Top 10 Pulmonary Hypertension Stories of 2021

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by Marisa Wexler MS |

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Pulmonary Hypertension News brought you the latest coverage of advances in treatment, clinical trials, and scientific research related to pulmonary hypertension (PH) throughout 2021.

We look forward to continuing to report more news to patients, family members, and caregivers dealing with PH in the new year. Here are the most-read PH news stories of 2021, with a brief description of what made them relevant for the PH community.

No. 10 – “‘Blueprint’ of Healthy Lung Cells May Guide Research Into Lung Disease

The cells that line the surface of the lung’s blood vessels, called endothelial cells, are dysregulated in PH and other lung disorders. By analyzing tens of thousands of cells from dozens of people, an international group of scientists created a detailed “cellular blueprint” of healthy endothelial cells in the human lung.

It revealed some previously unrecognized differences between endothelial cells. For example, cells lining tiny blood vessels called capillaries tended to express more genes related to the movement of gasses, while those lining the thin-walled veins expressed genes that enabled white blood cells — part of the immune system — to pass through the walls and access other tissues.

This blueprint may provide a useful standard against which to compare diseased cells, in order to better understand their dysregulation.

No. 9 – “Potential Oral Treatment Reverses Advanced PAH in Rats, Study Shows

BI113823 is an investigational oral molecule that blocks the activity of pro-inflammatory proteins called kinin B1 receptors.

A team of researchers in South Korea tested BI113823 in a rat model of severe pulmonary arterial hypertension (PAH). Results showed that the treatment significantly lowered blood pressure in the lungs, improved heart function, suppressed inflammatory responses, and increased rates of survival.

No. 8 – “Severe COVID-19 Infection Can Lead to Acute PH, Raising Risk of Death

Since the emergence of COVID-19 in late 2019, researchers have been trying to understand the effects of the disease. When COVID-19 is severe, it can cause inflammation and clotting in the lungs, which could theoretically set the stage for PH to develop.

Scientists in Sweden looked at health records for 67 people who were treated for severe COVID-19 infection, and found that more than one in three (39%) had acute PH related to the disease. Compared to those without this complication, patients with acute PH were at a great risk of needing ventilation or of dying.

No. 7 – “Dry-powder Tyvaso Found More Convenient Than Nebulizer for PAH Patients

Tyvaso, an inhaled formulation of treprostinil sold by United Therapeutics, is approved in the U.S. to treat PAH, and was also recently approved for PH associated with interstitial lung disease.

The Phase 1 BREEZE clinical trial (NCT03950739) tested a new dry-powder formulation of Tyvaso, called Tyvaso DPI, in 51 PAH patients who had previously been on the approved nebulized formulation. Top-line data suggested both effectiveness and greater patient satisfaction with the dry-powder formulation.

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The U.S. Food and Drug Administration (FDA), however, declined to approve Tyvaso DPI in October due to an inspection issue at a third-party manufacturing facility. United Therapeutics announced an intention to address this single issue.

No. 6 – “Uptravi Found to Reduce Risk, by Half, of PAH Disease Progression

The Phase 3 clinical trials GRIPHON (NCT01106014) and TRITON (NCT02558231) both tested the effects of Janssen’s Uptravi (selexipag) in people with PAH. Using data from these completed trials, researchers identified 649 participants who either started with Uptravi or were randomized to a placebo within six months of being diagnosed with PAH.

Results showed that Uptravi reduced the risk of disease progression — assessed via criteria like worsening physical ability, hospitalization, or a need for additional therapies — by more than 50% for these recently diagnosed patients.

No. 5 – “Peptide May Prevent PAH, Rat Study Finds

BPC 157 is a peptide (a small protein) that is normally found in stomach juice and it is thought to help prevent ulcers by protecting cells from damage. The peptide’s cell-protecting effects are being investigated for potential therapeutic application in a number of diseases.

Scientists in Croatia tested BPC 157 in a rat model of PH. Results showed that the peptide could prevent or reverse PH symptoms, including weight loss and diminished physical activity. The treatment also improved heart health. These results support further development of BPC 157 as a potential PH therapy, according to the researchers.

No. 4 – “Claritas Gaining Global Rights to R-107, Varied Nitric Oxide PAH Therapy

Nitric oxide is a gas that acts as a vasodilator — a substance that prompts blood vessels to widen, thereby lowering blood pressure. R-107 is a liquid formulation of nitric oxide that may be administered orally or via injection, whereas most nitric oxide-based treatments are inhaled.

Under an agreement between Claritas Pharmaceuticals and the Salzman Group, Claritas acquired exclusive global rights to develop R-107 in treating PAH and, depending on the outcome of clinical trials, potentially market this therapy.

No. 3 – “Statins Found to Benefit Patients with 2 Chronic Lung Disorders” 

Statins are a group of medications commonly prescribed to help lower cholesterol levels. Scientists in Taiwan, working with data from the country’s national health insurance program, identified hundreds of thousands of people with chronic obstructive pulmonary disease (COPD), of whom many developed PH, a common complication of COPD.

Results showed that patients given a statin were significantly less likely to die of causes related to PH, leading the researchers to suggest that statins have protective effects and their use can benefit patients with both PH and COPD.

No. 2 – “R-107 Stops PAH Progression, Reverses Hypertension in Rats

R-107, the liquid formulation of nitric oxide now is being developed by Claritas, was tested in a rat model of PAH. Results showed that the experimental therapy rapidly and powerfully eased acute symptoms, and also stopped the progression of damage to the lung’s blood vessels.

According to Claritas, these results suggest that R-107 is a “potentially revolutionary new treatment for PAH.” The company announced plans to begin clinical testing of R-107 in people.

No. 1 – “Low-carb, High-fat Diet Seen to Safely Treat PH With Metabolic Syndrome

Metabolic syndrome refers to a group of conditions characterized by high levels of fats and sugars in the blood, high blood pressure, and central obesity (excess fat around the waist).

Researchers described the case of a 62-year-old woman who had PH as well as metabolic syndrome and fatty liver disease. She was prescribed a statin to lower her cholesterol and was started on a supervised ketogenic (“keto”) diet, which involves eating fewer carbohydrates and more fats. Over the course of a year, the woman lost about 75 lbs (34 kg), and measures of lung function and blood flow parameters markedly improved.

According to the research team, these results suggest that addressing metabolic syndrome via a supervised ketogenic diet may have a key role in treating PH in patients with this disease and metabolic syndrome.

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We at Pulmonary Hypertension News hope these stories and our reporting throughout 2022 will help to inform and improve the lives of everyone affected by PH.

We wish all our readers a happy 2022.