Pulmonary hypertension (PH) is a condition that causes increased blood pressure in the pulmonary arteries and the right side of the heart. The pulmonary arteries carry blood from the heart and into the lungs.
Five types of PH exist. One common type is called pulmonary arterial hypertension (PAH). It’s a severe disease that progresses rapidly and usually causes right heart failure. In many cases, PAH is fatal and leads to death.
What Causes PAH?
The pulmonary arteries carry blood from the heart to the lungs, where the blood picks up oxygen. In people with PAH and IPAH, the small arteries become narrowed and constricted. One or more of these three things typically causes the narrowed arteries:
- Tiny blood clots form in the smallest arteries in the lungs and block blood flow.
- Increased cell growth in the blood vessels causes abnormally thick artery walls.
- Artery walls squeeze and make blood flow more difficult.
IPAH and Genes
Some people with IPAH may have a genetic predisposition to the disease. In other words, an inherited gene may play a role in determining who develops unexplained PAH and who doesn’t.
The first thing a doctor will do when they detect PAH is to search for a cause that explains the high blood pressure. But, people diagnosed with IPAH show no recognizable cause for pulmonary high blood pressure.
What are the signs and symptoms of IPAH?
- Weakness and tiredness
- Weight gain or lack of appetite
- Joint pain
- Shortness of breath with exercise
- Abdominal swelling
- Chest pain or heart palpitations (strong, fast heartbeats)
- Dizziness or feeling faint
How is IPAH diagnosed?
Your caregiver will ask about your medical history and examine you. You may need any of the following tests:
Blood tests: You may need blood taken from your hand, arm, or IV. This is done to help caregivers find out what is causing your IPAH.
Blood vessel test: This is done to see if the small arteries in your lungs will widen (dilate) when you are given a medicine. The pressure in your pulmonary artery will be measured before and after the medicine is given.
ECG: This is also called an EKG. An ECG is done to check for damage or problems in your heart. A short period of electrical activity in your heart is recorded.
Cardiac catheterization: A thin, bendable tube inserted into an arm, neck, or groin vein is moved into your heart. Your caregiver may use an x-ray to guide the tube to the right place. Dye may be put into your vein so the pictures show up better on a monitor.
Chest x-ray or echocardiogram: A chest x-ray is a picture of your lungs and heart. An echocardiogram is a type of ultrasound. Sound waves are used to show the structure, movement, and blood vessels of your heart.
CT scan: This test is also called a CAT scan. An x-ray uses a computer to take pictures of your heart and lungs. You may be given dye before the pictures are taken to help caregivers see the pictures better.
Tell caregivers if you are allergic to iodine or seafood. You may also be allergic to the dye.
MRI: This scan uses powerful magnets and a computer to take pictures of your heart and lungs. You may be given dye to help the pictures show up better. Tell caregivers if you are allergic to iodine or seafood. You may also be allergic to the dye.
Do not enter the MRI room with anything metal. Metal can cause serious injury. Tell caregivers if you have any metal in or on your body.
Pulmonary function tests: Pulmonary function tests (PFTs) help caregivers learn how well your body uses oxygen. You breathe into a mouthpiece connected to a machine. The machine measures how much air you breathe in and out over a certain amount of time. PFTs help your caregivers decide the best treatment for you.
V/Q Scan: This is a ventilation (V) and perfusion (Q) test. This test is also called a VP scan. A V/Q scan is a two-part test which takes pictures of your lungs to look for certain lung problems.
During the perfusion part of the test, radioactive dye is put into your vein (blood vessel). The blood carries the dye to the blood vessels in your lungs. Pictures are taken to see how blood flows in your lungs.
During the ventilation part of the test, you breathe in special gas. Pictures are taken to see how well your lungs take in oxygen.
How is IPAH treated?
There is no known cure for IPAH. The goals of treatment are to improve your condition and stop it from getting worse. You may have any of the following:
Blood thinners: These prevent blood clots. They may make you bruise or bleed more easily. Use a soft toothbrush and an electric shaver to prevent bleeding.
Diuretics: These help your body get rid of extra fluid and protect your heart from more damage. You may urinate more often while you are taking diuretics.
Vasodilators: These improve blood flow by making the vessels in your heart and lungs wider. These may be given as a pill, an inhaler, or IV.
You may need extra oxygen if your blood oxygen level is lower than it should be. You may get oxygen through a mask placed over your nose and mouth or through small tubes placed in your nostrils.
Ask your healthcare provider before you take off the mask or oxygen tubing.
Shunt: This is surgery to help blood flow from one part of the heart to another using a tube called a shunt. This increases the blood flow and oxygen delivery to your body.
Transplant: This is surgery to remove and replace a damaged organ with a healthy organ from a donor. You may need a lung or heart transplant if other treatments do not work and your condition is severe.
Life Expectancy with IPAH
Ultimately, IPAH and most forms of PAH are fatal. Treatments, including medicines and surgeries, can only slow the progression of the aggressive disease, not stop it.
If you’ve been diagnosed with IPAH, work with your doctor and healthcare professionals to create a disease management plan.
This plan should include treatment options, exercise regimens, and lifestyle changes that can make living with IPAH more comfortable.
Together, you and your healthcare team can make dealing with IPAH less burdensome and make your days more fulfilling.