Pulmonary Hypertension

Pulmonary hypertension (PH) is a serious condition where high blood pressure develops in the blood vessels of the lungs, straining the heart and reducing oxygen flow. UC Health offers the region's only nationally designated Adult Pulmonary Hypertension Association (PHA) Care Center, giving patients access to expert evaluation, advanced treatments, and coordinated care.

Pulmonary hypertension occurs when the arteries in the lungs become narrowed, blocked, stiff, or otherwise damaged. As blood flow through these vessels becomes restricted, pressure builds. The right side of the heart must work harder to move blood through the lungs, which can cause the heart muscle to weaken over time.

One specific form, pulmonary arterial hypertension (PAH), involves progressive narrowing or destruction of the small arteries in the lungs. PAH can be inherited, related to other medical conditions, caused by certain medications, or occur without a known cause.

Pulmonary hypertension often develops slowly. In some people, it can become life-threatening. There is currently no cure, but comprehensive treatments can help you feel better, stay active, and live a fuller life.

Pulmonary hypertension symptoms can be subtle at first and may take months or years to appear. Symptoms typically worsen as the condition progresses.

Common symptoms include:

• Shortness of breath—first with exertion, and eventually at rest

• Blue or gray skin tone (depending on skin color)

• Chest pain or pressure

• Dizziness or fainting

• Fast or pounding heartbeat

• Fatigue or reduced ability to exercise

• Swelling in the ankles, legs, or abdomen

Because these symptoms can resemble other conditions, it’s important to see a healthcare provider for an accurate diagnosis.

Pulmonary hypertension occurs when changes in the cells lining the lung arteries cause the vessel walls to thicken, stiffen, or narrow. This restricts blood flow and raises pressure in the pulmonary arteries.

Physicians classify pulmonary hypertension into five groups, based on the underlying cause:

Group 1: Pulmonary Arterial Hypertension (PAH)

Causes include:

• Unknown cause (idiopathic PAH)

• Inherited gene changes (heritable PAH)

• Certain medications or illicit drugs, including methamphetamine

• Congenital heart defects

• Autoimmune or liver diseases such as scleroderma, lupus, or cirrhosis

Group 2: Pulmonary Hypertension Due to Left-Sided Heart Disease

This is the most common form and may result from:

• Left heart failure

• Mitral or aortic valve disease

Group 3: Pulmonary Hypertension Due to Lung Disease

Causes include:

• Pulmonary fibrosis

• COPD

• Sleep apnea

• Long-term exposure to high altitudes in at-risk individuals

Group 4: Pulmonary Hypertension from Blockages in the Pulmonary Arteries

Often caused by:

• Chronic blood clots

• Tumors obstructing the pulmonary artery

Group 5: Pulmonary Hypertension with Multifactorial or Unclear Causes

May be related to:

• Blood disorders

• Inflammatory diseases such as sarcoidosis

• Metabolic disorders

• Kidney disease

Eisenmenger Syndrome and Pulmonary Hypertension

Eisenmenger syndrome is a long-term complication of an untreated congenital heart defect, such as a ventricular septal defect. Abnormal blood flow increases pressure in the pulmonary arteries, eventually leading to pulmonary hypertension.

Pulmonary hypertension can affect anyone, but is more common in adults aged 30–60. Risks increase with:

• Family history of pulmonary hypertension

• Congenital heart defects

• Overweight or obesity

• Smoking

• Blood-clotting disorders

• Long-term asbestos exposure

• Living at high altitude (8,000 ft or higher)

• Certain medications, including some weight-loss drugs

• Illicit drug use, such as cocaine or methamphetamine

Without treatment, pulmonary hypertension can lead to serious complications, including:

• Right-sided heart enlargement and heart failure (cor pulmonale)

• Blood clots in the pulmonary arteries

• Arrhythmias (irregular heartbeats), which can be life-threatening

• Bleeding in the lungs, including coughing up blood

• Pregnancy complications that may be life-threatening for both the mother and baby