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Pulmonary Arterial Hypertension

Pulmonary hypertension is a life-threatening condition that gets worse over time, but treatments can help your symptoms so you can live better with the disease. It may take some planning, but plenty of people who have it find ways to do all the things they love, just as they did before they were diagnosed.

Having pulmonary arterial hypertension (PAH) means that you have high blood pressure in the arteries that go from your heart to your lungs. It's different from having regular high blood pressure.

With PAH, the tiny arteries in your lungs become narrow or blocked. It's harder for blood to flow through them, and that raises the blood pressure in your lungs. Your heart has to work harder to pump blood through those arteries, and after a while the heart muscle gets weak. Eventually, it can lead to heart failure.

Sometimes doctors can't find a reason for high blood pressure in the lungs. In that case, the condition is called idiopathic pulmonary hypertension. Genes may play a role in why some people get it.

In other cases, there is another condition that's causing the problem. Any of these illnesses can lead to high blood pressure in your lungs:

You may not notice any symptoms for a while. The main one is shortness of breath when you're active. It usually starts slowly and gets worse as time goes on. You may notice that you can't do some of the things you used to without getting winded.

Other symptoms include:

If you have shortness of breath and see your doctor, they will ask you about your medical history. They may also ask you:

Do you smoke?

Does anyone in your family have heart or lung disease?

When did your symptoms start?

What makes your symptoms better or worse?

Do your symptoms ever go away?

Your doctor may order tests, including:

Echocardiogram: This ultrasound picture of the beating heart can check blood pressure in the pulmonary arteries.

CT scan: This can show enlarged pulmonary arteries. A CT scan can also spot other problems in the lungs that could cause shortness of breath.

Ventilation-perfusion scan (V/Q scan): This test can help find blood clots that can cause high blood pressure in the lungs.

Electrocardiogram (EKG or ECG): An EKG traces the heart's activity and can show whether the right side of the heart is under strain. That's a warning sign of pulmonary hypertension.

Chest X-ray: An X-ray can show if your arteries or heart are enlarged. Chest X-rays can help find other lung or heart conditions that may be causing the problems.

Exercise testing: Your doctor may want you to run on a treadmill or ride a stationary bike while you are hooked up to a monitor, so they can see any changes in your oxygen levels, heart function, lung pressure, or other things.

Your doctor may also do blood tests to check for HIV and conditions like rheumatoid arthritis or lupus.

If these tests show that you might have pulmonary hypertension, your doctor will need to do a right heart catheterization to be sure. Here's what happens during that test:

The doctor places a catheter into a large vein, most often the jugular vein in your neck or femoral vein in your leg, and then threads it into the right side of your heart.

A monitor records the pressures in the right side of the heart and in the pulmonary arteries.

The doctor may also inject medicines into the catheter to see if the pulmonary arteries are stiff. This is called a vasoreactivity test.

Right heart catheterization is safe. The doctor will give you a sedative and use local anesthesia. You can usually go home the same day, although you will need someone to drive you home.

You may want to write down a list of questions before your appointment, so you can make sure you ask your doctor everything you want to. It can also help to have a friend or family member with you to help you get the answers you want.

Some possible questions are:

What's the best treatment for me?

How often should I see a doctor for my condition?

Do I need to see a specialist?

When should I go to the emergency room?

Do I need to limit the salt or fluids in my diet?

What kind of exercise can I do?

Are there any activities I should stay away from?

Should I get a pneumonia vaccine, a flu shot , and a COVID-19 vaccine?

Pulmonary hypertension varies from person to person, so your treatment plan will be specific to your needs. Ask your doctor what your options are and what to expect.

First, your doctor will treat the cause of your condition. For example, if emphysema is causing the problem, you'll need to treat that to improve your pulmonary hypertension.

Most people also get treatment to improve their breathing, which makes it easier to be active and do daily tasks. Oxygen therapy, when you breathe pure oxygen through prongs that fit in your nose, will help if you're short of breath and have low oxygen levels in your blood. It helps you live longer when you have pulmonary hypertension. If you are at risk for blood clots your doctor will recommend blood thinners. Other medicines improve how well your heart works and keep fluid from building up in your body.

If you have severe pulmonary hypertension, your doctor may prescribe medications called calcium channel blockers. These medicines lower blood pressure in the lungs and the rest of the body.

If calcium channel blockers aren't enough, your doctor may refer you to a specialized treatment center. You may need more targeted therapies that can open up your narrowed blood vessels. They may be pills, medicines you breathe in, or drugs that are given through an IV. Options include:

Pills: ambrisentan (Letairis), bosentan (Tracleer), macitentan (Opsumit), riociguat (Adempas), selexipag (Uptravi), sildenafil (Revatio), tadalafil (Adcirca), treprostinil (Orenitram)

Inhalers: Iloprost tromethamine (Ventavis), treprostinil (Tyvaso)

IV drugs: epoprostenol sodium (Flolan, Veletri), treprostinil (Remodulin)

In more severe cases, or if medicines don't help, your doctor may recommend a lung transplant or a procedure called atrial septostomy. A surgeon creates an opening between the right and left sides of the heart. This surgery can have serious side effects.

One of the best things you can do for yourself is to stay active, even if you have shortness of breath. Regular exercise, like taking a walk, will help you breathe better and live better. Talk to your doctor first to find out what kind of exercise is best for you, and how much you should do. Some people may need to use oxygen when they exercise.

Get plenty of rest, too. Pulmonary hypertension makes you tired, so get a good night's sleep and take naps when you need to.

Just like anyone else, it's good for you to eat a healthy diet with lots of fruits, vegetables, and whole grains. That's important for your overall health.

A lot depends on what's causing your pulmonary hypertension. Treating an underlying condition will help you feel better. There's no cure for pulmonary hypertension, but the earlier it's diagnosed, the easier it is to live with.

If you have idiopathic pulmonary hypertension -- the kind where doctors can't find a cause -- your symptoms may get worse over time. But treatment can slow down the progress of the disease and help you live longer.

Remember that each person is different, and there are good treatments available. Work with your doctor to find what's right for you.

The Pulmonary Hypertension Association offers in-depth information on everything from medications to tips on making daily tasks easier. It also has an active online support community.

Another Doctor Appointment, But With Unexpected Good News

It's not often that pulmonary arterial hypertension (PAH) patients enter a visit with their physician and hear what they want to hear. Many times for my husband, Tim, visits have been a gross reminder of his condition and how limiting it can be. A typical, predictable rundown: check-in, vital signs, six-minute walk test (always trying, even subconsciously, to beat his last effort), echocardiogram, chest X-ray, and labs.

The results of those standard tests may vary, but they rarely improve significantly. Such a result would be unexpected. Running instead of walking, having endurance instead of stopping to rest, and taking the stairs instead of the elevator as a choice instead of a need: They're all significant improvements, but they aren't something that happens without divine intervention.

The standard for Tim would be small improvements and an activity level that allows him to enjoy his life and work. Hearing that his cardiac function hasn't worsened feels like a huge win. Oxygen levels staying greater than 90% during a six-minute walk test can inspire a fist pump and encouragement.

Tim's way of working through his PAH diagnosis has always been to push himself with treatments, keep current with research news, and stay active in his care. That last one is how he deals with the reality that PAH is progressive. Looking for large improvements can be futile.

As I help care for him, I choose to offer advice and support when asked, in whatever way he chooses. That works for us and keeps our expectations managed. Support, for me, means trusting him and being there for the provider visits. I'm present to pick up his mood when his PAH news is not so great, or to celebrate with him when news exceeds his expectations.

But he often leaves an appointment reminded that he's sick, which makes simply maintaining his health good news.

There was a moment a few years ago, however, at a routine appointment with his PAH physician, in which Tim's optimism served him well. It clicked for me then that I'll continue to support him handling his disease in his own way.

Tim's physician looked over his six-minute walk test results, labs, and studies with her usual precision and said, "Well, Tim, looks like you'll die from something other than PAH."

I started tearing up, with years of suppressed feelings and fear spilling out. Tim simply smiled at me, winked, and said it was what they'd been working toward for 18 long years. It's what we both needed to hear.

Note: Pulmonary Hypertension News is strictly a news and information website about the disease. It does not provide medical advice, diagnosis, or treatment. This content is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a medical condition. Never disregard professional medical advice or delay in seeking it because of something you have read on this website. The opinions expressed in this column are not those of Pulmonary Hypertension News or its parent company, BioNews, and are intended to spark discussion about issues pertaining to pulmonary hypertension.

PAH Today Broadcast Series Returns With Advice On Managing Disease

The PAH Initiative's PAH Today national broadcast series returns in September and November with presentations from a pulmonary arterial hypertension (PAH) specialist for adult patients and their caregivers on managing the disease.

This year's series, which offers insights and perspectives on coping with PAH, includes two programs covering what PAH specialists look for when evaluating echocardiogram images of the heart, and what patients should know before a next appointment, according to a PAH Initiative press release shared with Pulmonary Hypertension News.

The first broadcast, "How Specialists Use Echo to Monitor Your PAH," will be presented on Sept. 6 from 8-9 p.M. ET and on Sept. 16 from 2-3 p.M. ET.

The second presentation, "5 Things You Need to Know Before Your Next PAH Appointment," is set for Nov. 9 from 8-9 p.M. ET and Nov. 11 from 4-5 p.M. ET. Registration is required, limited to adults, and can be done for both events at the same time.

PAH Initiative presentations aiming to advance disease care

Each presentation will include a question-and-answer segment with the speaker, Lana Melendres-Groves, MD, a pulmonologist and medical director of the pulmonary hypertension program at the University of New Mexico.

The PAH Initiative, sponsored by United Therapeutics, seeks to advance PAH care by providing patients and caregivers with reliable information, and inspiration, to live as full lives as possible. The disease, a rare type of pulmonary hypertension (PH), is characterized by a narrowing of the pulmonary arteries, small blood vessels that transport blood through the lungs, making it for the heart to pump blood throughout the body.

United Therapeutics markets various PAH treatments in the U.S., including Adcirca (tadalafil), Orenitram (extended-release treprostinil), Remodulin (treprostinil; generics available), and Tyvaso (inhaled treprostinil).

A proper PH diagnosis can be difficult and delay the start of treatment, because disease symptoms can be similar to other heart or lung conditions. Physicians commonly use an echocardiogram, a noninvasive test of the heart's electrical activity, to confirm a diagnosis.

With the September presentations, patients will better understand how echocardiograms also can help with treatment planning and symptom management, and learn the importance of seeking a PAH specialist. They also will be able to view echocardiogram results to learn what the images mean and be able to ask better questions when discussing test results with their specialist.

"Today, noninvasive assessments like the Echo enable PAH specialists to routinely monitor your PAH and take action earlier—with the goal of delaying or preventing symptom worsening," according to the PAH Today webpage about the event. "You will learn more about how PAH specialists monitor your heart to optimize a treatment plan that may help you feel better and be more active."

Goals of the November broadcasts include providing a clear understanding of PAH's impact on the heart and lungs, as well as the fundamentals of how PAH medications work. Attendees also will explore the benefits of treating multiple PAH pathways and learn about topics they might want to discuss with their PAH specialist to improve a treatment plan.

"This program will cover a range of important topics – how amazing your heart is, how PAH medications work (and why it matters), and how to have more honest, 'real' discussions with your doctor," the webpage states.

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