By Katherine Alexis Athanasiou, PA-C 

Updated on October 30, 2023

 Medically reviewed by Sanja Jelic, MD

Pulmonary hypertension (PH) is a form of high blood pressure affecting the pulmonary artery, the vessel that carries deoxygenated blood into the lungs to pick up oxygen. It can lead to difficulty breathing and a reduction in usually performed activities, taking a physical and emotional toll on an affected individual's overall well-being. Pulmonary hypertension is a serious and progressive disease with several different causes.

Untreated pulmonary hypertension can lead to heart failure and early death, so proper and adequate management and treatment are important in controlling the disease and slowing its progression.

This article will review the different forms, common symptoms, and current treatments available for pulmonary hypertension.

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Classifying Pulmonary Hypertension (PH)

Pulmonary hypertension can be further classified into five subcategories based on the cause or associated diseases. The five classifications or groups of pulmonary hypertension are:¹

1. Pulmonary arterial hypertension

2. Pulmonary hypertension due to left-sided heart disease

3. Pulmonary hypertension due to underlying respiratory diseases

4. Chronic thromboembolic pulmonary hypertension (CTEPH) due to blood clots

5. Idiopathic pulmonary hypertension, which has no known cause

What Symptoms Could Be Related to Pulmonary Hypertension?

Symptoms of pulmonary hypertension can be difficult to recognize as several medical conditions can cause them. Some symptoms of pulmonary hypertension include:²¹

• Shortness of breath with activity (aka dyspnea on exertion)

• Fatigue

• Chest pain

• Dizziness

• Fainting

• Swelling of the legs and ankles

• Cough (can sometimes produce blood)

Pulmonary Hypertension Progression

The disease process of pulmonary hypertension is different in every case since there are several different causes. In most cases, specifically in pulmonary arterial hypertension, symptoms may start gradually over time before worsening to significant heart and lung complications.¹

Treatments to Manage Pulmonary Hypertension

There is no cure for pulmonary hypertension but there are treatments available that help minimize discomfort and symptoms.³ Treatment plans for pulmonary hypertension are individualized and based on the type, disease severity and progression, and other co-morbid conditions. Some treatment options include medications such as:⁴³

• Vasodilators: Medications known as calcium channel blockers, endothelin receptor antagonists, and phosphodiesterase type 5 inhibitors can relax blood vessels and decrease blood pressure in the pulmonary artery.

• Digoxin/digitalis: These medications can help regulate the rate at which the heart pumps.

• Blood thinners: These medications can help prevent the formation of blood clots, which is particularly helpful for people with group 4 PH.

• Prostacyclin inhalers: Can help relieve shortness of breath.

• At-home oxygen: This can be used when oxygen saturation levels fall very low.

In very severe cases, some people may be candidates for a heart and lung transplant. However, they are both serious surgical procedures, and it is best to discuss all other options first with a healthcare provider.⁴

Lifestyle Habits

In people with pulmonary hypertension, healthy lifestyle habits can help decrease the chance of serious complications. Some important general healthy lifestyle habits include:⁵

• Regular exercise: Make sure to speak with a healthcare provider before starting any new fitness routine. Often they can help direct you on safe exercise plans.

• A heart-healthy diet: Focus on fruits, vegetables, and high-protein foods like fish, lean meats, and legumes. Also, try to avoid saturated fats and limiting salt intake, while encouraging a variety of vegetables.

• Quit smoking: Tobacco use increases the risk of developing heart and lung diseases.

Support Groups, Resources, and Clinical Trials

Pulmonary hypertension can be both a physically and mentally taxing disease. Numerous resources for people living with PH can be found at The Pulmonary Hypertension Association website. Connecting with a community of people who also have PH can be helpful in navigating how to live with PH. Pulmonary hypertension is a big area of interest in medicine with hundreds of clinical trials already completed and many more in progress or currently recruiting. Clinical trial information can be found at ClinicalTrials.gov.

Summary

Pulmonary hypertension is a type of high blood pressure that affects blood flow into the lungs. It can cause chest pain, dizziness, fainting, and shortness of breath. Disease progression depends on the cause. Treatments can include different medications and lung or heart transplants in extreme cases. It can be an intense disease to deal with, but support groups are available.