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2. Defining Echocardiographic Degrees of Right Heart Size and Function in Pulmonary Vascular Disease From the PVDOMICS Study

Author

Mukherjee, Monica, Mathai, Stephen C., Jellis, Christine, Freed, Benjamin H., Yanek, Lisa R., Agoglia, Hannah, Chiu, Caitlin, Jani, Vivek P., Simpson, Catherine E., Brittain, Evan L., Tang, W.H. Wilson, Park, Margaret M., Hemnes, Anna R., Rosenzweig, Erika B., Rischard, Franz P., Frantz, Robert P., Hassoun, Paul M., Beck, Gerald, Hill, Nicholas S., Erzurum, Serpil, Thomas, James D., Kwon, Deborah, Leopold, Jane A., Horn, Evelyn M., and Kim, Jiwon

Published
2024
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3. Metabolomic Profiles Differentiate Scleroderma-PAH From Idiopathic PAH and Correspond With Worsened Functional Capacity

Author

Alotaibi, Mona, Shao, Junzhe, Pauciulo, Michael W, Nichols, William C, Hemnes, Anna R, Malhotra, Atul, Kim, Nick H, Yuan, Jason X-J, Fernandes, Timothy, Kerr, Kim M, Alshawabkeh, Laith, Desai, Ankit A, Bujor, Andreea M, Lafyatis, Robert, Watrous, Jeramie D, Long, Tao, Cheng, Susan, Chan, Stephen Y, and Jain, Mohit

Subjects
Medical Biochemistry and Metabolomics, Biomedical and Clinical Sciences, Autoimmune Disease, Scleroderma, Lung, Rare Diseases, Clinical Research, Orphan Drug, Inflammatory and immune system, Humans, Familial Primary Pulmonary Hypertension, Hypertension, Pulmonary, Scleroderma, Systemic, Prognosis, Lipids, biomarkers, metabolomics, pulmonary hypertension, scleroderma, Clinical Sciences, Respiratory System, Cardiovascular medicine and haematology, Clinical sciences
Abstract

BackgroundThe prognosis and therapeutic responses are worse for pulmonary arterial hypertension associated with systemic sclerosis (SSc-PAH) compared with idiopathic pulmonary arterial hypertension (IPAH). This discrepancy could be driven by divergence in underlying metabolic determinants of disease.Research questionAre circulating bioactive metabolites differentially altered in SSc-PAH vs IPAH, and can this alteration explain clinical disparity between these PAH subgroups?Study design and methodsPlasma biosamples from 400 patients with SSc-PAH and 1,082 patients with IPAH were included in the study. Another cohort of 100 patients with scleroderma with no PH and 44 patients with scleroderma with PH was included for external validation. More than 700 bioactive lipid metabolites, representing a range of vasoactive and immune-inflammatory pathways, were assayed in plasma samples from independent discovery and validation cohorts using liquid chromatography/high-resolution mass spectrometry-based approaches. Regression analyses were used to identify metabolites that exhibited differential levels between SSc-PAH and IPAH and associated with disease severity.ResultsFrom hundreds of circulating bioactive lipid molecules, five metabolites were found to distinguish between SSc-PAH and IPAH, as well as associate with markers of disease severity. Relative to IPAH, patients with SSc-PAH carried increased levels of fatty acid metabolites, including lignoceric acid and nervonic acid, as well as eicosanoids/oxylipins and sex hormone metabolites.InterpretationPatients with SSc-PAH are characterized by an unfavorable bioactive metabolic profile that may explain the poor and limited response to therapy. These data provide important metabolic insights into the molecular heterogeneity underlying differences between subgroups of PAH.

Published
2023

5. Association of Male Sex With Worse Right Ventricular Function and Survival in Pulmonary Hypertension in the Redefining Pulmonary Hypertension Through Pulmonary Vascular Disease Phenomics Cohort

Author

Shelburne, Nicholas J., Nian, Hui, Beck, Gerald J., Casanova, Nancy G., Desai, Ankit A., DuBrock, Hilary M., Erzurum, Serpil, Frantz, Robert P., Hassoun, Paul M., Hill, Nicholas S., Horn, Evelyn M., Jacob, Miriam S., Jellis, Christine L., Joseloff, Elizabeth, Kwon, Deborah H., Larive, A. Brett, Leopold, Jane A., Park, Margaret M., Rischard, Franz P., Rosenzweig, Erika B., Vanderpool, Rebecca R., Yu, Chang, and Hemnes, Anna R.

Published
2024
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6. Seralutinib in adults with pulmonary arterial hypertension (TORREY): a randomised, double-blind, placebo-controlled phase 2 trial

Author

Adir, Yochai, Baillie, Timothy, Baratz, David, Burger, Charles, Chakinala, Murali M., Cifrián Martínez, Jose Manual, Delcroix, Marion, Dwyer, Nathan, Elwing, Jean M., Fisher, Micah, Franco, Veronica, Grünig, Ekkehard, Highland, Kristin, Hill, Nicholas, Hirani, Naushad, Hoeper, Marius, Jansa, Pavel, Keogh, Anne, Kingrey, John, Lopez-Meseguer, Manuel, McConnell, John W., Mehta, Sanjay, Melendres-Groves, Lana, Opitz, Christian, Pepke-Zaba, Joanna, Pillutla, Priya, Rahaghi, Franck, Raina, Amresh, Raviv, Yael, Robinson, Jeffrey, Ryan, John, Sager, Jeffrey, Shapiro, Shelley, Simon, Marc, Smith, Kerri, Sobol, Irina R., Sood, Namita, Spikes, Leslie A., Stadler, Stefan, Stevens, Wendy, Sulica, Roxana, White, R. James, Frantz, Robert P, McLaughlin, Vallerie V, Sahay, Sandeep, Escribano Subías, Pilar, Zolty, Ronald L, Benza, Raymond L, Channick, Richard N, Chin, Kelly M, Hemnes, Anna R, Howard, Luke S, Sitbon, Olivier, Vachiéry, Jean-Luc, Zamanian, Roham T, Cravets, Matt, Roscigno, Robert F, Mottola, David, Osterhout, Robin, Bruey, Jean-Marie, Elman, Erin, Tompkins, Cindy-ann, Parsley, Ed, Aranda, Richard, Zisman, Lawrence S, and Ghofrani, Hossein-Ardeschir

Published
2024
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9. Diagnosis and Treatment of Right Heart Failure in Pulmonary Vascular Diseases: A National Heart, Lung, and Blood Institute Workshop.

Author

Leopold, Jane A, Kawut, Steven M, Aldred, Micheala A, Archer, Stephen L, Benza, Ray L, Bristow, Michael R, Brittain, Evan L, Chesler, Naomi, DeMan, Frances S, Erzurum, Serpil C, Gladwin, Mark T, Hassoun, Paul M, Hemnes, Anna R, Lahm, Tim, Lima, Joao AC, Loscalzo, Joseph, Maron, Bradley A, Rosa, Laura Mercer, Newman, John H, Redline, Susan, Rich, Stuart, Rischard, Franz, Sugeng, Lissa, Tang, WH Wilson, Tedford, Ryan J, Tsai, Emily J, Ventetuolo, Corey E, Zhou, YouYang, Aggarwal, Neil R, and Xiao, Lei

Subjects
Humans, Hypertension, Pulmonary, Ventricular Dysfunction, Right, Pulmonary Circulation, Ventricular Function, Right, United States, Heart Failure, National Heart, Lung, and Blood Institute (U.S.), Hematology, Rare Diseases, Heart Disease, Lung, Cardiovascular, Respiratory, Good Health and Well Being, cardiovascular disease, hemodynamics, morbidity, prognosis, vascular diseases, Biochemistry and Cell Biology, Cardiorespiratory Medicine and Haematology, Medical Physiology, Cardiovascular System & Hematology
Abstract

Right ventricular dysfunction is a hallmark of advanced pulmonary vascular, lung parenchymal, and left heart disease, yet the underlying mechanisms that govern (mal)adaptation remain incompletely characterized. Owing to the knowledge gaps in our understanding of the right ventricle (RV) in health and disease, the National Heart, Lung, and Blood Institute (NHLBI) commissioned a working group to identify current challenges in the field. These included a need to define and standardize normal RV structure and function in populations; access to RV tissue for research purposes and the development of complex experimental platforms that recapitulate the in vivo environment; and the advancement of imaging and invasive methodologies to study the RV within basic, translational, and clinical research programs. Specific recommendations were provided, including a call to incorporate precision medicine and innovations in prognosis, diagnosis, and novel RV therapeutics for patients with pulmonary vascular disease.

Published
2021

10. Defining the clinical validity of genes reported to cause pulmonary arterial hypertension

Author

Callejo, Emily P., Day, Kristina M., Macaya, Daniela, Maldonado-Velez, Gabriel, Archer, Stephen L., Auckland, Kathryn, Austin, Eric D., Badagliacca, Roberto, Barberà, Joan-Albert, Belge, Catharina, Bogaard, Harm Jan, Bonnet, Sébastien, Boomars, Karin A., Boucherat, Olivier, Chakinala, Murali M., Condliffe, Robin, Damico, Rachel Lynn, Delcroix, Marion, Desai, Ankit A., Doboszynska, Anna, Elliott, C. Greg, Eyries, Melanie, Escribano Subías, Maria Pilar, Gall, Henning, Ghio, Stefano, Ghofrani, Ardeschir-Hossein, Grünig, Ekkehard, Hamid, Rizwan, Harbaum, Lars, Hassoun, Paul M., Hemnes, Anna R., Hinderhofer, Katrin, Howard, Luke S., Humbert, Marc, Kiely, David G., Langleben, David, Lawrie, Allan, Loyd, Jim E., Moledina, Shahin, Montani, David, Morrell, Nichols W., Nichols, William C., Olschewski, Andrea, Olschewski, Horst, Papa, Silvia, Pauciulo, Mike W., Provencher, Steve, Quarck, Rozenn, Rhodes, Christopher J., Scelsi, Laura, Seeger, Werner, Stewart, Duncan J., Sweatt, Andrew, Swietlik, Emilia M., Treacy, Carmen, Trembath, Richard C., Tura-Ceide, Olga, Vizza, Carmine Dario, Vonk Noordegraaf, Anton, Wilkins, Martin R., Zamanian, Roham T., Zateyshchikov, Dmitry, Welch, Carrie L., Aldred, Micheala A., Balachandar, Srimmitha, Dooijes, Dennis, Eichstaedt, Christina A., Gräf, Stefan, Houweling, Arjan C., Machado, Rajiv D., Pandya, Divya, Prapa, Matina, Shaukat, Memoona, Southgate, Laura, Tenorio-Castano, Jair, and Chung, Wendy K.

Published
2023
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13. Classification and Predictors of Right Ventricular Functional Recovery in Pulmonary Arterial Hypertension

Author

Rischard, Franz P., Bernardo, Roberto J., Vanderpool, Rebecca R., Kwon, Deborah H., Acharya, Tushar, Park, Margaret M., Katrynuik, Austin, Insel, Michael, Kubba, Saad, Badagliacca, Roberto, Larive, A. Brett, Naeije, Robert, Garcia, Joe G.N., Beck, Gerald J., Erzurum, Serpil C., Frantz, Robert P., Hassoun, Paul M., Hemnes, Anna R., Hill, Nicholas S., Horn, Evelyn M., Leopold, Jane A., Rosenzweig, Erika B., Tang, W.H. Wilson, and Wilcox, Jennifer D.

Published
2023
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15. Clinical Characteristics and Transplant-Free Survival Across the Spectrum of Pulmonary Vascular Disease

Author

Hemnes, Anna R., Leopold, Jane A., Radeva, Milena K., Beck, Gerald J., Abidov, Aiden, Aldred, Micheala A., Barnard, John, Rosenzweig, Erika B., Borlaug, Barry A., Chung, Wendy K., Comhair, Suzy A.A., Desai, Ankit A., Dubrock, Hilary M., Erzurum, Serpil C., Finet, J. Emanuel, Frantz, Robert P., Garcia, Joe G.N., Geraci, Mark W., Gray, Michael P., Grunig, Gabriele, Hassoun, Paul M., Highland, Kristin B., Hill, Nicholas S., Hu, Bo, Kwon, Deborah H., Jacob, Miriam S., Jellis, Christine L., Larive, A. Brett, Lempel, Jason K., Maron, Bradley A., Mathai, Stephen C., McCarthy, Kevin, Mehra, Reena, Nawabit, Rawan, Newman, John H., Olman, Mitchell A., Park, Margaret M., Ramos, Jose A., Renapurkar, Rahul D., Rischard, Franz P., Sherer, Susan G., Tang, W.H. Wilson, Thomas, James D., Vanderpool, Rebecca R., Waxman, Aaron B., Wilcox, Jennifer D., Yuan, Jason X.-J., and Horn, Evelyn M.

Published
2022
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16. Myeloid-Specific JAK2 Contributes to Inflammation and Salt Sensitivity of Blood Pressure.

Author

Saleem, Mohammad, Aden, Luul A., Mutchler, Ashley L., Basu, Chitra, Ertuglu, Lale A., Quanhu Sheng, Penner, Niki, Hemnes, Anna R., Park, Jennifer H., Ishimwe, Jeanne A., Laffer, Cheryl L., Elijovich, Fernando, Wanjalla, Celestine N., de la Visitacion, Nestor, Kastner, Paul D., Albritton, Claude F., Ahmad, Taseer, Haynes, Alexandria P., Yu, Justin, and Graber, Meghan K.

Published
2024
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18. Assessment of Right Ventricular Function in the Research Setting: Knowledge Gaps and Pathways Forward. An Official American Thoracic Society Research Statement

Author

Lahm, Tim, Douglas, Ivor S, Archer, Stephen L, Bogaard, Harm J, Chesler, Naomi C, Haddad, Francois, Hemnes, Anna R, Kawut, Steven M, Kline, Jeffrey A, Kolb, Todd M, Mathai, Stephen C, Mercier, Olaf, Michelakis, Evangelos D, Naeije, Robert, Tuder, Rubin M, Ventetuolo, Corey E, Vieillard-Baron, Antoine, Voelkel, Norbert F, Vonk-Noordegraaf, Anton, and Hassoun, Paul M

Subjects
Cardiovascular, Heart Disease, Lung, Rare Diseases, Animals, Humans, Research, Societies, Medical, United States, Ventricular Dysfunction, Right, Ventricular Function, Right, right ventricle, pulmonary hypertension, pulmonary embolism, acute respiratory distress syndrome, pulmonary circulation, American Thoracic Society Assembly on Pulmonary Circulation, Medical and Health Sciences, Respiratory System
Abstract

BackgroundRight ventricular (RV) adaptation to acute and chronic pulmonary hypertensive syndromes is a significant determinant of short- and long-term outcomes. Although remarkable progress has been made in the understanding of RV function and failure since the meeting of the NIH Working Group on Cellular and Molecular Mechanisms of Right Heart Failure in 2005, significant gaps remain at many levels in the understanding of cellular and molecular mechanisms of RV responses to pressure and volume overload, in the validation of diagnostic modalities, and in the development of evidence-based therapies.MethodsA multidisciplinary working group of 20 international experts from the American Thoracic Society Assemblies on Pulmonary Circulation and Critical Care, as well as external content experts, reviewed the literature, identified important knowledge gaps, and provided recommendations.ResultsThis document reviews the knowledge in the field of RV failure, identifies and prioritizes the most pertinent research gaps, and provides a prioritized pathway for addressing these preclinical and clinical questions. The group identified knowledge gaps and research opportunities in three major topic areas: 1) optimizing the methodology to assess RV function in acute and chronic conditions in preclinical models, human studies, and clinical trials; 2) analyzing advanced RV hemodynamic parameters at rest and in response to exercise; and 3) deciphering the underlying molecular and pathogenic mechanisms of RV function and failure in diverse pulmonary hypertension syndromes.ConclusionsThis statement provides a roadmap to further advance the state of knowledge, with the ultimate goal of developing RV-targeted therapies for patients with RV failure of any etiology.

Published
2018

20. Identifying consistent echocardiographic thresholds for risk stratification in pulmonary arterial hypertension

Author

Celestin, Bettia E., primary, Bagherzadeh, Shadi P., additional, Ichimura, Kenzo, additional, Santana, Everton J., additional, Sanchez, Pablo Amador, additional, Tobore, Tobore, additional, Hemnes, Anna R., additional, Noordegraaf, Anton Vonk, additional, Salerno, Michael, additional, Zamanian, Roham T., additional, Sweatt, Andrew J., additional, and Haddad, Francois, additional

Published
2024
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23. NHLBI-CMREF Workshop Report on Pulmonary Vascular Disease Classification: JACC State-of-the-Art Review

Author

Oldham, William M., Hemnes, Anna R., Aldred, Micheala A., Barnard, John, Brittain, Evan L., Chan, Stephen Y., Cheng, Feixiong, Cho, Michael H., Desai, Ankit A., Garcia, Joe G.N., Geraci, Mark W., Ghiassian, Susan D., Hall, Kathryn T., Horn, Evelyn M., Jain, Mohit, Kelly, Rachel S., Leopold, Jane A., Lindstrom, Sara, Modena, Brian D., Nichols, William C., Rhodes, Christopher J., Sun, Wei, Sweatt, Andrew J., Vanderpool, Rebecca R., Wilkins, Martin R., Wilmot, Beth, Zamanian, Roham T., Fessel, Joshua P., Aggarwal, Neil R., Loscalzo, Joseph, and Xiao, Lei

Published
2021
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29. Seralutinib in adults with pulmonary arterial hypertension (TORREY): a randomised, double-blind, placebo-controlled phase 2 trial.

Author

Frantz, Robert P, McLaughlin, Vallerie V, Sahay, Sandeep, Escribano Subías, Pilar, Zolty, Ronald L, Benza, Raymond L, Channick, Richard N, Chin, Kelly M, Hemnes, Anna R, Howard, Luke S, Sitbon, Olivier, Vachiéry, Jean-Luc, Zamanian, Roham T, Cravets, Matt, Roscigno, Robert F, Mottola, David, Osterhout, Robin, Bruey, Jean-Marie, Elman, Erin, and Tompkins, Cindy-ann

Subjects
PULMONARY arterial hypertension, C-kit protein, MACROPHAGE colony-stimulating factor, PLATELET-derived growth factor receptors, VASCULAR remodeling, COUGH, PULMONARY hypertension
Abstract

Morbidity and mortality in pulmonary arterial hypertension (PAH) remain high. Activation of platelet-derived growth factor receptor, colony stimulating factor 1 receptor, and mast or stem cell growth factor receptor kinases stimulates inflammatory, proliferative, and fibrotic pathways driving pulmonary vascular remodelling in PAH. Seralutinib, an inhaled kinase inhibitor, targets these pathways. We aimed to evaluate the efficacy and safety of seralutinib in patients with PAH receiving standard background therapy. The TORREY trial was a phase 2, randomised, multicentre, multinational, double-blind, placebo-controlled study. Patients with PAH from 40 hospital and community sites were randomly assigned 1:1 via interactive response technologies to receive seralutinib (60 mg twice daily for 2 weeks, then increased to 90 mg twice daily as tolerated) or placebo by dry powder inhaler twice daily for 24 weeks. Randomisation was stratified by baseline pulmonary vascular resistance (PVR; <800 dyne·s/cm5 and ≥800 dyne·s/cm5). Patients were eligible if classified as WHO Group 1 PH (PAH), WHO Functional Class II or III, with a PVR of 400 dyne·s/cm5 or more, and a 6 min walk distance of between 150 m and 550 m. The primary endpoint was change in PVR from baseline to 24 weeks. Analyses for efficacy endpoints were conducted in randomly assigned patients (intention-to-treat population). Safety analyses included all patients who received the study drug. TORREY was registered with ClinicalTrials.gov (NCT04456998) and EudraCT (2019-002669-37) and is completed. From Nov 12, 2020, to April 20, 2022, 151 patients were screened for eligibility, and following exclusions, 86 adults receiving PAH background therapy were randomly assigned to seralutinib (n=44; four male, 40 female) or placebo (n=42; four male, 38 female), and comprised the intention-to-treat population. At baseline, treatment groups were balanced except for a higher representation of WHO Functional Class II patients in the seralutinib group. The least squares mean change from baseline to week 24 in PVR was 21·2 dyne·s/cm5 (95% CI −37·4 to 79·8) for the placebo group and −74·9 dyne·s/cm5 (−139·7 to −10·2) for the seralutinib group. The least squares mean difference between the seralutinib and placebo groups for change in PVR was −96·1 dyne·s/cm5 (95% CI −183·5 to −8·8; p=0·03). The most common treatment-emergent adverse event in both treatment groups was cough: 16 (38%) of 42 patients in the placebo group; 19 (43%) of 44 patients in the seralutinib group. Treatment with inhaled seralutinib significantly decreased PVR, meeting the primary endpoint of the study among patients receiving background therapy for PAH. Gossamer Bio. [ABSTRACT FROM AUTHOR]

Published
2024
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30. Carnitine consumption and effect of oral supplementation in human pulmonary arterial hypertension: A pilot study.

Author

Brittain, Evan L., Lindsey, Alisha, Burke, Kelly, Agrawal, Vineet, Robbins, Ivan, Pugh, Meredith, Calcutt, M. Wade, Mallugari, Ravi, West, James, Nian, Hui, and Hemnes, Anna R.

Subjects
PULMONARY arterial hypertension, DIETARY supplements, CARNITINE, MITOCHONDRIAL membranes, FOOD consumption
Abstract

Carnitine is required to transport fatty acid across the mitochondrial membrane to undergo beta oxidation. In addition to disorders of fatty acid metabolism, a relative carnitine deficiency has been reported in pulmonary arterial hypertension (PAH). Here we performed an observational study in which food and supplement consumption were collected in an observation period followed by open label administration of a carnitine supplement to determine feasibility of increasing plasma carnitine levels in humans PAH. We confirmed that relative carnitine deficiency in PAH is not due to reduced dietary consumption and that plasma levels of carnitine can be increased in PAH patients with supplementation that is well tolerated. [ABSTRACT FROM AUTHOR]

Published
2024
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31. Spectral analysis of non‐contrast fluoroscopy for evaluation of pulmonary perfusion: Feasibility and sensitivity testing with a phantom.

Author

Smith, Matthew R., Grice, Jared V., Zhou, Dennis W., Emmons, Erica C., Rollins, Allman T., Hemnes, Anna R., O'Leary, Jared M., and Smith, Gary T.

Subjects
FLUOROSCOPY, PULMONARY artery diseases, PERFUSION, FOURIER transforms, DIAGNOSTIC imaging, FREQUENCIES of oscillating systems
Abstract

Background: Oscillating x‐ray attenuation in the lungs provides an opportunity to evaluate pulmonary perfusion without contrast. Recent intensity‐based methods have been compared to pulmonary scintigraphy and CT angiography but lack rigorous phantom studies. Purpose: A new method to quantify the periodic signal amplitude was employed using spectral analysis. Performance was characterized using a water phantom capable of creating an oscillating x‐ray attenuation at physiologic amplitudes. Feasibility in detecting abnormal perfusion was performed on a volunteer with pulmonary vascular disease and compared to pulmonary angiography, the clinical gold standard. Methods: For each fluoroscopic acquisition, the normalized temporal signal from each pixel was decomposed into its frequency components using Fourier transformation, and the spectral amplitude, defined as the x‐ray pulsatility index (XPI), was determined at the desired frequency using a band‐pass filter. XPI was displayed as a pixel‐wise parametric colormap. Based on XPI maps generated using two human volunteers, a water bath phantom was constructed with a fluctuating fluid height and a 1 cm diameter pulsatility defect. Contrast‐to‐noise (CNR) of the defect was measured using fluoroscopy images acquired at variable fluid height fluctuation (0.1–1.9 mm) and oscillation frequency (30–60 bpm). Various sampling frame rates (3–30 fps) and acquisition durations (1.8–8 s) using truncated datasets were reconstructed from full datasets. Fluoroscopic images were obtained in a patient just prior to pulmonary angiography in the same projection. Results: XPI maps in human subjects showed high signal to background contrast with high central XPI measuring up to 0.5. Phantom experiments revealed CNR was linearly correlated to fluid height change (r2 = 0.998). CNR is proportional to increasing sampling frame rate and increasing acquisition duration as expected with Fourier analysis. XPI map displayed multifocal perfusion defects in good agreement with pulmonary angiography. Conclusion: Spectral analysis is an accurate and sensitive method to detect small changes in periodic x‐ray attenuation using a short fluoroscopic acquisition. This method demonstrated good agreement to pulmonary angiography and shows promise for clinical imaging of pulmonary perfusion using standard fluoroscopic methods. [ABSTRACT FROM AUTHOR]

Published
2024
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33. Age-related differences in hemodynamics and functional status in pulmonary arterial hypertension: Baseline results from the Pulmonary Hypertension Association Registry

Author

Allen, Roblee, Badesch, David, Bakshi, Sahil, Bartolome, Sonja, Bull, TM, Burger, Charles D, Cadaret, Linda M, Chakinala, Murali, De Marco, Teresa, Duncan, Michael, Eggert, Michael, Elwing, Jean M., Feldman, Jeremy, Fineman, Jeff, Foley, Raymond J., Ford, Hubert James, Frantz, Robert P, Grinnan, Daniel, Hemnes, Anna R, Hirsch, Russel, Horn, Evelyn M, Ivy, D Dunbar, Kawut, Steven M, Klinger, James R, Lammi, Matthew R., Leary, Peter J, Mathai, Stephen C, Mazimba, Sula, McConnell, John W., Parikh, Kishan S, Presberg, Kenneth W, Raina, Amresh, Ramani, Gautam, Robinson, Jeffrey C, Rosenzweig, Erika B, Runo, James R, Ryan, John J, Sager, Jeffrey S, Shlobin, Oksana A, Simon, Marc A, Swisher, John W, Thenappan, Thenappan, Varghese, Nidhy P, Ventetuolo, Corey E, White, R. James, Williamson, Timothy, Yung, Delphine, Zamanian, Roham T, Zwicke, Dianne L, DesJardin, Jacqueline T., Kolaitis, Nicholas A., Kime, Noah, Kronmal, Richard A., Benza, Raymond L., Presberg, Kenneth W., Sager, Jeffrey S., and Shlobin, Oksana A.

Published
2020
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35. Correlates of Plasma NT‐proBNP/Cyclic GMP Ratio in Heart Failure With Preserved Ejection Fraction: An Analysis of the RELAX Trial

Author

Chiu, Leonard, primary, Agrawal, Vineet, additional, Armstrong, David, additional, Brittain, Evan, additional, Collins, Sheila, additional, Hemnes, Anna R., additional, Hill, Joseph A., additional, Lindenfeld, JoAnn, additional, Shah, Sanjiv J., additional, Stevenson, Lynne W., additional, Wang, Thomas J., additional, and Gupta, Deepak K., additional

Published
2024
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36. Pulmonary primary oxysterol and bile acid synthesis as a predictor of outcomes in pulmonary arterial hypertension

Author

Alotaibi, Mona, primary, Harvey, Lloyd David, additional, Nichols, William, additional, Pauciulo, Michael W, additional, Hemnes, Anna R, additional, Long, Tao, additional, Watrous, Jeramie D, additional, Begzati, Arjana, additional, Tuomilehto, Jaakko, additional, Havulinna, Aki S, additional, Niiranen, Teemu, additional, Jousilahti, Pekka, additional, Salomaa, Veikko, additional, Bertero, Thomas, additional, Kim, Nick H, additional, Desai, Ankit A, additional, Malhotra, Atul, additional, Yuan, Jason X.-J., additional, Cheng, Susan, additional, Chan, Stephen Y, additional, and Jain, Mohit, additional

Published
2024
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39. A roadmap for therapeutic discovery in pulmonary hypertension associated with left heart failure. A scientific statement of the Heart Failure Association (HFA) of the ESC and the ESC Working Group on Pulmonary Circulation & Right Ventricular Function.

Author

Ameri, Pietro, Mercurio, Valentina, Pollesello, Piero, Anker, Markus S., Backs, Johannes, Bayes‐Genis, Antoni, Borlaug, Barry A., Burkhoff, Daniel, Caravita, Sergio, Chan, Stephen Y., de Man, Frances, Giannakoulas, George, González, Aránzazu, Guazzi, Marco, Hassoun, Paul M., Hemnes, Anna R., Maack, Cristoph, Madden, Brendan, Melenovsky, Vojtech, and Müller, Oliver J.

Subjects
PULMONARY circulation, PULMONARY arterial hypertension, HEART failure, PULMONARY hypertension
Abstract

Pulmonary hypertension (PH) associated with left heart failure (LHF) (PH‐LHF) is one of the most common causes of PH. It directly contributes to symptoms and reduced functional capacity and negatively affects right heart function, ultimately leading to a poor prognosis. There are no specific treatments for PH‐LHF, despite the high number of drugs tested so far. This scientific document addresses the main knowledge gaps in PH‐LHF with emphasis on pathophysiology and clinical trials. Key identified issues include better understanding of the role of pulmonary venous versus arteriolar remodelling, multidimensional phenotyping to recognize patient subgroups positioned to respond to different therapies, and conduct of rigorous pre‐clinical studies combining small and large animal models. Advancements in these areas are expected to better inform the design of clinical trials and extend treatment options beyond those effective in pulmonary arterial hypertension. Enrichment strategies, endpoint assessments, and thorough haemodynamic studies, both at rest and during exercise, are proposed to play primary roles to optimize early‐stage development of candidate therapies for PH‐LHF. [ABSTRACT FROM AUTHOR]

Published
2024
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40. Myeloid Cell Derived IL1β Contributes to Pulmonary Hypertension in HFpEF

Author

Agrawal, Vineet, primary, Kropski, Jonathan A., additional, Gokey, Jason J., additional, Kobeck, Elizabeth, additional, Murphy, Matthew B., additional, Murray, Katherine T., additional, Fortune, Niki L., additional, Moore, Christy S., additional, Meoli, David F., additional, Monahan, Ken, additional, Ru Su, Yan, additional, Blackwell, Thomas, additional, Gupta, Deepak K., additional, Talati, Megha H., additional, Gladson, Santhi, additional, Carrier, Erica J., additional, West, James D., additional, and Hemnes, Anna R., additional

Published
2023
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41. Sleep-Related Hypoxia, Right Ventricular Dysfunction, and Survival in Patients With Group 1 Pulmonary Arterial Hypertension

Author

Lowery, Megan M., primary, Hill, Nicholas S., additional, Wang, Lu, additional, Rosenzweig, Erika B., additional, Bhat, Aparna, additional, Erzurum, Serpil, additional, Finet, J. Emanuel, additional, Jellis, Christine L., additional, Kaur, Sunjeet, additional, Kwon, Deborah H., additional, Nawabit, Rawan, additional, Radeva, Milena, additional, Beck, Gerald J., additional, Frantz, Robert P., additional, Hassoun, Paul M., additional, Hemnes, Anna R., additional, Horn, Evelyn M., additional, Leopold, Jane A., additional, Rischard, Franz P., additional, Mehra, Reena, additional, Hill, N., additional, Xiao, L., additional, Fu, Y.-P., additional, Postow, L., additional, Schmetter, B., additional, Stanton, K., additional, Tian, X., additional, Gray, M., additional, Wong, B., additional, Leopold, J., additional, Waxman, A., additional, DiCarli, M., additional, Lawler, L., additional, Maron, B., additional, Segrera, S., additional, Systrom, D., additional, Yu, P., additional, Rosenzweig, E.B., additional, Arcasoy, S., additional, Brady, D., additional, Chung, W., additional, Payne, D., additional, Grunig, G., additional, Haythe, J., additional, Krishnan, U., additional, Horn, E., additional, Akat, K., additional, Borczuk, A., additional, Devereux, R., additional, Gordon, J., additional, Kaner, R., additional, Karas, M., additional, Min, J., additional, Narula, N., additional, Ricketts, M., additional, Sobol, I., additional, Spiera, R., additional, Singh, H., additional, Tuschl, T., additional, Weinsaft, J., additional, Hassoun, P., additional, Mathai, S., additional, Barnes, K., additional, Damico, R., additional, Enobun, B., additional, Gao, L., additional, Halushka, M., additional, Kass, D., additional, Kolb, T., additional, Lin, T., additional, Tedford, R., additional, Zimmerman, S., additional, Frantz, R., additional, Behfar, A., additional, Block, L., additional, Borlaug, B., additional, Durst, L., additional, Foley, T., additional, Hammer, T., additional, Johnson, B., additional, Johnson, G., additional, Kane, G., additional, Krowka, M., additional, McNallan, A., additional, Olson, T., additional, Redfield, M., additional, Rohwer, K., additional, Terzic, A., additional, Williamson, E., additional, Rischard, F., additional, Yuan, J., additional, Abidov, A., additional, Garcia, J., additional, Cordery, A., additional, Desai, A., additional, Erickson, H., additional, Hansen, L., additional, Khalpey, Z., additional, Knox, K., additional, Lussier, Y., additional, Simon, M., additional, Vanderpool, R., additional, Hemnes, A., additional, Newman, J., additional, Austin, E., additional, Brittain, E., additional, Cunningham, J., additional, LaRochelle, C., additional, Pugh, M., additional, Robbins, I., additional, Wheeler, L., additional, Beck, G., additional, Erzurum, S., additional, Aldred, M., additional, Asosingh, K., additional, Barnard, J., additional, Collart, C., additional, Comhair, S., additional, DiFilippo, F., additional, Drinko, J., additional, Dweik, R., additional, Flinn, A., additional, Geraci, M., additional, Hu, B., additional, Jaber, W., additional, Jacob, M., additional, Jellis, C., additional, Kalhan, S., additional, Kassimatis, K., additional, Kirsop, J., additional, Koo, M., additional, Kwon, D., additional, Larive, B., additional, Lempel, J., additional, Li, M., additional, MacKrell, J., additional, Matuska, B., additional, McCarthy, K., additional, Mehra, R., additional, Neumann, D., additional, Nawabit, R., additional, Olman, M., additional, Park, M., additional, Radeva, M., additional, Sharp, J., additional, Sherer, S., additional, Tang, W., additional, Thomas, J., additional, Wiggins, K., additional, Willard, B., additional, Rounds, S., additional, Benza, R., additional, Bull, T., additional, Cadigan, J., additional, Fang, J., additional, Gomberg-Maitland, M., additional, and Page, G., additional

Published
2023
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42. Kynurenine pathway metabolism evolves with development of preclinical and scleroderma-associated pulmonary arterial hypertension

Author

Simpson, Catherine E., primary, Ambade, Anjira S., additional, Harlan, Robert, additional, Roux, Aurelie, additional, Aja, Susan, additional, Graham, David, additional, Shah, Ami A., additional, Hummers, Laura K., additional, Hemnes, Anna R., additional, Leopold, Jane A., additional, Horn, Evelyn M., additional, Berman-Rosenzweig, Erika S., additional, Grunig, Gabrielle, additional, Aldred, Micheala A., additional, Barnard, John, additional, Comhair, Suzy A.A., additional, Tang, W. H. Wilson, additional, Griffiths, Megan, additional, Rischard, Franz, additional, Frantz, Robert P., additional, Erzurum, Serpil C., additional, Beck, Gerald J., additional, Hill, Nicholas S., additional, Mathai, Stephen C., additional, Hassoun, Paul M., additional, and Damico, Rachel L., additional

Published
2023
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43. DIFFUSING CAPACITY FOR CARBON MONOXIDE IN PULMONARY VASCULAR DISEASE: INSIGHTS FROM THE PVDOMICS STUDY

Author

PADMANABHAN MENON, DIVYA, primary, FRANTZ, ROBERT P, additional, RISCHARD, FRANZ P, additional, HASSOUN, PAUL M, additional, GOCHANOUR, BENJAMIN, additional, MATHAI, STEPHEN C, additional, HILL, NICHOLAS S, additional, HEMNES, ANNA R, additional, EMANUEL FINET, J., additional, FARHA, SAMAR, additional, BECK, GERALD, additional, MCCARTHY, KEVIN, additional, OLMAN, MITCHELL A, additional, HORN, EVELYN M, additional, GRUNIG, GABRIELE, additional, CEM ERZURUM, SERPIL, additional, BORLAUG, BARRY, additional, and DUBROCK, HILARY M, additional

Published
2023
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44. GROUND-GLASS OPACITIES IN GROUP 1 PULMONARY HYPERTENSION: FINDINGS FROM THE PVDOMICS STUDY

Author

PADMANABHAN MENON, DIVYA, primary, FRANTZ, ROBERT P, additional, RISCHARD, FRANZ P, additional, HASSOUN, PAUL M, additional, GOCHANOUR, BENJAMIN, additional, MATHAI, STEPHEN C, additional, HILL, NICHOLAS S, additional, HEMNES, ANNA R, additional, EMANUEL FINET, J., additional, LEMPEL, JASON, additional, FARHA, SAMAR, additional, WAXMAN, AARON B, additional, HORN, EVELYN M, additional, RENAPURKAR, RAHUL, additional, GRUNIG, GABRIELE, additional, CEM ERZURUM, SERPIL, additional, BORLAUG, BARRY, additional, and DUBROCK, HILARY M, additional

Published
2023
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45. RIGHT VENTRICULAR VOLUME INTER-READER VARIABILITY INCREASES WITH VENTRICULAR DILATION: RESULTS FROM THE PVDOMICS STUDY

Author

TAN, RACHEL, primary, KWON, DEBORAH, additional, ACHARYA, TUSHAR, additional, PARK, MARGARET, additional, VANDERPOOL, REBECCA, additional, KATRYNUIK, AUSTIN, additional, BECK, GERALD, additional, FRANTZ, ROBERT P, additional, HASSOUN, PAUL M, additional, HEMNES, ANNA R, additional, HILL, NICHOLAS S, additional, HORN, EVELYN M, additional, LEOPOLD, JANE, additional, BERMAN ROSENZWEIG, ERIKA, additional, H. WILSON TANG, W., additional, MATHAI, STEPHEN C, additional, ZIMMERMAN, STEFAN, additional, AMBALE VENKATESH, BHARATH, additional, KIM, JIWON, additional, DILORENZO, MICHAEL, additional, KWONG, RAYMOND, additional, FRANCOIS, CHRISTOPHER, additional, BRITTAIN, EVAN, additional, and RISCHARD, FRANZ P, additional

Published
2023
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46. CHARACTERISTICS OF PATIENTS WITH PULMONARY ARTERIAL HYPERTENSION RECEIVING SELEXIPAG IN THE SPHERE REGISTRY BY RACE AND ETHNICITY

Author

FARBER, HARRISON, primary, CHAKINALA, MURALI M, additional, HEMNES, ANNA R, additional, CHIN, KELLY, additional, HIGHLAND, KRISTIN B, additional, MCLAUGHLIN, VALLERIE, additional, MELENDRES-GROVES, LANA, additional, CHO, MICHELLE, additional, DOAD, GURINDERPAL, additional, FATEHI, ELHAM, additional, HAN, MICHELLE, additional, RAHMAN, MOHAMMAD, additional, STRACHAN, PAUL M, additional, TOBORE, TOBORE, additional, and KIM, NICK H, additional

Published
2023
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50. Disease characteristics, treatments, and outcomes of patients with pulmonary arterial hypertension treated with selexipag in real-world settings from the SPHERE registry (SelexiPag: tHe usErs dRug rEgistry)

Author

McLaughlin, Vallerie, primary, Farber, Harrison W., additional, Highland, Kristin B., additional, Hemnes, Anna R., additional, Chakinala, Murali M., additional, Chin, Kelly M., additional, Han, Michelle, additional, Cho, Michelle, additional, Tobore, Tobore, additional, Rahman, Mohammad, additional, and Kim, Nick H., additional

Published
2023
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