Shortness of breath, dizziness, swelling of the legs and chest pain or pressure are symptoms that could indicate a variety of medical conditions, including pulmonary hypertension. 

This is different from the hypertension people monitor with blood pressure cuffs squeezing their arms. Hypertension is high blood pressure affecting the vessels of the body. Pulmonary hypertension involves just the vessels that carry blood to the lungs.

“Early diagnosis and more aggressive treatment up front increases the survival,” says Upstate pulmonologist Birendra Sah, MD. “There’s no cure for pulmonary hypertension, but if we diagnose it early and if we treat them with proper drugs, that can lead to increased survival.”

 Pulmonary hypertension develops when blood vessels in the lungs become thickened, narrowed, blocked or destroyed.

Krithika Ramachandran, MBBS, is a colleague of Sah’s, specializing in critical care and pulmonary diseases. “Usually the way I explain it to my patients is, I tell them to imagine a balloon with a straw attached to the end. If the diameter of the straw just keeps getting smaller and smaller, that balloon has to be squeezed harder and harder to send flow through the straw.

“That’s essentially what’s happening with the right side of the heart in this situation.”

Diagnosis

A diagnosis can be challenging because the first symptoms are generally nonspecific. A person may feel short of breath, or tired. As the disease progresses, legs may swell, or the belly may swell as fluid is retained.

Pulmonary hypertension is dangerous because untreated, it leads to congestive heart failure. The chambers of the heart dilate, and their ability to push blood into the lungs diminishes. This reduces the supply of oxygen to the brain and the rest of the body.

To treat pulmonary hypertension appropriately, health providers begin by identifying what type of pulmonary hypertension a patient has.

One type, pulmonary arterial hypertension, occurs when the walls of the artery become stiff. This may develop because of a gene passed down through families, congenital heart disease, use of certain drugs or illegal substances, connective tissue disorders such as lupus or scleroderma, liver disease or infection with the human immunodeficiency virus. Some people develop this condition without ever learning its cause. 

Sah says pulmonary arterial hypertension is typically treated with vasodilator medications, which help the blood vessels relax. In addition, patients may require supplemental oxygen, or medications to thin the blood, or medicine to help the body remove excess water. Another type, chronic thromboembolic pulmonary hypertension, which occurs due to longstanding blood clots in the lung blood vessels, can be treated with surgery to remove the blood clots in select patients, or with vasodilators in patients who are not candidates for surgery.

The other types of pulmonary hypertension are generally treated with supportive care, he says. That means some patients may need supplemental oxygen. Some may require blood thinners. Some may need inhaled vasodilators. Most benefit from careful measurement of their fluid and salt intake and body weight.

Other concerns

People with pulmonary hypertension are managing a disease that can complicate their lives in multiple ways.

“It’s a very precarious line they toe when they’re trying to keep themselves healthy. So anything and everything needs to be evaluated as a risk-benefit ratio before taking it,” says Ramachandran.

Most patients are taking a medication to help maintain their body’s salt and fluid balance, so they have to be careful about the salt content of their foods and about taking other medications.

If they develop a cold, they may not be   able to take a decongestant because it can increase pressure in the lungs and make it harder for the heart to work properly. And the pain reliever ibuprofen can affect liver function and kidney function.

Most of the drugs that treat atrial fibrillation, a common heart rhythm disturbance, work by slowing the heart rate. This can be a particular concern for someone with pulmonary hypertension because it can depress the heart’s already-impaired ability to pump blood.

Exercise has to be approached with caution, too. Lifting weights above the head is a no-no because of the stress it puts on the heart. Traveling to high altitudes, or on an airplane, may require the use of supplemental oxygen.

Multiple causes

The cardiovascular system in a woman with pulmonary hypertension cannot handle the stress of pregnancy and can be life-threatening to her. Also, many of the medications used to treat the disease can cause fetal defects. So women with pulmonary hypertension are advised not to become pregnant.

Pulmonary hypertension caused by left-sided heart disease can be the result of mitral valve or aortic valve disease or failure of the  left ventricle because of a heart attack or another disease.

Pulmonary hypertension caused by lung disease can be the result of chronic obstructive pulmonary diseases including emphysema and bronchitis, pulmonary fibrosis or obstructive sleep apnea. 

Other health conditions — including inflammatory diseases such as sarcoidosis and vasculitis, blood disorders, metabolic disorders, kidney disease and others – can trigger pulmonary hypertension. And, people with clotting disorders or pulmonary emboli are at risk for developing pulmonary hypertension caused by chronic blood clots.

“The landscape for treating this disease has changed dramatically in the last 10 to 15 years,” Ramachandran says. In the 1990s, a diagnosis of pulmonary hypertension was like a death sentence, with more than half of patients dead within three years. “But now, it can be one of those diseases that you die with instead of die from.”

She explains that multiple medications are available today to help dilate the blood vessels and reduce the pressure, so the heart can pump blood through the lungs.

It’s a complicated disease that requires careful medical management.

Sah says patients with pulmonary hypertension keep in close contact with their pulmonologists, making regular visits for checkups. 

This article appears in the spring 2023 issue of Upstate Health magazine.