{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,5]],"date-time":"2026-05-05T16:13:52Z","timestamp":1777997632799,"version":"3.51.4"},"reference-count":25,"publisher":"Oxford University Press (OUP)","issue":"5","license":[{"start":{"date-parts":[[2017,5,1]],"date-time":"2017-05-01T00:00:00Z","timestamp":1493596800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/pages\/standard-publication-reuse-rights"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2017,5,1]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Rationale<\/jats:title>\n                    <jats:p>Patients with systemic sclerosis\u2013associated pulmonary arterial hypertension (SSc-PAH) continue to have an unacceptably high mortality rate despite the progress achieved with pulmonary arterial vasodilator therapies.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Objectives<\/jats:title>\n                    <jats:p>We sought to determine whether SSc-PAH is a clinically distinct pulmonary vascular disease phenotype when compared with idiopathic pulmonary arterial hypertension (IPAH) on the basis of progression of echocardiographic right ventricular (RV) dysfunction.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>Retrospective analysis of echocardiographic data in 13 patients with SSc-PAH and 11 patients with IPAH was used to delineate the progression of RV dysfunction during single or combination pulmonary arterial vasodilator therapy. All patients had right heart catheterization\u2013confirmed pulmonary arterial hypertension as well as complete baseline (at the time of diagnosis) and follow-up (most recent) echocardiograms. We excluded patients with significant scleroderma-associated interstitial lung disease. Adjusting for time of follow-up and disease duration, we performed mixed model regression analyses comparing the changes between the two groups for different echocardiographic variables: tricuspid annular plane systolic excursion, tricuspid regurgitation jet velocity, right atrial area, and RV diameter.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>The mean ages for the SSc-PAH and IPAH groups were 60.8 and 48.2 years, respectively. The mean follow-up periods for the two groups were 3.8 and 1.95 years, respectively. Tricuspid annular plane systolic excursion did not improve in patients with SSc-PAH, whereas it increased in the patients with IPAH (\u22120.38 mm, P\u2009=\u20090.87; vs. +5.6 mm, P\u2009=\u20090.02). The other echocardiographic variables showed a trend toward worsening in the SSc-PAH group and improvement in the IPAH group.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>Our results indicate that, in patients with SSc-PAH, echocardiographic RV function does not improve over time compared with that of patients with IPAH, despite institution of pulmonary artery vasodilator therapies.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1513\/annalsats.201608-655oc","type":"journal-article","created":{"date-parts":[[2017,3,10]],"date-time":"2017-03-10T16:39:08Z","timestamp":1489163948000},"page":"682-689","source":"Crossref","is-referenced-by-count":15,"title":["Differences in Right Ventricular Functional Changes during Treatment between Systemic Sclerosis\u2013associated Pulmonary Arterial Hypertension and Idiopathic Pulmonary Arterial Hypertension"],"prefix":"10.1093","volume":"14","author":[{"given":"Rahul G.","family":"Argula","sequence":"first","affiliation":[{"name":"Division of Pulmonary, Critical Care, Allergy, and Sleep Medicine"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Abhijit","family":"Karwa","sequence":"additional","affiliation":[{"name":"Division of Internal Medicine"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Abigail","family":"Lauer","sequence":"additional","affiliation":[{"name":"Department of Public Health Sciences"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"David","family":"Gregg","sequence":"additional","affiliation":[{"name":"Division of Cardiology, and"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Richard M.","family":"Silver","sequence":"additional","affiliation":[{"name":"Division of Rheumatology, Medical University of South Carolina, Charleston, South Carolina; and"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Carol","family":"Feghali-Bostwick","sequence":"additional","affiliation":[{"name":"Division of Rheumatology, Medical University of South Carolina, Charleston, South Carolina; and"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Lynn M.","family":"Schanpp","sequence":"additional","affiliation":[{"name":"Division of Pulmonary, Critical Care, Allergy, and Sleep Medicine"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kim","family":"Egbert","sequence":"additional","affiliation":[{"name":"Division of Pulmonary, Critical Care, Allergy, and Sleep Medicine"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Bruce W.","family":"Usher","sequence":"additional","affiliation":[{"name":"Division of Cardiology, and"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Viswanathan","family":"Ramakrishnan","sequence":"additional","affiliation":[{"name":"Department of Public Health Sciences"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Paul M.","family":"Hassoun","sequence":"additional","affiliation":[{"name":"Division of Pulmonary and Critical Care Medicine, Johns Hopkins University School of Medicine, Baltimore, Maryland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Charlie","family":"Strange","sequence":"additional","affiliation":[{"name":"Division of Pulmonary, Critical Care, Allergy, and Sleep Medicine"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"286","published-online":{"date-parts":[[2017,5,1]]},"reference":[{"key":"2026020403063026800_bib1","doi-asserted-by":"crossref","first-page":"06","DOI":"10.12703\/P7-06","article-title":"Connective tissue disease-associated pulmonary arterial hypertension","volume":"7","author":"Condliffe","year":"2015","journal-title":"F1000Prime Rep"},{"key":"2026020403063026800_bib2","doi-asserted-by":"crossref","first-page":"151","DOI":"10.1164\/rccm.200806-953OC","article-title":"Connective tissue disease\u2013associated pulmonary arterial hypertension in the modern treatment era","volume":"179","author":"Condliffe","year":"2009","journal-title":"Am J Respir Crit Care Med"},{"key":"2026020403063026800_bib3","doi-asserted-by":"crossref","first-page":"2412","DOI":"10.1002\/art.38029","article-title":"Survival and prognostic factors in systemic sclerosis-associated pulmonary hypertension: a systematic review and meta-analysis","volume":"65","author":"Lef\u00e8vre","year":"2013","journal-title":"Arthritis Rheum"},{"key":"2026020403063026800_bib4","doi-asserted-by":"crossref","first-page":"1282","DOI":"10.1378\/chest.12-0653","article-title":"Survival in systemic sclerosis with pulmonary arterial hypertension has not improved in the modern era","volume":"144","author":"Rubenfire","year":"2013","journal-title":"Chest"},{"key":"2026020403063026800_bib5","doi-asserted-by":"crossref","first-page":"1545","DOI":"10.3899\/jrheum.121380","article-title":"The 15% rule in scleroderma: the frequency of severe organ complications in systemic sclerosis. A systematic review","volume":"40","author":"Muangchan","year":"2013","journal-title":"J Rheumatol"},{"key":"2026020403063026800_bib6","doi-asserted-by":"crossref","first-page":"156","DOI":"10.1161\/CIRCULATIONAHA.109.911818","article-title":"Survival in patients with idiopathic, familial, and anorexigen-associated pulmonary arterial hypertension in the modern management era","volume":"122","author":"Humbert","year":"2010","journal-title":"Circulation"},{"key":"2026020403063026800_bib7","doi-asserted-by":"crossref","first-page":"489","DOI":"10.1002\/acr.22121","article-title":"Survival and predictors of mortality in systemic sclerosis-associated pulmonary arterial hypertension: outcomes from the pulmonary hypertension assessment and recognition of outcomes in scleroderma registry","volume":"66","author":"Chung","year":"2014","journal-title":"Arthritis Care Res (Hoboken)"},{"key":"2026020403063026800_bib8","doi-asserted-by":"crossref","first-page":"371","DOI":"10.1183\/09031936.00106008","article-title":"Pulmonary arterial hypertension in limited cutaneous systemic sclerosis: a distinctive vasculopathy","volume":"34","author":"Overbeek","year":"2009","journal-title":"Eur Respir J"},{"key":"2026020403063026800_bib9","doi-asserted-by":"crossref","first-page":"647","DOI":"10.1093\/rheumatology\/keu350","article-title":"Assessment of myocardial fibrosis and microvascular damage in systemic sclerosis by magnetic resonance imaging and coronary angiotomography","volume":"54","author":"Rodr\u00edguez-Reyna","year":"2015","journal-title":"Rheumatology (Oxford)"},{"key":"2026020403063026800_bib10","doi-asserted-by":"crossref","first-page":"e78808","DOI":"10.1371\/journal.pone.0078808","article-title":"Right bundle branch block: a predictor of mortality in early systemic sclerosis","volume":"8","author":"Draeger","year":"2013","journal-title":"PLoS One"},{"key":"2026020403063026800_bib11","doi-asserted-by":"crossref","first-page":"982","DOI":"10.1016\/j.amjmed.2013.06.025","article-title":"Increased risk of acute myocardial infarction in systemic sclerosis: a nationwide population-based study","volume":"126","author":"Chu","year":"2013","journal-title":"Am J Med"},{"key":"2026020403063026800_bib12","doi-asserted-by":"crossref","first-page":"2078","DOI":"10.1002\/art.30380","article-title":"Atherosclerosis in systemic sclerosis: a systematic review and meta-analysis","volume":"63","author":"Au","year":"2011","journal-title":"Arthritis Rheum"},{"key":"2026020403063026800_bib13","doi-asserted-by":"crossref","first-page":"685","DOI":"10.1016\/j.echo.2010.05.010","article-title":"Guidelines for the echocardiographic assessment of the right heart in adults: a report from the American Society of Echocardiography endorsed by the European Association of Echocardiography, a registered branch of the European Society of Cardiology, and the Canadian Society of Echocardiography","volume":"23","author":"Rudski","year":"2010","journal-title":"J Am Soc Echocardiogr"},{"key":"2026020403063026800_bib14","doi-asserted-by":"crossref","first-page":"1111","DOI":"10.1164\/rccm.201507-1456OC","article-title":"Comparison of treatment response in idiopathic and connective tissue disease\u2013associated pulmonary arterial hypertension","volume":"192","author":"Rhee","year":"2015","journal-title":"Am J Respir Crit Care Med"},{"key":"2026020403063026800_bib15","doi-asserted-by":"crossref","first-page":"953","DOI":"10.1161\/CIRCHEARTFAILURE.112.000008","article-title":"Right ventricular dysfunction in systemic sclerosis-associated pulmonary arterial hypertension","volume":"6","author":"Tedford","year":"2013","journal-title":"Circ Heart Fail"},{"key":"2026020403063026800_bib16","doi-asserted-by":"crossref","first-page":"327","DOI":"10.1086\/680356","article-title":"Right ventricular remodeling in idiopathic and scleroderma-associated pulmonary arterial hypertension: two distinct phenotypes","volume":"5","author":"Kelemen","year":"2015","journal-title":"Pulm Circ"},{"key":"2026020403063026800_bib17","doi-asserted-by":"crossref","first-page":"3043","DOI":"10.1002\/art.22069","article-title":"Clinical differences between idiopathic and scleroderma-related pulmonary hypertension","volume":"54","author":"Fisher","year":"2006","journal-title":"Arthritis Rheum"},{"key":"2026020403063026800_bib18","doi-asserted-by":"crossref","first-page":"1102","DOI":"10.1164\/rccm.201507-1398OC","article-title":"Ambrisentan and tadalafil up-front combination therapy in scleroderma-associated pulmonary arterial hypertension","volume":"192","author":"Hassoun","year":"2015","journal-title":"Am J Respir Crit Care Med"},{"key":"2026020403063026800_bib19","doi-asserted-by":"crossref","first-page":"2413","DOI":"10.1161\/CIRCULATIONAHA.116.022082","article-title":"Right ventricular functional reserve in pulmonary arterial hypertension","volume":"133","author":"Hsu","year":"2016","journal-title":"Circulation"},{"key":"2026020403063026800_bib20","doi-asserted-by":"crossref","first-page":"749","DOI":"10.1001\/archinte.1943.00210060010002","article-title":"Scleroderma heart disease: with a consideration of certain other visceral manifestations of scleroderma","volume":"71","author":"Weiss","year":"1943","journal-title":"Arch Intern Med"},{"key":"2026020403063026800_bib21","first-page":"656","article-title":"A controlled clinicopathologic study of myocardial fibrosis in systemic sclerosis (scleroderma)","volume":"17","author":"Follansbee","year":"1990","journal-title":"J Rheumatol"},{"key":"2026020403063026800_bib22","doi-asserted-by":"crossref","first-page":"38","DOI":"10.7326\/0003-4819-94-1-38","article-title":"The prevalence of conduction defects and cardiac arrhythmias in progressive systemic sclerosis","volume":"94","author":"Roberts","year":"1981","journal-title":"Ann Intern Med"},{"key":"2026020403063026800_bib23","doi-asserted-by":"crossref","first-page":"17","DOI":"10.1016\/S0022-0736(96)80107-5","article-title":"Electrophysiologic abnormalities of cardiac function in progressive systemic sclerosis","volume":"29","author":"Rokas","year":"1996","journal-title":"J Electrocardiol"},{"key":"2026020403063026800_bib24","doi-asserted-by":"crossref","first-page":"2511","DOI":"10.1016\/j.jacc.2011.06.068","article-title":"Progressive right ventricular dysfunction in patients with pulmonary arterial hypertension responding to therapy","volume":"58","author":"van de Veerdonk","year":"2011","journal-title":"J Am Coll Cardiol"},{"key":"2026020403063026800_bib25","doi-asserted-by":"crossref","first-page":"218","DOI":"10.1183\/09059180.00003512","article-title":"A critical analysis of survival in pulmonary arterial hypertension","volume":"21","author":"O\u2019Callaghan","year":"2012","journal-title":"Eur Respir Rev"}],"container-title":["Annals of the American Thoracic Society"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/academic.oup.com\/annalsats\/article-pdf\/14\/5\/682\/66714030\/annalsats_14_5_682.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"syndication"},{"URL":"https:\/\/academic.oup.com\/annalsats\/article-pdf\/14\/5\/682\/66714030\/annalsats_14_5_682.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,2,4]],"date-time":"2026-02-04T08:06:41Z","timestamp":1770192401000},"score":1,"resource":{"primary":{"URL":"https:\/\/academic.oup.com\/annalsats\/article\/14\/5\/682\/8454364"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2017,5,1]]},"references-count":25,"journal-issue":{"issue":"5","published-online":{"date-parts":[[2017,5,1]]},"published-print":{"date-parts":[[2017,5,1]]}},"URL":"https:\/\/doi.org\/10.1513\/annalsats.201608-655oc","relation":{"has-review":[{"id-type":"doi","id":"10.3410\/f.727388738.793568457","asserted-by":"object"}]},"ISSN":["2329-6933","2325-6621"],"issn-type":[{"value":"2329-6933","type":"print"},{"value":"2325-6621","type":"electronic"}],"subject":[],"published-other":{"date-parts":[[2017,5]]},"published":{"date-parts":[[2017,5,1]]}}}