Since 2008, Rare Disease Day — the last day of February — has brought together patients, caregivers, family members, friends, and advocates from around the world to raise awareness and improve equity for the more than 7,000 known rare diseases that affect more than 300 million people. In 2022, the…
Breathing reserve, a measure of lung function calculated during exercise, predicted survival in adults with congenital heart disease associated with pulmonary arterial hypertension (PAH), a study reported for the first time. Breathing reserve assessment in other diseases affecting blood vessels in the lungs also may predict survival outcomes, the…
A passive leg raise is equally as effective as exercise in measuring pulmonary vascular distensibility, a physiological marker to predict outcomes in people with pulmonary hypertension (PH), a study suggests. This finding is important for patients who cannot exercise or do not have access to clinical exercise facilities, the…
Identification of mutations in the ATP13A3 gene, which are associated with a severe form of pulmonary arterial hypertension (PAH), led doctors to select a riskier treatment approach for a 21-year-old woman to prevent her symptoms from worsening, according to a case study. “Short-term follow-up is encouraging, and the patient…
A calcium channel called TRPC6 may offer a new target for the development of treatments for pulmonary hypertension (PH), including pulmonary arterial hypertension (PAH), a mouse study suggests. Researchers also found that a calcium channel blocker called BI-749327, given orally, reversed PH in a mouse model of the…
Among people selected for kidney transplant, the presence of pre-transplant pulmonary hypertension (PH) is associated with an increased risk of transplanted kidney dysfunction at five years, according to a single-center study in the U.S. looking at 350 patients. However, this risk did not affect the five-year survival of patients…
Plasma volume status (PVS) — an indirect, non-invasive measure of heart congestion — is a predictor of short-term hospital stays and mortality outcomes in people with multiple types of pulmonary hypertension (PH), a study reported. According to the researchers, plasma volume could be used to monitor…
Researchers in the U.K. will evaluate whether the levels of NT-proBNP — a heart damage biomarker that serves as a prognostic marker of pulmonary hypertension (PH) — can be accurately measured in a finger-prick blood sample that can be taken by patients at home and sent by mail to…
Measuring levels of protein sLR11 in the blood could be useful in testing for pulmonary hypertension (PH) in people who have heart failure, according to a new study. The study, “Circulating sLR11 levels predict severity of pulmonary hypertension due to left heart disease,” was published in…
High blood levels of NT-proBNP — a validated and widely accepted prognostic marker of pulmonary hypertension (PH) — may serve as a biomarker to diagnose PH among people with chronic obstructive pulmonary disease (COPD) exacerbations, according to a study in China. However, no significant association was detected between…
The use of imaging to evaluate the right side of the heart could be useful for non-invasively diagnosing and monitoring pulmonary arterial hypertension (PAH) in people with connective tissue diseases, according to a new study. The study, “Evaluation of pulmonary arterial pressure in patients with connective…
Gene activity analysis of whole blood collected from people with pulmonary arterial hypertension (PAH) identified three patient subgroups associated with distinct clinical features and outcomes, a study concluded. These findings may provide molecular insights into the development of PAH, improve disease risk assessments, and potentially guide individual treatment strategies,…
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