Another Kind of High Blood Pressure: Pulmonary Hypertension

November 04, 2024

Most adults are familiar with routine blood pressure readings at the doctor’s office, and many take medications for high blood pressure, or hypertension.

A different kind of blood pressure that’s not as well known – and isn’t routinely tested for – is called pulmonary hypertension. It’s high blood pressure that occurs inside the arteries of the lungs. For many cases of pulmonary hypertension, treating the underlying disease is the key, says Robert Schilz, DO, PhD, Director of the Pulmonary Vascular Disease Program at University Hospitals.

What Is Pulmonary Hypertension?

Pulmonary hypertension is most often caused by other diseases, particularly left heart disease and lung disease. The condition may be reversible, depending on the type.

Left heart disease, also called left-sided heart failure, happens when the pumping power of the heart is diminished. “If you have minor increases in pulmonary blood pressure because of problems in the left heart and you fix that, or you have a bad heart valve and fix that, the pulmonary hypertension is often fixed as well,” he says.

Other possible causes of pulmonary hypertension are congenital heart disease, lung diseases, sleep apnea, blood clots in the lungs, sickle cell disease and connective tissue disorders.

Pulmonary hypertension can be serious. Symptoms may be similar to symptoms of other conditions. They include:

Shortness of breath
Dizziness
Chest pain
Swelling of the feet or ankles
Fatigue
Blueish color of lips or skin

A Rare But Severe Type

A rare but severe form of the disease is called pulmonary arterial hypertension (PAH).

Unlike other types of pulmonary hypertension, PAH involves narrowing or thickening of the small blood vessels of the lungs. The arterial damage may be caused by a genetic mutation, certain recreational drugs such as methamphetamine, or it can have no known cause (idiopathic).

Idiopathic PAH occurs in about one in 300,000 people. The incidence may be higher in people with systemic diseases such as scleroderma, advanced liver failure, cirrhosis and, in rare cases, HIV.

“With this type of pulmonary hypertension, it’s the only time we treat the pulmonary hypertension rather than the underlying disease.” he says. PAH is not curable and can be fatal, but it can be treated with specialized medications.

This type of pulmonary hypertension is almost always associated with significant enlargement of the right side of the heart. “That's the side of the heart that pushes blood through the lungs,” Dr. Schilz says.

Narrowing or destruction of these small blood vessels causes the heart muscle to work harder and can lead to heart failure.

Risk Factors for Pulmonary Hypertension

Factors that can increase the risk of pulmonary hypertension include:

Family history of the disease.
Family history of blood clots in the lungs.
Genetic disorders, such as Down syndrome, congenital heart disease, and Gaucher disease.
Smoking and illegal drug use.
Congenital heart defects.

The disease is usually diagnosed in people between ages 30 and 60, but it can occur at any age.

How is Pulmonary Hypertension Diagnosed?

Symptoms can be difficult to detect in the early stages of the disease. As symptoms progress, your doctor may order an electrocardiogram, echocardiogram, blood tests or heart and lung imaging tests.

Echocardiograms estimate pulmonary pressures. “But they can often be wrong, especially in healthy people, which leads to inappropriate concern among patients,” Dr. Schilz says.

Heart catheterization is the most definitive test to diagnose pulmonary hypertension. “The only way that you can for sure know that there's high blood pressures in the lungs is by doing a heart catheterization.”