Researchers looked at whether cigarette smoking affected the diagnosis and disease progression of patients with pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH). They found that smoking tended to delay the diagnosis of PAH but not CTEPH. They also found that male smokers with PAH fared worse than female smokers. Read the Pulmonology article

This study tried to identify new environmental triggers or genetic causes in heritable pulmonary arterial hypertension. They found several potential genes for further exploration, but they also found consistent toxin exposure on one of the families they were investigating. They conclude that environmental triggers should also be investigated in heritable PAH. Read the Scientific Reports article

This study identified factors associated with a delayed diagnosis of PAH and found that earlier diagnosis (<6 months) and treatment initiation were associated with significantly improved survival. More early diagnosis patients saw a pulmonologist before another provider. Read the Pulmonary Circulation article

A review of 12 studies evaluating selexipag in pediatric patients with pulmonary arterial hypertension found that it improved functional capacity and mPAP, especially when added to existing therapies. About 60% of pediatric patients had side effects, mostly mild. Read the Pediatric Cardiology article

Patients with portopulmonary hypertension were not included in clinical trials for sotatercept. This case report found that sotatercept helped one patient with portopulmonary hypertension, who was then able to get a liver transplant. However, they did note some risks. Read the Communications Medicine article

Researchers conducted a meta-analysis, looking at 13 studies with about 700 participants in total with pulmonary hypertension (PH). They found that exercise improved 6-minute walk distance and was consistently well-tolerated by PH patients, and there were no serious adverse events directly linked to the exercise activities in the studies. Read the European Journal of Applied Physiology article

A study found that chronic thromboembolic pulmonary hypertensio (CTEPH) patients had significantly more of an antioxidant enzyme (peroxiredoxin 2, or PRDX2) in their bloodstream compared to healthy patients. Using rats, the researchers determined that PRDX2 helped reduce inflammation, suggesting that it has a protective role and might have therapeutic use. Read the Scientific Reports article

This study examined the causes of delayed diagnosis (diagnosis after approximately 22 months) of pulmonary arterial hypertension (PAH) and whether an earlier diagnosis (within six months) leads to better outcomes. They identified several factors associated with delayed diagnosis and found that earlier diagnosis and treatment were associated with better survival. Read the Pulmonary Circulation article

A relatively simple, inexpensive, and minimally invasive test for idiopathic pulmonary arterial hypertension (IPAH) would aid in the diagnosis of this disease. This study found that levels of the NOTCH3 extracellular domain (NOTCH3-ECD) shed into blood serum could be used as a biomarker for IPAH. They found that NOTCH3-ECD levels did correlate with the presence and severity of IPAH. Read the Nature Medicine article

As pulmonary arterial hypertension (PAH) patients became sicker, their self-reported quality of life was significantly worse than their doctors recognized. This discordance was associated both with worse health-related quality of life scores and a higher WHO functional class score, so the sickest patients had the least agreement with their doctors. The researchers note that "it's self-evident that providers cannot deliver optimal treatment strategies nor best outcomes if they

Looking at the racial and ethnic breakdown of patients with PAH and patients who take part in clinical trials, the authors conclude that the goal of equitable representation has not yet been reached. The article includes a useful table of potential barriers to inclusive clinical trials and potential solutions. Read the Advances in Pulmonary Hypertension article

Air pollution is known to be bad for heart and lung diseases, but this study looked at whether it affected pulmonary arterial hypertension (PAH) in particular. They followed a cohort of patients for about three years and found that not only does exposure to high levels of air pollution increase the risk of death or lung transplantation for PAH patients, it made their pulmonary hypertension worse. People who lived in highly polluted areas were more likely to be categorized as.

A small study of patients with stable pulmonary arterial hypertension (PAH) were randomly assigned to either no exercise program or an exercise program using kettlebells three days per week for 8 weeks. The kettlebell group had improved functional capacity, quality of life, and physical activity level, and reduced fatigue, dyspnea, anxiety and depression. Read the Respiratory Medicine article

A study found that people taking infused prostacyclin analogues for pulmonary hypertension had a significantly lower risk of developing age-related macular degeneration, an eye problem that causes vision loss in older adults. The protection was robust -- patients using systemic prostacyclin analogues were about 60% less likely to develop several types of age-related macular degeneration -- and the effect was sustained over time. Read the Eye article

For very preterm babies, inhaled nitric oxide in the first week of life seems to help both persistent pulmonary hypertension of the newborn (PPHN) and retinopathy. After the first week, though, inhaled nitric oxide wasn't associated with better outcomes. Inhaled nitric oxide could be reframed as multi-organ protection for very premature babies with PPHN. Read the Nature article

This study compared the costs of an 8-week centre-based pulmonary rehabilitation program to a virtual program. Costs and outcomes were...

Because a drug trial for another drug similar to sotatercept was stopped when it was found to be associated with pericardial effusion...

In chronic thromboembolic pulmonary hypertension (CTEPH), this study found that the cells lining blood vessels (the endothelium) are...

A study looked at 262 very premature babies diagnosed with pulmonary hypertension who were given inhaled nitric oxide. For babies whose...

In the body, variations in several genes were associated with a more than 20-fold increased risk of pulmonary arterial hypertension...