Science-Backed Ways To Manage Pulmonary Hypertension

Pulmonary hypertension is high blood pressure specifically in the arteries that lead to your lungs. This can be related to heart or lung disease or appear for no apparent reason. In the latter case, it is typically hereditary.

Like other forms of high blood pressure, there is no cure for PH, but there are ways you can manage the condition.

Symptoms of pulmonary hypertension

Unlike more generalized hypertension that often has no warning signs, pulmonary hypertension (PH) does have symptoms. These include:

• Chest pain

• Racing heartbeat

• Pain on the upper right side of the abdomen

• Decreased appetite

• Fatigue

• Shortness of breath during routine activities

Pulmonary hypertension tends to worsen over time and can significantly limit your ability to do any physical activity, including those that we associate with normal, daily living.

If you have these symptoms, your doctor will likely recommend an echocardiogram, an ultrasound scan of your heart that can also show the pressure in the pulmonary arteries. This may be done while you are exercising. You may also get a chest x-ray to inspect your lungs and an electrocardiogram to check for abnormal heartbeats.

How to manage pulmonary hypertension

There is no cure for pulmonary hypertension, and treatment focuses on managing symptoms and slowing the progress of the disease. It can take some time to find the right treatment for your pulmonary hypertension, but there are options.

Treating any underlying condition

Pulmonary hypertension is sometimes caused by lung disease or a heart condition. In these cases, doctors will focus primarily on treating the underlying cause. This may or may not be enough to get hypertension under control, and sometimes, pulmonary hypertension symptoms may be the reason a heart or lung condition is discovered.

Lifestyle changes

Several lifestyle changes have been shown to help with pulmonary hypertension. They include:

Staying as active as possible

While pulmonary hypertension limits physical activity, if you can stay active, you should do so. It used to be common wisdom that patients with PH should be restricted from pretty much all unnecessary exercise, but a 2006 study showed that patients who exercised (within limits and under supervision) increased their exercise tolerance, had no severe adverse outcomes, and gained a better quality of life.

Quitting smoking

While we don't know if smoking causes PH in humans, it does in animals, and it may be a risk factor¹, particularly for men. Talk to your doctor about quitting smoking, and don't simply switch to vaping. You should also avoid secondhand smoke as much as possible.

Avoid pregnancy

If you have pulmonary hypertension and get pregnant, you are at high risk of becoming ill or dying². This is because you can't increase your cardiac output to support the baby properly, so you should take steps to avoid getting pregnant.

Unfortunately, combined pill birth control is also known to worsen PH symptoms, and barrier methods are not considered sufficiently effective. Doctors typically recommend progesterone-only pills or implants, or ideally a levonorgestrel-releasing IUD, which you should get inserted in a hospital.

Many doctors will recommend termination if you get pregnant or are diagnosed with pulmonary hypertension during pregnancy, many doctors will recommend termination. If you choose to continue the pregnancy, you will need to be managed heavily by a multi-disciplinary team and have a cesarean.

Avoid high altitudes

High altitude³ can make your symptoms worse because of the lower air pressure, and you are also more likely to experience serious altitude sickness. If you must travel at altitude, then you will need a pre-trip assessment and will likely have to use oxygen during the trip. If you live at an altitude of 8,000 feet or higher, your doctor will probably advise that you move to a lower altitude as soon as it is financially viable.

Ask your doctor about flying

Airline cabins are pressurized to a height of about 7,000 to 8,000 feet. Many people with pulmonary hypertension can fly safely⁴, but you should talk to your doctor as in-flight side effects can be severe. If you do fly, make sure to:

1. Drink plenty of water

2. Avoid high sodium in-flight foods and snacks

3. Avoid alcohol and caffeine

4. Wear compression stockings

Talk to your doctor about whether you should use supplemental oxygen during the flight. If you do, make sure to get an FAA-approved portable oxygen concentrator, give the airline a heads up, and get a medical certificate from your doctor.

Eat a healthy diet

While everyone should try to eat a healthy diet with whole grains, fruits and vegetables, and low fat, it's particularly important for people with pulmonary hypertension.

Your doctor may also recommend a low-sodium diet, similar to the Dietary Approaches to Stop Hypertension (DASH) plan. Talk to a nutritionist, if you can, about creating a diet plan that is healthy but still fits your preferences and values.

There are promising signs that a whole food plant-based diet⁵ can also help reduce pulmonary hypertension. While this is far from proven, it might be worth talking to your nutritionist about it, especially if you prefer to avoid meat.

Also, be careful not to drink too much water. While dehydration is not good for you, people with pulmonary hypertension retain water, and this can cause swelling and increase water weight.

Avoid hot baths and long showers

Hot baths, hot tubs, saunas, and long showers can all cause your blood pressure to drop significantly. This can cause you to faint, especially if you are on medications. Fainting in a hot tub can result in a significant danger of drowning, so you should avoid this risk entirely.

Avoid heavy lifting

Talk to your doctor about how much you are allowed to lift. Lifting heavy weights, whether for exercise or because you are moving boxes in the basement, can put too much strain on your heart.

Get enough sleep

Many people with pulmonary hypertension have issues getting enough sleep, and your doctor may prescribe medication to help. If you snore or wake up feeling not refreshed, a sleep study can be of great help. You may have sleep apnea, which can worsen your condition.

Medication for pulmonary hypertension

Most people with pulmonary hypertension will require medication, and several varieties are available. You may need more than one type, and try several regimens before you find the one that works best for you. Be patient, and tell your doctor right away if you experience side effects that you can't handle. Typical medications include:

Guanylate cyclase stimulators

These increase nitric oxide, relaxing your pulmonary arteries and lowering the pressure in the lungs. Common side effects are nausea, dizziness, and fainting. These should not be used while pregnant.

Endothelin receptor antagonists, such as Tracleer and Opsumit

These improve energy levels and symptoms by reversing the effect of endothelin, which is a peptide that causes blood vessels to narrow. However, this type of medication does put a strain on your liver, and you may need monthly blood tests.  This medicine should not be used while pregnant, as it can harm the fetus.

Sildenafil and tadalafil

Sildenafil is otherwise known as Viagra. The most common use of these drugs is to treat erectile dysfunction, which they do by opening blood vessels. For PH patients, they help open the blood vessels in the lungs. Side effects commonly include nausea, headache, and vision problems.

Calcium channel blockers

These don't work for everyone, but they can relax the muscles in your blood vessel walls. They are very effective for the small number of people they work for.

Vasodilators

These relax and open narrow blood vessels. Typically, doctors prescribe epoprostenol, but the downside is that this drug needs to be taken through a continuous IV, meaning will have to wear a pump on your belt or shoulder.

Some other vasodilators can be inhaled, injected, or taken as pills, but they are less commonly prescribed. Iloprost is another widely used vasodilator, taken through a nebulizer (similar to asthma meds).

Side effects of epoprostenol are jaw pain, nausea, diarrhea, leg cramps, and pain at the IV site. Side effects of iloprost are headache, nausea, and diarrhea.

Warfarin

Warfarin is prescribed if you have a risk of blood clots in the pulmonary arteries. Like all blood thinners, it increases the risk of bleeding. If you have to have surgery, talk to your doctor, as you will likely have to stop taking it for a few days before the procedure.

Digoxin

This drug strengthens your heart and helps regulate your heartbeat.

Diuretics

"Water" pills are commonly prescribed to help reduce the fluid buildup common in people with PH by causing you to urinate more.

While not a medication, you may also be prescribed oxygen therapy, particularly if you live at altitude or have sleep apnea. You may also be recommended oxygen therapy under certain circumstances, such as during prescribed exercise or if you need to fly.

Surgery for pulmonary hypertension

If standard management techniques don't work, surgery might be recommended. In a few cases, surgery can cure PAH, but only if it is caused by specific issues, such as obstruction of the pulmonary arteries.

One procedure is called pulmonary thromboendarterectomy, which removes blood clots from the arteries leading to the lungs. However, as PH is also a risk factor for severe surgery complications, it's important to consider all of your options.

A lung transplant or heart-lung transplant is sometimes performed in younger patients with severe idiopathic pulmonary hypertension (that is to say, PH with no apparent reason). Transplant surgery is complex and requires taking immunosuppressants for the rest of your life.

The lowdown

Managing pulmonary hypertension generally requires lifestyle changes and medication. These lifestyle changes are backed by science and known to help improve your quality of life and extend your lifespan.

In extreme cases, surgery may be recommended, and in some instances, surgery may cure PH by removing the cause. However, you should discuss this with your doctor and family.

The most important things are exercising as recommended, eating a healthy, low-sodium diet, and attending routine follow-up visits with your doctor.