Top 10 Pulmonary Hypertension Stories of 2022

Improving patient outcomes, evaluating treatments were hot topics this year

Marisa Wexler, MS avatar

by Marisa Wexler, MS |

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This Top Ten illustration features a host of red blood cells.

Over the past year, the team here at Pulmonary Hypertension News has brought you up-to-date coverage of the latest developments in research, care, and treatment related to pulmonary hypertension (PH).

We hope to continue to be a resource for the PH community in the coming year. Here is a list of the top 10 most-read articles of 2022, with a brief description of each.

No. 10 – Early Detection of PH Essential for Optimal Care, 2022 Guidelines Stress

The European Society of Cardiology and the European Respiratory Society teamed up in August to publish a new set of guidelines for diagnosing and managing PH. The guidelines highlight the disease’s complexity and how care has changed with recent advances in treatment. The societies also stressed the importance of an early diagnosis, and provided a three-step procedure aimed at streamlining a PH diagnosis. The guidelines also note that pregnancy can carry substantial health risks for people with PH, emphasizing that healthcare providers need to have honest discussions with patients about these risks.

No. 9 – Oxygen Therapy Is Only Effective Treatment for COPD With PH: Study

Chronic obstructive pulmonary disease, or COPD, is a group of conditions characterized by inflammation in the lungs and shortness of breath. Some people with COPD develop co-occurring PH, and this often is linked with poorer clinical outcomes. Here, researchers conducted a review of scientific literature to assess treatment outcomes for people with COPD-related PH. Studies on long-term oxygen therapy generally showed improvements in survival, as well as in blood pressure in the lungs. Other treatments included in the assessment did not consistently show an effect on clinical or survival outcomes.

No. 8 – Study Gauges Long-term Survival for PAH Patients on Opsumit

Opsumit (macitentan) is a daily oral therapy that’s approved to treat pulmonary arterial hypertension (PAH). In this study, researchers analyzed data from the Phase 3 clinical trial SERAPHIN (NCT00660179) and its open-label extension (NCT00667823) to evaluate the therapy’s long-term effect on survival. Nearly three-quarters (73.3%) of patients were alive at five years, and more than half (52.7%) were alive after nine years on Opsumit, the study found.

No. 7 – Pulmonary Hypertension Tied to Increased Cancer Risk

People with PH are known to be at increased risk of certain other health conditions, but it has not been clear whether there is an association between PH and cancer. To learn more, researchers compared cancer rates among more than 11,000 people with PH and an equal number of individuals without the disease. Results showed that PH patients were about 30% more likely to have cancer, and the association was particularly strong for lung and skin cancers.

No. 6 – Sotatercept Added to PAH Therapy Improves Exercise Ability in Patients

Sotatercept is an experimental treatment designed to “trap” certain signaling molecules to reduce the abnormal blood cell growth that drives PH. The Phase 3 clinical trial STELLAR (NCT04576988) tested sotatercept against a placebo, on top of standard-of-care therapy, in more than 300 people with PAH. Merck, sotatercept’s developer, announced in October that the trial had met its main goal, with the treatment leading to statistically significant and clinically meaningful improvement in the distance patients could walk in six minutes, a common test for PH. Based on the results, Merck announced plans to apply for regulatory approvals of sotatercept.

No. 5 – PAH Mortality Risk ‘Unacceptably High’ in the Modern Era, Study Finds

There have been marked advances in treatment for PAH in recent years, but most estimates of the disease’s mortality were from studies conducted before these developments. To assess PAH mortality risk in the modern era, researchers analyzed outcomes from a registry that has been collecting patient data since 2015. The overall mortality rates at one, two, and three years after diagnosis were 8%, 16%, and 21%, respectively. Estimated mortality rates were higher among patients with worse disease risk scores. The researchers called that risk “unacceptably high.”

No. 4 – Nearly a Third of COVID-19 Patients Show PH Signs in Study

Since the onset of the COVID-19 pandemic in late 2019, scientists have been working to understand how the disease can impact the human body. In this study, researchers performed echocardiograms, or scans of heart movement, in more than 100 people who had been diagnosed with COVID-19. Among these patients, 29.7% showed signs of PH, and those with both illnesses had worse survival outcomes. The researchers stressed that it’s impossible to tell whether these patients might have had PH prior to contracting COVID-19. But they said the results highlight the importance of vigilant testing for people with COVID-19 who show signs suggestive of PH.

No. 3 – PAH Patients Prefer Yutrepia Over Tyvaso in INSPIRE Study

Yutrepia is an inhaled formulation of treprostinil, a vasodilator that lowers blood pressure by making blood vessels relax and widen. The therapy’s developer, Liquidia, sponsored a Phase 3 trial called INSPIRE (NCT03399604) that evaluated Yutrepia in more than 100 people with PAH. Among them, 55 had previously been on Tyvaso, an older inhaled version of treprostinil. Most patients experienced side effects from Yutrepia, such as cough, headache, fatigue, and respiratory infections. However, these adverse events were generally not severe and were consistent with the known safety profile of treprostinil, according to researchers. Nearly all (98.2%) patients who switched from Tyvaso to Yutrepia said they preferred the inhaler device used with Yutrepia.

No. 2 – Sotatercept Safely Maintains Efficacy in PAH Up to 2 Years: New Data

A Phase 2 study called PULSAR (NCT03496207) compared the experimental therapy sotatercept against a placebo in more than 100 people with PAH. After the initial six-month trial, patients had the option to continue into an open-label extension (OLE) in which all were treated with sotatercept for an additional 18 months. Results from the OLE showed that improvements in lung blood pressure seen after six months were sustained after two years of treatment. Patients who switched from the placebo to sotatercept in the OLE experienced improvements in measures of lung blood pressure and exercise capacity. Rates of safety-related events generally decreased with prolonged sotatercept use.

No. 1 – Leading Causes of Death Differ by PH Type, Study Shows

PH is classified into five groups based on the underlying cause. Better understanding how each underlying type affects cause of death in PH is important for improving survival outcomes, according to researchers. Here, a team in Japan compared the causes of death for 80 PH patients seen at their clinic, including individuals with group 1 (PAH), group 3 (PH due to lung disease or low oxygen levels), or group 4 (PH due to blood clots) disease. PH itself was found to be the leading cause of death for those with group 1 disease. In group 3 patients, the leading cause of death was respiratory failure due to underlying lung disease, while in most group 4 patients cancer contributed at least partially to patient deaths. The goal now, the researchers say, is to improve outcomes in each patient group.


We at PH News look forward to continuing to serve the PH community in 2023, and we wish all our readers a happy new year!

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