What Does Vaping Do to Your Lungs?
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:
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:
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:
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:
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.
Keeping Track Of Your Pulmonary Arterial Hypertension
If you have pulmonary arterial hypertension (PAH), it's possible to lead an active, productive life. The key is to work closely with a specialist, usually a lung doctor (pulmonologist) or a heart doctor (cardiologist).
"The cardiologists who do this have an appreciation for the effect on the lungs, and the pulmonologists who do it have an appreciation for the impact on the heart. There are a lot of wonderful specialists," says Jamie Garfield, MD, a pulmonologist and associate professor of thoracic medicine and surgery at Temple University.
Because PAH is a condition that can get worse over time, it's important to get certain medical tests every few months. These help your doctor understand how well your treatments are working or if they're not.
"This disease is not like one and done: You don't just come in, you get started on a pill, and you're done," Garfield says.
Two exams your specialist might order are the 6-minute walk test and the echocardiogram.
What Is a 6-Minute Walk Test?The goal is simply to walk as far as you can at a normal pace for 6 minutes. The course you walk on is a flat, hard surface indoors, and it can be as basic as a 100-foot hallway with a chair or cone that you pace back and forth to.
"You walk around a set course for 6 minutes, and we monitor how your oxygen level handles that walk and what happens to your heart rate," Garfield says.
Your blood pressure, pulse, and oxygen level are measured before you begin walking. You'll be asked to rate how tired you feel and if you're having any trouble breathing.
Once you start walking, the person giving you the test will let you know how much time is left after each minute goes by. It's OK to slow down, rest, or stop at any point. Let them know if you have any symptoms, like chest pain or trouble breathing. They'll be ready to help you right away if you need it.
After the 6 minutes are up, they'll ask you again to rate your breathing and fatigue level, and they'll measure how far you walked. They may measure your oxygen level and check your pulse again, too.
What Is an Echocardiogram?It's an imaging test that uses sound waves to see inside your body and give your doctor a look at your heart.
"It's similar to an ultrasound that you use on pregnant women," Garfield says. "An echocardiogram shows us the size of the heart, the chambers of the heart, and the flow of the blood through these chambers. Most importantly, we can use it to measure pressure in the heart and in the pulmonary arteries."
Before the test, you'll take off your clothes from the waist up and put on a hospital gown. A technician will put three small, sticky patches called electrodes on your chest. These are attached to a monitor that tracks your heart's electrical activity during the exam. The technician might also have you wear a mask that tracks how well your heart and lungs use oxygen and carbon dioxide.
They may have you lie on your left side on an exam table. But some people get an echocardiogram while pedaling a stationary bike or walking on a treadmill.
During the test, your technician places a handheld wand over your chest, and it gives off high-frequency sound waves to make pictures of your heart. The wand will have some gel on its end, which helps make clearer images. If the technician has you lie down for the test, they may ask you to change positions or hold your breath at times.
The test shouldn't cause you any pain or serious discomfort. The gel on the wand may feel cool on your skin, and you might feel a slight pressure from the wand itself.
How Does Your Doctor Use Your Test Results?If the results of a 6-minute walk test or echocardiogram show that your PAH is getting worse, the doctor may change your treatment plan. Depending on your specific situation, they might:
Your doctor might also recommend more tests, like right-heart catheterization, Garfield says. Doctors usually use this test to help diagnose the condition, she says, but they may need to do it again if other tests show your PAH is getting worse.
Right-heart catheterization is when your doctor puts a small, thin, flexible tube called a catheter into a large vein, usually in your neck or groin.
"We thread that catheter through the blood vessels until we reach the heart, and in the heart we're able to measure pressures," Garfield says.
Before the procedure, your doctor gives you medicine that numbs the body part where the catheter goes in. You'll lie down on a table during the procedure and stay awake. During right-heart catheterization, your doctor may ask you to do things like hold your breath, bear down, and cough. This all takes about an hour.
As you're getting tests to track your pulmonary arterial hypertension, keep going to all your follow-up appointments, and be sure to tell your doctor how you're feeling.
Considering Therapy Based On PH And PAH Subpopulations
Elucidation of the various treatment strategies used in PH and PAH subpopulations: CTEPH, CTD-PAH, and POPH.
Transcript
Charles D. Burger, MD: As the physician is making decisions with the patient regarding the best treatment approach, there are several factors that impact those decisions. Most importantly is the severity of the disease, which guides what types of therapies we use in terms of potency, method of delivery, and what we know about them from a science perspective. But subpopulations of Group 1 PAH [pulmonary arterial hypertension] do impact that decision as well. For example, we know that PAH in association with connective tissue disease [CTD] is extremely challenging. The reason the patient has PAH is the systemic connective tissue disease, which of course isn't going away. That remains forever. There's ongoing risk to the pulmonary circulation forever because there is an association with the underlying connective tissue disease.
In general, we want to treat those patients as aggressively as possible because of that risk. We know that if we are not as aggressive as we should be, the patients struggle more than perhaps some of the other subgroups. It's also very interesting that some of the information has shown that using 2 oral medications in different pathways as soon after diagnosis as possible—what's called upfront combination oral therapy, which was shown in the AMBITION trial to be beneficial to Group 1 PAH patients—also holds value in connective tissue disease. Based on that scientific information and personal experience, I advocate for patients with connective tissue disease—associated mild or intermediate PAH to start 2 oral drugs as early as possible after diagnosis. Obviously, if they're sicker, they may have to be on an infusion therapy or triplet therapy.
For patients with liver disease and portopulmonary hypertension [POPH], the goal is not only to treat the pulmonary hypertension [PH] but also to allow the patient to safely receive a liver transplant. Ultimately, the problem is end-stage liver disease or hepatic cirrhosis. If that's not remedied, even if you control and improve the pulmonary hypertension, patients are still at risk of having complications from cirrhosis or even dying from liver failure. The goal is to get pressures down to a certain level, typically a mean pulmonary artery pressure of 35 mm Hg or less, with low pulmonary vascular resistance and normal right ventricular function by echocardiography, which then allows the patients to safely receive a liver transplant.
We work carefully with our GI [gastrointestinal] hepatologists and our liver transplant surgeons. We discuss these patients carefully at multidisciplinary conferences regarding the best therapy and the goals of therapy that can get them in an appropriate condition such that they can undergo a liver transplant if they're otherwise eligible, which has a very good chance of improving the pulmonary hypertension as well. It's not a guarantee that pulmonary hypertension would go away completely, but there is often a very favorable response, at least allowing for less medication after the transplant. Certainly, patients feel better if their liver is now working properly.
For Group 4 chronic thromboembolic pulmonary hypertension [CTEPH], decisions around drug therapy really hinge on if the patient is eligible for surgery—pulmonary thromboendarterectomy—which if successful, has a very high cure rate. About 10% of patients may have persistent pulmonary hypertension after the surgery, so they may require ongoing medication. Almost always, the initial therapy in that group, if they're not going to have surgery, is riociguat.
A new intervention that's receiving more study is balloon pulmonary angioplasty. There is some information showing that it can have a very positive effect on pulmonary pressures and pulmonary vascular resistance with similar or even slightly better benefit than riociguat, based on 1 study. But remember, it's an intervention, so it's going to have complications. Complications can be severe when you're working with a catheter in pulmonary circulation. The potential complications may balance out any potential improvement over drug therapy. That becomes an individualized decision between the physician and the patient. But we're very excited about the possible role of balloon pulmonary angioplasty in these patients who would not otherwise be eligible for the more curative surgery, pulmonary thromboendarterectomy.
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