What is Pulmonary Arterial Hypertension (PAH)?
Pulmonary arterial hypertension (PAH) is a progressive disorder with high blood pressure in the arteries of the lungs.
Every year in the United States, about 1,000 patients are diagnosed with PAH.
However, unfortunately, there is no cure for PAH.
After PAH diagnosis, the mean survival time of PAH patients is about 2.8 years.
Every year in the United States, about 1,000 patients are diagnosed with PAH.
However, unfortunately, there is no cure for PAH.
After PAH diagnosis, the mean survival time of PAH patients is about 2.8 years.
What do the arteries in PAH patients look like?
As the above figure shows, pulmonary arteries can be changed significantly under different conditions. Healthy pulmonary arteries have large diameter. However, with PAH, cell division is promoted but cell death is prevented in the arteries. These result in an overgrowth of cells. Therefore, pulmonary arteries narrow in diameter, increasing the resistance to blood flow
What does a PAH heart look like?
Left figure shows a healthy heart and right figure shows a heart with PAH.
The narrowed pulmonary arteries in PAH cause high resistance to blood flows to lungs. Then in order to overcome the high resistance, pressure would increase in the pulmonary arteries and in the right ventricle, which pumps the blood into the pulmonary arteries. To provide more blood to the pulmonary arteries and lungs, right heart and heart wall muscle become larger. However, because of the enlarged right heart, left heart becomes smaller.
The narrowed pulmonary arteries in PAH cause high resistance to blood flows to lungs. Then in order to overcome the high resistance, pressure would increase in the pulmonary arteries and in the right ventricle, which pumps the blood into the pulmonary arteries. To provide more blood to the pulmonary arteries and lungs, right heart and heart wall muscle become larger. However, because of the enlarged right heart, left heart becomes smaller.
What are the symptoms of PAH?
PAH worsens over time and is life-threatening because the pressure in a patient's pulmonary arteries rises to dangerously high levels, putting a strain on the heart. Therefore, it is important to be diagnosed before PAH worsens.
Symptoms of PAH are:
However, it may be difficult for a doctor to diagnose PAH because the symptoms of PAH are similar to other heart and lung problems. Those disorders include asthma, pneumonia, chronic obstructive pulmonary disease (COPD), left heart failure and coronary disease. For this reason, Patients and doctors might overlook the disorder until symptoms become severe.
Symptoms of PAH are:
- Chest pain, usually in the front of the chest
- Dizziness
- Fainting
- Fatigue
- Leg swelling
- Light-headedness during exercise
- Shortness of breath during activity
- Weakness
However, it may be difficult for a doctor to diagnose PAH because the symptoms of PAH are similar to other heart and lung problems. Those disorders include asthma, pneumonia, chronic obstructive pulmonary disease (COPD), left heart failure and coronary disease. For this reason, Patients and doctors might overlook the disorder until symptoms become severe.
How can PAH be diagnosed?
There are several ways to diagnose PAH. In early stages of the disease, a physical exam may be normal or almost normal. Therefore, it may take your doctor several months to diagnose PAH. A doctor who suspects a patient may have PAH usually runs a series of tests to measure blood pressure in the pulmonary arteries, decide how well the heart and lungs are working, and rule out other diseases.
Tests include:
Tests include:
- Chest X-Ray (of the heart and lungs)
- Electrocardiogram (an electrical measure of your heart rate and how regularly your heart is beating)
- Echocardiogram (an ultrasound picture of the heart to help determine how well it is working)
- Right-heart catheterization (a special device that the doctor threads through a vein into the heart and arteries of the lungs to measure pressure in the arteries and test how well the heart is pumping)
- Pulmonary function tests (breathing tests that measure lung function)
- Perfusion lung scan (an image that shows blood flow through the lungs)
- Six-minute walk test (a measure of the patient's ability to do exercise)
Is there any treatment for PAH?
There is no cure for PAH, but there are several treatment options.
Treatments include medications and surgery. patients also make lifestyle changes as their PAH worsens and limits daily activity. Treatments for PAH aim to reduce symptoms, improve quality of life, and slow down how quickly the disease progresses.
Treatments include medications and surgery. patients also make lifestyle changes as their PAH worsens and limits daily activity. Treatments for PAH aim to reduce symptoms, improve quality of life, and slow down how quickly the disease progresses.
There are online communities to share information about PAH and medications such as Tyvaso, Ventavis, and Letairis to treat PAH. Click those if you want to know more about PAH treatments.
If the video doesn't work, please click PLAY VIDEO button and it will connect you to Youtube.
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Reference
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- Image: http://www.greenbarleymanila.com/wp-content/uploads/2012/11/bp-flow.jpg
- Image: Heart patient icon. (n.d.). Dreamstime. Retrieved May 16, 2014, from http://www.dreamstime.com/royalty-free-stock-photography-heart-patient-icon-image650957
- Image: Cure For All Diseases: Does Exist? by eNaturalHealthCures.com. (n.d.). e Natural Health Cures. Retrieved May 16, 2014, from http://www.enaturalhealthcures.com/cure-for-all-diseases.html
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- ABOUT PAH*. (n.d.). What is pulmonary Arterial Hypertension*. Retrieved May 16, 2014, from http://www.4ventavis.com/patient_about_pah.asp
- Welcome to Living PAH. (n.d.). wwwLivingPAHcom. Retrieved May 16, 2014, from http://www.livingpah.com
- Breathe In the Power of a Prostanoid. (n.d.). Tyvaso® (treprostinil) Inhalation Solution. Retrieved May 16, 2014, from http://www.tyvaso.com/dtc
- A SECOND WIND®. (n.d.). Official PAH* and Ventavis Information. Retrieved May 16, 2014, from http://www.4ventavis.com
- Letairis® (ambrisentan) | Official Site. (n.d.). Letairis® (ambrisentan) | Official Site. Retrieved May 16, 2014, from https://www.letairis.com/patients/
- Video: Pathophysiology of Pulmonary Arterial Hypertension (PAH). (n.d.). YouTube. Retrieved May 16, 2014, from http://www.youtube.com/watch?v=9a4untSzLzg