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1. Direct observation of the complex S(IV) equilibria at the liquid-vapor interface

Author

Tillmann Buttersack, Ivan Gladich, Shirin Gholami, Clemens Richter, Rémi Dupuy, Christophe Nicolas, Florian Trinter, Annette Trunschke, Daniel Delgado, Pablo Corral Arroyo, Evelyne A. Parmentier, Bernd Winter, Lucia Iezzi, Antoine Roose, Anthony Boucly, Luca Artiglia, Markus Ammann, Ruth Signorell, and Hendrik Bluhm

Subjects
Science
Abstract

Abstract The multi-phase oxidation of S(IV) plays a crucial role in the atmosphere, leading to the formation of haze and severe pollution episodes. We here contribute to its understanding on a molecular level by reporting experimentally determined pK a values of the various S(IV) tautomers and reaction barriers for SO2 formation pathways. Complementary state-of-the-art molecular-dynamics simulations reveal a depletion of bisulfite at low pH at the liquid-vapor interface, resulting in a different tautomer ratio at the interface compared to the bulk. On a molecular-scale level, we explain this with the formation of a stable contact ion pair between sulfonate and hydronium ions, and with the higher energetic barrier for the dehydration of sulfonic acid at the liquid-vapor interface. Our findings highlight the contrasting physicochemical behavior of interfacial versus bulk environments, where the pH dependence of the tautomer ratio reported here has a significant impact on both SO2 uptake kinetics and reactions involving NO x and H2O2 at aqueous aerosol interfaces.

Published
2024
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4. Evidence and unresolved questions in pulmonary hypertension: Insights from the 5th French Pulmonary Hypertension Network Meeting

Author

Boucly, Athénaïs, Bertoletti, Laurent, Fauvel, Charles, Dewavrin, Mélanie Gallant, Gerges, Christian, Grynblat, Julien, Guignabert, Christophe, Hascoet, Sébastien, Jaïs, Xavier, Jutant, Etienne-Marie, Lamblin, Nicolas, Meyrignac, Olivier, Riou, Marianne, Savale, Laurent, Tromeur, Cécile, Turquier, Ségolène, Valentin, Simon, Simonneau, Gérald, Humbert, Marc, Sitbon, Olivier, and Montani, David

Published
2024
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5. Immunovirological status in people with perinatal and adult-acquired HIV-1 infection: a multi-cohort analysis from FranceResearch in context

Author

Rémonie Seng, Pierre Frange, Albert Faye, Catherine Dollfus, Jérôme le Chenadec, Faroudy Boufassa, Asma Essat, Tessa Goetghebuer, Elisa Arezes, Véronique Avettand-Fènoël, Jean-Joël Bigna, Stéphane Blanche, Cécile Goujard, Laurence Meyer, Josiane Warszawski, Jean-Paul Viard, H. Aumaitre, E. Froguel, F. Caby, S. Dellion, L. Gerard, F. Lucht, C. Chirouze, M. Dupon, Jl Schmit, C. Goujard, T. Allegre, B. Cazenave, G. Hittinger, P. De Truchis, J. Cailhol, C. Duvivier, A. Canestri, O. Bouchaud, M. Karmochkine, D. Salmon-Ceron, D. Zucman, E. Mortier, R. Tubiana, P.M. Girard, C. Pintado, A. Cabie, V. Rabier, P. Morlat, D. Neau, C. Genet, D. Makhloufi, S Bregigeon Ronot, J. Ghosn, V. Reliquet, P. Perré, Jl Pellegrin, C. Arvieux, C. Cheneau, L. Bernard, P. Delobel, R. Verdon, C. Jacomet, L. Piroth, F. Ajana, S. Bevilacqua, Y. Debab, A.L. Lecapitaine, L. Cotte, S. Mokhtari, P. Mercie, P. Poubeau, V. Garrait, Ma Khuong, G. Beck-Wirth, L. Blum, S. Blanche, F. Boccara, T. Prazuck, C. Barbuat, J.P. Viard, S. Stegmann-Planchard, B. Martha, J.M. Treluyer, E. Dore, C. Gaud, M. Niault, E. Fernandes, H. Hitoto, A. Compagnucci, N. Elenga, A. Faye, C. Dollfus, A. Chace, M. Levine, S.A. Martha, C. Floch-Tudal, K. Kebaïli, N. Entz-Werle, J. Tricoire, F. Mazingue, P. Bolot, P. Brazille, T. Goetghebuer, A.F. Gennotte, D. Van Der Linden, V. Schmitz, M. Moutschen, C. Crenn-Hebert, F. Habibi, A. Coursol, E. Guesdon, P.F. Ceccaldi, M. Dehlinger – Paul, E. Pannier, V. Marcou, C. Elleau, M. Achkar, M.O. Vareil, S. Couderc, C. Routier, M.A. Bouldouyre, L. Selleret, A. Chabrol, C. Bellahcene, C. Pluchart, A. Yangui, D. Vignes, A. Alissa, A. Johnson, E. Lachassinne, A. Benbara, L. Karaoui, A. Bongain, B. Yakeu, J.L. Schmit, L. Cravello, C. Hubert, P. Faucher, D. Pinquier, C. Borie, D. Rocchi, C. Brunet-Cartier, C. Briandet, J. Brouard, A. Chalvon-Demersay, M. Rajguru, K. Billiemaz, A. Fresard, A. Moulin, P. Fialaire, L. Mesnard, E. Werner, E. Vintejoux, J. Marian, S. Ranaivojaona, F. Bissuel, M. Abdelhadi, Y. Hammou, C. Genet-Villeger, Y. Hatchuel, G. Bachelard, M. Medus, J. Dendale – Nguyen, T.S. Guimard, A. Martha, M. Rouha, P. Perfezou, L. De Saint Martin, S. Jaffuel, R. Buzele, M. Gousseff, C. Cudeville, V. Vitrat, C. Michau, G. Palenzuela, M. Driessen, B. Heller-Roussin, J.M. Labaune, B. Muanza, J. Massardier, M. Partisani, I. Hau, C. Runel-Belliard, C. Brehin, K. Kebaili, M. Lalande, M. Lagree, K. Lacombe, J.-M. Molina, J. Reynes, O. Robineau, F. Raffi, A. Becker, L. Weiss, T. Allègre, G. Pialoux, F. Souala, A. Rami, C. Katlama, A. Cabié, J.-P. Viard, F. Bastides, C. Michel, D. Salmon, J-D Le Lièvre, A. Sotto, E. Rouveix, A. Naqvi, S. Brégigeon, R. Rodet, A. Simon-Coutelier, J.-L. Esnault, R. Buzelé, A. Stein, C. Godin-Colet, G. Pichancourt, P. Caraux-Paz, M Mohseni Zadeh, L. Gérard, C. Lascaux-Cametz, L. Bodard, J.-L. Pellegrin, N. Ettahar, A. Uludag, E. Rosenthal, F. Prevoteau du Clary, S. Jaureguiberry, P. Philibert, A.-L. Lecapitaine, E. Chakvetadze, H. Champagne, V. Daneluzzi, J. Goupil de Bouillé, A. Leprêtre, I. Lamaury, I. Darasteanu, B. Abraham, D. Garipuy, J.-L. Berger, J.-L. Schmit, K. Diallo, F. Gourdon, O. Vaillant, V. Gaborieau, J. Doll, D. Quinsat, L. Geffray, J.-J. Girard, D. Houlbert, V. Perronne, E. Klement, O. Antioniotti, C. Rouzioux, V. Avettand-Fenoel, O. Lortholary, S. Boucly, A. Maignan, R. Thiebaut, L. Meyer, F. Boufassa, M.A. Charles, R. Dray-Spira, C. Legeai, V. Amon, N. Benammar, R. Seng, L. Slama, P. Bonnard, C. Chakvetadze, T. L’Yavanc, J. Capeau, C. Vigouroux, S. Fellahi, J.P. Bastard, E. Oksenhendler, J.F. Bourge, V. Bajzik, D. Sereni, C. Lascoux-Combe, O. Taulera, L.V. Dien, J. Delgado, J.M. Molina, T. Saint-Marc, S. Ferret, J. Pavie, J.F. Bergmann, M. Parrinello, BLefebvre, C. Boudraa, B. Diallo, C. Lupin, S. Herson, A. Simon, N. Edeb, L. Guillevin, T. Tahi, M.P. Pietri, D. Tisne-Dessus, C. Jalbert, P. Yeni, S. Matheron, G. Pahlavan, B. Phung, N. El-Alami Talbi, Z. Ramani, G. Catalano, C. Godard, F. Boue, V. Chambrin, D. Bornarel, H. Schoen, R. Carlier, B. Fantin, C. Poder, R. Dhote, M. Bentata, P. Honore, Xuan Tuyet, J.F. Delfraissy, F. Chaix, M.T. Rannou, Y. Levy, A. Sobel, C. Dumont, S. Abel, S. Pierre-François, V. Beaujolais, I. Poizot-Martin, O. Zaegel-Faucher, C. Debreux, J. Moreau, E. Van Der Gheynst, M.C. Thiebaut-Drobacheff, A. Foltzer, B. Hoen, J.F. Faucher, H. Gil, J.M. Ragnaud, I. Raymond, I. Louis, M. Hessamfar, V. Baillat, C Merle De Boever, C. Tramoni, A. Soufflet, P. Guadagnin, P. Choutet, O. Mounoury, D. Brosseau, H. Hue, T. May, S. Wassoumbou, M. Stenzel, M.P. Bouillon, Y. Yazdanpanah, T. Huleux, E. Aissi, S. Pavel, D. Rey, P. Fischer, G. Blaison, M. Martinot, A. Pachart, F. Jeanblanc, J.L. Touraine, C. Trepo, P. Miailhes, K. Kouadjo, V. Thoirain, C. Brochier, P. Perre, S. Leautez, J.L. Esnault, and I. Suaud

Subjects
Perinatal HIV infection, Cohort, Viral failure, Immunological outcome, Epidemiology, Public aspects of medicine, RA1-1270
Abstract

Summary: Background: No study has compared the virological and immunological status of young people with perinatally-acquired HIV infection (P-HIV) with that of people with HIV adulthood (A-HIV) having a similar duration of infection. Methods: 5 French cohorts of P-HIV and A-HIV patients with a known date of HIV-infection and receiving antiretroviral treatment (ART), were used to compare the following proportions of: virological failure (VF) defined as plasma HIV RNA ≥ 50 copies/mL, CD4 cell percentages and CD4:CD8 ratios, at the time of the most recent visit since 2012. The analysis was stratified on time since infection, and multivariate models were adjusted for demographics and treatment history. Findings: 310 P-HIV were compared to 1515 A-HIV (median current ages 20.9 [IQR:14.4–25.5] and 45.9 [IQR:37.9–53.5] respectively). VF at the time of the most recent evaluation was significantly higher among P-HIV (22.6%, 69/306) than A-HIV (3.3%, 50/1514); p ≤ 0.0001. The risk of VF was particularly high among the youngest children (2–5 years), adolescents (13–17 years) and young adults (18–24 years), compared to A-HIV with a similar duration of infection: adjusted Odds-Ratio (aOR) 7.0 [95% CI: 1.7; 30.0], 11.4 [4.2; 31.2] and 3.3 [1.0; 10.8] respectively. The level of CD4 cell percentages did not differ between P-HIV and A-HIV. P-HIV aged 6–12 and 13–17 were more likely than A-HIV to have a CD4:CD8 ratio ≥ 1: 84.1% vs. 58.8% (aOR = 3.5 [1.5; 8.3]), and 60.9% vs. 54.7% (aOR = 1.9 [0.9; 4.2]) respectively. Interpretation: P-HIV were at a higher risk of VF than A-HIV with a similar duration of infection, even after adjusting for treatment history, whereas they were not at a higher risk of immunological impairment. Exposure to viral replication among young patients living with HIV since birth or a very early age, probably because of lower adherence, could have an impact on health, raising major concerns about the selection of resistance mutations and the risk of HIV transmission. Funding: Inserm - ANRS MIE.

Published
2024
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6. Immunovirological status in people with perinatal and adult-acquired HIV-1 infection: a multi-cohort analysis from France

Author

Aumaitre, H., Froguel, E., Caby, F., Dellion, S., Gerard, L., Lucht, F., Chirouze, C., Dupon, M., Schmit, Jl, Goujard, C., Allegre, T., Cazenave, B., Hittinger, G., De Truchis, P., Cailhol, J., Duvivier, C., Canestri, A., Bouchaud, O., Karmochkine, M., Salmon-Ceron, D., Zucman, D., Mortier, E., Tubiana, R., Girard, P.M., Pintado, C., Cabie, A., Rabier, V., Morlat, P., Neau, D., Genet, C., Makhloufi, D., Ronot, S Bregigeon, Ghosn, J., Reliquet, V., Perré, P., Pellegrin, Jl, Arvieux, C., Cheneau, C., Bernard, L., Delobel, P., Verdon, R., Jacomet, C., Piroth, L., Ajana, F., Bevilacqua, S., Debab, Y., Lecapitaine, A.L., Cotte, L., Mokhtari, S., Mercie, P., Poubeau, P., Garrait, V., Khuong, Ma, Beck-Wirth, G., Blum, L., Blanche, S., Boccara, F., Prazuck, T., Barbuat, C., Viard, J.P., Stegmann-Planchard, S., Martha, B., Treluyer, J.M., Dore, E., Gaud, C., Niault, M., Fernandes, E., Hitoto, H., Compagnucci, A., Elenga, N., Faye, A., Dollfus, C., Chace, A., Levine, M., Martha, S.A., Floch-Tudal, C., Kebaïli, K., Entz-Werle, N., Tricoire, J., Mazingue, F., Bolot, P., Brazille, P., Goetghebuer, T., Gennotte, A.F., Van Der Linden, D., Schmitz, V., Moutschen, M., Crenn-Hebert, C., Habibi, F., Coursol, A., Guesdon, E., Ceccaldi, P.F., Dehlinger – Paul, M., Pannier, E., Marcou, V., Elleau, C., Achkar, M., Vareil, M.O., Couderc, S., Routier, C., Bouldouyre, M.A., Selleret, L., Chabrol, A., Bellahcene, C., Pluchart, C., Yangui, A., Vignes, D., Alissa, A., Johnson, A., Lachassinne, E., Benbara, A., Karaoui, L., Bongain, A., Yakeu, B., Schmit, J.L., Cravello, L., Hubert, C., Faucher, P., Pinquier, D., Borie, C., Rocchi, D., Brunet-Cartier, C., Briandet, C., Brouard, J., Chalvon-Demersay, A., Rajguru, M., Billiemaz, K., Fresard, A., Moulin, A., Fialaire, P., Mesnard, L., Werner, E., Vintejoux, E., Marian, J., Ranaivojaona, S., Bissuel, F., Abdelhadi, M., Hammou, Y., Genet-Villeger, C., Hatchuel, Y., Bachelard, G., Medus, M., Dendale – Nguyen, J., Guimard, T.S., Martha, A., Rouha, M., Perfezou, P., De Saint Martin, L., Jaffuel, S., Buzele, R., Gousseff, M., Cudeville, C., Vitrat, V., Michau, C., Palenzuela, G., Driessen, M., Heller-Roussin, B., Labaune, J.M., Muanza, B., Massardier, J., Partisani, M., Hau, I., Runel-Belliard, C., Brehin, C., Kebaili, K., Lalande, M., Lagree, M., Lacombe, K., Molina, J.-M., Reynes, J., Robineau, O., Raffi, F., Becker, A., Weiss, L., Allègre, T., Pialoux, G., Souala, F., Rami, A., Katlama, C., Cabié, A., Viard, J.-P., Bastides, F., Michel, C., Salmon, D., Lièvre, J-D Le, Sotto, A., Rouveix, E., Naqvi, A., Brégigeon, S., Rodet, R., Simon-Coutelier, A., Esnault, J.-L., Buzelé, R., Stein, A., Godin-Colet, C., Pichancourt, G., Caraux-Paz, P., Zadeh, M Mohseni, Gérard, L., Lascaux-Cametz, C., Bodard, L., Pellegrin, J.-L., Ettahar, N., Uludag, A., Rosenthal, E., Prevoteau du Clary, F., Jaureguiberry, S., Philibert, P., Lecapitaine, A.-L., Chakvetadze, E., Champagne, H., Daneluzzi, V., Goupil de Bouillé, J., Leprêtre, A., Lamaury, I., Darasteanu, I., Abraham, B., Garipuy, D., Berger, J.-L., Schmit, J.-L., Diallo, K., Gourdon, F., Vaillant, O., Gaborieau, V., Doll, J., Quinsat, D., Geffray, L., Girard, J.-J., Houlbert, D., Perronne, V., Klement, E., Antioniotti, O., Rouzioux, C., Avettand-Fenoel, V., Lortholary, O., Boucly, S., Maignan, A., Thiebaut, R., Meyer, L., Boufassa, F., Charles, M.A., Dray-Spira, R., Legeai, C., Amon, V., Benammar, N., Seng, R., Slama, L., Bonnard, P., Chakvetadze, C., L’Yavanc, T., Capeau, J., Vigouroux, C., Fellahi, S., Bastard, J.P., Oksenhendler, E., Bourge, J.F., Bajzik, V., Sereni, D., Lascoux-Combe, C., Taulera, O., Dien, L.V., Delgado, J., Molina, J.M., Saint-Marc, T., Ferret, S., Pavie, J., Bergmann, J.F., Parrinello, M., BLefebvre, Boudraa, C., Diallo, B., Lupin, C., Herson, S., Simon, A., Edeb, N., Guillevin, L., Tahi, T., Pietri, M.P., Tisne-Dessus, D., Jalbert, C., Yeni, P., Matheron, S., Pahlavan, G., Phung, B., El-Alami Talbi, N., Ramani, Z., Catalano, G., Godard, C., Boue, F., Chambrin, V., Bornarel, D., Schoen, H., Carlier, R., Fantin, B., Poder, C., Dhote, R., Bentata, M., Honore, P., Tuyet, Xuan, Delfraissy, J.F., Chaix, F., Rannou, M.T., Levy, Y., Sobel, A., Dumont, C., Abel, S., Pierre-François, S., Beaujolais, V., Poizot-Martin, I., Zaegel-Faucher, O., Debreux, C., Moreau, J., Van Der Gheynst, E., Thiebaut-Drobacheff, M.C., Foltzer, A., Hoen, B., Faucher, J.F., Gil, H., Ragnaud, J.M., Raymond, I., Louis, I., Hessamfar, M., Baillat, V., De Boever, C Merle, Tramoni, C., Soufflet, A., Guadagnin, P., Choutet, P., Mounoury, O., Brosseau, D., Hue, H., May, T., Wassoumbou, S., Stenzel, M., Bouillon, M.P., Yazdanpanah, Y., Huleux, T., Aissi, E., Pavel, S., Rey, D., Fischer, P., Blaison, G., Martinot, M., Pachart, A., Jeanblanc, F., Touraine, J.L., Trepo, C., Miailhes, P., Kouadjo, K., Thoirain, V., Brochier, C., Perre, P., Leautez, S., Esnault, J.L., Suaud, I., Seng, Rémonie, Frange, Pierre, Faye, Albert, Dollfus, Catherine, le Chenadec, Jérôme, Boufassa, Faroudy, Essat, Asma, Goetghebuer, Tessa, Arezes, Elisa, Avettand-Fènoël, Véronique, Bigna, Jean-Joël, Blanche, Stéphane, Goujard, Cécile, Meyer, Laurence, Warszawski, Josiane, and Viard, Jean-Paul

Published
2024
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8. ERS International Congress 2023: highlights from the Pulmonary Vascular Diseases Assembly

Author

Sarah Cullivan, Athénaïs Boucly, Mitja Jevnikar, Benoit Lechartier, Silvia Ulrich, Laurent Bertoletti, Olivier Sitbon, Anton Vonk-Noordegraaf, Aleksandar Bokan, Da-Hee Park, Leon Genecand, Julien Guiot, Etienne-Marie Jutant, Lucilla Piccari, and Mona Lichtblau

Subjects
Medicine
Abstract

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.

Published
2024
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9. Pulmonary veno-occlusive disease: illustrative cases and literature review

Author

Benoit Lechartier, Athénaïs Boucly, Sabina Solinas, Deepa Gopalan, Peter Dorfmüller, Teodora Radonic, Olivier Sitbon, and David Montani

Subjects
Diseases of the respiratory system, RC705-779
Abstract

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 EIF2AK4 gene in heritable forms of disease. Distinguishing PVOD from PAH is critical for guiding appropriate management. Chest computed tomography typically displays interlobular septal thickening, ground-glass opacities and mediastinal lymphadenopathy. Life-threatening pulmonary oedema is a complication of pulmonary vasodilator therapy that can occur with any class of PAH drugs in PVOD. Early referral to a lung transplant centre is essential due to the poor response to therapy when compared with other forms of PAH. Histopathological analysis of lung explants reveals microvascular remodelling with typical fibrous veno-occlusive lesions. This review covers the main features distinguishing PVOD from PAH and two clinical cases that illustrate the challenges of PVOD management.

Published
2024
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10. Outcomes and risk assessment in pulmonary veno-occlusive disease

Author

Athénaïs Boucly, Sabina Solinas, Antoine Beurnier, Xavier Jaïs, Sophia Keddache, Mélanie Eyries, Andrei Seferian, Mitja Jevnikar, Anne Roche, Sophie Bulifon, Arnaud Bourdin, Ari Chaouat, Vincent Cottin, Laurent Bertoletti, Laurent Savale, Marc Humbert, Olivier Sitbon, and David Montani

Subjects
Medicine
Abstract

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.

Published
2024
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13. Real‐world evidence to advance knowledge in pulmonary hypertension: Status, challenges, and opportunities. A consensus statement from the Pulmonary Vascular Research Institute's Innovative Drug Development Initiative's Real‐world Evidence Working Group

Author

Kellie Morland, Christian Gerges, Jean Elwing, Scott H. Visovatti, Jason Weatherald, Kari R. Gillmeyer, Sandeep Sahay, Stephen C. Mathai, Athénaïs Boucly, Paul G. Williams, Sivadasanpillai Harikrishnan, Evan P. Minty, Lukas Hobohm, Arun Jose, Roberto Badagliacca, Edmund M. T. Lau, Zhi‐Cheng Jing, Rebecca R. Vanderpool, Charles Fauvel, Jose Leonidas Alves Jr., Geoff Strange, Tomas Pulido, Junyan Qian, Mengtao Li, Valentina Mercurio, Jason G. E. Zelt, Victor M. Moles, Meghan M. Cirulis, Sylvia M. Nikkho, Raymond L. Benza, and C. Gregory Elliott

Subjects
administrative claims data, electronic health records, patient‐generated health data, real‐world data, registries, Diseases of the circulatory (Cardiovascular) system, RC666-701, Diseases of the respiratory system, RC705-779
Abstract

Abstract This manuscript on real‐world evidence (RWE) in pulmonary hypertension (PH) incorporates the broad experience of members of the Pulmonary Vascular Research Institute's Innovative Drug Development Initiative Real‐World Evidence Working Group. We aim to strengthen the research community's understanding of RWE in PH to facilitate clinical research advances and ultimately improve patient care. Herein, we review real‐world data (RWD) sources, discuss challenges and opportunities when using RWD sources to study PH populations, and identify resources needed to support the generation of meaningful RWE for the global PH community.

Published
2023
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14. Effect of weight-adjusted intermediate-dose versus fixed-dose prophylactic anticoagulation with low-molecular-weight heparin on venous thromboembolism among noncritically and critically ill patients with COVID-19: the COVI-DOSE trial, a multicenter, randomised, open-label, phase 4 trial

Author

Zuily, Stéphane, Couturaud, Francis, Goehringer, François, Laporte, Silvy, Mismetti, Patrick, Presles, Emilie, Rossignol, Patrick, Thilly, Nathalie, Wahl, Denis, Lecompte, Thomas, Oger, Emmanuel, Sevestre, Marie-Antoinette, Parent, Florence, Benhamou, Ygal, Roy, Pierre-Marie, Gigante, Tristan, Gilg, Morgane, Maclot, Pierre-Luc, Rossignol, Bénédicte, Udot, Jonathan, Bouteille, Hélène, Buchmuller, Andréa, Peurière, Céline, Siaghy, El Mehdi, Bouaziz, Valérie, Daguin, Charlotte, Grosjean, Benjamin, Saunier, Véronique, Verger, Laurence, Jacquot, Madlyne, Petitpain, Nadine, Charly, Martin, Tordella, Laurent, Baronnet, Guillaume, Baux, Elisabeth, Benetos, Athanase, Blanvillain, Jean-Baptiste, Conrad, Marie, Courte, Guilhem, Cravoisy-Popovic, Aurélie, Dufrost, Virginie, Gibot, Sébastien, Guerci, Philippe, Joly, Laure, Kimmoun, Antoine, Koszutski, Matthieu, Larue, Alexandrine, Levy, Bruno, Losser, Marie-Reine, Mattei, Mathieu, Nace, Lionel, Novy, Emmanuel, Perez, Pierre, Pertek, Jean-Pierre, Rigaux, Camille, Sauvage, Alexis, Schvoerer, Evelyne, Thivilier, Carine, Volkov, Lev, Zieminski, Piotr, Benlounes, Manil, Cheng, Charles, Fréminville, Jean-Baptiste de, Détriché, Grégoire, Flammarion, Emmanuel, Goudot, Guillaume, Hamdan, Amer, Hindré, Raphaël, Manoli, Corina, Messas, Emmanuel, Michon, Adrien, Mirault, Tristan, Pastré, Jean, Penet, Marie-Aude, Planquette, Benjamin, Volle, Geoffroy, Hamdan, Rémy, Laubriet-Jazayeri, Aline, Petit, Vincent, Piroth, Lionel, Quenot, Jean Pierre, Saccu, Mélissa, Barraud, Damien, Cavalli, Zoé, Gaci, Rostane, Andreu, Mathilde, Bertoletti, Laurent, De Magalhaes, Elodie, Bulifon, Sophie, Boucly, Athénaïs, Ebstein, Nathan, Humbert, Marc, Jaïs, Xavier, Jevnikar, Mitja, Savale, Laurent, Seferian, Andrei Horia, Colin, Charlotte, Ganem, Timothée, Roumila, Mehdi, Chopard, Romain, Besutti, Matthieu, Mouhat, Basile, Andrejak, Claire, Dupas, Stéphane, Le Roy, Gaëlle, Samy-Modeliar, Santhi, Coste, Anne, Fauche, Alexandre, Goetghebeur, David, Gut-Gobert, Christophe, Hoffmann, Clément, Hourmant, Baptiste, L'hévéder, Cécile, Lemoigne, Emmanuelle, Nepveu, Olivier, Paret, Raphaël, Picart, Gaël, Azerkan, Saïd, Boudaa, Chadia, Campagne, Julien, Eszto, Peter, Godbert, Benoît, Guichard, Jean-François, Heschung, Marion, Legoff, Antoine, Mariot, Jacques, Martin, Pascale, Mercy, Magalie, Perrin, Julie, Raymond, Stéphane, Vernier, Nathalie, Fesler, Pierre, Henneton, Pierrick, Mercuzot, Cédric, Pansu, Nathalie, Perez, Lucas, Andre, Loïc, Bakhache, Edgar, Chopin, Marie-Charlotte, Gilbert, Marie, Lambert, Marc, Pokeerbux, Mohammad Ryadh, Danion, François, Hansmann, Yves, Rougier, Estelle, Ruch, Yvon, Stéphan, Dominique, Ursenbach, Axel, Connerade, Isabelle, Gravier, Simon, Kayser, Damien, Michel, Jean-Marc, Mohseni, Mahsa, Younes, Waël, Burlacu, Ruxandra, Lopes, Amanda, Mouly, Stéphane, Nassarmadji, Kladoum, Sène, Damien, Siguret, Virginie, Stepanian, Alain, Annweiler, Cédric, Brangier, Antoine, Dubee, Vincent, Henni, Samir, Hersant, Jeanne, Loison, Jocelyne, Kern, Léa, Laine, Jean-Baptiste, Neveux-Brecheteau, Claire, Perez, Lucia, Benainous, Ruben, Giroux-Leprieur, Bénédicte, Lopez-Sublet, Marilucy, Khaled-Jousselin, Saïda, Bernard, Yohann, Amiot, Amélie, Breistroff, Jessica, Detry, Emilie, Diallo, Kadidiatou, Didier, Agnès, Dumont, Nathalie, Egensperger, Julie, Emmerich, Aurélie, François, Nelly, Gallo, Fanny, George, Valérie, Gérome, Quentin, Gutehrle, Aurélie, Lehman, Laure, Petit, Séverine, Piard, Vanessa, Saint-Gilles, Maximilien, Terenzi, Olivier, Marquette, Amélie, Mortelette, Hélène, Audry, Mathilde, Cransac, Amélie, Maillard, Marine, Boyer, Anaïs, Gallo, Floriana, Urbing, Arielle, Zahaf, Imane, Byczko, Alexandra, Chaalal, Amina, Berlier, Georgette, Bernabe, Corinne, Bezzeghoud, Souad, Chaudier, Caroline, Chauvet, Carole, Davier, Marina, Labruyere, Carine, Perrin, Estelle, Pierre, Michaël, Chekirine, Claire-Annissa, Voivret, Florence, Meftali, Ramdane, Sabri, Ouaffa, Beulaygue, Anaïs, Gall, Julie, Girard, Laure, Haddaoui, Soumia, Rami, Scheherazade, Couderc, Auriane, Le Breton, Aude, Perruche, Marie-Line, Claudon, Cindy, Roussel, Ludivine, Barnier, Aude, Blanchard, Tiphaine, Le Gall, Bénédicte, Pelouin, Mélanie, Veillon, Anne-Sophie, Aquereburu, Quam, Delaygue, Charlène, Ait Idir, Zahoua, Drugeon, Jérémy, Dubrulle, Déborah, Tezkratt, Rabah, Frantz, Anne-Sophie, Drouaine, Julie, Dubois, Jacqueline, Eyriey, Magali, Haerrel, Elina, Beaudenon, Mélinda, Guenet, Mialy, Lecerf, Thibaud, Marechal-Girault, Stéphanie, Rehaiem, Sami, Simon, Romain, Dangeul-Potier, Florence, Goulvent, Morgane, Fliss, Souha, Messani, Fadhila, Mizejewski, Béatrice, Mugnier, Brigitte, Opderbeck, Valérie, Risse, Brigitte, Lefèvre, Benjamin, Sanchez, Olivier, Empis de Vendin, Ombeline, de Ciancio, Guillaume, Arlet, Jean-Benoît, Khider, Lina, Terriat, Béatrice, Greigert, Hélène, Robert, Céline S., Louis, Guillaume, Trinh-Duc, Albert, Rispal, Patrick, Accassat, Sandrine, Thiery, Guillaume, Montani, David, Azarian, Réza, Meneveau, Nicolas, Soudet, Simon, Le Mao, Raphaël, Maurier, François, Le Moing, Vincent, Quéré, Isabelle, Yelnik, Cécile M., Lefebvre, Nicolas, Martinot, Martin, and Delrue, Maxime

Published
2023
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17. Serum and Pulmonary Expression Profiles of the Activin Signaling System in Pulmonary Arterial Hypertension

Author

Guignabert, Christophe, Savale, Laurent, Boucly, Athénaïs, Thuillet, Raphaël, Tu, Ly, Ottaviani, Mina, Rhodes, Christopher J., De Groote, Pascal, Prévot, Grégoire, Bergot, Emmanuel, Bourdin, Arnaud, Howard, Luke S., Fadel, Elie, Beurnier, Antoine, Roche, Anne, Jevnikar, Mitja, Jaïs, Xavier, Montani, David, Wilkins, Martin R., Sitbon, Olivier, and Humbert, Marc

Published
2023
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18. Functional respiratory complaints among COVID-19 survivors: a prospective cohort study

Author

Antoine Beurnier, Laurent Savale, Xavier Jaïs, Romain Colle, Tai Pham, Luc Morin, Sophie Bulifon, Nicolas Noël, Athénaïs Boucly, Benoit Delbarre, Nathan Ebstein, Samy Figueiredo, Matthieu Gasnier, Anatole Harrois, Etienne-Marie Jutant, Mitja Jevnikar, Sophia Keddache, Anne-Lise Lecoq, Olivier Meyrignac, Florence Parent, Jérémie Pichon, Mariana Preda, Anne Roche, Andrei Seferian, Marie-France Bellin, Thomas Gille, Emmanuelle Corruble, Olivier Sitbon, Laurent Becquemont, Xavier Monnet, Marc Humbert, David Montani, and and the Comebac Investigators

Subjects
Medicine
Abstract

Background Dyspnoea is a common persistent symptom after COVID-19. Whether it is associated with functional respiratory disorders remains unclear. Methods We assessed the proportion and characteristics of patients with “functional respiratory complaints” (FRCs) (as defined by Nijmegen Questionnaire >22) among 177 post-COVID-19 individuals who benefited from outclinic evaluation in the COMEBAC study (i.e., symptomatic and/or intensive care unit (ICU) survivors at 4 months). In a distinct explanatory cohort of 21 consecutive individuals with unexplained post-COVID-19 dyspnoea after routine tests, we also analysed the physiological responses to incremental cardiopulmonary exercise testing (CPET). Findings In the COMEBAC cohort, 37 patients had significant FRCs (20.9%, IC95: 14.9–26.9). The prevalence of FRCs ranged from 7.2% (ICU patients) to 37.5% (non-ICU patients). The presence of FRCs was significantly associated with more severe dyspnoea, lower 6-min walk distance, more frequent psychological and neurological symptoms (cognitive complaint, anxiety, depression, insomnia and post-traumatic stress disorders) and poorer quality of life (all p

Published
2023
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19. Germanium surface segregation in highly doped Ge:β-Ga2O3 grown by molecular beam epitaxy observed by synchrotron radiation hard x-ray photoelectron spectroscopy.

Author

Boucly, Anthony, Back, Tyson C., Asel, Thaddeus J., Noesges, Brenton A., Evans, Prescott E., Weiland, Conan, and Barrett, Nick

Subjects
*X-ray photoelectron spectroscopy, *MOLECULAR beam epitaxy, *PHOTOELECTRON spectroscopy, *SYNCHROTRON radiation, *SURFACE segregation
Abstract

We present a study of Ge segregation at the surface of highly germanium-doped gallium oxide (2.5 × 1020 cm−3 nominal doping level) grown by molecular beam epitaxy. We probed the dopant concentration as a function of depth by hard x-ray photoelectron spectroscopy and standard laboratory photoemission spectroscopy. We notably found that there is germanium segregation within the top 2 nm where its concentration is 3 times the nominal doping level. This increased dopant concentration leads to a threefold enhancement of surface conductivity. The results suggest a reliable method for delta doping for power electronics applications. [ABSTRACT FROM AUTHOR]

Published
2024
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22. Behavioural disinhibition in frontotemporal dementia investigated within an ecological framework

Author

Batrancourt, Bénédicte, Azuar, Carole, Dubois, Bruno, Lecouturier, Karen, Araujo, Carla M., Janvier, Estelle, Jourdain, Aline, Rametti-Lacroux, Armelle, Coriou, Sophie, Brochard, Vanessa B., Gaudebout, Cécile, Ferrand-Verdejo, Johan, Bonnefous, Louis, Pochan-Leva, Flore, Jeanne, Lucie, Joulié, Mathilde, Provost, Myriam, Renaud, Rozenn, Hachemi, Sarah, Guillemot, Vincent, Bendetowicz, David, Carle, Guilhem, Socha, Julie, Pineau, Fanny, Marin, Frédéric, Liu, Yongjian, Mullot, Pierre, Mousli, Aymen, Blossier, Armelle, Visentin, Giulia, Tanguy, Delphine, Godefroy, Valérie, Sezer, Idil, Boucly, Mathilde, Cabrol-Douat, Blandine, Odobez, Raphaëlle, Marque, Constance, Tessereau-Barbot, Daphné, Raud, Anaïs, Funkiewiez, Aurélie, Chamayou, Céline, Cognat, Emmanuel, Le Bozec, Manon, Bouzigues, Arabella, Le Du, Vincent, Bombois, Stéphanie, Simard, Camille, Fulcheri, Paolo, Guitton, Hortense, Peltier, Caroline, Lejeune, François-Xavier, Jorgensen, Lars, Mariani, Louise-Laure, Corvol, Jean-Christophe, Valero-Cabre, Antoni, Garcin, Béatrice, Volle, Emmanuelle, Le Ber, Isabelle, Migliaccio, Raffaella, Levy, Richard, Saracino, Dario, Morandi, Xavier, and Jannin, Pierre

Published
2022
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23. Pulmonary Hypertension in Patients with Common Variable Immunodeficiency

Author

Thoré, Pierre, Jaïs, Xavier, Savale, Laurent, Dorfmuller, Peter, Boucly, Athénaïs, Devilder, Matthieu, Meyrignac, Olivier, Pichon, Jérémie, Mankikian, Julie, Riou, Marianne, Boiffard, Emmanuel, Boissin, Clément, De Groote, Pascal, Chabanne, Céline, Gagnadoux, Frédéric, Bergeron, Anne, Noel, Nicolas, Sitbon, Olivier, Humbert, Marc, and Montani, David

Published
2021
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24. Respiratory symptoms and radiological findings in post-acute COVID-19 syndrome

Author

Etienne-Marie Jutant, Olivier Meyrignac, Antoine Beurnier, Xavier Jaïs, Tai Pham, Luc Morin, Athénaïs Boucly, Sophie Bulifon, Samy Figueiredo, Anatole Harrois, Mitja Jevnikar, Nicolas Noël, Jérémie Pichon, Anne Roche, Andrei Seferian, Samer Soliman, Jacques Duranteau, Laurent Becquemont, Xavier Monnet, Olivier Sitbon, Marie-France Bellin, Marc Humbert, Laurent Savale, and David Montani

Subjects
Medicine
Abstract

Rationale The characteristics of patients with respiratory complaints and/or lung radiologic abnormalities after hospitalisation for coronavirus disease 2019 (COVID-19) are unknown. The objectives were to determine their characteristics and the relationships between dyspnoea, radiologic abnormalities and functional impairment. Methods In the COMEBAC (Consultation Multi-Expertise de Bicêtre Après COVID-19) cohort study, 478 hospital survivors were evaluated by telephone 4 months after hospital discharge, and 177 who had been hospitalised in an intensive care unit (ICU) or presented relevant symptoms underwent an ambulatory evaluation. New-onset dyspnoea and cough were evaluated, and the results of pulmonary function tests and high-resolution computed tomography of the chest were collected. Results Among the 478 patients, 78 (16.3%) reported new-onset dyspnoea, and 23 (4.8%) new-onset cough. The patients with new-onset dyspnoea were younger (56.1±12.3 versus 61.9±16.6 years), had more severe COVID-19 (ICU admission 56.4% versus 24.5%) and more frequent pulmonary embolism (18.0% versus 6.8%) (all p≤0.001) than patients without dyspnoea. Among the patients reassessed at the ambulatory care visit, the prevalence of fibrotic lung lesions was 19.3%, with extent

Published
2022
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26. Survival Improved in Patients Aged ≤ 70 Years With Systemic Sclerosis-Associated Pulmonary Arterial Hypertension During the Period 2006 to 2017 in France

Author

Hachulla, Eric, Launay, David, Boucly, Athénaïs, Mouthon, Luc, de Groote, Pascal, Cottin, Vincent, Pugnet, Grégory, Prévôt, Grégoire, Bourlier, Delphine, Dauphin, Claire, Chaouat, Ari, Weatherald, Jason, Simonneau, Gérald, Montani, David, Humbert, Marc, Sitbon, Olivier, and Giovannelli, Jonathan

Published
2020
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31. Immunization and multiple sclerosis: Recommendations from the French Multiple Sclerosis Society

Author

Abadie, V., Achour, C., Ader, F., Alchaar, H., Alkhedr, A., Andreux, F., Androdias, G., Arjmand, R., Audoin, B., Audry, D., Aufauvre, D., Autreaux, C., Ayrignac, X., Bailbe, M., Benazet, M., Bensa, C., Bensmail, D., Berger, E., Bernady, P., Bertagna, Y., Biotti, D., Blanchard-Dauphin, A., Bonenfant, J., Bonnan, M., Bonnemain, B., Borgel, F., Botelho-Nevers, E., Boucly, S., Bourre, B., Boutière, C., Branger, P., Brassat, D., Bresch, S., Breuil, V., Brochet, B., Brugeilles, H., Bugnon, P., Cabre, P., Camdessanché, J.-P., Carra-Dalière, C., Casez, O., Chamouard, J.-M., Chassande, B., Chataignier, P., Chbicheb, M., Chenet, A., Ciron, J., Clavelou, P., Cohen, M., Colamarino, R., Collongues, N., Coman, I., Corail, P.-R., Courtois, S., Coustans, M., Creange, A., Creisson, E., Daluzeau, N., Davenas, C., De Seze, J., Debouverie, M., Depaz, R., Derache, N., Divio, L., Douay, X., Dulau, C., Durand-Dubief, F., Edan, G., Elias, Z., Fagniez, O., Faucher, M., Faucheux, J.-M., Fournier, M., Gagneux-Brunon, A., Gaida, P., Galli, P., Gallien, P., Gaudelus, J., Gault, D., Gayou, A., Genevray, M., Gentil, A., Gere, J., Gignoux, L., Giroux, M., Givron, P., Gout, O., Grimaud, J., Guennoc, A.-M., Hadhoum, N., Hautecoeur, P., Heinzlef, O., Jaeger, M., Jeannin, S., Kremer, L., Kwiatkowski, A., Labauge, P., Labeyrie, C., Lachaud, S., Laffont, I., Lanctin-Garcia, C., Lannoy, J., Lanotte, L., Laplaud, D., Latombe, D., Lauxerois, M., Le Page, E., Lebrun-Frenay, C., Lejeune, P., Lejoyeux, P., Lemonnier, B., Leray, E., Loche, C.-M., Louapre, C., Lubetzki, C., Maarouf, A., Mada, B., Magy, L., Maillart, E., Manchon, E., Marignier, R., Marque, P., Mathey, G., Maurousset, A., Mekies, C., Merienne, M., Michel, L., Milor, A.-M., Moisset, X., Montcuquet, A., Moreau, T., Morel, N., Moussa, M., Naudillon, J.-P., Normand, M., Olive, P., Ouallet, J.-C., Outteryck, O., Pacault, C., Papeix, C., Patry, I., Peaureaux, D., Pelletier, J., Pichon, B., Pittion, S., Planque, E., Pouget, M.-C., Pourcher, V., Radot, C., Robert, I., Rocher, F., Ruet, A., Saint-Val, C., Salle, J.-Y., Salmon, A., Sartori, E., Schaeffer, S., Stankhof, B., Taithe, F., Thouvenot, E., Tizon, C., Tourbah, A., Tourniaire, P., Vaillant, M., Vermersch, P., Vidil, S., Wahab, A., Warter, M.-H., Wiertlewski, S., Wiplosz, B., Wittwer, B., Zaenker, C., Zephir, H., Lebrun, C., and Vukusic, S.

Published
2019
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34. Outcomes and risk assessment in pulmonary veno-occlusive disease.

Author

Boucly, Athénaïs, Solinas, Sabina, Beurnier, Antoine, Jaïs, Xavier, Keddache, Sophia, Eyries, Mélanie, Seferian, Andrei, Jevnikar, Mitja, Roche, Anne, Bulifon, Sophie, Bourdin, Arnaud, Chaouat, Ari, Cottin, Vincent, Bertoletti, Laurent, Savale, Laurent, Humbert, Marc, Sitbon, Olivier, and Montani, David

Published
2024
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35. Pulmonary veno-occlusive disease: illustrative cases and literature review.

Author

Lechartier, Benoit, Boucly, Athénaïs, Solinas, Sabina, Gopalan, Deepa, Dorfmüller, Peter, Radonic, Teodora, Sitbon, Olivier, and Montani, David

Subjects
PULMONARY arterial hypertension, PULMONARY veins, CANCER chemotherapy, MITOMYCINS, LYMPHADENITIS
Abstract

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 EIF2AK4 gene in heritable forms of disease. Distinguishing PVOD from PAH is critical for guiding appropriate management. Chest computed tomography typically displays interlobular septal thickening, ground-glass opacities and mediastinal lymphadenopathy. Life-threatening pulmonary oedema is a complication of pulmonary vasodilator therapy that can occur with any class of PAH drugs in PVOD. Early referral to a lung transplant centre is essential due to the poor response to therapy when compared with other forms of PAH. Histopathological analysis of lung explants reveals microvascular remodelling with typical fibrous veno-occlusive lesions. This review covers the main features distinguishing PVOD from PAH and two clinical cases that illustrate the challenges of PVOD management. [ABSTRACT FROM AUTHOR]

Published
2024
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36. Pharmacological management of connective tissue disease-associated pulmonary arterial hypertension.

Author

Kularatne, Mithum, Boucly, Athénaïs, Savale, Laurent, Solinas, Sabina, Cheron, Céline, Roche, Anne, Jevnikar, Mitja, Jaïs, Xavier, Montani, David, Humbert, Marc, and Sitbon, Olivier

Abstract

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. This review discusses the epidemiology of CTD-associated PAH, outlines the complex diagnosis approach, and finishes with an in-depth discussion on the current treatment paradigm. Focus is placed on challenges faced in the treatment of CTD-associated PAH, (decreased efficacy and poorer tolerance of pharmacological therapies) and includes a discussion on the future investigational treatments. Despite significant advances over the past decades with more aggressive treatment algorithms, CTD-associated PAH patients continue to have poorer survival compared to those with idiopathic PAH. This review highlights factors leading to disparate outcomes compared to other forms of PAH, and discusses on further improvements that may increase quality of life and survival for CTD-associated PAH patients. [ABSTRACT FROM AUTHOR]

Published
2023
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37. Management of Acutely Decompensated Pulmonary Hypertension.

Author

Savale, Laurent, Kularatne, Mithum, Roche, Anne, Pichon, Jérémie, Baron, Audrey, Boucly, Athenaïs, Sitbon, Olivier, and Humbert, Marc

Subjects
HEART failure, PULMONARY hypertension, PULMONARY arterial hypertension, ARTIFICIAL blood circulation, HEART diseases, HEALTH care teams
Abstract

Pulmonary arterial hypertension is a severe life-threatening condition associated with increased pulmonary vascular resistance and resulting right heart dysfunction. Admission to intensive care unit with acutely decompensated right heart failure is a significant negative prognostic event with a high risk of multisystem organ dysfunction and death. Presentations are heterogenous and may combine signs of both diastolic and systolic dysfunction complicating management. Renal dysfunction is often present, but other organ systems can be involved resulting in findings such as acute hepatic dysfunction or bowel wall congestion and ischemia. The goals of therapy are to rapidly reverse ventriculo-arterial decoupling and reduce right ventricular afterload to prevent progression to refractory or irreversible right heart failure. Triggering events must be investigated for and addressed urgently if identified. Volume status management is critical and both noninvasive and invasive testing can aid in prognostication and guide management, including the use of inotropes and vasopressors. In cases of refractory right heart dysfunction, consideration of urgent lung transplantation and mechanical circulatory support is necessary. These patients should be managed at expert centers in an intensive care setting with a multidisciplinary team of practitioners experienced in the management of right heart dysfunction given the high short- and long-term mortality resulting from acute decompensated right heart failure. [ABSTRACT FROM AUTHOR]

Published
2023
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38. Clinical relevance and prognostic value of renal Doppler in acute decompensated precapillary pulmonary hypertension.

Author

Pichon, Jérémie, Roche, Anne, Fauvel, Charles, Boucly, Athénais, Mercier, Olaf, Ebstein, Nathan, Beurnier, Antoine, Cortese, Jonathan, Jevnikar, Mitja, Jaïs, Xavier, Fartoukh, Muriel, Fadel, Elie, Sitbon, Olivier, Montani, David, Voiriot, Guillaume, Humbert, Marc, and Savale, Laurent

Subjects
PULMONARY arterial hypertension, INTENSIVE care units, BLOOD pressure, ECHOCARDIOGRAPHY, STATISTICS, CENTRAL venous pressure, CONFIDENCE intervals, PATIENTS, TRICUSPID valve, MANN Whitney U Test, HOSPITAL admission & discharge, T-test (Statistics), DOPPLER ultrasonography, HEART atrium, DESCRIPTIVE statistics, DATA analysis, LOGISTIC regression analysis, LONGITUDINAL method, HEART failure, PROPORTIONAL hazards models
Abstract

Aims We aim to evaluate the clinical relevance and the prognostic value of arterial and venous renal Doppler in acute decompensated precapillary pulmonary hypertension (PH). Methods and results The renal resistance index (RRI) and the Doppler-derived renal venous stasis index (RVSI) were monitored at admission and on Day 3 in a prospective cohort of precapillary PH patients managed in intensive care unit for acute right heart failure (RHF). The primary composite endpoint included death, circulatory assistance, urgent transplantation, or rehospitalization for acute RHF within 90 days following inclusion. Ninety-one patients were enrolled (58% female, age 58 ± 16 years). The primary endpoint event occurred in 32 patients (33%). In univariate logistic regression analysis, variables associated with RRI higher than the median value were non-variable parameters (age and history of hypertension), congestion (right atrial pressure and renal pulse pressure), cardiac function [tricuspid annular plane systolic excursion (TAPSE) and left ventricular outflow tract- velocity time integral], systemic pressures and NT-proBNP. Variables associated with RVSI higher than the median value were congestion (high central venous pressure, right atrial pressure, and renal pulse pressure), right cardiac function (TAPSE), severe tricuspid regurgitation, and systemic pressures. Inotropic support was more frequently required in patients with high RRI (P = 0.01) or high RVSI (P = 0.003) at the time of admission. At Day 3, a RRI value <0.9 was associated with a better prognosis after adjusting to the estimated glomerular filtration rate. Conclusion Renal Doppler provides additional information to assess the severity of patients admitted to the intensive care unit for acute decompensated precapillary PH. [ABSTRACT FROM AUTHOR]

Published
2023
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39. Real‐world evidence to advance knowledge in pulmonary hypertension: Status, challenges, and opportunities. A consensus statement from the Pulmonary Vascular Research Institute's Innovative Drug Development Initiative's Real‐world Evidence Working Group.

Author

Morland, Kellie, Gerges, Christian, Elwing, Jean, Visovatti, Scott H., Weatherald, Jason, Gillmeyer, Kari R., Sahay, Sandeep, Mathai, Stephen C., Boucly, Athénaïs, Williams, Paul G., Harikrishnan, Sivadasanpillai, Minty, Evan P., Hobohm, Lukas, Jose, Arun, Badagliacca, Roberto, Lau, Edmund M. T., Jing, Zhi‐Cheng, Vanderpool, Rebecca R., Fauvel, Charles, and Leonidas Alves, Jose

Abstract

This manuscript on real‐world evidence (RWE) in pulmonary hypertension (PH) incorporates the broad experience of members of the Pulmonary Vascular Research Institute's Innovative Drug Development Initiative Real‐World Evidence Working Group. We aim to strengthen the research community's understanding of RWE in PH to facilitate clinical research advances and ultimately improve patient care. Herein, we review real‐world data (RWD) sources, discuss challenges and opportunities when using RWD sources to study PH populations, and identify resources needed to support the generation of meaningful RWE for the global PH community. [ABSTRACT FROM AUTHOR]

Published
2023
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40. In-depth characterization of pulmonary arterial hypertension in mixed connective tissue disease: a French national multicentre study.

Author

Chaigne, Benjamin, Chevalier, Kevin, Boucly, Athenaïs, Agard, Christian, Baudet, Antoine, Bourdin, Arnaud, Chabanne, Céline, Cottin, Vincent, Fesler, Pierre, Goupil, François, Jego, Patrick, Launay, David, Lévesque, Hervé, Maurac, Arnaud, Mohamed, Shirine, Tromeur, Cécile, Rottat, Laurence, Sitbon, Olivier, Humbert, Marc, and Mouthon, Luc

Subjects
MORTALITY risk factors, PULMONARY arterial hypertension, RESEARCH, PERICARDITIS, MULTIVARIATE analysis, INTERSTITIAL lung diseases, CONNECTIVE tissue diseases, RISK assessment, KAPLAN-Meier estimator, DESCRIPTIVE statistics, ARTHRITIS, THROMBOCYTOPENIA, SMOKING, SYSTEMIC lupus erythematosus, DISEASE risk factors, DISEASE complications
Abstract

Objective Pulmonary arterial hypertension (PAH) is a leading cause of death in MCTD. We aimed to describe PAH in well-characterized MCTD patients. Methods MCTD patients enrolled in the French Pulmonary Hypertension Registry with a PAH diagnosis confirmed by right heart catheterization were included in the study and compared with matched controls: MCTD patients without PAH, SLE patients with PAH and SSc patients with PAH. Survival rates were estimated by the Kaplan–Meier method and risk factors for PAH in MCTD patients and risk factors for mortality in MCTD-PAH were sought using multivariate analyses. Results Thirty-six patients with MCTD-PAH were included in the study. Comparison with MCTD patients without PAH and multivariate analysis revealed that pericarditis, polyarthritis, thrombocytopenia, interstitial lung disease (ILD) and anti-Sm antibodies were independent predictive factors of PAH/PH in MCTD. Estimated survival rates at 1, 5 and 10 years following PAH diagnosis were 83%, 67% and 56%, respectively. MCTD-PAH presentation and survival did not differ from SLE-PAH and SSc-PAH. Multivariate analysis revealed that tobacco exposure was an independent factor predictive of mortality in MCTD-PAH. Conclusion PAH is a rare and severe complication of MCTD associated with a 56% 10-year survival. We identified ILD, pericarditis, thrombocytopenia and anti-Sm antibodies as risk factors for PAH in MCTD and tobacco exposure as a predictor of mortality in MCTD-PAH. [ABSTRACT FROM AUTHOR]

Published
2023
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41. Clinical Phenotype and Outcomes of Pulmonary Hypertension Associated with Myeloproliferative Neoplasms: A Population-based Study.

Author

Montani, David, Thoré, Pierre, Mignard, Xavier, Jaïs, Xavier, Boucly, Athénaïs, Jevnikar, Mitja, Seferian, Andrei, Jutant, Etienne-Marie, Cottin, Vincent, Fadel, Elie, Simonneau, Gérald, Savale, Laurent, Sitbon, Olivier, and Humbert, Marc

Subjects
MYELOFIBROSIS, MYELOPROLIFERATIVE neoplasms, PULMONARY hypertension, POLYCYTHEMIA vera, VASCULAR resistance, PHYSICIANS
Abstract

Rationale: Precapillary pulmonary hypertension (PH) is a rare and largely unrecognized complication of myeloproliferative neoplasms (MPNs), including polycythemia vera (PV), essential thrombocythemia (ET), and primary myelofibrosis (MF). Objectives: To describe characteristics and outcomes of MPNassociated PH. Methods: We report clinical, functional, and hemodynamic characteristics, classification, and outcomes of patients with PV, ET, or primary MF in the French PH registry. Measurements and Main Results: Ninety patients with MPN (42 PV, 35 ET, 13 primary MF) presented with precapillary PH with severe hemodynamic impairment, with a median mean pulmonary arterial pressure and pulmonary vascular resistance of 42mmHg and 6.7 Wood units, respectively, and impaired clinical conditions, with 71% in New York Heart Association functional classes III/IV and having a median 6-minute-walk distance of 310 m. Half of the patients were diagnosed with chronic thromboembolic PH (CTEPH); the other half were considered to have group 5 PH. MF was preferentially associated with group 5 PH, whereas PV and ET were generally related to CTEPH. Proximal lesions were diagnosed in half of the patients with CTEPH. Thromboendarterectomy was performed in 18 selected patients with high risk of complications (5 early deaths). Overall survival at 1, 3, and 5 years was 67%, 50%, and 34% in group 5 PH and 81%, 66%, and 42% in CTEPH, respectively. Conclusions: PH is a life-threatening condition potentially occurring inMPN. There are multiple mechanisms, with equal diagnoses of CTEPH and group 5 PH. Physicians should be aware that PH strongly affects the burden of patients with MPN, especially in group 5 PH, with unknown pathophysiological mechanisms. [ABSTRACT FROM AUTHOR]

Published
2023
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42. In-depth characterization of pulmonary arterial hypertension in mixed connective tissue disease: a French national multicenter study

Author

Benjamin Chaigne, Kevin Chevalier, Athenaïs Boucly, Christian Agard, Antoine Baudet, Arnaud Bourdin, Céline Chabanne, Vincent Cottin, Pierre Fesler, François Goupil, Patrick Jego, David Launay, Hervé Lévesque, Arnaud Maurac, Shirine Mohamed, Cécile Tromeur, Laurence Rottat, Olivier Sitbon, Marc Humbert, Luc Mouthon, Hôpital Cochin [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Centre hospitalier universitaire de Nantes (CHU Nantes), Physiologie & médecine expérimentale du Cœur et des Muscles [U 1046] (PhyMedExp), Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS)-Université de Montpellier (UM), Centre Hospitalier Régional Universitaire [Montpellier] (CHRU Montpellier), CHU Pontchaillou [Rennes], Laboratoire Traitement du Signal et de l'Image (LTSI), Université de Rennes (UR)-Institut National de la Santé et de la Recherche Médicale (INSERM), Infections Virales et Pathologie Comparée - UMR 754 (IVPC), École Pratique des Hautes Études (EPHE), Université Paris sciences et lettres (PSL)-Université Paris sciences et lettres (PSL)-Université Claude Bernard Lyon 1 (UCBL), Université de Lyon-Université de Lyon-Institut National de Recherche pour l’Agriculture, l’Alimentation et l’Environnement (INRAE), Institut de recherche en santé, environnement et travail (Irset), Université d'Angers (UA)-Université de Rennes (UR)-École des Hautes Études en Santé Publique [EHESP] (EHESP)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Structure Fédérative de Recherche en Biologie et Santé de Rennes ( Biosit : Biologie - Santé - Innovation Technologique ), Institute for Translational Research in Inflammation - U 1286 (INFINITE (Ex-Liric)), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Lille-Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), Institut Fédératif de Recherches Multidisciplinaires sur les Peptides (IFRMP 23), CHU Rouen, Normandie Université (NU)-Normandie Université (NU)-Université Le Havre Normandie (ULH), Normandie Université (NU)-Université de Rouen Normandie (UNIROUEN), Normandie Université (NU)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre de Lutte Contre le Cancer Henri Becquerel Normandie Rouen (CLCC Henri Becquerel)-Centre National de la Recherche Scientifique (CNRS), Centre Hospitalier Régional Universitaire de Nancy (CHRU Nancy), Groupe d'Etude de la Thrombose de Bretagne Occidentale (GETBO), Université de Brest (UBO)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Institut Brestois Santé Agro Matière (IBSAM), Université de Brest (UBO)-Université de Brest (UBO), and Hôpital Bicêtre

Subjects
Rheumatology, systemic lupus erythematosus, [SDV.MHEP.RSOA]Life Sciences [q-bio]/Human health and pathology/Rhumatology and musculoskeletal system, systemic sclerosis, pulmonary arterial hypertension, [SDV]Life Sciences [q-bio], mixed connective tissue disease, Pharmacology (medical), survival
Abstract

International audience; Abstract Objective pulmonary arterial hypertension (PAH) is a leading cause of death in mixed connective tissue disease (MCTD). We aimed to describe PAH in well-characterized MCTD patients. Methods MCTD patients enrolled in the French Pulmonary Hypertension Registry with a PAH diagnosis confirmed by right heart catheterization were included in the study and compared with matched controls: MCTD patients without PAH, systemic lupus erythematous (SLE) patients with PAH, and systemic sclerosis (SSc) patients with PAH. Survival rates were estimated by the Kaplan-Meier method and risk factors for PAH in MCTD patients and risk factors for mortality in MCTD-PAH were sought using multivariate analyses. Results thirty-six patients with MCTD-PAH were included in the study. Comparison with MCTD patients without PAH and multivariate analysis revealed that pericarditis, polyarthritis, thrombocytopenia, interstitial lung disease (ILD), and anti-Sm antibodies were independent predictive factors of PAH/PH in MCTD. Estimated survival rates at 1 year, 5 years, and 10 years, following PAH diagnosis were 83%, 67%, and 56%, respectively. MCTD-PAH presentation and survival did not differ from SLE-PAH and SSc-PAH. Multivariate analysis revealed that tobacco exposure was an independent factor predictive of mortality in MCTD-PAH. Conclusion PAH is a rare and severe complication of MCTD, associated with a 56% 10-year survival. We identified ILD, pericarditis, thrombocytopenia, and anti-Sm antibodies as risk factors for PAH in MCTD and tobacco exposure as predictor of mortality in MCTD-PAH.

Published
2023

43. Improving time-resolution and sensitivity of in situ X-ray photoelectron spectroscopy of a powder catalyst by modulated excitation

Author

Roger, Maneka, Artiglia, Luca, Boucly, Anthony, Buttignol, Filippo, Agote-Arán, Miren, van Bokhoven, Jeroen A., Kröcher, Oliver, and Ferri, Davide

Subjects
co oxidation, pd(111) oxidation, pressure, alumina interface, oxide formation, xps, surface, palladium, performance, combustion
Abstract

Ambient pressure X-ray photoelectron spectroscopy (APXPS) is a powerful tool to characterize the surface structure of heterogeneous catalysts in situ. In order to improve the time resolution and the signal-to-noise (S/N) ratio of photoemission spectra, we collected consecutive APXP spectra during the periodic perturbation of a powder Pd/Al2O3 catalyst away from its equilibrium state according to the modulated excitation approach (ME). Averaging of the spectra along the alternate pulses of O-2 and CO improved the S/N ratio demonstrating that the time resolution of the measurement can be limited solely to the acquisition time of one spectrum. Through phase sensitive analysis of the averaged time-resolved spectra, the formation/consumption dynamics of three oxidic species, two metal species, adsorbed CO on Pd-0 as well as Pdn+ (n > 2) was followed along the gas switches. Pdn+ and 2-fold surface PdO species were recognised as most reactive to the gas switches. Our approach demonstrates that phase sensitive detection of time-resolved XPS data allows following the dynamics of reactive species at the solid-gas interface under different reaction environments with unprecedented precision., Chemical Science, ISSN:2041-6520, ISSN:2041-6539

Published
2023

44. Orcinol and resorcinol induce local ordering of water molecules near the liquid-vapor interface

Author

Yang, Huanyu, Gladich, Ivan, Boucly, Anthony, Artiglia, Luca, and Ammann, Markus

Abstract

Resorcinol and orcinol are simple members of the family of phenolic compounds present in particulate matter in the atmosphere; they are amphiphilic in nature and thus surface active in aqueous solution. Here, we used X-ray photoelectron spectroscopy to probe the concentration of resorcinol (benzene-1,3-diol) and orcinol (5-methylbenzene-1,3-diol) at the liquid-vapor interface of aqueous solutions. Qualitatively consistent surface propensity and preferential orientation was obtained by molecular dynamics simulations. Auger electron yield near-edge X-ray absorption fine structure (NEXAFS) spectroscopy was used to probe the hydrogen bonding (HB) structure, indicating that the local structure of water molecules near the surface of the resorcinol and orcinol solutions tends towards a larger fraction of tetrahedrally coordinated molecules than observed at the liquid-vapor interface of pure water. The order parameter obtained from the molecular dynamics simulations confirm these observations. This effect is being discussed in terms of the formation of an ordered structure of these molecules at the surface leading to patterns of hydrated OH groups with distances among them that are relatively close to those in ice. These results suggest that the self-assembly of phenolic species at the aqueous solution-air interface could induce freezing similar to the case of fatty alcohol monolayers and, thus, be of relevance for ice nucleation in the atmosphere. We also attempted at looking at the changes of the O 1b(1), 3a(2) and 1b(2) molecular orbitals of liquid water, which are known to be sensitive to the HB structure as well, in response to the presence of resorcinol and orcinol. However, these changes remained negligible within uncertainty for both experimentally obtained valence spectra and theoretically calculated density of states., Environmental Science: Atmospheres, 2 (6), ISSN:2634-3606

Published
2022

45. Surface spectroscopic analysis of aqueous phase iodine oxides : Electron binding energy and surface propensity

Author

Roose, Antoine, Opoku, Richard, Boucly, Anthony, Yang, Huanyu, Lezzi, Lucia, Toubin, Céline, Vallet, Valérie, Halbert, Loïc, Severo Pereira Gomes, André, Ammann, Markus, Artiglia, Luca, Laboratory of Environmental Chemistry [Villigen] (LUC), Paul Scherrer Institute (PSI), Physico-Chimie Moléculaire Théorique (PCMT), Laboratoire de Physique des Lasers, Atomes et Molécules - UMR 8523 (PhLAM), Université de Lille-Centre National de la Recherche Scientifique (CNRS)-Université de Lille-Centre National de la Recherche Scientifique (CNRS), Institute of Atmospheric and Climate Science, Eidgenössische Technische Hochschule - Swiss Federal Institute of Technology [Zürich] (ETH Zürich), ANR-16-IDEX-0004,ULNE,ULNE(2016), and ANR-11-LABX-0005,Cappa,Physiques et Chimie de l'Environnement Atmosphérique(2011)

Subjects
[CHIM.THEO]Chemical Sciences/Theoretical and/or physical chemistry, [PHYS.PHYS.PHYS-CHEM-PH]Physics [physics]/Physics [physics]/Chemical Physics [physics.chem-ph]
Abstract

International audience; Iodine chemistry is implicated in atmospheric chemistry and can form several oxides such as HOI, I2, IO, OIO, and finally I2O5 or HIO3, which may nucleate as nanoparticles relevant for cloud formation in remote environments. These oxides can be formed through reaction with oxidants or other halogen compounds in the gas phase or the particle phase. Most of the iodide oxidation processes have been suggested to be enhanced at interfaces, similar to other halogen species, either due to the surface propensity of intermediates or the iodine species itself. However, no data are available about the surface concentration of iodine species other than iodide. After two decades of research into the surface propensity of iodide and bromide, the picture emerges that their surface propensity is not as extreme as initially thought. Liquid jet X-ray photoelectron spectroscopy (XPS) experiments have been carried out at the SIM beamline at the Swiss Light Source. Acquisition of kinetic energy dependent (thus at different probing depth) I3d, I4d core level and valence level spectra has been done for iodide, iodate and iodic acid. This allows to retrieve the surface propensity of these iodine species at the aqueous solution – air interface. Theoretical computations were performed to calculate the spectra using the so-called Frozen Embedding Method where DFT SAOP is coupled with CVS-EOM-IP-CCSD/d-aug-dy-all.ac2vz via a molecular mean-field X2C including the Gaunt interaction. The computation of the iodide core binding energies in the aqueous have been determined and compared to experimental measurements.

Published
2022

50. Acute decompensated pulmonary hypertension

Author

Laurent Savale, Jason Weatherald, Xavier Jaïs, Constance Vuillard, Athénaïs Boucly, Mitja Jevnikar, David Montani, Olaf Mercier, Gerald Simonneau, Elie Fadel, Olivier Sitbon, and Marc Humbert

Subjects
Diseases of the respiratory system, RC705-779
Abstract

Acute right heart failure in chronic precapillary pulmonary hypertension is characterised by a rapidly progressive syndrome with systemic congestion resulting from impaired right ventricular filling and/or reduced right ventricular flow output. This clinical picture results from an imbalance between the afterload imposed on the right ventricle and its adaptation capacity. Acute decompensated pulmonary hypertension is associated with a very poor prognosis in the short term. Despite its major impact on survival, its optimal management remains very challenging for specialised centres, without specific recommendations. Identification of trigger factors, optimisation of fluid volume and pharmacological support to improve right ventricular function and perfusion pressure are the main therapeutic areas to consider in order to improve clinical condition. At the same time, specific management of pulmonary hypertension according to the aetiology is mandatory to reduce right ventricular afterload. Over the past decade, the development of extracorporeal life support in refractory right heart failure combined with urgent transplantation has probably contributed to a significant improvement in survival for selected patients. However, there remains a considerable need for further research in this field.

Published
2017
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