Symptoms & Causes

Pulmonary Hypertension

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What is Pulmonary Hypertension?

Pulmonary hypertension is a rare type of high blood pressure that affects the arteries in the lungs and the right side of the heart. The pulmonary arteries thicken and become stiff, making them unable to expand to allow blood flow. This causes excess stress on the right side of the heart, eventually causing it to weaken, leading to heart failure.

Pulmonary hypertension is more common in people with preexisting heart or lung conditions; however, it can affect people of all ages.

Pulmonary Hyperternsion

Types of Pulmonary Hypertension

Types

Pulmonary hypertension is usually brought on by underlying conditions that cause the pulmonary arteries to harden, slowing down blood flow.

There are five classification groups for pulmonary hypertension. The World Health Organization's (WHO) assessment of the causes of pulmonary hypertension will determine which of the five groups it is categorized:

Group 1: Pulmonary arterial hypertension (PAH) is broken down into further classifications:
- Idiopathic pulmonary arterial hypertension, unknown cause
- Heritable pulmonary arterial hypertension, gene changes that are passed down through families
- Drug use
- Congenital heart diseases
- Other chronic health conditions such as HIV, liver (cirrhosis) disease, or connective tissue disorders (lupus)
Group 2: Pulmonary hypertension caused by left-sided heart disease
- Mitral valve or aortic valve disease
- Left ventricle failure
Group 3: Pulmonary hypertension caused by lung disease
- Chronic obstructive pulmonary disease (COPD)
- Pulmonary fibrosis
- Obstructive sleep apnea
- Long-term exposure to high altitudes
Group 4: Pulmonary hypertension caused by chronic blood clots
- Pulmonary emboli
- Other blood clotting disorders
Group 5: Pulmonary hypertension triggered by other rare health conditions:
- Polycythemia vera and essential thrombocythemia
- Sarcoidosis and vasculitis
- Glycogen storage disease and other metabolic disorders
- Kidney disease
- Tumors

Stages of Pulmonary Hypertension

Stages

According to the World Health Organization (WHO), the severity of the symptoms of pulmonary hypertension determines which of the four stages it will be categorized:

Class 1: No noticeable symptoms are present during normal physical activity; therefore, no limitation of physical activity is required.
Class 2:Minor limitation of physical activity when active; no symptoms noticed during rest.
Class 3:This stage substantially limits physical movement during mild exertion and ordinary activity, but not while resting.
Class 4:This stage will cause total loss of the ability to carry out any physical activity, even during resting periods. Right-sided heart failure is common during this stage.

Pulmonary Hypertension

Types of Pulmonary Hypertension

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Signs & Symptoms of Pulmonary Hypertension

Symptoms

Symptoms of pulmonary hypertension develop slowly over time. Yet, once the symptoms appear, the disease can quickly progress and symptoms will worsen.Symptoms of pulmonary hypertension can include:

Cyanosis – your skin or lips may turn blue
Chest pressure – chest pain when inhaling
Syncope – fainting or dizziness can occur
Palpitations – your heart may race, or your pulse will be fast
Fatigue – easily tired when performing normal activities
Dyspnea – short of breath when performing normal activities and eventually all the time
Edema – Swelling in the legs, ankles, and abdomen due to fluid build-up

What Causes Pulmonary Hypertension?

Causes

The heart contains two upper chambers (atria) and two lower chambers (ventricles). The heart's lower chamber (right ventricle) pumps blood to the lungs through a large blood vessel called the pulmonary artery. The blood in the lungs then releases carbon dioxide in exchange for oxygen.

Normally, this transfer of blood to the left ventricle is easy. Cells that line the pulmonary arteries may become altered, causing them to thicken and become rigid. This cell change can affect blood flow through the lungs resulting in pulmonary hypertension.

There are preexisting conditions that can trigger pulmonary hypertension. Some common causes of pulmonary hypertension include:

High blood pressure
Congenital heart disease
Connective tissue disease
Coronary artery disease
Liver disease (cirrhosis)
Chronic lung diseases such as emphysema or COPD
Blood clots

Risk Factors for Pulmonary Hypertension

Risk Factors

Risk factors for pulmonary hypertension can range from genetic to environmental or even behavioral. Most people diagnosed with pulmonary hypertension are between the ages of 30 to 60. Age becomes more of a risk factor for older adults affected by pulmonary arterial hypertension (PAH) (Group 1). However, pulmonary arterial hypertension resulting from an unknown source is more common among younger adults.

Risk factors for pulmonary hypertension include:

A family history of pulmonary hypertension
Obesity
Blood clotting disorder or family history of one
Exposure to asbestos
Congenital heart disease
Living in high-altitude areas
Illegal drugs such as cocaine or methamphetamine or certain medications for weight loss
Use of selective serotonin reuptake inhibitors (SSRIs) used to treat anxiety and depression