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- W2552562530 abstract "Introduction: Survival in SSc-PAH is worse than IPAH despite milder haemodynamics. Methods: 651 patients fulfilling criteria for PAH were identified. Patients with ≥2 cardiovascular risk factors or evidence of >limited lung disease were excluded leaving 183 IPAH and 192 SSc-PAH patients. MRI and CT assessment was performed in a subset. Results: Compared with IPAH, SSc-PAH patients were older with milder haemodynamics but lower exercise capacity and DLco and poorer survival (5-yr 35% v 65%, p<0.001). Age-adjusted compliance for a given resistance was lower(p<0.05). Oscillatory power fraction (marker of wasted energy) was higher and right ventricular stroke work index was lower. SSc, exercise capacity, SVO2 and FVC/DLco were independent prognosticators. RV mass was lower but no difference was seen when corrected for afterload. Ground glass changes located centrally within the lung were present in 30% compared with 4% of IPAH(p Conclusion: Survival differences are not just due to age and may reflect differences in pulmonary vasculopathy and RV adaptation to increased afterload." @default.
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- W2552562530 date "2016-09-01" @default.
- W2552562530 modified "2023-09-27" @default.
- W2552562530 title "Idiopathic and systemic sclerosis-associated PAH: A comprehensive haemodynamic and radiological comparison" @default.
- W2552562530 doi "https://doi.org/10.1183/13993003.congress-2016.pa2423" @default.
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