{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,18]],"date-time":"2026-08-18T14:14:49Z","timestamp":1787062489214,"version":"build-2736575974"},"reference-count":17,"publisher":"MDPI AG","issue":"6","license":[{"start":{"date-parts":[[2020,6,18]],"date-time":"2020-06-18T00:00:00Z","timestamp":1592438400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["JCM"],"abstract":"<jats:p>Background and Aim: Isolated exercise-induced pulmonary hypertension (ExPH) associates with cardiovascular (CV) events in patients with left heart disease. We investigated its prognostic significance in scleroderma patients at risk for pulmonary arterial hypertension (PAH). Methods: In 26 consecutive scleroderma female patients with either low (n = 13) or intermediate probability (n = 13) of pulmonary hypertension (PH) at rest, we evaluated, both at time 0 and 1 year, prognostic determinants of CV risk: onset or progression of heart failure\/syncope; worsening of functional class; functional performance at the 6-minute walking test and at cardiopulmonary exercise test; right atrial area; and pericardial effusion. We assigned a severity score 1\u20133 to each prognostic determinant, derived an overall CV risk score, and its 0\u20131 year change. Isolated ExPH during the cardiopulmonary exercise test (CPET) was defined as absence of PH at rest, reduced peak VO2, VE\/VCO2 &gt;30 at anaerobic threshold, reduced O2 pulse, and \u0394VO2\/\u0394W &lt;9 mL\/min\/W. We then correlated ExPH at time 0 with clinical worsening (risk score increase &gt;20% after 1 year). Results: ExPH was strongly associated with clinical worsening compared to patients without ExPH (p = 0.005). In patients without ExPH, none had &gt; 20% increased CV risk score after 1 year. Conversely, about 50% of patients with ExPH had such an increase, suggesting a worsening of prognosis. Conclusions: Isolated ExPH associates with higher cardiovascular risk and thus clinical worsening in scleroderma patients. The assessment of ExPH by CPET can thus contribute to a better risk stratification and the planning of a more adequate follow-up.<\/jats:p>","DOI":"10.3390\/jcm9061910","type":"journal-article","created":{"date-parts":[[2020,6,18]],"date-time":"2020-06-18T12:21:46Z","timestamp":1592482906000},"page":"1910","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":11,"title":["Isolated Exercise-Induced Pulmonary Hypertension Associates with Higher Cardiovascular Risk in Scleroderma Patients"],"prefix":"10.3390","volume":"9","author":[{"given":"Rosalinda","family":"Madonna","sequence":"first","affiliation":[{"name":"Institute of Cardiology, University of Pisa, 56124 Pisa, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7783-0262","authenticated-orcid":false,"given":"Riccardo","family":"Morganti","sequence":"additional","affiliation":[{"name":"Institute of Epidemiology, University of Pisa, 56124 Pisa, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Francesco","family":"Radico","sequence":"additional","affiliation":[{"name":"Institute of Cardiology, G. d\u2019Annunzio University\u2014Chieti-Pescara, and SS. Annunziata Hospital, 56100 Chieti, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Piergiusto","family":"Vitulli","sequence":"additional","affiliation":[{"name":"Institute of Cardiology, G. d\u2019Annunzio University\u2014Chieti-Pescara, and SS. Annunziata Hospital, 56100 Chieti, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marco","family":"Mascellanti","sequence":"additional","affiliation":[{"name":"Cardiology Division, Ospedale Santo Spirito, 65127 Pescara, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1589-9672","authenticated-orcid":false,"given":"Paolo","family":"Amerio","sequence":"additional","affiliation":[{"name":"Department of Medicine and Aging Sciences, Dermatology Clinic, G. d\u2019Annunzio University\u2014Chieti-Pescara, 56100 Chieti, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1637-574X","authenticated-orcid":false,"given":"Raffaele","family":"De Caterina","sequence":"additional","affiliation":[{"name":"Institute of Cardiology, University of Pisa, 56124 Pisa, Italy"},{"name":"Fondazione Villa Serena per la Ricerca, Citt\u00e0 S. Angelo, 65013 Pescara, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2020,6,18]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"67","DOI":"10.1093\/eurheartj\/ehv317","article-title":"2015 ESC\/ERS Guidelines for the Diagnosis and Treatment of Pulmonary Hypertension: The Joint 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: Association for European Paediatric and Congenital Cardiology (AEPC), International Society for Heart and Lung Transplantation (ISHLT)","volume":"37","author":"Humbert","year":"2016","journal-title":"Eur. Heart J."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"55","DOI":"10.1016\/j.jacc.2009.04.011","article-title":"Diagnosis and Assessment of Pulmonary Arterial Hypertension","volume":"54","author":"Badesch","year":"2009","journal-title":"J. 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