News

Measuring blood vessel function in the arm in a noninvasive way can help predict pulmonary hypertension (PH) when combined with certain heart measurements, a study has found.

The study, “Endothelial Function Correlates With Pulmonary Pressures in Subjects With Clinically Suspected Pulmonary Hypertension,” was published in The American Journal of Cardiology.

PH occurs when blood pressure in the pulmonary arteries that carry blood from the heart to the lungs is too high. This increased pressure can lead to serious problems, such as heart failure. To understand PH better, it’s important to study how blood flows through these arteries. read more

In the world of chronic illness, medications can be many things. At best, they’re a blessing, but more often than not, I’ve found them to be a necessary evil.

While living with pulmonary hypertension (PH) for 19 years, I remained on a fairly consistent medication regimen. My doctors occasionally adjusted my dosages and added a new treatment every once in a while. But for the most part, the group of drugs I was prescribed didn’t change.

The side effects weren’t the greatest, but dealing with them was a part of my routine. Plus, even if they became intolerable, I had few alternatives, considering the limited range of treatments available for PH. read more

Do you ever feel like a fraud because of your rare disease?

For the past two decades while managing pulmonary hypertension (PH), I’ve battled self-doubt, shame, and feelings of inadequacy. I know I’m not alone. We all face these emotions at different points in life, and through conversations with friends and colleagues who have PH or another rare disease, I’ve found that many of us question ourselves and feel like we don’t belong.

I even stumbled across a column by a friend with cystic fibrosis, Brad Dell, called “Impostor syndrome is an injustice to all we’ve endured with CF.” While his struggle is different from mine, our illnesses have caused both of us to doubt ourselves at times. read more

Two molecules, 5-hydroxyindoleacetic acid (5-HIAA) and insulin-like growth factor 1 (IGF-1), showed a potential to diagnose pulmonary hypertension  early in children with congenital heart disease based on their levels in blood and urine samples examined in a study.

5-HIAA is the main product of the breakdown of serotonin, a signaling molecule that regulates vascular tone — the degree of blood vessel constriction — while IGF-1 is reported to influence blood vessel widening and heart muscle cell survival. read more

Healthcare costs were found to be more than eight times higher for people with pulmonary arterial hypertension (PAH) compared with patients without the disease in a real-world study — which contributed to the greater economic burden seen for those being treated for PAH.

These costs were mainly driven by expenses in PAH medications and hospitalizations, which increased significantly in the last months of patients’ lives, according to the study, led by scientists from Merck & Co. and IQVIA, a clinical research services company. read more

Sildenafil — a medication that relaxes blood vessels, thereby increasing blood flow — may possibly be used to treat neonatal pulmonary hypertension in babies born at high altitudes, where less oxygen is available for breathing.

This is according to clinical observations by Alexandra Heath-Freudenthal, MD, a pediatric cardiologist, while providing care in the Bolivian cities of La Paz and El Alto, located more than 2.2 miles above sea level on the Andes mountains in South America.

While the observations are preliminary, they may provide ground for extending the indication of sildenafil, approved under the brand names Revatio and Liqrev for patients with pulmonary arterial hypertension, to newborns.  read more

I have pulmonary hypertension (PH), which is difficult to manage, but it doesn’t affect only me. It also affects the people closest to me, including my husband, Manny, who is my rock.

Unfortunately, living with a rare disease can strain a marriage at times. When that happens, it helps to put yourself in your partner’s shoes. Many couples find that facing obstacles together can actually strengthen their bond.

Unless you’ve experienced having PH yourself, fully understanding what a partner with the disease is going through can be challenging. We PH patients need more rest than a healthy person does, and we might not always feel like going out to do things. PH limits our ability to work, do chores, and complete other everyday tasks. I try to compensate for lost time by doing several activities over the course of a day or two, when I feel capable. read more

High blood levels of resistin, a hormone produced by fat cells, may help identify pulmonary arterial hypertension (PAH) patients at higher risk of clinical worsening, a large U.S. study suggests.

Resistin levels also could separate people with idiopathic, or unknown cause, PAH (iPAH) or scleroderma-associated PAH (SSc-PAH) from healthy controls.

The findings support resistin as a “novel biomarker for PAH prognostication and may indicate a new therapeutic avenue,” the study’s researchers wrote in “Resistin predicts disease severity and survival in patients with pulmonary arterial hypertension,” which was published in Respiratory Research. read more

When I make plans to go to a concert, amusement park, or sporting event, my first thought turns to accessibility issues. It’s unfortunate, but with today’s heightened security and limits on what you can bring into a venue, getting in with a medical device sometimes feels like a small miracle.

Last year, a friend and I traveled to Nashville, Tennessee, to sing our faces off at a Taylor Swift concert. During the height of the pandemic in 2020, we’d taken a deep dive into Swift’s discography, courtesy of the “Every Single Album” podcast. We agreed to see the pop superstar perform next time she was on tour. read more

The U.S. Food & Drug Administration (FDA) has tentatively approved Alembic Pharmaceuticals‘ generic formulation of injectable selexipag as a treatment for pulmonary arterial hypertension (PAH).

As a generic drug, which contains the same chemical substance as an approved treatment, it’s equivalent to Uptravi (selexipag) for injection (1,800 mcg/vial), sold by Johnson & Johnson.

Tentative approval means Alembic’s application met all the requirements for approval, but patents or exclusivities block final approval. The application contains a Paragraph IV certification under provisions of the Hatch-Waxman Act, which allows Alembic to seek FDA approval to market the generic drug before the expiration of brand-name drug patents. read more