News

Elevated blood levels of uric acid are associated with a higher risk of pulmonary arterial hypertension (PAH) at the genetic level, a data study reported.

“Overall, this study confirmed, from a genetic perspective, the causal relationship between serum UA [uric acid] levels and PAH,” the researchers wrote. They also suggested that treatments working to lower these levels might benefit PAH patients.

The study, “Serum uric acid and pulmonary arterial hypertension: A two-sample Mendelian randomization study,” was conducted by a team in China and published in the journal Heart & Lung. read more

Cereno Scientific’s investigational therapy CS014 was found to reverse remodeling, meaning alterations in structure and arrangement, of pulmonary arteries in a preclinical model of pulmonary arterial hypertension (PAH).

According to the company, the new preclinical data “provide the most compelling evidence to date that CS014 offers a disease modifying approach to PAH and related pulmonary vascular diseases by robustly reversing pulmonary pathological [disease-causing] vascular remodeling and fibrosis,” or tissue thickening and scarring. read more

I grew up in Minnesota, the “Land of 10,000 Lakes.” In fact, my childhood home was located on one of the largest lakes in the state. The summers in our lake town centered on water activities, from tubing behind a boat to deep dives off the dock. Being in the water was a significant part of the local culture.

It was a beautiful place to grow up. But it was also a bit inconvenient for me, given that I couldn’t swim.

This was always a shocking reveal when making new friends. “What do you mean you can’t swim?” It took some explaining to convey that I had a central line in my chest with a pump attached to it, neither of which could get wet. I was diagnosed with pulmonary hypertension (PH) at the age of 4 and placed on IV Flolan (epoprostenol GM) soon after, so swimming was something I had never learned how to do. read more

In August, I had the amazing opportunity to attend the Pulmonary Hypertension Association’s (PHA) International PH Conference in Indianapolis. Meeting newly diagnosed patients and caregivers was a vibrant experience. For many, it was their first time attending such an event, and the energy was contagious. Even the pharmaceutical and healthcare teams appeared hopeful.

The conference offered educational and group support sessions, workshops, and panels, with a focus on the latest research and therapies for those of us with pulmonary hypertension (PH). The excitement around new treatments for PH was palpable, and hope buzzed in the air. read more

Differences in Uptravi (selexipag) maintenance doses have no impact on the risk of hospitalization among adults with pulmonary arterial hypertension (PAH), a real-world claims analysis suggests.

While different maintenance doses did not affect treatment adherence or discontinuations among U.S. patients in the real world, dose adjustments were more frequent for those who took lower regular doses, the data analysis found.

“The findings in this diverse, real-world population of patients with PAH reinforced an individualized approach to the dosing scheme to maximize benefit-risk and achieve the highest tolerated dose with [Uptravi],” the researchers wrote. read more

Oral carnitine supplements were well tolerated and blood levels of the molecule increased in people with pulmonary arterial hypertension (PAH), a pilot study shows.

Carnitine transports fatty acids — the building blocks of fatty molecules — into the cells’ powerhouses, the mitochondria, where they are broken down to produce energy. Some studies indicate PAH patients may have a carnitine deficiency, which can be linked to heart issues.

“Our study demonstrated the feasibility of carnitine supplementation in PAH and that relative carnitine deficiency may be rapidly overcome through this intervention,” the researchers wrote. Supplementation didn’t change measures of heart function, heart damage, and exercise capacity, however. read more

Treatment with Adempas (riociguat) is generally safe and effective for people with pulmonary arterial hypertension (PAH) and coexisting cardiometabolic conditions, or those that affect the heart and metabolism, according to a pooled analysis of clinical trials.

“Efficacy and risk assessment results suggest that [Adempas] can be beneficial for patients with PAH irrespective of the presence of comorbidities [coexisting conditions],” researchers wrote.

The study, “Safety and efficacy of riociguat in patients with pulmonary arterial hypertension and cardiometabolic comorbidities: Data from interventional clinical trials,” was published in The Journal of Heart and Lung Transplantation. read more

Companionship and physical intimacy are universal needs that play an important physical, psychological, emotional, and social role in our lives. But living with a rare condition like pulmonary hypertension (PH) can complicate matters. Intimacy often becomes secondary when I am exhausted, in pain, or short of breath — all common symptoms of PH.

This isn’t a topic I’ve been shy about discussing. In a 2022 column titled “Keeping Physical Intimacy Alive, Even With PH,” I shared how I maintain a fulfilling sex life with my husband, Manny, while managing PH. It takes communication and some adaptations. read more

Having fewer very fine blood vessels in the fold of skin at the base of the fingernails appears to be linked to an increased risk of clinical worsening in pulmonary arterial hypertension (PAH), a study in the Netherlands suggests.

People with chronic thromboembolic pulmonary hypertension (CTEPH) also had fewer fine blood vessels, called capillaries, in their nail folds than healthy people, but this didn’t change after a surgical procedure to relieve high blood pressure in their lungs.

This suggests a loss of capillaries isn’t unique to PAH or directly caused by how the disease affects blood flow. Instead, shared mechanisms may cause changes to both the pulmonary arteries that supply the lungs and the capillaries throughout the body. The study, “Low nailfold capillary density in patients with pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension: biomarker of clinical outcome?” was published in Scientific Reports. read more