Pulmonary Hypertension Treatment

Pulmonary hypertension (PH) is a serious condition that affects the arteries in the lungs. While there is no cure for PH, treatment options are available to help manage symptoms and improve quality of life. Early diagnosis is crucial for better overall treatment outcomes.¹

Who treats pulmonary hypertension?

PH is a complex condition. It may require many doctors and specialists to take part in the diagnosis and treatment plan. The care team may include any or all of the following.^1,2

Cardiologists

Cardiologists are doctors who specialize in the diagnosis, treatment, and prevention of heart diseases. A cardiologist is often involved in the diagnosis of pulmonary hypertension. Once the condition has been diagnosed, the cardiologist works with other specialists to develop a treatment plan. They also regularly follow up to see how treatment is going.^2,3

Pulmonologists

Pulmonologists are doctors who specialize in the respiratory system, which includes the lungs and airways. Because a person’s PH may be linked to a lung disease, this doctor works to treat the underlying cause of PH.^2,3

Rheumatologists

Rheumatologists are doctors who specialize in autoimmune and inflammatory diseases that affect the joints, muscles, and connective tissues. Some autoimmune diseases are linked to PH. That is why this doctor may be brought on to the treatment team.²

Types of treatment for pulmonary hypertension

Treatment can help people with PH live fulfilling lives. The main goal of PH treatment is to treat its underlying causes and manage its symptoms. Because PH can be brought on by various causes and risk factors, treatments can vary widely. They may include:^1,4,5

• Medicines
• Oxygen therapy
• Lifestyle changes
• Medical procedures
• Surgeries

Medicines

Many different medicines are used to treat PH, including:^1,4,5

• Vasodilators – These medicines help widen (dilate) the blood vessels. This helps reduce the strain on the heart and improve blood flow in the lungs. Examples of these drugs include:
• Prostacyclin analogs like treprostinil (Orenitram®)
• Endothelin receptor antagonists like ambrisentan (Letairis®), bosentan (Tracleer®), and macitentan (Opsumit®)
• Phosphodiesterase-5 (PDE5) inhibitors like sildenafil (Revatio®) and tadalafil (Adcirca®)
• Soluble guanylate cyclase (sGC) stimulators – These drugs help relax the pulmonary arteries. This lowers pressure in the lungs. An example of an sGC stimulator used for PH is riociguat (Adempas®).
• Diuretics – Fluid retention is a common symptom of PH. These drugs – also called water pills – help flush out excess fluid in the body.
• Blood thinners – Also known as anticoagulants, these drugs help prevent blood clots. Blood clots can be a serious complication of PH. Examples include warfarin (Coumadin®), apixaban (Eliquis®), rivaroxaban (Xarelto®), and dabigatran etexilate (Pradaxa®).
• Calcium channel blockers – These drugs help lower blood pressure in the pulmonary arteries as well as other areas of the body. Some examples of calcium channel blockers used for PH are amlodipine (Norvasc®), diltiazem (Cardizem®), and felodipine (Plendil®, Cardioplen®).

Oxygen therapy

Some people with PH may need supplemental oxygen to help them breathe. Some may only need oxygen from to time. Others may need to be on oxygen all the time, depending on how severe their PH is.^1,4,5

Lifestyle changes

These healthy habits are recommended to improve heart health and manage PH symptoms:^1,4

• Get regular exercise. While more intense activities may not be right for people with PH, a tailored exercise program can help improve overall well-being.
• Eat a healthy diet. Certain diets can help manage fluid retention and put less stress on the heart. A heart-healthy diet centers around fruits, vegetables, and healthy carbs, and is low in salt.
• Avoid high altitudes. High altitudes can make PH symptoms worse. This is because oxygen levels decrease as elevation increases.
• Avoid activities that could rapidly lower blood pressure. These include things like using a sauna or hot tub, or taking long, hot showers.
• Stay up to date on vaccines. Vaccines help you stay healthy and avoid respiratory illnesses like the flu. These illnesses can cause complications for people with PH.

Medical procedures

Sometimes, medicine is not enough to manage PH symptoms. Medical procedures may be needed to reduce the risk of complications. They may include:^1,4

• Balloon pulmonary angioplasty (BPA) – In BPA, a catheter with a balloon at its tip is used to widen narrowed or blocked pulmonary arteries. This improves blood flow in the lungs.
• Balloon atrial septostomy (BAS) – In severe cases, BAS may be performed to create a small hole in the atrial septum. This relieves pressure on the right side of the heart. BAS is mostly used to help keep people stable while they wait for a lung transplant.

Surgeries

In more severe cases of PH, surgery may be necessary. Surgeries for PH include:^1,4,5

• Pulmonary endarterectomy – This procedure removes blood clots from the pulmonary arteries. It is used specifically for chronic thromboembolic pulmonary hypertension (CTEPH).
• Lung transplant – In advanced stages of PH, a lung transplant may be a last resort. This is typically considered only when other treatment options have failed.

Can pulmonary hypertension be prevented?

It may not be possible to prevent all cases of PH, especially those that are passed down from family members. But certain lifestyle choices can contribute to overall heart health and reduce a person’s risk of developing PH:^1,4,5

• Do not smoke. Smoking is a big risk factor for PH. Quitting can contribute to better lung and heart health.
• Stick to an exercise plan. Regular, moderate physical activity (like a 30-minute brisk walk each day) can improve heart health and reduce the risk of heart disease. Your doctor can help you choose an exercise plan that is safe for you.
• Maintain a healthy weight. Having a higher body weight can strain your heart and lungs. Work with a nutritionist if you need to lose weight.