{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,17]],"date-time":"2026-03-17T12:05:25Z","timestamp":1773749125816,"version":"3.50.1"},"reference-count":32,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2025,1,10]],"date-time":"2025-01-10T00:00:00Z","timestamp":1736467200000},"content-version":"vor","delay-in-days":9,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"},{"start":{"date-parts":[[2025,1,1]],"date-time":"2025-01-01T00:00:00Z","timestamp":1735689600000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"funder":[{"DOI":"10.13039\/501100019491","name":"National Natural Science Foundation of China - State Grid Corporation Joint Fund for Smart Grid","doi-asserted-by":"publisher","award":["82071827;81701610"],"award-info":[{"award-number":["82071827;81701610"]}],"id":[{"id":"10.13039\/501100019491","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Int J of Rheum Dis"],"published-print":{"date-parts":[[2025,1]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>Right ventricular (RV) failure is a well\u2010recognized pivotal prognostic factor of adverse outcomes in pulmonary artery hypertension (PAH), while RV dilation provides significant implications for adaptive or maladaptive changes. PAH is a predominant cause of mortality among patients with connective tissue disease (CTD). This study aims to elucidate the prognostic significance of RV morphology, as assessed by echocardiography (ECHO), in with CTD associated with PAH (CTD\u2010PAH).<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>In this ambispective cohort study, 143 CTD\u2010PAH patients diagnosed by right\u2010sided heart catheterization (RHC) from 2013 to 2023 were enrolled. Clinical characteristics, laboratory data, echocardiographic parameters (right ventricular end\u2010diastolic basal diameter index (RVDDI), tricuspid annular plane systolic excursion (TAPSE) and pulmonary arterial systolic pressure (PASP)) and therapy were recorded. The primary endpoint was defined as clinical worsening within a five\u2010year timeframe. Analytical methods included Kaplan\u2013Meier survival analyses, the log\u2010rank test, and multivariable Cox proportional hazards regression to evaluate prognostic factors.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      The study enrolled a total of 143 patients with CTD\u2010PAH, with a notable female predominance (95.1%) and a median age of 41.67\u2009years; SLE\u2010PAH (49%) and pSS\u2010PAH (34%) were the most common subtypes, and 94% of the participants were in WHO\u2010FC II\u2010III. Among the participants, 34 (23.8%) patients experienced clinical worsening during a median follow\u2010up period of 21\u2009months. After adjusting for confounders such as age and sex, RVDDI, as determined by ECHO was correlated with clinical worsening (HR 1.090; 95% CI: 1.019\u20131.166;\n                      <jats:italic>p<\/jats:italic>\n                      \u2009=\u20090.012). RVDDI &gt;\u200925.81\u2009mm\/m\n                      <jats:sup>2<\/jats:sup>\n                      predicts higher incidence of clinical worsening in CTD\u2010PAH. In the subgroup of TAPSE\/PASP &gt;\u20090.19\u2009mm\/mmHg, patients with RVDDI &gt;\u200925.81\u2009mm\/m\n                      <jats:sup>2<\/jats:sup>\n                      had a higher incidence of clinical worsening. The estimated event\u2010free survival rates at 1 and 3\u2009years were 93.5% and 53.7%, respectively.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>The study demonstrates that RVDDI, as evaluated by ECHO, is a significant prognostic indicator for clinical worsening in CTD\u2010PAH. Its inclusion in the assessment of RV function and risk stratification may provide valuable incremental prognostic information for this CTD\u2010PAH population.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1111\/1756-185x.70061","type":"journal-article","created":{"date-parts":[[2025,1,10]],"date-time":"2025-01-10T07:45:30Z","timestamp":1736495130000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["The Prognostic Value of Right Ventricular End\u2010Diastolic Basal Diameter Index by Echocardiography in Connective Tissue Diseases Associated With Pulmonary Artery Hypertension"],"prefix":"10.1111","volume":"28","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-4365-7216","authenticated-orcid":false,"given":"Wu","family":"Qianwen","sequence":"first","affiliation":[{"name":"Department of Rheumatology The First Affiliated Hospital of Nanjing Medical University  Nanjing 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