{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,16]],"date-time":"2026-06-16T23:52:50Z","timestamp":1781653970929,"version":"3.54.5"},"reference-count":45,"publisher":"Springer Science and Business Media LLC","issue":"1","license":[{"start":{"date-parts":[[2026,6,8]],"date-time":"2026-06-08T00:00:00Z","timestamp":1780876800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"},{"start":{"date-parts":[[2026,6,8]],"date-time":"2026-06-08T00:00:00Z","timestamp":1780876800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"}],"funder":[{"DOI":"10.13039\/100000050","name":"National Heart, Lung, and Blood Institute","doi-asserted-by":"publisher","award":["R01HL172830"],"award-info":[{"award-number":["R01HL172830"]}],"id":[{"id":"10.13039\/100000050","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000050","name":"National Heart, Lung, and Blood Institute","doi-asserted-by":"publisher","award":["R01HL162851"],"award-info":[{"award-number":["R01HL162851"]}],"id":[{"id":"10.13039\/100000050","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000050","name":"National Heart, Lung, and Blood Institute","doi-asserted-by":"publisher","award":["R01HL162851"],"award-info":[{"award-number":["R01HL162851"]}],"id":[{"id":"10.13039\/100000050","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000090","name":"Congressionally Directed Medical Research Programs","doi-asserted-by":"publisher","award":["PR191839"],"award-info":[{"award-number":["PR191839"]}],"id":[{"id":"10.13039\/100000090","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000090","name":"Congressionally Directed Medical Research Programs","doi-asserted-by":"publisher","award":["PR191839"],"award-info":[{"award-number":["PR191839"]}],"id":[{"id":"10.13039\/100000090","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000090","name":"Congressionally Directed Medical Research Programs","doi-asserted-by":"publisher","award":["PR231648"],"award-info":[{"award-number":["PR231648"]}],"id":[{"id":"10.13039\/100000090","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000069","name":"National Institute of Arthritis and Musculoskeletal and Skin Diseases","doi-asserted-by":"publisher","award":["K24080217"],"award-info":[{"award-number":["K24080217"]}],"id":[{"id":"10.13039\/100000069","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100019921","name":"National Scleroderma Foundation","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100019921","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Echo Res Pract"],"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>Systemic sclerosis (SSc) is a multisystem autoimmune disease frequently complicated by pre- and post-capillary pulmonary hypertension (PH). Within SSc, progressive diastolic dysfunction and heart failure with preserved ejection fraction (HFpEF) are key contributors, often presenting as isolated postcapillary PH (Ipc-PH) or combined pre- and postcapillary PH (Cpc-PH). The ability to differentiate these hemodynamic phenotypes is critical for risk stratification, yet echocardiographic markers specific to each subtype in SSc-HFpEF are poorly defined.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>\n                      We investigated 147 adults with SSc-HFpEF with echocardiograms and right heart catheterization (RHC) assessments performed within one year. Patients were classified as Ipc-PH (\n                      <jats:italic>n<\/jats:italic>\n                      \u2009=\u200946) or Cpc-PH (\n                      <jats:italic>n<\/jats:italic>\n                      \u2009=\u2009101) based on guideline-defined hemodynamic criteria. Echocardiographic parameters, including conventional measures, strain indices, and coupling metrics were analyzed. A random forest (RF) classifier was used to identify top echocardiographic predictors of Cpc-PH, and further assessed using multivariable logistic regression. Separate RF models and Cox regression analyses were used to determine echocardiographic predictors of mortality within each group. Survival was assessed using Kaplan-Meier analysis.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>Patients with Cpc-PH exhibited significantly greater right heart remodeling, higher pulmonary pressures, and impaired right ventricle (RV)-pulmonary artery (PA) coupling. The top echocardiographic predictors of Cpc-PH included reduced RV free wall strain (RVFWS), decreased RVFWS\/PA systolic pressure (PASP) and fractional area change (FAC)\/PASP ratios, elevated PASP, lower septal e\u2032 velocity, and higher systolic LV eccentricity index (LV EI). In adjusted Cox models, elevated LVEI (HR 1.39), increased RV internal diastolic diameter (HR 2.15), and reduced left and right atrial strain (HR 0.91 and 0.94, respectively) were independently associated with mortality in Cpc-PH. In Ipc-PH, mortality was linked to reduced FAC\/PASP, lower left ventricular global longitudinal strain (LVGLS), increased LV mass index, elevated PASP, and lower RVFWS.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>In the present study, we demonstrate key echocardiographic differences between Ipc-PH and Cpc-PH within the SSc-HFpEF population, emphasizing the central role of right heart remodeling, RV-PA coupling, atrial and septal mechanics in phenotypic differentiation and prognostication. Strain-based parameters and RV-PA coupling indices offer incremental value for risk stratification and may guide more tailored therapeutic strategies in this heterogeneous population.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1186\/s44156-026-00116-4","type":"journal-article","created":{"date-parts":[[2026,6,7]],"date-time":"2026-06-07T23:01:53Z","timestamp":1780873313000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Echocardiographic characterization of combined postcapillary and precapillary pulmonary hypertension in systemic sclerosis with preserved ejection fraction"],"prefix":"10.1186","volume":"13","author":[{"given":"Ahmad","family":"Daoud","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Garrett","family":"Goldin","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lea","family":"Goren","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Arindam","family":"Bagga","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hoda","family":"Mombeini","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ryan","family":"Osgueritchian","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Vivek P.","family":"Jani","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Steven","family":"Hsu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fredrick M.","family":"Wigley","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Matthew R.","family":"Lammi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Paul M.","family":"Hassoun","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ami A.","family":"Shah","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stephen C.","family":"Mathai","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Monica","family":"Mukherjee","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2026,6,8]]},"reference":[{"key":"116_CR1","doi-asserted-by":"publisher","first-page":"68","DOI":"10.1007\/s11926-019-0867-0","volume":"21","author":"G Hung","year":"2019","unstructured":"Hung G, Mercurio V, Hsu S, Mathai SC, Shah AA, Mukherjee M. Progress in Understanding, Diagnosing, and Managing Cardiac Complications of Systemic Sclerosis. Curr Rheumatol Rep. 2019;21:68. https:\/\/doi.org\/10.1007\/s11926-019-0867-0.","journal-title":"Curr Rheumatol Rep"},{"key":"116_CR2","doi-asserted-by":"publisher","first-page":"3","DOI":"10.1007\/s11886-024-02164-w","volume":"27","author":"R Osgueritchian","year":"2025","unstructured":"Osgueritchian R, Mombeini H, Jani VP, Hsu S, Hummers LK, Wigley FM, Mathai SC, Shah AA, Mukherjee M. Myocardial Disease in Systemic Sclerosis: Recent Updates and Clinical Implications. Curr Cardiol Rep. 2025;27:3. https:\/\/doi.org\/10.1007\/s11886-024-02164-w.","journal-title":"Curr Cardiol Rep"},{"key":"116_CR3","doi-asserted-by":"publisher","first-page":"3618","DOI":"10.1093\/eurheartj\/ehac237","volume":"43","author":"M Humbert","year":"2022","unstructured":"Humbert M, Kovacs G, Hoeper MM, Badagliacca R, Berger RM, Brida M, Carlsen J, Coats AJ, Escribano-Subias P, Ferrari P. 2022 ESC\/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension: Developed by the task force for the diagnosis and treatment of pulmonary hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS). Endorsed by the International Society for Heart and Lung Transplantation (ISHLT) and the European Reference Network on rare respiratory diseases (ERN-LUNG). Eur Heart J. 2022;43:3618\u2013731.","journal-title":"Eur Heart J"},{"key":"116_CR4","doi-asserted-by":"publisher","unstructured":"Haque A, Kiely DG, Kovacs G, Thompson AAR, Condliffe R. Pulmonary hypertension phenotypes in patients with systemic sclerosis. Eur Respir Rev. 2021;30. https:\/\/doi.org\/10.1183\/16000617.0053-2021.","DOI":"10.1183\/16000617.0053-2021"},{"key":"116_CR5","doi-asserted-by":"publisher","unstructured":"Launay D, Sobanski V, Hachulla E, Humbert M. Pulmonary hypertension in systemic sclerosis: different phenotypes. Eur Respir Rev. 2017;26. https:\/\/doi.org\/10.1183\/16000617.0056-2017.","DOI":"10.1183\/16000617.0056-2017"},{"key":"116_CR6","doi-asserted-by":"publisher","first-page":"1340","DOI":"10.1136\/annrheumdis-2013-203301","volume":"73","author":"JG Coghlan","year":"2014","unstructured":"Coghlan JG, Denton CP, Grunig E, Bonderman D, Distler O, Khanna D, Muller-Ladner U, Pope JE, Vonk MC, Doelberg M, et al. Evidence-based detection of pulmonary arterial hypertension in systemic sclerosis: the DETECT study. Ann Rheum Dis. 2014;73:1340\u20139. https:\/\/doi.org\/10.1136\/annrheumdis-2013-203301.","journal-title":"Ann Rheum Dis"},{"key":"116_CR7","doi-asserted-by":"publisher","first-page":"312","DOI":"10.1164\/rccm.202204-0731OC","volume":"207","author":"HJ Hassan","year":"2023","unstructured":"Hassan HJ, Naranjo M, Ayoub N, Housten T, Hsu S, Balasubramanian A, Simpson CE, Damico RL, Mathai SC, Kolb TM, et al. Improved Survival for Patients with Systemic Sclerosis-associated Pulmonary Arterial Hypertension: The Johns Hopkins Registry. Am J Respir Crit Care Med. 2023;207:312\u201322. https:\/\/doi.org\/10.1164\/rccm.202204-0731OC.","journal-title":"Am J Respir Crit Care Med"},{"key":"116_CR8","doi-asserted-by":"publisher","first-page":"849","DOI":"10.1002\/art.42054","volume":"74","author":"AM Hinze","year":"2022","unstructured":"Hinze AM, Perin J, Woods A, Hummers LK, Wigley FM, Mukherjee M, Shah AA. Diastolic Dysfunction in Systemic Sclerosis: Risk Factors and Impact on Mortality. Arthritis Rheumatol. 2022;74:849\u201359. https:\/\/doi.org\/10.1002\/art.42054.","journal-title":"Arthritis Rheumatol"},{"key":"116_CR9","first-page":"S30","volume":"30","author":"M Hinchcliff","year":"2012","unstructured":"Hinchcliff M, Desai CS, Varga J, Shah SJ. Prevalence, prognosis, and factors associated with left ventricular diastolic dysfunction in systemic sclerosis. Clin Exp Rheumatol. 2012;30:S30\u201337.","journal-title":"Clin Exp Rheumatol"},{"key":"116_CR10","doi-asserted-by":"publisher","first-page":"1804","DOI":"10.1016\/j.jacc.2018.07.068","volume":"72","author":"AH Tenn\u00f8e","year":"2018","unstructured":"Tenn\u00f8e AH, Murbr\u00e6ch K, Andreassen JC, Fretheim H, Garen T, Gude E, Andreassen A, Aakhus S, Molberg \u00d8, Hoffmann-Vold A-M. Left ventricular diastolic dysfunction predicts mortality in patients with systemic sclerosis. J Am Coll Cardiol. 2018;72:1804\u201313.","journal-title":"J Am Coll Cardiol"},{"key":"116_CR11","doi-asserted-by":"publisher","first-page":"409","DOI":"10.1177\/2045893217700438","volume":"7","author":"KI Bourji","year":"2017","unstructured":"Bourji KI, Kelemen BW, Mathai SC, Damico RL, Kolb TM, Mercurio V, Cozzi F, Tedford RJ, Hassoun PM. Poor survival in patients with scleroderma and pulmonary hypertension due to heart failure with preserved ejection fraction. Pulmonary Circulation. 2017;7:409\u201320.","journal-title":"Pulmonary Circulation"},{"key":"116_CR12","doi-asserted-by":"publisher","first-page":"e12249","DOI":"10.1002\/pul2.12249","volume":"13","author":"G Babu","year":"2023","unstructured":"Babu G, Annis JS, Garry JD, Freiberg MS, Hemnes AR, Brittain EL. Clinical features do not identify risk of progression from isolated postcapillary pulmonary hypertension to combined pre-and postcapillary pulmonary hypertension. Pulmonary Circulation. 2023;13:e12249.","journal-title":"Pulmonary Circulation"},{"key":"116_CR13","doi-asserted-by":"publisher","first-page":"141","DOI":"10.1016\/j.echo.2025.01.006","volume":"38","author":"M Mukherjee","year":"2025","unstructured":"Mukherjee M, Rudski LG, Addetia K, Afilalo J, D\u2019Alto M, Freed BH, Friend LB, Gargani L, Grapsa J, Hassoun PM, et al. Guidelines for the Echocardiographic Assessment of the Right Heart in Adults and Special Considerations in Pulmonary Hypertension: Recommendations from the American Society of Echocardiography. J Am Soc Echocardiogr. 2025;38:141\u201386. https:\/\/doi.org\/10.1016\/j.echo.2025.01.006.","journal-title":"J Am Soc Echocardiogr"},{"key":"116_CR14","doi-asserted-by":"publisher","DOI":"10.1016\/j.echo.2025.07.013","author":"R Osgueritchian","year":"2025","unstructured":"Osgueritchian R, Jani VP, Mombeini H, Gami A, Goldin G, Hsu S, Hummers LK, Wigley FM, Lammi MR, Ambale-Venkatesh B, et al. Refining Risk Prediction in Systemic Sclerosis-Associated Pulmonary Arterial Hypertension. J Am Soc Echocardiogr. 2025. https:\/\/doi.org\/10.1016\/j.echo.2025.07.013.","journal-title":"J Am Soc Echocardiogr"},{"key":"116_CR15","doi-asserted-by":"publisher","first-page":"2737","DOI":"10.1002\/art.38098","volume":"65","author":"F van den Hoogen","year":"2013","unstructured":"van den Hoogen F, Khanna D, Fransen J, Johnson SR, Baron M, Tyndall A, Matucci-Cerinic M, Naden RP, Medsger TA Jr., Carreira PE, et al. 2013 classification criteria for systemic sclerosis: an American College of Rheumatology\/European League against Rheumatism collaborative initiative. Arthritis Rheum. 2013;65:2737\u201347. https:\/\/doi.org\/10.1002\/art.38098.","journal-title":"Arthritis Rheum"},{"key":"116_CR16","first-page":"202","volume":"15","author":"EC LeRoy","year":"1988","unstructured":"LeRoy EC, Black C, Fleischmajer R, Jablonska S, Krieg T, Medsger TA Jr., Rowell N, Wollheim F. Scleroderma (systemic sclerosis): classification, subsets and pathogenesis. J Rheumatol. 1988;15:202\u20135.","journal-title":"J Rheumatol"},{"key":"116_CR17","doi-asserted-by":"publisher","unstructured":"Kovacs G, Bartolome S, Denton CP, Gatzoulis MA, Gu S, Khanna D, Badesch D, Montani D. Definition, classification and diagnosis of pulmonary hypertension. Eur Respir J. 2024;64. https:\/\/doi.org\/10.1183\/13993003.01324-2024.","DOI":"10.1183\/13993003.01324-2024"},{"key":"116_CR18","doi-asserted-by":"publisher","first-page":"233","DOI":"10.1093\/ehjci\/jev014","volume":"16","author":"RM Lang","year":"2015","unstructured":"Lang RM, Badano LP, Mor-Avi V, Afilalo J, Armstrong A, Ernande L, Flachskampf FA, Foster E, Goldstein SA, Kuznetsova T, et al. Recommendations for cardiac chamber quantification by echocardiography in adults: an update from the American Society of Echocardiography and the European Association of Cardiovascular Imaging. Eur Heart J Cardiovasc Imaging. 2015;16:233\u201370. https:\/\/doi.org\/10.1093\/ehjci\/jev014.","journal-title":"Eur Heart J Cardiovasc Imaging"},{"key":"116_CR19","doi-asserted-by":"publisher","DOI":"10.1016\/j.echo.2025.07.007","author":"JD Thomas","year":"2025","unstructured":"Thomas JD, Edvardsen T, Abraham T, Appadurai V, Badano L, Banchs J, Cho GY, Cosyns B, Delgado V, Donal E, et al. Clinical Applications of Strain Echocardiography: A Clinical Consensus Statement From the American Society of Echocardiography Developed in Collaboration With the European Association of Cardiovascular Imaging of the European Society of Cardiology. J Am Soc Echocardiogr. 2025. https:\/\/doi.org\/10.1016\/j.echo.2025.07.007.","journal-title":"J Am Soc Echocardiogr"},{"key":"116_CR20","doi-asserted-by":"publisher","first-page":"1516","DOI":"10.1016\/j.jcmg.2023.08.011","volume":"16","author":"J Nyberg","year":"2023","unstructured":"Nyberg J, Jakobsen EO, Ostvik A, Holte E, Stolen S, Lovstakken L, Grenne B, Dalen H. Echocardiographic Reference Ranges of Global Longitudinal Strain for All Cardiac Chambers Using Guideline-Directed Dedicated Views. JACC Cardiovasc Imaging. 2023;16:1516\u201331. https:\/\/doi.org\/10.1016\/j.jcmg.2023.08.011.","journal-title":"JACC Cardiovasc Imaging"},{"key":"116_CR21","doi-asserted-by":"publisher","first-page":"112","DOI":"10.1093\/bioinformatics\/btr597","volume":"28","author":"DJ Stekhoven","year":"2012","unstructured":"Stekhoven DJ, B\u00fchlmann P. MissForest\u2014non-parametric missing value imputation for mixed-type data. Bioinformatics. 2012;28:112\u20138.","journal-title":"Bioinformatics"},{"key":"116_CR22","doi-asserted-by":"publisher","unstructured":"Vonk Noordegraaf A, Chin KM, Haddad F, Hassoun PM, Hemnes AR, Hopkins SR, Kawut SM, Langleben D, Lumens J, Naeije R. Pathophysiology of the right ventricle and of the pulmonary circulation in pulmonary hypertension: an update. Eur Respir J. 2019;53. https:\/\/doi.org\/10.1183\/13993003.01900-2018.","DOI":"10.1183\/13993003.01900-2018"},{"key":"116_CR23","doi-asserted-by":"publisher","unstructured":"Tello K, Ghofrani HA, Heinze C, Krueger K, Naeije R, Raubach C, Seeger W, Sommer N, Gall H, Richter MJ. A simple echocardiographic estimate of right ventricular-arterial coupling to assess severity and outcome in pulmonary hypertension on chronic lung disease. Eur Respir J. 2019;54. https:\/\/doi.org\/10.1183\/13993003.02435-2018.","DOI":"10.1183\/13993003.02435-2018"},{"key":"116_CR24","doi-asserted-by":"publisher","first-page":"H1373","DOI":"10.1152\/ajpheart.00157.2013","volume":"305","author":"M Guazzi","year":"2013","unstructured":"Guazzi M, Bandera F, Pelissero G, Castelvecchio S, Menicanti L, Ghio S, Temporelli P, Arena R. Tricuspid annular plane systolic excursion and pulmonary arterial systolic pressure relationship in heart failure: an index of right ventricular contractile function and prognosis. Am J Physiol Heart Circ Physiol. 2013;305:H1373\u201381.","journal-title":"Am J Physiol Heart Circ Physiol"},{"key":"116_CR25","doi-asserted-by":"publisher","first-page":"41","DOI":"10.1016\/j.amjcard.2023.03.016","volume":"196","author":"M Mukherjee","year":"2023","unstructured":"Mukherjee M, Ogunmoroti O, Jani V, Kapoor K, Beussink-Nelson L, Freed BH, Hays AG, Shah SJ, Michos ED. Characteristics of Right Ventricular to Pulmonary Arterial Coupling and Association With Functional Status Among Older Aged Adults from the Multi-Ethnic Study of Atherosclerosis. Am J Cardiol. 2023;196:41\u201351. https:\/\/doi.org\/10.1016\/j.amjcard.2023.03.016.","journal-title":"Am J Cardiol"},{"key":"116_CR26","doi-asserted-by":"publisher","DOI":"10.1111\/echo.15432","author":"V Jani","year":"2022","unstructured":"Jani V, Kapoor K, Meyer J, Lu J, Goerlich E, Metkus TS, Madrazo JA, Michos E, Wu K, Bavaro N, et al. Unsupervised machine learning demonstrates the prognostic value of TAPSE\/PASP ratio among hospitalized patients with COVID-19. Echocardiography. 2022. https:\/\/doi.org\/10.1111\/echo.15432.","journal-title":"Echocardiography"},{"key":"116_CR27","doi-asserted-by":"publisher","first-page":"e009047","DOI":"10.1161\/CIRCIMAGING.119.009047","volume":"12","author":"K Tello","year":"2019","unstructured":"Tello K, Wan J, Dalmer A, Vanderpool R, Ghofrani HA, Naeije R, Roller F, Mohajerani E, Seeger W, Herberg U, et al. Validation of the Tricuspid Annular Plane Systolic Excursion\/Systolic Pulmonary Artery Pressure Ratio for the Assessment of Right Ventricular-Arterial Coupling in Severe Pulmonary Hypertension. Circ Cardiovasc Imaging. 2019;12:e009047. https:\/\/doi.org\/10.1161\/CIRCIMAGING.119.009047.","journal-title":"Circ Cardiovasc Imaging"},{"key":"116_CR28","doi-asserted-by":"publisher","first-page":"425","DOI":"10.1093\/ehjci\/jex133","volume":"19","author":"TM Gorter","year":"2018","unstructured":"Gorter TM, van Veldhuisen DJ, Voors AA, Hummel YM, Lam CSP, Berger RMF, van Melle JP, Hoendermis ES. Right ventricular-vascular coupling in heart failure with preserved ejection fraction and pre- vs. post-capillary pulmonary hypertension. Eur Heart J Cardiovasc Imaging. 2018;19:425\u201332. https:\/\/doi.org\/10.1093\/ehjci\/jex133.","journal-title":"Eur Heart J Cardiovasc Imaging"},{"key":"116_CR29","doi-asserted-by":"publisher","DOI":"10.1016\/j.echo.2024.09.010","author":"A Gami","year":"2024","unstructured":"Gami A, Jani VP, Mombeini H, Osgueritchian R, Cubero Salazar IM, Kauffman M, Simpson CE, Damico RL, Kolb TM, Shah AA, et al. Prognostic Value of Echocardiographic Coupling Metrics in Systemic Sclerosis-Associated Pulmonary Vascular Disease. J Am Soc Echocardiogr. 2024. https:\/\/doi.org\/10.1016\/j.echo.2024.09.010.","journal-title":"J Am Soc Echocardiogr"},{"key":"116_CR30","doi-asserted-by":"publisher","first-page":"873","DOI":"10.1002\/ejhf.664","volume":"19","author":"S Ghio","year":"2017","unstructured":"Ghio S, Guazzi M, Scardovi AB, Klersy C, Clemenza F, Carluccio E, Temporelli PL, Rossi A, Faggiano P, Traversi E, et al. Different correlates but similar prognostic implications for right ventricular dysfunction in heart failure patients with reduced or preserved ejection fraction. Eur J Heart Fail. 2017;19:873\u20139. https:\/\/doi.org\/10.1002\/ejhf.664.","journal-title":"Eur J Heart Fail"},{"key":"116_CR31","doi-asserted-by":"publisher","first-page":"1518","DOI":"10.1016\/j.healun.2023.07.004","volume":"42","author":"ZA Rako","year":"2023","unstructured":"Rako ZA, Yogeswaran A, Lakatos BK, Fabian A, Yildiz S, da Rocha BB, Vadasz I, Ghofrani HA, Seeger W, Gall H, et al. Clinical and functional relevance of right ventricular contraction patterns in pulmonary hypertension. J Heart Lung Transpl. 2023;42:1518\u201328. https:\/\/doi.org\/10.1016\/j.healun.2023.07.004.","journal-title":"J Heart Lung Transpl"},{"key":"116_CR32","doi-asserted-by":"publisher","DOI":"10.1016\/j.amjcard.2024.03.043","author":"VP Jani","year":"2024","unstructured":"Jani VP, Strom JB, Gami A, Beussink-Nelson L, Patel R, Michos ED, Shah SJ, Freed BH, Mukherjee M. Optimal Method for Assessing Right Ventricular to Pulmonary Arterial Coupling and Subclinical Right Ventricular Dysfunction in Older Aged Healthy Adults: The Multi-Ethnic Study of Atherosclerosis. Am J Cardiol. 2024. https:\/\/doi.org\/10.1016\/j.amjcard.2024.03.043.","journal-title":"Am J Cardiol"},{"key":"116_CR33","doi-asserted-by":"publisher","first-page":"e017074","DOI":"10.1161\/CIRCIMAGING.124.017074","volume":"17","author":"M Mukherjee","year":"2024","unstructured":"Mukherjee M, Mathai SC, Jellis C, Freed BH, Yanek LR, Agoglia H, Chiu C, Jani VP, Simpson CE, Brittain EL, et al. Defining Echocardiographic Degrees of Right Heart Size and Function in Pulmonary Vascular Disease From the PVDOMICS Study. Circ Cardiovasc Imaging. 2024;17:e017074. https:\/\/doi.org\/10.1161\/CIRCIMAGING.124.017074.","journal-title":"Circ Cardiovasc Imaging"},{"key":"116_CR34","doi-asserted-by":"publisher","first-page":"395","DOI":"10.1016\/j.healun.2014.11.002","volume":"34","author":"R Badagliacca","year":"2015","unstructured":"Badagliacca R, Poscia R, Pezzuto B, Nocioni M, Mezzapesa M, Francone M, Giannetta E, Papa S, Gambardella C, Sciomer S, et al. Right ventricular remodeling in idiopathic pulmonary arterial hypertension: adaptive versus maladaptive morphology. J Heart Lung Transpl. 2015;34:395\u2013403. https:\/\/doi.org\/10.1016\/j.healun.2014.11.002.","journal-title":"J Heart Lung Transpl"},{"key":"116_CR35","doi-asserted-by":"publisher","DOI":"10.1016\/j.chest.2025.04.007","author":"M Mukherjee","year":"2025","unstructured":"Mukherjee M, Jani VP, Osgueritchian R, Mombeini H, Abidov A, Beck G, Erzurum S, Frantz RP, Hassoun PM, Hemnes AR, et al. Echocardiographic Parameters Enhance Risk Prediction Scores in Pulmonary Arterial Hypertension: Insights from the PVDOMICS Network. Chest. 2025. https:\/\/doi.org\/10.1016\/j.chest.2025.04.007.","journal-title":"Chest"},{"key":"116_CR36","doi-asserted-by":"publisher","unstructured":"Mukherjee M, Chung SE, Ton VK, Tedford RJ, Hummers LK, Wigley FM, Abraham TP, Shah AA. Unique Abnormalities in Right Ventricular Longitudinal Strain in Systemic Sclerosis Patients. Circ Cardiovasc Imaging. 2016;9. https:\/\/doi.org\/10.1161\/CIRCIMAGING.115.003792.","DOI":"10.1161\/CIRCIMAGING.115.003792"},{"key":"116_CR37","doi-asserted-by":"publisher","first-page":"1701436","DOI":"10.1183\/13993003.01436-2017","volume":"50","author":"M Mukherjee","year":"2017","unstructured":"Mukherjee M, Mercurio V, Tedford RJ, Shah AA, Hsu S, Mullin CJ, Sato T, Damico R, Kolb TM, Mathai SC. Right ventricular longitudinal strain is diminished in systemic sclerosis compared with idiopathic pulmonary arterial hypertension. Eur Respir J. 2017;50:1701436.","journal-title":"Eur Respir J"},{"key":"116_CR38","doi-asserted-by":"publisher","first-page":"2360","DOI":"10.1161\/circulationaha.117.033147","volume":"137","author":"S Hsu","year":"2018","unstructured":"Hsu S, Kokkonen-Simon KM, Kirk JA, Kolb TM, Damico RL, Mathai SC, Mukherjee M, Shah AA, Wigley FM, Margulies KB, et al. Right Ventricular Myofilament Functional Differences in Humans With Systemic Sclerosis-Associated Versus Idiopathic Pulmonary Arterial Hypertension. Circulation. 2018;137:2360\u201370. https:\/\/doi.org\/10.1161\/circulationaha.117.033147.","journal-title":"Circulation"},{"key":"116_CR39","doi-asserted-by":"publisher","first-page":"1919","DOI":"10.1161\/circulationaha.123.064717","volume":"147","author":"V Jani","year":"2023","unstructured":"Jani V, Aslam MI, Fenwick AJ, Ma W, Gong H, Milburn G, Nissen D, Cubero Salazar IM, Hanselman O, Mukherjee M, et al. Right Ventricular Sarcomere Contractile Depression and the Role of Thick Filament Activation in Human Heart Failure With Pulmonary Hypertension. Circulation. 2023;147:1919\u201332. https:\/\/doi.org\/10.1161\/circulationaha.123.064717.","journal-title":"Circulation"},{"key":"116_CR40","doi-asserted-by":"publisher","first-page":"D100","DOI":"10.1016\/j.jacc.2013.10.033","volume":"62","author":"JL Vachiery","year":"2013","unstructured":"Vachiery JL, Adir Y, Barbera JA, Champion H, Coghlan JG, Cottin V, De Marco T, Galie N, Ghio S, Gibbs JS, et al. Pulmonary hypertension due to left heart diseases. J Am Coll Cardiol. 2013;62:D100\u2013108. https:\/\/doi.org\/10.1016\/j.jacc.2013.10.033.","journal-title":"J Am Coll Cardiol"},{"key":"116_CR41","first-page":"1321","volume":"17","author":"SF Nagueh","year":"2016","unstructured":"Nagueh SF, Smiseth OA, Appleton CP, Byrd BF, Dokainish H, Edvardsen T, Flachskampf FA, Gillebert TC, Klein AL, Lancellotti P. Recommendations for the evaluation of left ventricular diastolic function by echocardiography: an update from the American Society of Echocardiography and the European Association of Cardiovascular Imaging. Eur J Echocardiography. 2016;17:1321\u201360.","journal-title":"Eur J Echocardiography"},{"key":"116_CR42","doi-asserted-by":"publisher","first-page":"108","DOI":"10.1016\/j.echo.2014.09.004","volume":"28","author":"M D\u2019Alto","year":"2015","unstructured":"D\u2019Alto M, Romeo E, Argiento P, Pavelescu A, M\u00e9lot C, D\u2019Andrea A, Correra A, Bossone E, Calabr\u00f2 R, Russo MG. Echocardiographic prediction of pre-versus postcapillary pulmonary hypertension. J Am Soc Echocardiogr. 2015;28:108\u201315.","journal-title":"J Am Soc Echocardiogr"},{"key":"116_CR43","doi-asserted-by":"publisher","first-page":"e006984","DOI":"10.1161\/circimaging.117.006984","volume":"11","author":"F Alenezi","year":"2018","unstructured":"Alenezi F, Mandawat A, Il\u2019Giovine ZJ, Shaw LK, Siddiqui I, Tapson VF, Arges K, Rivera D, Romano MMD, Velazquez EJ, et al. Clinical Utility and Prognostic Value of Right Atrial Function in Pulmonary Hypertension. Circ Cardiovasc Imaging. 2018;11:e006984. https:\/\/doi.org\/10.1161\/circimaging.117.006984.","journal-title":"Circ Cardiovasc Imaging"},{"key":"116_CR44","doi-asserted-by":"publisher","unstructured":"Hasselberg NE, Kagiyama N, Soyama Y, Sugahara M, Goda A, Ryo-Koriyama K, Batel O, Chakinala M, Simon MA, Gorcsan J 3. The Prognostic Value of Right Atrial Strain Imaging in Patients with Precapillary Pulmonary Hypertension. J Am Soc Echocardiogr. 2021;34:851\u2013e861851. https:\/\/doi.org\/10.1016\/j.echo.2021.03.007.","DOI":"10.1016\/j.echo.2021.03.007"},{"key":"116_CR45","doi-asserted-by":"crossref","unstructured":"Hirose K, Nakanishi K, Daimon M, Seki H, Yoshida Y, Hirokawa M, Nakao T, Kimura K, Minatsuki S, Hatano M. Association of left atrial dysfunction with pulmonary hemodynamics and cardiovascular outcomes in patients with systemic sclerosis. CJC Open. 2025.","DOI":"10.1016\/j.cjco.2025.02.006"}],"container-title":["Echo Research &amp; Practice"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1186\/s44156-026-00116-4.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/article\/10.1186\/s44156-026-00116-4","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1186\/s44156-026-00116-4.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,6,16]],"date-time":"2026-06-16T23:01:17Z","timestamp":1781650877000},"score":1,"resource":{"primary":{"URL":"https:\/\/link.springer.com\/10.1186\/s44156-026-00116-4"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,6,8]]},"references-count":45,"journal-issue":{"issue":"1","published-online":{"date-parts":[[2026,12]]}},"alternative-id":["116"],"URL":"https:\/\/doi.org\/10.1186\/s44156-026-00116-4","relation":{},"ISSN":["2055-0464"],"issn-type":[{"value":"2055-0464","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,6,8]]},"assertion":[{"value":"29 April 2025","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"7 April 2026","order":2,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"8 June 2026","order":3,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Declarations"}},{"value":"The study was approved by the Johns Hopkins University Institutional Review Board. Written informed consent was obtained from all participants upon entry into the registry.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Human ethics and consent to participate"}},{"value":"Not applicable.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Consent to publish"}},{"value":"The authors declare no competing interests.","order":4,"name":"Ethics","group":{"name":"EthicsHeading","label":"Competing interests"}}],"article-number":"18"}}