Understanding Pulmonary Hypertension: Symptoms & Treatments

When most people hear the word hypertension, they think of high blood pressure. While not as common as high blood pressure, pulmonary hypertension, which affects the lungs, can be harder to diagnose, more challenging to treat, and, tragically, often comes with fatal consequences.

Dr. Talal Dahhan, Division Chief for Pulmonary & Critical Care at Baystate Pulmonary explains, “Nearly 3 to 8% of the world’s population is affected by pulmonary hypertension (PH). A serious condition brought on by abnormally high pressure in the blood vessels between the lungs and the heart, most cases of PH cannot be cured. But with early detection and proper treatment, it is possible to improve symptoms and slow the disease’s progression.”

What is Pulmonary Hypertension?

While the signs and symptoms of pulmonary hypertension are felt in the lungs, the cause starts in the heart. Dr. Dahhan explains, “In a healthy body, the heart does its job pumping blood around the body and delivering oxygen to organs and tissue. The oxygen gets taken up by the body and the blood cycles back to the heart. When all things are working well, the blood flows back into the heart and travels to the lungs via the pulmonary artery, picks up fresh oxygen, and the whole cycle begins again. However, in cases of PH, the walls of the pulmonary artery become stiff, damaged and narrow and the amount of oxygen going out to the rest of the body drops to dangerously low levels. Sensing the low levels of oxygen, the heart begins to pump harder to push oxygen-low blood to the lungs. Over time, the low level of oxygen in the blood and the added pressure on the heart can lead to serious complications, including heart failure.”

What Causes Pulmonary Hypertension?

There are a number of conditions and issues that can lead to PH. Once determined, the cause is used to classify a patient’s condition by a Group number (Group 1 PH, Group 2 PH, etc.). This grouping information is later used to help identify appropriate treatment options.

Here’s a look at the Groups and causes for PH:

Group 1 PH is due to pulmonary arterial hypertension (PAH), a condition in which the tiny arteries in your lung become thickened and narrowed. While PAH can occur due to unknow reasons (what physicians call idiopathic), some common causes include:

• Connective tissue disorders, including scleroderma
• HIV
• Certain drugs and/or medications
• Pulmonary veno-occlusive disease (PVOC), blockage of small arteries
• Pulmonary capillary hemangiomatous (PCH), a disease affecting the tiny blood vessels between the arteries and veins of the lungs

Group 2 PH is due to left-sided heart disease, the most common cause of PH in the United States, left-sided heart disease causes blood to back up in the heart, raising the pressure in the pulmonary arteries.

Group 3 PH is due to lung disease, including interstitial lung disease, chronic obstructive pulmonary disease (COPD), certain sleep apneas, pulmonary fibrosis, and emphysema, all of which reduce the flow of blood through the lungs and oxygen uptake.

Group 4 PH is due to blockages in the lungs and arteries, including clots and scars from chronic thromboembolic pulmonary hypertension (CTEPH).

Group 5 PH is due to other diseases and conditions, including blood disorders, kidney disease, inflammatory disorders, and metabolic disorders.

Dr. Dahhan notes that “PH can also be caused by a specific congenital heart called Eisenmenger syndrome. This occurs when there’s a hole, or more than one, between the heart chambers, which causes blood to flow incorrectly in the heart, often increasing the blood flow and pressure in the pulmonary arteries.”

No matter the cause, Dr. Dahhan notes that PH in all forms is a very serious condition and left untreated can be life threatening.

What Are the Symptoms of Pulmonary Hypertension?

According to Dr. Dahhan, “PH symptoms aren’t ‘all of the sudden’ type symptoms. They develop slowly over the years and are often like symptoms of common heart and lung conditions making them easy to miss.”

Pulmonary hypertension symptoms include:

• Unexplained shortness of breath
• Swelling (edema) in the ankles, legs and/or belly
• Dizziness or fainting spells (syncope), particularly during exercise or exertion
• Fatigue
• Heart palpitations
• A blue or grayish tint to the lips or skin

Dr. Dahhan adds, “Most people first recognize a problem when they become short of breath when walking. They may be fine on a flat surface but if there’s an incline or they’re going up stairs, they may find themselves suddenly out of breath. As the disease progresses, they may even feel out of breath when seated. Typically, that’s alarming enough to drive someone to seek medical help.”

How is Pulmonary Hypertension Diagnosed?

As noted, diagnosing PH can be challenging as its symptoms often resemble those of other lung and heart conditions. In addition to performing a physical exam and reviewing your medical history, your doctor may perform the following:

Diagnostic Tests for raising index of suspicion if we have a possibility of Pulmonary Hypertension

• Echocardiogram
• Chest X-ray (does not diagnose PH, but is part of the process)
• Electrocardiogram (ECG)
• Pulmonary function tests
• Blood tests

Right Heart Catheterization

The most definite test for diagnosing PH, right heart catheterization measures the blood pressure in the pulmonary arteries. This information identifies where the high pressure exists in your heart and arteries and the specific type (group) of PH the patient has.

What is the Treatment for Pulmonary Hypertension?

While there's no cure for most forms of pulmonary hypertension, various treatments are determined by the type of PH. Treatment can help manage symptoms and improve quality of life.

• Diuretics: to reduce any fluid accumulation
• Oxygen: to prevent spasms of the arteries, which can reduce the flow of oxygen
• Blood thinners (anticoagulants): specifically for patients with CTEPH, or if we have a high-risk atrial fibrillation patient with PH
• Treatment for anemia: typically, in the form of iron supplements and dietary changes
• Salt and fluid restriction: excess fluid through consumption or retention can put unnecessary strain on the heart
• Lifestyle changes: including quitting smoking, maintaining a healthy weight, avoiding high altitudes, and physical activity at a level that doesn’t overtax the heart

The next course of treatment options are medications. “There are a number of medication therapies available to help with different types of PH,” says Dr. Dahhan, “Depending upon the type of PH a patient has, we may recommend medications to relax or widen blood vessels and arteries, relax muscles in blood vessels, or to increase blood flow. These don’t treat PH, but they can treat and help with complications from PH.”

A last resort option, surgery may be recommended for patients with CTEPH, however, as Dr. Dahhan notes, not all PH patients are candidates for thromboendarterectomy. But there are procedures to consider in absence of CTEPH. Options include:

• Atrial septostomy to relieve pressure on the right side of the heart
• Lung or heart-lung transplantation in advanced cases

Living with pulmonary hypertension can be challenging, but with proper management and care, it is possible to maintain a good quality of life. “Early diagnosis and treatment are crucial,” says Dr. Dahhan. “If you are experiencing persistent shortness of breath or other symptoms, reach out to your doctor to schedule an appointment as soon as possible. The more information you can bring to the appointment—how long have you had symptoms, what makes them worse or better, etc.—the easier it will be to identify if PH is behind your issue and the faster you can begin treatment.”