ERS Congress 2024: highlights from the Pulmonary Vascular Diseases Assembly.

https://bit.ly/3Yvb7VR.

[Screening and diagnosis of pulmonary hypertension].

SCREENING AND DIAGNOSIS OF PULMONARY HYPERTENSION. Diagnosis of pulmonary hypertension is often delayed due to the low specificity of clinical signs, dominated by exertional dyspnea. The European recommendations of the learned societies of pneumology and cardiology, published in 2022, have specified the diagnostic algorithm.

Pulmonary veno-occlusive disease: a paradigm of diagnosis and therapeutic challenges in pulmonary hypertension.

Purpose Of Review: Pulmonary veno-occlusive disease (PVOD) is a rare and life-threatening form of precapillary pulmonary hypertension. This review aims to outline its genetic and environmental risk factors, highlight key diagnostic challenges, and discuss current treatment options.

Recent Findings: PVOD can occur sporadically or as a hereditary autosomal recessive condition with biallelic eukaryotic translation initiation factor 2 alpha kinase 4 ( EIF2AK4) mutations, leading to nearly complete disease penetrance.

Pulmonary arterial hypertension in adults with Still's disease: another pulmonary manifestation associated with HLA-DRB1*15.

Objectives: Inflammatory lung disease in Still's disease (SD) has recently been described. Among its manifestations, pulmonary arterial hypertension (PAH) is a rare and life-threatening event, with only a few case reports published. The objective was to report the largest adult cohort of PAH occurring in the context of SD.

Clustering Patients with Pulmonary Hypertension Using the Plasma Proteome.

Patients with pulmonary hypertension are classified according to clinical criteria to inform treatment decisions. Knowledge of the molecular drivers of pulmonary hypertension might better inform treatment choice. To investigate plasma protein clusters in patients with a diagnosis of pulmonary hypertension.

Drugs targeting novel pathways in pulmonary arterial hypertension.

Over the past three decades, several drugs have been developed to target three major dysfunctional pathways in pulmonary arterial hypertension (PAH), including the prostacyclin, endothelin and nitric oxide pathways. Despite these advances, PAH remains incurable, necessitating further drug discovery efforts. New therapies focus on previously untargeted pathways, especially the bone morphogenetic protein (BMP)/transforming growth factor (TGF)-β signalling pathway.

Risk stratification in Eisenmenger syndrome.

Pulmonary arterial hypertension complicating congenital heart disease can progress to the life-threatening irreversible form known as Eisenmenger syndrome. When conservative treatments are inadequate, the risk of death as a result of the disease must be weighed against the risk associated with transplantation. Risk stratification has become a fundamental tool for the prediction of outcomes and the guidance of treatment in pulmonary arterial hypertension.

Effect of Balloon Pulmonary Angioplasty and Riociguat on Right Ventricular Afterload and Function in CTEPH: Insights From the RACE Trial.

Background: Riociguat and balloon pulmonary angioplasty (BPA) improve hemodynamics in inoperable chronic thromboembolic pulmonary hypertension. Importantly, comparative effects of riociguat and BPA on different components of right ventricular (RV) afterload and function remain not fully elucidated.

Methods: We conducted a post hoc analysis including patients from the RACE trial (Riociguat Versus Balloon Pulmonary Angioplasty in Non-Operable Chronic Thromboembolic Pulmonary Hypertension) with complete data for the primary end point assessment (49 riociguat and 51 BPA).

Initial therapy in patients with pulmonary arterial hypertension and cardiovascular comorbidities.

Background: European guidelines recommend initial monotherapy in pulmonary arterial hypertension patients with cardiovascular comorbidities based on the limited evidence for combination therapy in this growing population.

Methods: A retrospective analysis was conducted on incident pulmonary arterial hypertension patients enrolled in the French Pulmonary Hypertension Registry between 2009 and 2020. Propensity score matching was used to investigate initial dual oral combination therapy oral monotherapy in patients with at least one cardiovascular comorbidity ( hypertension, obesity, diabetes and coronary artery disease).

Effect of sotatercept on circulating proteomics in pulmonary arterial hypertension.

https://bit.ly/3Z5AZJ3

Risk stratification and treatment goals in pulmonary arterial hypertension.

Risk stratification has gained an increasing role in predicting outcomes and guiding the treatment of patients with pulmonary arterial hypertension (PAH). The most predictive prognostic factors are three noninvasive parameters (World Health Organization functional class, 6-min walk distance and natriuretic peptides) that are included in all currently validated risk stratification tools. However, suffering from limitations mainly related to reduced specificity of PAH severity, these variables may not always be adequate in isolation for guiding individualised treatment decisions.

Role of Exercise Hemodynamics in the Prediction of Pulmonary Arterial Hypertension in BMPR2 Mutation Carriers.

Background: Exercise hemodynamics are recommended for early detection of pulmonary arterial hypertension (PAH) and have been suggested to be predictive of future development of PAH in high-risk populations such as BMPR2 mutation carriers. However, the optimal exercise hemodynamic screening parameter remains to be determined. Recent data suggest that pulmonary vascular distensibility (α) may serve as a useful parameter for early detection of PAH.

Evidence and unresolved questions in pulmonary hypertension: Insights from the 5th French Pulmonary Hypertension Network Meeting.

Pulmonary hypertension (PH) continues to present significant challenges to the medical community, both in terms of diagnosis and treatment. The advent of the updated 2022 European Society of Cardiology (ESC) and European Respiratory Society (ERS) guidelines has introduced pivotal changes that reflect the rapidly advancing understanding of this complex disease. These changes include a revised definition of PH, updates to the classification system, and treatment algorithm.

Characteristics and outcomes of patients developing pulmonary hypertension associated with proteasome inhibitors.

Background: Pulmonary arterial hypertension (PAH) has been described in patients treated with proteasome inhibitors (PIs). Our objective was to evaluate the association between PIs and PAH.

Methods: Characteristics of incident PAH cases previously treated with carfilzomib or bortezomib were analysed from the French pulmonary hypertension registry and the VIGIAPATH programme from 2004 to 2023, concurrently with a pharmacovigilance disproportionality analysis using the World Health Organization (WHO) global database (VigiBase) and a meta-analysis of randomised controlled trials.

Risk stratification refinements with inclusion of haemodynamic variables at follow-up in patients with pulmonary arterial hypertension.

Background: Haemodynamic variables are prognostic factors in pulmonary arterial hypertension (PAH). However, right heart catheterisation (RHC) is not systematically recommended to assess the risk status during follow-up. This study aimed to assess the added value of haemodynamic variables in prevalent patients to predict the risk of death or lung transplantation according to their risk status assessed by the non-invasive four-strata model as recommended by the European guidelines.

Sarcoidosis-Associated Pulmonary Hypertension.

Pulmonary hypertension is a life-threatening complication of advanced sarcoidosis. Many mechanisms can cause an elevation of pulmonary pressure in sarcoidosis, leading to precapillary or postcapillary pulmonary hypertension. Sarcoidosis-associated pulmonary hypertension contributes to severe exertional dyspnea, reduced exercise capacity, and notably compromised the survival.

Pulmonary veno-occlusive disease: illustrative cases and literature review.

Pulmonary veno-occlusive disease (PVOD), also known as "pulmonary arterial hypertension (PAH) with overt features of venous/capillary involvement", is a rare cause of PAH characterised by substantial small pulmonary vein and capillary involvement, leading to increased pulmonary vascular resistance and right ventricular failure. Environmental risk factors have been associated with the development of PVOD, such as occupational exposure to organic solvents and chemotherapy, notably mitomycin. PVOD may also be associated with a mutation in the gene in heritable forms of disease.

Outcomes and risk assessment in pulmonary veno-occlusive disease.

Introduction: Pulmonary veno-occlusive disease (PVOD) is a rare and severe subtype of pulmonary arterial hypertension (PAH). Although European Society of Cardiology/European Respiratory Society (ESC/ERS) guidelines advise assessing PAH severity at baseline and during follow-up, no existing risk assessment methods have been validated for PVOD. This study aimed to identify prognostic factors, examine the impact of treatment strategies and evaluate risk assessment methods for PVOD patients.

Pharmacological management of connective tissue disease-associated pulmonary arterial hypertension.

Introduction: Pulmonary arterial hypertension (PAH) is a severe, progressive pulmonary vasculopathy (Group 1 Pulmonary Hypertension (PH)) that complicates the course of many connective tissue diseases (CTD). Detailed testing is required to differentiate PAH from other types of PH caused by CTD such as left heart disease (Group 2 PH), pulmonary parenchymal disease (Group 3 PH), and chronic thromboembolic pulmonary hypertension (Group 4 PH). PAH is most frequently seen in systemic sclerosis but can also be seen with systemic lupus erythematosus, mixed CTD, and primary Sjogren's syndrome.