH. For example, lasix help take extra fluid off your body, thereby helping to relieve edema and easing the workload of your heart and arteries.

Maintenance

Your doctor will develop a treatment/maintenance plan for you. Personally, I go to my doctor’s appointments in downtown Houston in the Texas Medical Center a few times a year (I visit my doctor more if I am having issues), and complete an Echo and 6 minute walk test. Sometimes, I also have heart catheterization procedures.

Lastly, if you do have PAH, I strongly suggest finding a doctor that is a PAH specialist if possible. Not a pulmonary hypertension specialist, someone who works ONLY in Pulmonary Arterial Hypertension. Your PAH specialist might require you to visit other doctors as well, like a pulmonologist or a cardiologist, depending on what they need to treat you best.

References

What is PAH?

About PH

Types of Pulmonary Hypertension: The WHO Groups

https://www.heart.org/en/health-topics/heart-failure/what-is-heart-failure

https://www.cdc.gov/chronicdisease/about/index.htm