Developed by psychologists, and supported by the UK’s pulmonary hyeprtension association, PHA UK, this four-week programme consists of a series of workbooks based on Cognitive Behavioural Therapy (CBT), to help patients change their thinking habits and behaviours. Find our more at this link on the PHA UK website
The emPHasis-10 questionnaire is a specialized tool for assessing health-related quality of life in pulmonary arterial hypertension patients. A comprehensive validation process was undertaken by researchers in Thailand including forward-backward translation, expert content validity assessment using Item-Objective Congruence (IOC) index by five specialists, and reliability testing with 20 Thai PAH patients using both internal consistency
Pulmonary Hypertension News published a report on February 2, 2026, about a case series and literature review found that ECMO (extracorporeal membrane oxygenation)—a life-support machine that temporarily replaces heart and lung function—significantly improves survival in pregnant women with severe pulmonary arterial hypertension (PAH). The study documented five cases where the machine was used as rescue
An Italian study examined whether echocardiographic patterns of right heart changes could improve risk prediction in pulmonary arterial hypertension patients beyond current tools. Researchers defined four phenotypes based on right ventricular size and function, then followed patients for nearly 4 years. They found that these echo-based classifications provided additional prognostic information independent of existing risk
This multicenter, open-label, single-arm phase 3 study aimed to assess the efficacy and safety of sotatercept in Japanese patients with PAH. Forty-six adult Japanese patients with pulmonary arterial hypertension receiving stable background therapy were enrolled in a multicenter, open-label, single-arm phase 3 study to assess the efficacy and safety of sotatercept in Japanese patients. They
The most common genetic cause of heritable pulmonary arterial hypertension is a pathogenic variant in the BMPR2 gene, which encodes a receptor in the TGF-β signaling pathway. While 18 pulmonary arterial hypertension genes have been identified and can be tested simultaneously through gene panel sequencing, approximately 17% of heritable pulmonary arterial hypertension patients show no
Women are disproportionately affected by both pulmonary arterial hypertension (PAH) and breast cancer. BMPR2 (bone morphogenetic protein receptor type 2) mutations are the leading genetic cause of familial pulmonary arterial hypertension. The findings of a study published in Circulation indicate that breast cancer and pulmonary arterial hypertension share a common genetic link involving the BMPR2
Current joint European Society of Cardiology and European Respiratory Society pulmonary hypertension clinical practice guidelines recommend double oral therapy for intermediate-risk pulmonary arterial hypertension patients . They recommend upfront triple combination therapy for high-risk patients only, but retrospective data suggests triple therapy including parenteral prostacyclins may be better. The phase IV, multicentre, prospective, randomised, two-arm,
The UK Pulmonary Hypertension Association’s free form has been designed to help patients prepare for remote medical appointments. Completing it it beforehand can help make the most of the visits. Download the form at this link on the PHA UK website
A recent study published in Pulmonary Circulation, summarised in an article by Andrea Lob on Pulmonary Hypertension News, found that early diagnosis of pulmonary arterial hypertension (PAH) – within six months of first healthcare contact for symptoms – improves five-year survival by 42 percentage points compared to delayed diagnosis (two years or more). Five-year survival
UC San Diego researchers published findings in Nature Medicine demonstrating that a simple blood test can detect pulmonary arterial hypertension (PAH) before it becomes life-threatening. What follows is a brief summary from an article about this study published on ABC 10 New San Diego (see link below). The test identifies a specific piece of the
This comprehensive Nature Reviews Disease Primers paper provides an authoritative overview of pulmonary hypertension (PH) globally, with particular attention to disparities between high-income (HICs) and low- and middle-income countries (LMICs). Key Points Classification and Prevalence: pulmonary hypertension is defined as mean pulmonary artery pressure >20 mmHg and comprises five groups: Group 1 (pulmonary arterial hypertension/PAH
Pulmonary hypertension, Nature Reviews Disease Primers, January 4, 2024 Read Post »
Twelve pulmonary arterial hypertension patients followed at the Fuwai Central China Cardiovascular Hospital in Zhengzhou City in the Henan Province, China, were interviewed to understand their self-management challenges. The findings were published in the Patient Preference and Adherence Journal on January 17, 2026, and reveal significant barriers to effective disease self-management across two main categories.
Research published in Respiratory Medicine on January 12, 2026, explored adherence to pulmonary arterial hypertension-specific therapies across different therapeutic classes, identified factors associated with non-adherence and evaluated its impact on clinical outcomes. Adherence was measured by proportion of days covered (PDC) during the treatment period, defined as 60 days after first therapy until treatment discontinuation/censor.
A prospective analysis from the Spanish Registry of pulmonary arterial hypertension (REHAP) examined 59 patients diagnosed with toxic oil syndrome-associated pulmonary arterial hypertension (TOS-PAH) between 1997 and 2025, providing new clinical, pathological and genetic insights into this historically significant form of drug-induced pulmonary arterial hypertension. Key findings: The patient cohort was 63% female with a
The Pulmonary Hypertension Global Patient Survey (PH GPS), the largest survey of its kind in the pulmonary hypertension field, was collaboratively designed through a multidisciplinary consensus process and disseminated by pulmonary hypertension organizations globally. The survey analyzed data from 3,329 adult patients (mean age 52.2 years, 81.7% female) and 135 paediatric patients, examining healthcare provision,
The Pulmonary Hypertension Association of Canada, PHA Canada, has published an article reporting on the findings of a survey where they asked a small group of patients what they valued about having a right heart catheterization (RHC), and how they would like their care providers to share the information. The article is not open access
During right heart catheterization (RHC), the “acute vasodilator test” offers valuable insights into pulmonary circulation by evaluating vasoreactivity. A positive result identifies a rare pulmonary arterial subtype that may benefit from long-term calcium-channel blocker therapy (the so called “responders”). A recent study re-examines the applications, criteria, and objectives of acute vasodilator testing in pulmonary hypertension
A retrospective cohort study using data from 103 healthcare organizations compared 454 pulmonary arterial hypertension patients receiving sotatercept to 963.386 pulmonary arterial hypertension patients not receiving sotatercept. After propensity score matching to balance baseline characteristics, 346 patients per group were analyzed over an average follow-up of approximately 6-8 months. The sotatercept group demonstrated dramatically lower
The Pulmonary Vascular Research Institute (PVRI) GoDeep meta-registry integrates data from international pulmonary hypertension registries, from which 21,123 incident hemodynamically fully characterised patients were analysed to investigate the influence of sex on survival of patients. Findings revealed that male patients have significantly higher mortality rates than female patients across all types of pulmonary hypertension. This survival
