Lung & Respiratory

Pulmonary Arterial Hypertension: Understanding Symptoms, Causes, Treatments & Survival Rates

Author Bowtie Team

Updated on 2025-08-14

Disclaimer: This article is translated with the assistance of AI.

According to the Hong Kong Rare Disease Alliance, pulmonary arterial hypertension is an extremely rare yet serious condition, affecting only 15 to 50 people per million. Once diagnosed, it can rapidly worsen and even lead to death. What causes pulmonary hypertension? And what are the available diagnosis and treatment options?

What is Pulmonary Hypertension?

Pulmonary arterial hypertension (commonly known as pulmonary hypertension, or PH in English) is a rare disease characterized by persistently elevated pressure in the pulmonary arteries, reaching a systolic pressure of ≥25 mmHg. This can cause severe damage to the heart and lungs. Pulmonary hypertension is typically classified into 5 categories, including:

1. Idiopathic pulmonary arterial hypertension: A type of pulmonary hypertension with unknown causes.

2. Pulmonary hypertension caused by other diseases: Such as pulmonary fibrosis, chronic obstructive pulmonary disease (COPD), pulmonary embolism, etc.

3. Pulmonary hypertension due to left heart failure: When the left heart fails, it can’t properly pump blood back from the lungs, leading to increased pulmonary blood pressure.

4. Pulmonary hypertension due to right heart failure: When the right side of the heart can’t pump blood to the lungs effectively, pulmonary blood pressure rises.

5. Pulmonary vascular hypertension: A rare genetic disorder that causes constriction and blockage of blood vessels in the lungs.

What are Pulmonary Hypertension Symptoms?

The symptoms of pulmonary arterial hypertension (pulmonary hypertension) can be divided into early and late-stage symptoms. Early symptoms might be mild, but late-stage ones can be more severe. Here are some common symptoms of pulmonary hypertension:

Shortness of breath : This is one of the most common symptoms of pulmonary arterial hypertension. Patients may feel breathless, especially after activity or exercise.
Fatigue : Patients may feel tired or weak, possibly because the lungs can’t deliver enough oxygen to other parts of the body.
Chest pain : Patients may experience chest pain or a feeling of pressure.
Fainting : In the late stages of pulmonary arterial hypertension, patients may frequently faint or lose consciousness, as the heart can’t pump blood to other parts of the body.
Cough : Patients may develop a dry cough or persistent coughing, due to constriction and blockage of pulmonary blood vessels.
Swollen feet : In the late stages of pulmonary arterial hypertension, patients may experience swollen feet or leg edema, because the heart can’t properly pump blood back from other parts of the body.

Keep in mind that symptoms of pulmonary arterial hypertension can vary from person to person and may resemble those of other conditions. If you suspect you have pulmonary arterial hypertension, consult a doctor for diagnosis and treatment.

What Causes Pulmonary Hypertension?

Pulmonary hypertension has many causes, potentially involving the lungs, heart, blood disorders, medications, or toxins, among other factors. Conditions like pulmonary embolism, chronic obstructive pulmonary disease, interstitial lung fibrosis, sleep apnea syndrome, and congenital heart disease can all lead to pulmonary hypertension.

Pulmonary Hypertension Risk Factors

Pulmonary hypertension can occur at any age, including in children, and the risk increases with age.
Pulmonary hypertension is more common in women, African Americans, and people aged 75 or older.

How is Pulmonary Hypertension Diagnosed?

Doctors can use various tests to determine if you have pulmonary arterial hypertension, with the most common being cardiac catheterization and echocardiography . Normal pulmonary artery pressure ranges from 11 to 20 mm Hg. If the pressure is too high, you may have pulmonary arterial hypertension. If cardiac catheterization measures 25 mm Hg or higher, or echocardiography measures 35 to 40 mm Hg or higher, it indicates you have pulmonary hypertension.

Other tests may include:

Blood tests

To check for blood clots, heart strain, or anemia.

Cardiac imaging tests

Such as cardiac magnetic resonance imaging (MRI), which provides detailed insights into the structure and function of the heart and surrounding blood vessels.

Lung imaging tests

Such as chest X-rays, to examine the size and shape of the heart and surrounding blood vessels, including the pulmonary arteries.

Electrocardiogram (ECG or EKG)

To check for changes in your heart’s electrical activity. This can help detect if certain parts of the heart are damaged or overworked. In pulmonary arterial hypertension, the heart may become overworked due to damage or changes in the pulmonary arteries.

Pulmonary Hypertension Treatment Options

The treatment goals for pulmonary hypertension are to lower pulmonary artery pressure and improve symptoms. Here are some common treatment methods:

Drug Treatment: Including vasodilators, anticoagulants, diuretics, and more—these medications can lower pulmonary artery pressure, improve heart function, and alleviate symptoms.
Oxygen Therapy: Improves breathing and reduces symptoms by providing extra oxygen.
Surgical Treatment: For some severe cases of pulmonary hypertension, surgery may be necessary. For example, lung transplantation can serve as a last resort.
Lifestyle Changes: Quitting smoking, maintaining a healthy weight, engaging in appropriate exercise, avoiding high altitudes, and preventing overexertion can all help alleviate symptoms.

Is Pulmonary Hypertension Fatal?

Pulmonary hypertension can be fatal. If not treated promptly, it may lead to arrhythmias, heart failure, blood clots, sudden cardiac death, and other serious complications.

What is the Survival Rate for Pulmonary Hypertension?

According to a 2019 French study , the 5-year survival rate for pulmonary hypertension ranges from 62% to 85%.

Sources

Author

Bowtie Team

The Bowtie Life Insurance Company Limited is an authorized life insurance company and Hong Kong’s very first virtual insurer approved. Through the use of modern technology and medical expertise, Bowtie offers an agent-free, commission-free and more convenient online platform for customers to quote, apply and claim for Voluntary Health Insurance Scheme (VHIS) anytime anywhere. Bowtie is ranked first in the direct channel sales throughout 2021. Stay up to date at www.bowtie.com.hk