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In patients with very severe CTEPH eligible for BPA, it is possible to achieve major haemodynamic improvement with upfront triple PH therapy including epoprostenol and then to perform angioplasties https://bit.ly/3vZZvib., Competing Interests: Conflict of interest: The authors have nothing to disclose., (Copyright ©The authors 2024.)

Pulmonary vascular diseases such as pulmonary embolism and pulmonary hypertension are important and frequently under-recognised conditions. This article provides an overview of key highlights in pulmonary vascular diseases from the European Respiratory Society International Congress 2023. This includes insights into disease modification in pulmonary arterial hypertension and novel therapies such as sotatercept and seralutinib. Exciting developments in our understanding of the mechanisms underpinning pulmonary hypertension associated with interstitial lung disease are also explored. A comprehensive overview of the complex relationship between acute pulmonary embolism and chronic thromboembolic pulmonary hypertension (CTEPH) is provided along with our current understanding of the molecular determinants of CTEPH. The importance of multidisciplinary and holistic care cannot be understated, and this article also addresses advances beyond medication, with a special focus on exercise training and rehabilitation., Competing Interests: Conflict of interest: S. Cullivan reports travel grant from Janssen outside the submitted work. Conflict of interest: A. Boucly reports honoraria for lectures or travel support from AOP Orphan, Janssen, Ferrer and MSD outside the submitted work. Conflict of interest: M. Jevnikar reports travel grants from MSD, Janssen and AstraZeneca, and consulting fees from Janssen, outside the submitted work. Conflict of interest: B. Lechatier reports participation on advisory boards and travel grants from MSD and Janssen outside the submitted work. Conflict of interest: S. Ulrich receives grant money from the Swiss National Science Foundation and Swiss Lung League; and received grant money, travel fees, consultancy and for patient enrolment into trials from Janssen SA, MSD SA, Novartis SA and OrPha Swiss. Conflict of interest: L. Bertoletti reports personal fees and nonfinancial support from BMS/Pfizer, LEO-Pharma and Viatris; grants from Bayer; and grants, personal fees and nonfinancial support from MSD, outside the submitted work. Conflict of interest: O. Sitbon reports relationships with pharmaceutical companies including Merck, Janssen, Enzyvant, Gossamer Bio, Respira Therapeutics, Ferrer and AOP Orphan, outside the submitted work; relationships include grants for research and educational programmes, fees for lectures and membership of scientific advisory boards. Conflict of interest: A. Vonk-Noordegraaf reports participation on advisory boards for Ferrer, MSD and Johnson & Johnson, outside the submitted work. A. Bokan has nothing to declare. D-H. Park reports support for attending meetings and/or travel from Janssen, outside the submitted work. Conflict of interest: L. Genecand has nothing to declare. Conflict of interest: J. Guiot reports grants paid to his institution from Roche, Janssen and Merk; consulting fees from Oncoradiomics, Janssen, Boehringer Ingelheim, Pfizer and AstraZeneca; honoraria from Janssen, SMB, GSK and Chiesi; and travel support from Merck and Chiesi, all outside the submitted work. Conflict of interest: E-M. Jutant reports consulting fees from Chiesi; honoraria from Chiesi, GSK, MSD and AstraZeneca; and travel support from Janssen and MSD. Conflict of interest: L. Piccari reports grants from Janssen and Ferrer; lecture honoraria from Janssen, Ferrer, MSD and United Therapeutics; participation on advisory boards with Janssen, Ferrer and United Therapeutics; and travel support from Janssen, Ferrer and MSD, outside the submitted work. Conflict of interest: M. Lichtblau reports travel support, lecture honoraria and participation on advisory boards from Janssen, MSD and Orpha Swiss, outside the submitted work., (Copyright ©The authors 2024.)

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., Methods: The study analysed all incident PVOD patients included in the French Pulmonary Hypertension Registry between 2006 and 2021. Survival was assessed based on initial treatment strategy and risk status and compared to a matched (age, sex, pulmonary vascular resistance) PAH group. Six risk assessment methods (number of four low-risk and three noninvasive low-risk variables, ESC/ERS guidelines three-strata and four-strata models, REVEAL 2.0 and Lite 2) were applied at baseline and early follow-up, and their accuracy was compared using Harrell's c-statistic., Results: Among the 327 included PVOD patients, survival rates at 1, 3 and 5 years were 86%, 50% and 27%, respectively. Multivariate analysis showed that only 6-min walk distance was associated with survival, with no significant difference based on initial treatment strategy. All six risk assessment methods could discriminate mortality risk, and the ESC/ERS four-strata model was the most accurate at both baseline and follow-up (C-index 0.64 and 0.74). PVOD survival rates were consistently lower than PAH when comparing baseline risk status using the ESC/ERS four-strata model., Conclusion: PVOD is associated with poor outcomes, and initial treatment strategies do not significantly affect survival. Risk assessment methods can be useful in predicting survival for PVOD patients., Competing Interests: Conflict of interest: A. Boucly reports grants or contracts from Acceleron, Janssen and MSD, outside the submitted work; payment or honoraria for lectures, presentations, speakers’ bureaus, manuscript writing or educational events from Janssen, Merck, AOP Orphan and Ferrer, outside the submitted work; support for attending meetings and/or travel from Janssen and MSD, outside the submitted work. Conflict of interest: X. Jaïs reports grants or contracts from Acceleron, Janssen, MSD and Bayer HealthCare, outside the submitted work; payment or honoraria for lectures, presentations, speakers’ bureaus, manuscript writing or educational events from Janssen, and MSD, outside the submitted work. Conflict of interest: M. Jevnikar reports payment or honoraria for lectures, presentations, speakers’ bureaus, manuscript writing or educational events from Janssen, outside the submitted work. Conflict of interest: A. Bourdin reports grants or contracts from AstraZeneca and Boehringer Ingelheim, outside the submitted work; consulting fees from AstraZeneca, GSK, Novartis, Sanofi Regeneron, Boehringer Ingelheim and Chiesi, outside the submitted work; payment or honoraria for lectures, presentations, speakers’ bureaus, manuscript writing or educational events from AstraZeneca, GSK, Novartis, Sanofi Regeneron, Boeringher Ingelheim and Chiesi, outside the submitted work; support for attending meetings and/or travel from AstraZeneca, GSK, Novartis, Sanofi Regeneron, Boehringer Ingelheim and Chiesi, outside the submitted work; and participation on a data safety monitoring or advisory board for AB Science, outside the submitted work. Conflict of interest: A. Chaouat reports payment or honoraria for lectures, presentations, speakers’ bureaus, manuscript writing or educational events from GSK, MSD and Chiesi, outside the submitted work; and support for attending meetings and/or travel from Janssen and AstraZeneca, outside the submitted work. Conflict of interest: V. Cottin reports consulting fees from Ferrer/United Therapeutics, outside the submitted work; payment for lectures and consulting from Ferrer/United Therapeutics, outside the submitted work; participation on data and safety monitoring board for Galapagos, Galecto and GSK, outside the submitted work; and adjudication committee for Fibrogen, outside the submitted work. Conflict of interest: L. Bertoletti reports grant to the institution for research studies from MSD and Bayer, outside the submitted work; consulting fees from MSD, outside the submitted work; payments for honoraria for lectures, presentations, speakers’ bureaus, manuscript writing or educational events from MSD, BMS/Pfizer, LEO-Pharma and Viatris, outside the submitted work; support for attending meetings and/or travel from Johnson and Johnson, BMS/Pfizer, and LEO-Pharma, outside the submitted work; participation on a data safety monitoring or advisory board for Bayer, outside the submitted work; receipt of medical writing services from BMS/Pfizer, outside the submitted work. Conflict of interest: L. Savale reports payment or honoraria for lectures, presentations, speakers’ bureaus, manuscript writing or educational events from Janssen, outside the submitted work; support for attending meetings and/or travel from Merck, outside the submitted work; and participation on a data safety monitoring or advisory board for Janssen, outside the submitted work. Conflict of interest: M. Humbert reports grants or contracts from Acceleron, AOP Orphan, Janssen, Merck and Shou Ti, outside the submitted work; consulting fees from Acceleron, Aerovate, Altavant, AOP Orphan, Bayer, Chiesi, Ferrer, Janssen, Merck, MorphogenIX, Shou Ti, Tiakis and United Therapeutics, outside the submitted work; payment or honoraria for lectures, presentations, speakers’ bureaus, manuscript writing or educational events from Janssen and Merck, outside the submitted work; and participation on a data safety monitoring or advisory board for Acceleron, Altavant, Janssen, Merck, and United Therapeutics, outside the submitted work. Conflict of interest: O. Sitbon reports grants or contracts from Acceleron (now MSD), AOP Orphan, Janssen (formerly Actelion) and MSD, outside the submitted work; consulting fees from Acceleron (now MSD), Altavant (now Enzyvant), AOP Orphan, Ferrer, Gossamer Bio, Janssen (formerly Actelion) and MSD, outside the submitted work; honoraria for speaking at conferences from AOP Orphan, Janssen (formerly Actelion), Ferrer and MSD, outside the submitted work; and honoraria received for Trial Steering Committee membership (topic: pulmonary hypertension) from Altavant (now Enzyvant), Gossamer Bio and Janssen (formerly Actelion), outside the submitted work. Conflict of interest: D. Montani reports grants or contracts from Acceleron, Janssen and Merck MSD, outside the submitted work; consulting fees from Acceleron, Janssen, Merck MSD and Ferrer, outside the submitted work; and payment or honoraria for speakers’ bureaus from Bayer, Janssen, Boehringer, Chiesi, GSK, Ferrer and Merck MSD, outside the submitted work., (Copyright ©The authors 2024.)

Evidence supporting the use of balloon pulmonary angioplasty #BPA, which has drastically changed management of non-operable chronic thromboembolic pulmonary hypertension #CTEPH, and rationale for a future multimodal approach with combined medical therapy https://bit.ly/3F7ccKD., Competing Interests: Conflict of interest: T. Gille reports personal fees from Roche S.A.S., other fees from Oxyvie (oxygen provider), other fees from Vivisol France (oxygen provider), other fees from Menarini France, outside the submitted work. L. Bertoletti reports grants or contracts from MSD and Janssen, outside the submitted work; support for attending meetings and/or travel from Janssen. The remaining authors have nothing to disclose., (Copyright ©ERS 2023.)

This article aims to summarise the latest research presented at the virtual 2021 European Respiratory Society (ERS) International Congress in the field of pulmonary vascular disease. In light of the current guidelines and proceedings, knowledge gaps are addressed and the newest findings of the various forms of pulmonary hypertension as well as key points on pulmonary embolism are discussed. Despite the comprehensive coverage of the guidelines for pulmonary embolism at previous conferences, discussions about controversies in the diagnosis and treatment of this condition in specific cases were debated and are addressed in the first section of this article. We then report on an interesting pro-con debate about the current classification of pulmonary hypertension. We further report on presentations on Group 3 pulmonary hypertension, with research exploring pathogenesis, phenotyping, diagnosis and treatment; important contributions on the diagnosis of post-capillary pulmonary hypertension are also included. Finally, we summarise the latest evidence presented on pulmonary vascular disease and COVID-19 and a statement on the new imaging guidelines for pulmonary vascular disease from the Fleischner Society., Competing Interests: Conflict of interest: M. Lichtblau has nothing to disclose. Conflict of interest: L. Piccari reports grants, personal fees and nonfinancial support from Janssen, and nonfinancial support from Menarini, outside the submitted work. Conflict of interest: S. Ramjug has nothing to disclose. Conflict of interest: A. Bokan has nothing to disclose. Conflict of interest: B. Lechartier has nothing to disclose. Conflict of interest: E-M. Jutant has nothing to disclose. Conflict of interest: M. Barata has nothing to disclose. Conflict of interest: A.R. Garcia has nothing to disclose. Conflict of interest: L.S. Howard reports grants and personal fees from Janssen, personal fees from MSD, GSK, Bayer, Third Pole, Gossamer Bio, Endotronix and United Therapeutics, outside the submitted work. Conflict of interest: Y. Adir reports personal fees from Teva, grants and personal fees from GSK and AstraZeneca, personal fees from Sanofi, grants and personal fees from Yansen, and personal fees from BI and Acceleron, outside the submitted work. Conflict of interest: M. Delcroix has nothing to disclose. Conflict of interest: L. Jara-Palomares has nothing to disclose. Conflict of interest: L. Bertoletti reports personal fees from Sanofi, personal fees and nonfinancial support from Leo-Pharma and Aspen, personal fees from Bayer, personal fees and nonfinancial support from BMS/Pfizer, and grants, personal fees and nonfinancial support from MSD, during the conduct of the study. Conflict of interest: O. Sitbon reports grants and personal fees from Acceleron Pharmaceuticals, personal fees from AOP Orphan, grants, personal fees and nonfinancial support from Bayer, personal fees from Ferrer and Gossamer Bio, grants from GlaxoSmithKline, and grants, personal fees and nonfinancial support from Janssen and MSD, outside the submitted work. Conflict of interest: S. Ulrich reports grants from Johnson and Johnson SA, the Swiss National Science Foundation, Zurich Lung and Orpha Swiss, and personal fees from MSD Switzerland and SA, outside the submitted work. Conflict of interest: A. Vonk Noordegraaf is supported by the Netherlands CardioVascular Research Initiative (CVON-2012-08 PHAEDRA and CVON-2017-10 DOLPHIN-GENESIS) and the Netherlands Organization for Scientific Research (NWO-VICI: 918.16.610). In addition, his institute has received speaker's money from Johnson & Johnson, MSD, Actelion, Bayer and Ferrer in the past 3 years. Finally, he served as a member of the scientific advisory boards of Morphogen-X, Ferrer and Johnson & Johnson., (Copyright ©The authors 2022.)

Pulmonary arterial hypertension (PAH) is a rare disease characterised by pulmonary vascular remodelling and elevated pulmonary pressure, which eventually leads to right heart failure and death. Registries worldwide have noted a female predominance of the disease, spurring particular interest in hormonal involvement in the disease pathobiology. Several experimental models have shown both protective and deleterious effects of oestrogens, suggesting that complex mechanisms participate in PAH pathogenesis. In fact, oestrogen metabolites as well as receptors and enzymes implicated in oestrogen signalling pathways and associated conditions such as BMPR2 mutation contribute to PAH penetrance more specifically in women. Conversely, females have better right ventricular function, translating to a better prognosis. Along with right ventricular adaptation, women tend to respond to PAH treatment differently from men. As some young women suffer from PAH, contraception is of particular importance, considering that pregnancy in patients with PAH is strongly discouraged due to high risk of death. When contraception measures fail, pregnant women need a multidisciplinary team-based approach. This article aims to review epidemiology, mechanisms underlying the higher female predominance, but better prognosis and the intricacies in management of women affected by PAH., Competing Interests: Conflict of interest: C. Cheron has nothing to disclose. Conflict of interest: S.A. McBride has nothing to disclose. Conflict of interest: F Antigny has nothing to disclose. Conflict of interest: B. Girerd has nothing to disclose. Conflict of interest: M. Chouchana has nothing to disclose. Conflict of interest: M-C. Chaumais has nothing to disclose. Conflict of interest: X. Jaïs reports grants, personal fees and non-financial support from Actelion/Janssen, personal fees and non-financial support from MSD, and grants from Bayer, outside the submitted work. Conflict of interest: L. Bertoletti reports grants, personal fees and non-financial support from Actelion, MSD, Bayer, BMS/Pfizer and Léo-Pharma, outside the submitted work. Conflict of interest: O. Sitbon reports grants, personal fees and non-financial support from Actelion; personal fees from Acceleron, Ferrer and Gossamer Bio; grants and personal fees from Bayer and MSD; and grants from GlaxoSmithKline, outside the submitted work. Conflict of interest: J. Weatherald reports grants, personal fees and non-financial support from Janssen Inc. and Actelion, personal fees and non-financial support from Bayer, and personal fees from Novartis, outside the submitted work. Conflict of interest: M. Humbert reports grants, personal fees and non-financial support from GlaxoSmithKline; personal fees from AstraZeneca, Novartis, Roche, Sanofi, Teva and Merck; grants and personal fees from Acceleron, Actelion and Bayer, outside the submitted work. Conflict of interest: D. Montani reports grants and personal fees from Actelion and Bayer; personal fees from GSK, Pfizer, Chiesi and Boerhinger; grants, personal fees and non-financial support from MSD; and non-financial support from Acceleron, outside the submitted work., (Copyright ©The authors 2021.)

We aimed to identify risk factors for recurrent venous thromboembolism (VTE) after unprovoked pulmonary embolism.Analyses were based on the double-blind randomised PADIS-PE trial, which included 371 patients with a first unprovoked pulmonary embolism initially treated during 6 months who were randomised to receive an additional 18 months of warfarin or placebo and followed up for 2 years after study treatment discontinuation. All patients had ventilation/perfusion lung scan at inclusion ( i.e. at 6 months of anticoagulation).During a median follow-up of 41 months, recurrent VTE occurred in 67 out of 371 patients (6.8 events per 100 person-years). In main multivariate analysis, the hazard ratio for recurrence was 3.65 (95% CI 1.33-9.99) for age 50-65 years, 4.70 (95% CI 1.78-12.40) for age >65 years, 2.06 (95% CI 1.14-3.72) for patients with pulmonary vascular obstruction index (PVOI) ≥5% at 6 months and 2.38 (95% CI 1.15-4.89) for patients with antiphospholipid antibodies. When considering that PVOI at 6 months would not be available in practice, PVOI ≥40% at pulmonary embolism diagnosis (present in 40% of patients) was also associated with a 2-fold increased risk of recurrence.After a first unprovoked pulmonary embolism, age, PVOI at pulmonary embolism diagnosis or after 6 months of anticoagulation and antiphospholipid antibodies were found to be independent predictors for recurrence., Competing Interests: Conflict of interest: Disclosures can be found alongside this article at erj.ersjournals.com, (Copyright ©ERS 2018.)

Treatment for pulmonary arterial hypertension (PAH) has been underpinned by single-agent therapy to which concomitant drugs are added sequentially when pre-defined treatment goals are not met.This retrospective analysis of real-world clinical data in 97 patients with newly diagnosed PAH (86% in New York Heart Association functional class III-IV) explored initial dual oral combination treatment with bosentan plus sildenafil (n=61), bosentan plus tadalafil (n=17), ambrisentan plus tadalafil (n=11) or ambrisentan plus sildenafil (n=8).All regimens were associated with significant improvements in functional class, exercise capacity, dyspnoea and haemodynamic indices after 4 months of therapy. Over a median follow-up period of 30 months, 75 (82%) patients were still alive, 53 (71%) of whom received only dual oral combination therapy. Overall survival rates were 97%, 94% and 83% at 1, 2 and 3 years, respectively, and 96%, 94% and 84%, respectively, for the patients with idiopathic PAH, heritable PAH and anorexigen-induced PAH. Expected survival rates calculated from the French equation for the latter were 86%, 75% and 66% at 1, 2 and 3 years, respectively.Initial combination of oral PAH-targeted medications may offer clinical benefits, especially in PAH patients with severe haemodynamic impairment., (Copyright ©ERS 2016.)

We analysed a cohort of patients with normotensive pulmonary embolism (PE) in order to assess whether combining echocardiography and biomarkers with the pulmonary embolism severity index (PESI) improves the risk stratification in comparison to the PESI alone. The PESI was calculated in normotensive patients with PE who also underwent echocardiography and assays of cardiac troponin I and brain natriuretic peptide. 30-day adverse outcome was defined as death, recurrent PE or shock. 529 patients were included, 25 (4.7%, 95% CI 3.2-6.9%) had at least one outcome event. The proportion of patients with adverse events increased from 2.1% in PESI class I-II to 8.4% in PESI class III-IV, and to 14.3% in PESI class V (p<0.001). In PESI class I-II, the rate of outcome events was significantly higher in patients with abnormal values of biomarkers or right ventricular dilatation. In multivariate analysis, the PESI (class III-IV versus I-II, OR 3.1, 95% CI 1.2-8.3; class V versus I-II, OR 5.5, 95% CI 1.5-25.5 and echocardiography (right ventricular/left ventricular ratio, OR (for an increase of 0.1) 1.3, 95% CI 1.1-1.5) were independent predictors of an adverse outcome. In patients with normotensive PE, biomarkers and echocardiography provided additional prognostic information to the PESI.

Chronic obstructive pulmonary disease (COPD) is a moderate risk factor for venous thromboembolism (VTE), but neither the clinical presentation nor the outcome of VTE in COPD patients is well known. The clinical presentation of VTE, namely pulmonary embolism (PE) or deep venous thrombosis (DVT), and the outcome at 3 months (death, recurrent VTE or bleeding) were compared between 2,984 COPD patients and 25,936 non-COPD patients included in the RIETE (Registro Informatizado de la Enfermedad TromboEmbólica) registry. This ongoing international, multi-centre registry includes patients with proven symptomatic PE or DVT. PE was the more frequent VTE presentation in COPD patients (n = 1,761, 59%). PE presentation was more significantly associated with COPD patients than non-COPD patients (OR 1.64, 95% CI 1.49-1.80). During the 3-month follow-up, mortality (10.8% versus 7.6%), minor bleeding (4.5% versus 2.3%) or first VTE recurrences as PE (1.5% versus 1.1%) were significantly higher in COPD patients than in non-COPD patients. PE was the most common cause of death. COPD patients presented more frequently with PE than DVT. It may explain the worse prognosis of COPD patients, with a higher risk of death, bleeding or VTE recurrences as PE compared with non-COPD patients. Further therapeutic options are needed.

Sleep-related disorders represent an important health burden and their prevalence increases with age. In patients with snoring or sleepiness, the presence of expiratory flow limitation (EFL), determined via the negative expiratory pressure (NEP) method, is related to the apnoea/hypopnoea index (AHI). In this study, we examined whether EFL can be used to predict obstructive sleep apnoea syndrome (OSAS) in healthy asymptomatic older subjects. A group of 72-yr-old subjects (n = 448, 44% males) with a mean body mass index of 25.5±3.8 kg·m(-2) were examined. All subjects underwent spirometry, NEP (-5 cmH(2)O, sitting position) and ventilatory polygraphy (VP). Spirometry was within normal values in 88% of the group and EFL was present in 143 (32%) subjects with a higher prevalence in females (89 out of 249 versus 54 out of 199 in females and males, respectively). VP showed an AHI<15 h(-1) in 238 subjects (53%) and OSAS with an AHI ≥15 h(-1) in 47%. EFL was found in 15% of subjects with OSAS. Consequently, EFL had low sensitivity and specificity in the prediction of OSAS (31.4% and 67.7%, respectively). We conclude that the prevalence of EFL is elevated in healthy older subjects and cannot be used to predict the presence of sleep-related disorders in an older population.

Acute right ventricular failure in the setting of pulmonary arterial hypertension (PAH) often requires hospitalisation in intensive care units (ICU) to manage the subsequent low cardiac output and its consequences. There are very few data on these acute events. We recorded demographic, clinical and biological data and therapy in consecutive patients suffering from acute right heart failure requiring catecholamine treatment in the ICU of the French referral centre for pulmonary hypertension. These variables were analysed according to the survival status in ICU. 46 patients were included, the mean age was 50.3 yrs. ICU mortality was 41%. We found no difference in terms of demographics, clinical data, last haemodynamic measurements at admission. Systemic arterial pressure was significantly lower in the subgroup of patients whose clinical course was fatal. Plasma brain natriuretic peptide (BNP), C-reactive protein (CRP), serum sodium and creatinine at admission correlated with survival. Demonstration of an infection during the ICU stay was associated with a worse prognosis. These preliminary results underline the importance of some simple clinical and biological parameters in the prognostic evaluation of acute heart failure in the setting of PAH. Whether these parameters can guide therapy needs to be further investigated.