Top 10 Pulmonary Hypertension Stories of 2018

Alice Melão, MSc avatar

by Alice Melão, MSc |

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Throughout the past year, Pulmonary Hypertension News has brought you information about important discoveries, treatment developments, clinical trials, and other events dealing with pulmonary hypertension (PH).

As we look ahead to 2019, here are the 10 most-read stories of 2018, with a summary of their significance to PH patients, family members, and caregivers.

No. 10 — “Six-minute Walk Test Still Key in Predicting Long-Term PAH Outcomes, Study Confirms”

The six-minute walk test (6MWT), the goal of which is to walk as far as possible in six minutes, is commonly used to evaluate exercise tolerance in patients with chronic respiratory disease and heart failure. Analysis of clinical data collected during the Phase 3 SERAPHIN trial (NCT00660179) in patients with pulmonary arterial hypertension (PAH) revealed that those who can walk more than 400 meters (437 yards) have a better long-term prognosis. Additionally, patients who had a better physical performance had a lower risk of PAH-related death or hospitalization. The study findings confirmed previous observations that the 6MWT can effectively reflect prognosis in PAH patients.

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No. 9 — “People with Mild Pulmonary Hypertension Have Increased Risk of Death, Study Indicates”

Diagnosis of PH is often based on the evaluation of mean pulmonary arterial pressure (mPAP), with a value of 25 mmHg considered the threshold above which PH is defined. However, research out of the Providence Veterans Affairs Medical Center in Rhode Island has found that current mPAP values may not be an effective diagnostic tool. Upon a review of available clinical data, the researchers found that patients with mild PH, with mPAP values between 19 and 21 mmHg, have a 1.52 times higher risk of death than control individuals with lower mPAP values. This finding suggests that redefinition of the mPAP threshold value to 19 mmHg could help identify patients who would not have been diagnosed with PH.

No. 8 — “PH Patients Seen to Have Little Daily Exercise, Leading to Lower Odds of Survival, Study Finds”

A Spanish study found that patients with PH had much lower levels of daily physical activity and more inactivity time than healthy people. They discovered that frequent moderate to vigorous exercise can help improve the outcome of patients, putting them in a “low risk” of death category, based on 6MWT results. The researchers agreed that patients should be encouraged to be physically active to improve their odds of survival.

No. 7 — “Traditional Chinese Medicine Shows Promise for Treating PAH, Study Reports”

A compound known as osthole that is used in traditional Chinese medicine, extracted from the plant Angelica pubescens Maxim, was found to lower lung blood pressure in rats with PAH. Treatment with osthole could also prevent enlargement of the heart’s right ventricle in the animals, which suggested it could inhibit the pulmonary vascular remodeling associated with PAH progression.

No. 6 — “Study Links Low Levels of Oxygen in Blood to Poor Pulmonary Hypertension Patient Outlook”

A study found that half of patients with idiopathic or heritable PAH have some degree of hypoxemia (reduced levels of oxygen) in the blood, either at rest or during physical activities. This manifestation was more prevalent in older and heavier patients, and in those who had worse scores on the walking test. Hypoxemia was also more frequent among patients who had cardiovascular and secondary respiratory diseases. Reduced levels of oxygen in the blood were found to be associated with worse long-term survival, which could be alleviated with supplementary oxygen.

No. 5 — “FDA Approves Medtronic’s Implantable System for Remodulin for Treatment of PAH”

Remodulin (treprostinil) is a vasodilator that has been marketed in the United States by United Therapeutics since 2002 for the treatment of PAH patients using external infusion pumps. In July, a new and more comfortable method of administering the medicine became available when the U.S. Food and Drug Administration approved Medtronic’s implantable system, which takes away some of the external pump’s burden and reduces the risk of complications such as infections. The new administration system is indicated for adults with class I, II, or III PAH receiving intravenous delivery of Remodulin.

No. 4 — Clinical Trial Expected in 2019 to Test Therapy C76 as Potential PAH Inhibitor”

Researchers are planning to launch a clinical trial this year to evaluate a new investigational therapy that may inhibit and possibly reverse vascular remodeling in patients with PAH. The therapy, known as C76, inhibits a gene called HIF-2α and was found to block the remodeling process of blood vessels, and reverse PAH and right heart failure in different rodent models of the disease.

No. 3 “New Diagnosis Guidelines Proposed to Include Patients At Risk of Pulmonary Hypertension”

A group of clinician-researchers with expertise in the field of cardiovascular and pulmonary diseases have proposed that the guidelines for PH diagnosis be changed to include patients who may be at risk of developing the disease. After examining the findings of several studies, experts believe that a broader definition of PH to include patients presenting mPAP values of 19 mmHg or higher (vs. 25 mmHg) would promote early patient care and potentially reduce mortality rates. The suggested guidelines were published in a commentary in The Lancet Respiratory Medicine.

No. 2 — “Mylan Announces U.S. Launch of 20 mg Tadalafil Tablets, 1st Generic of Adcirca for PAH Treatment

In August, the FDA approved the first generic formulation of Eli Lilly‘s Adcirca (tadalafil) for PAH treatment. The 20 mg tadalafil tablets were developed by Mylan, and are prescribed to improve exercise capacity in PAH patients. Like the original branded therapy, generic tadalafil tablets promote the widening of blood vessels, which, in turn, reduce pulmonary blood pressure and improve heart function, allowing patients to become more active.

No. 1 — “Exhaled Breath Temperature Can be Potential Biomarker in Patients with Pulmonary Hypertension”

Exhaled breath temperature (EBT) has been used as a marker of inflammation in patients with asthma and chronic obstructive pulmonary disease (COPD). Recently, Italian researchers have found that the temperature of exhaled breath may also help predict the outcome of PH patients. EBT levels were found to be inversely correlated to mPAP values. The lower the EBT level, the higher the mPAP values, which resulted in worse clinical severity of PH. So, determination of EBT may be a useful noninvasive method to evaluate PH patients.

Pulmonary Hypertension News hopes these stories, along with our continued reporting throughout 2019, will ultimately contribute to educating, informing, and improving the lives of patients with PH and their loved ones.

We wish all our readers a happy and inspiring 2019.