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Verfasst von:Xanthouli, Panagiota [VerfasserIn]   i
 Jordan, Suzana [VerfasserIn]   i
 Milde, Nicklas [VerfasserIn]   i
 Blank, Norbert [VerfasserIn]   i
 Egenlauf, Benjamin [VerfasserIn]   i
 Gorenflo, Matthias [VerfasserIn]   i
 Harutyunova, Satenik [VerfasserIn]   i
 Lorenz, Hanns-Martin [VerfasserIn]   i
 Nagel, Christian [VerfasserIn]   i
 Theobald, Vivienne [VerfasserIn]   i
 Grünig, Ekkehard [VerfasserIn]   i
 Benjamin, Nicola [VerfasserIn]   i
Titel:Haemodynamic phenotypes and survival in patients with systemic sclerosis
Titelzusatz:the impact of the new definition of pulmonary arterial hypertension
Verf.angabe:Panagiota Xanthouli, Suzana Jordan, Nicklas Milde, Alberto Marra, Norbert Blank, Benjamin Egenlauf, Matthias Gorenflo, Satenik Harutyunova, Hanns-Martin Lorenz, Christian Nagel, Vivienne Theobald, Mona Lichtblau, Charlotte Berlier, Silvia Ulrich, Ekkehard Grünig, Nicola Benjamin, Oliver Distler
Jahr:2020
Jahr des Originals:2019
Umfang:9 S.
Fussnoten:Published Online First 9 December 2019 ; Gesehen am 14.04.2020
Titel Quelle:Enthalten in: Annals of the rheumatic diseases
Ort Quelle:London : BMJ Publ. Group, 1939
Jahr Quelle:2020
Band/Heft Quelle:79(2020), 3, Seite 370-378
ISSN Quelle:1468-2060
Abstract:Background In this study, we investigated the impact of the new haemodynamic definition of pulmonary arterial hypertension (PAH) as proposed by the 6th PH World Symposium on phenotypes and survival in patients with systemic sclerosis (SSc). - Methods In SSc patients who were prospectively and consecutively screened for PAH including right heart catheterisation in Heidelberg or Zurich, haemodynamic and clinical variables have been reassessed according to the new PAH definition. Patients have been followed for 3.7±3.7 (median 3.4) years; Kaplan-Meier survival analysis was performed. Patients with significant lung or left heart disease were excluded from comparative analyses. - Results The final dataset included 284 SSc patients, 146 patients (49.2%) had mean pulmonary arterial pressure (mPAP) ≤20 mm Hg, 19.3% had mPAP 21-24 mm Hg and 29.4% had mPAP ≥25 mm Hg. In the group of mildly elevated mPAP, only four patients (1.4% of the whole SSc cohort) had pulmonary vascular resistance (PVR) values ≥3 Wood Units (WU) and could be reclassified as manifest SSc-APAH. Twenty-eight (9.8%) patients with mPAP of 21-24 mm Hg and PVR ≥2 WU already presented with early pulmonary vascular disease with decreased 6 min walking distance (6MWD) (p<0.001), TAPSE (p=0.004) and pulmonary arterial compliance (p<0.001). A PVR ≥2 WU was associated with reduced long-term survival (p=0.002). PVR and 6MWD were independent prognostic predictors in multivariate analysis. - Conclusion The data of this study show that a PVR threshold ≥3 WU is too high to enable an early diagnosis of PAH. A PVR threshold ≥2 WU was already associated with pulmonary vascular disease, significantly reduced survival and would be more appropriate in SSc patients with mild PAH.
DOI:doi:10.1136/annrheumdis-2019-216476
URL:Bitte beachten Sie: Dies ist ein Bibliographieeintrag. Ein Volltextzugriff für Mitglieder der Universität besteht hier nur, falls für die entsprechende Zeitschrift/den entsprechenden Sammelband ein Abonnement besteht oder es sich um einen OpenAccess-Titel handelt.

Volltext ; Verlag: https://doi.org/10.1136/annrheumdis-2019-216476
 Volltext: https://ard.bmj.com/content/79/3/370
 DOI: https://doi.org/10.1136/annrheumdis-2019-216476
Datenträger:Online-Ressource
Sprache:eng
Sach-SW:outcomes research
 systemic sclerosis
 treatment
K10plus-PPN:1694443051
Verknüpfungen:→ Zeitschrift

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