Pulmonary Hypertension: Overview

This information was reviewed and approved by Marjorie Patricia George, MD (1/31/2020).

Pulmonary hypertension refers to a condition in which high blood pressure exists within the vessels of the lungs. Normally, venous (low oxygen) blood returns from the body to the right side of the heart. The blood is pumped to the lungs via the pulmonary arteries. Oxygen is transferred into the blood at the alveolus-capillary interface, and then returns through the pulmonary veins to the left side of the heart, where it is pumped to the rest of the body to deliver oxygen to organs and tissues.

Pulmonary hypertension is high blood pressure in the arteries of the lungs and it makes people short of breath.

There are five different categories of pulmonary hypertension. The first type is pulmonary arterial hypertension, which is a disease of the arteries of the lungs themselves. The second type is pulmonary hypertension due to heart disease. The third type is pulmonary hypertension due to lung disease or in chronic low oxygen levels or sleep apnea. The fourth type is pulmonary hypertension due to chronic blood clots in the lungs. And the fifth type is pulmonary hypertension due to miscellaneous diseases.

Early pulmonary hypertension is manifest with shortness of breath when someone exerts themselves. As it progresses, people can do less and less in terms of an activity tolerance. As the disease progresses, people may develop swelling in their lower extremities or even in their abdomen.

Making the diagnosis of pulmonary hypertension can be challenging because it looks like a lot of other diseases. Oftentimes people are misdiagnosed with asthma or COPD or obesity or just being out of shape. It's really important to make an accurate diagnosis and the gold standard test to making the diagnosis is a right heart catheterization.

The goals and treatment of pulmonary hypertension are for us to make an early diagnosis. So number one, it's important to, when it's suspected, do the appropriate testing so that we can make an early diagnosis in this disease. And then we treat it aggressively. We have medications available and our goals are to help the patient breathe better, walk further, do more and live longer.

Classifications of Pulmonary Hypertension

There are five different types of pulmonary hypertension (PH). These types are categorized into groupings based on the causes and treatments of the disease. The groups are known as the World Health Organization (WHO) classification system.

WHO Groups of Pulmonary Hypertension

Group	Name	Caused by	Risk Factors
1	Pulmonary Arterial Hypertension (PAH) —due to a disease of the arteries themselves	Increased stiffness, narrowing or scarring of the pulmonary arteries stresses the heart, losing its ability to pump enough blood through the lungs.	Family history Connective Tissue Diseases (scleroderma, lupus) HIV Liver Disease Cocaine & Meth Use Unknown Cause (idiopathic)
2	PH Due to Left Heart Disease — heart failure or heart valve problems	The left side of the heart does not pump correctly, creating a back pressure into the lungs.	Atrial Fibrillation Coronary Artery Disease (CAD) Heart Valve Disorders HFpEF (diastolic heart failure)
3	PH Due to Chronic Lung Disease and Hypoxia (low oxygen levels) including Sleep Disorders	Shortage of oxygen causes the arteries in the lungs to tighten.	COPD ILD/Pulmonary Fibrosis Sleep Disordered Breathing Chronic High Elevation Exposure Lung Developmental Defects Alveolar Hypoventilation Disorders
4	PH Due to Chronic Arterial Obstructions — blockages in the pulmonary arteries	Blood clots that the body can’t dissolve or other obstructions block pulmonary arteries.	Recurring Blood Clots Blood Clotting Disorders Chronic Thromboembolic Pulmonary Hypertension
5	PH from Unknown Causes	Not well understood.	Blood Disorders (Sickle Cell Disease) Systemic Disorders (sarcoidosis & histiocytosis) Metabolic Disorders Chronic Kidney Failure Splenectomy

As the chart below shows, most patients are diagnosed with PH due to left heart disease (Group 2) and/or PH due chronic lung disease with low oxygen levels, Group 3.

Chart source: Wijeratne, D. T., et al. (2018). Increasing Incidence and Prevalence of World Health Organization Groups 1 to 4 Pulmonary Hypertension: A Population-Based Cohort Study in Ontario, Canada. Circulation. Cardiovascular Quality and Outcomes, 11(2), e003973. http://doi.org/10.1161/CIRCOUTCOMES.117.003973

Associated Conditions

Pulmonary hypertension can occur in isolation or, more commonly, with diseases of the lungs and heart. Pulmonary hypertension in the absence of other diseases is very rare and is often idiopathic, associated with autoimmune disease or familial in nature. This kind of pulmonary hypertension is referred as pulmonary arterial hypertension (PAH). Pulmonary arterial hypertension can also be associated with drug use, such as methamphetamines or diet drugs; human immunodeficiency virus (HIV); liver disease; and congenital heart disease.

Pulmonary hypertension is commonly associated with a variety of lung conditions with low oxygen levels. These include COPD, emphysema, interstitial lung disease, chronic pulmonary blood clots or sleep apnea. When pulmonary hypertension arises from cardiac conditions such as heart failure or heart valve disease, it is sometimes referred to as pulmonary venous hypertension. And while there are specific types of pulmonary hypertension, it is important to note that pulmonary hypertension can be associated with multiple causes.

See how your heart can affect your breathing in our infographic explaining the heart/lung connection.

Learn about the Pulmonary Hypertension Program at National Jewish Health.

Classifications of Pulmonary Hypertension

WHO Groups of Pulmonary Hypertension

Associated Conditions

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