The Food and Drug Administration (FDA) has approved sotatercept for the treatment of pulmonary arterial hypertension. This medication, which was given “breakthrough” status by the FDA to speed its evaluation and approval, has been shown in clinical trials to improve pulmonary arterial hypertension symptoms.

Below are some answers to questions you may have about this new medication.

What Is Sotatercept?

Sotatercept is sold under the brand name Winrevair. In March, the FDA approved sotatercept as a treatment for adults with pulmonary arterial hypertension to increase their capacity to exercise, improve their condition and reduce their risk of getting sicker.

It’s a new class of drug aimed at curbing the overproduction of cells that can narrow and block the lung’s arteries. By restricting blood flow, these blockages lead to high blood pressure in the lungs that puts strain on the right side of the heart. 

How Does Sotatercept Work?

Pulmonary arterial hypertension can develop when the body’s signals to produce more cells in the pulmonary artery outweigh the signals to hold off on cell production.

The drug improves the balance between signals to make more cells and the signals to stop producing cells. Sotatercept is a protein that is intended to bind and trap the proteins that spark cell growth — activins.

The drug is used in combination with traditional “background therapy” — a variety of cardiovascular drugs — widely used to manage the condition.

How Is Sotatercept Administered?

Sotatercept is a shot given in the tissue just under the skin (subcutaneously). Patients need close monitoring for potential side effects, but can administer the shot every three months themselves.

How Was Sotatercept Tested?

Because of its potential to benefit so many people with a life-threatening condition, the FDA designated sotatercept a “breakthrough” therapy. That means its approval was expedited, but the drug was still subject to rigorous testing at pulmonary hypertension treatment centers around the world, including Norton Pulmonary Specialists.

The 24-week trial included more than 320 adults receiving traditional background therapy for pulmonary arterial hypertension. The volunteers were given shots of sotatercept or a placebo and weren’t told which they received.

The study measured nine outcomes, but was primarily focused on how far the patient could walk in six minutes. By the end of the trial, patients receiving sotatercept were able to walk almost 40 yards farther (34.4 meters) in the six-minute walk test. Patients receiving the placebo added about 1 yard (1 meter) to their walking distance. Sotatercept also improved seven of the other eight measures significantly, with only a cognitive/emotional score not improving.

Results of the study were published in the New England Journal of Medicine in 2021.

What Are the Potential Side Effects of Sotatercept?

Side effects with sotatercept in the study included nosebleeds, dizziness, telangiectasia (widened blood vessels in the skin), increased hemoglobin levels, low platelet count and increased blood pressure. Your provider will monitor hemoglobin levels before each dose for at least the first five doses.

Who Is Eligible for Sotatercept?

Patients with a pulmonary arterial hypertension diagnosis who have symptoms are eligible for the drug.

An accredited pulmonary hypertension Center of Comprehensive Care is best equipped to manage this complex condition with a prompt and accurate diagnosis and treatment through the disease’s progression.

Norton Pulmonary Specialists was one of the first in the country accredited by the Pulmonary Hypertension Association more than 10 years ago and has the only accredited program in Kentucky or Southern Indiana.

How Is Pulmonary Arterial Hypertension Diagnosed?

The condition can mimic many other diseases and can be difficult to diagnose. Too many patients see various providers before landing at a comprehensive care center.

The diagnostic process requires ruling out other causes of symptoms, then examining the heart’s right ventricle with a minimally invasive catheterization procedure.

Does Insurance Cover Sotatercept?

Once you are diagnosed and your pulmonologist decides sotatercept is right for you, you’ll submit an application with your information to the drug’s maker, where insurance coverage will be evaluated and your copay calculated.

The drug is expensive, and many patients will need assistance with the copay. Staff at Norton Pulmonary Specialists are available to help navigate the process by submitting applications, following up and helping you find financial assistance to cover the copays.

How Does Sotatercept Compare With Other Pulmonary Arterial Hypertension Treatments?

Sotatercept takes an entirely new approach to treating pulmonary arterial hypertension by attempting to balance out the triggers for excess production of cells that build up in the pulmonary arteries.

Conventional cardiovascular treatments attempt to lower the pulmonary blood pressure with blood thinners and other drugs.

In the clinical trial, sotatercept was added to the traditional treatment and was found to improve nine of 10 metrics. They included the six-minute walk test and showed patients taking sotatercept were able to walk almost 40 meters farther by the end of the trial. Placebo patients’ distance was virtually unchanged.

Improvements were also recorded in pulmonary vascular resistance (a measure of the blood pressure in the lungs), the severity of patients’ disease as measured by the World Health Organization functional class, and delayed clinical worsening or death.

What Is Involved if I Am Eligible for Treatment With Sotatercept?

You’ll need to be monitored for potential harmful side effects, chiefly low hemoglobin levels. The drug is given by a shot into the skin, so there’s no visit to an infusion center, and patients are able to administer the shot themselves every three months.

Is Sotatercept a Cure for Pulmonary Arterial Hypertension?

Sotatercept is a new way of treating pulmonary arterial hypertension symptoms, but is not a cure. There are indications that the drug promotes narrowed or blocked pulmonary arteries to resume some of their natural shape, but the treatment doesn’t cure the disease.

Current Pulmonary Arterial Hypertension Clinical Trials

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The board-certified and fellowship-trained physicians with Norton Pulmonary Specialists are leaders in caring for Louisville and Southern Indiana patients with asthma, chronic obstructive pulmonary disease (COPD), emphysema and other lung diseases.

• More than two dozen board-certified and fellowship-trained physicians with extensive experience specialize in diagnosis and management of all forms of lung diseases and sleep disorders, so you can get the care you need.
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