Since sotatercept received Food and Drug Administration (FDA) approval for pulmonary arterial hypertension in March 2024, knowing when and how to use it has become an essential part of managing this serious condition — so say the authors of a recently published article in CHEST, who offer a practical guide for clinicians navigating this new […]
The authors of a study recently published on CHEST retrospectively analyzed data from adult patients enrolled in the French Pulmotension registry (Commission Nationale de l’Informatique et des Libertés No. 842063) from the Limoges Competence Center between May 1, 2020, and May 1, 2021. Inclusion criteria were the following: (1) confirmed pulmonary hypertension (PH) diagnosed by
This review covers the full clinical picture of pulmonary arterial hypertension (PAH) — from its pathophysiology and diagnosis through to current and emerging treatments. Understanding the Disease Pulmonary arterial hypertension is driven by progressive structural remodelling of the pulmonary arteries, creating a vicious cycle of vasoconstriction, inflammation, and uncontrolled cell growth that ultimately leads to
A Japanese study has confirmed that sotatercept works just as well in Asian patients, even though pulmonary arterial hypertension presents somewhat differently in the Asian population: patients tend to have longer disease duration, more severe baseline profiles, and are more frequently on intensive triple therapy including parenteral prostacyclin. This was a regional Phase 3, open-label
This International Women’s Day, the European Society of Cardiology (ESC) proudly announced the launch of the patient version of the 2025 guidelines on the management of cardiovascular disease (CVD) in pregnancy. Pregnancy is a life-changing experience — but for women living with cardiovascular disease, it comes with an additional layer of complexity, uncertainty, and anxiety.
A large US claims-based study has shed light on a worrying trend: pulmonary arterial hypertension linked to methamphetamine use (Meth-APAH) is rising fast — up 93% between 2018 and 2022, a rate disproportionate to the general rise in methamphetamine use itself. Compared to other pulmonary arterial hypertension patients, those with Meth-APAH tend to be younger,
A consensus document featuring 10 statements, the main focus of which was the use of parenteral prostanoids, was developed by eight experts in pulmonary arterial hypertension during in-person and web-based meetings. Forty-six Italian physicians were invited online to rate each statement, indicating their agreement, neutrality or disagreement. These consensus statements are intended to support physicians
A retrospective study based on data from the Spanish pulmonary arterial hypertension Registry (REHAP) set out to compare the demographic and clinical profiles, comorbidity burden, and survival rates of patients with connective tissue disease-associated pulmonary arterial hypertension (CTD-PAH) against those with idiopathic pulmonary arterial hypertension (IPAH). The study, covering the period 2014–2023, included 757 patients
Here is the AI translation of the index. Patient Community & Events Regional Association Updates (Landesverbände) Reports from local pulmonay hypertension chapters across Germany, covering Baden-Württemberg, Bavaria, Berlin/Brandenburg/Mecklenburg-Vorpommern, Hamburg/Schleswig-Holstein, Hesse, North Rhine-Westphalia, Saxony, and Saarland/Rhineland-Palatinate (pp. 16–33), plus a directory of regional leaders (p. 33). Medical & Scientific Topics Practical Patient Information Policy & Advocacy
The PROSERA study for seralutinib in pulmonary arterial hypertension findings were released yesterday and report that it did not meet its primary endpoint in the full population. It appears that unexpected placebo results in some geographical areas may have been responsible for this happening. While this is disappointing news for the patient community the program
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
