Abstract
The prognostic importance of follow-up haemodynamics and the validity of multidimensional risk assessment are not well established for systemic sclerosis-associated pulmonary arterial hypertension (SSc-PAH).
We assessed incident SSc-PAH patients to determine the association between clinical and haemodynamic variables at baseline and first follow-up right heart catheterization (RHC) with transplant-free survival. RHC variables included cardiac index (CI), stroke volume index (SVI), pulmonary arterial compliance (PCa) and pulmonary vascular resistance (PVR). Risk assessment was performed according to the number of low-risk criteria: functional class I or II, 6-minute walk distance (6 MWD) >440 m, right atrial pressure <8 mmHg and cardiac index ≥2.5 L·min−1·m−2.
Transplant-free survival from diagnosis (n=513) was 87%, 55%, and 35% at 1-, 3-, and 5-years, respectively. At baseline, 6 MWD was the only independent predictor. A follow-up RHC was available for 353 (median interval 4.6 months, IQR 3.9–6.4). The 6 MWD, functional class, CI, SVI, PCa and PVR were independently associated with transplant-free survival at follow-up, with SVI performing better than other haemodynamic variables. One-year outcomes were better with increasing number of low-risk criteria at baseline (AUC 0.63, 95% CI 0.56–0.69) and at first follow-up (AUC 0.71, 95% CI 0.64–0.78).
Follow-up haemodynamics and multidimensional risk assessment had greater prognostic significance than baseline in SSc-PAH.
Footnotes
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Conflict of interest: Dr. Weatherald reports grants from European Respiratory Society, grants from Canadian Thoracic Society, during the conduct of the study; personal fees and non-financial support from Actelion Pharmaceuticals, personal fees and non-financial support from Bayer, personal fees from Novartis, grants from Canadian Vascular Network, outside the submitted work.
Conflict of interest: Dr. BOUCLY reports non-financial support from Bayer, non-financial support from MSD, personal fees and non-financial support from Actelion, non-financial support from GSK, outside the submitted work.
Conflict of interest: Dr. LAUNAY reports grants from PFIZER, during the conduct of the study; personal fees from Actelion, grants and personal fees from GSK, outside the submitted work.
Conflict of interest: Dr. Cottin reports personal fees from Actelion, personal fees from Boehringer Ingelheim, personal fees from Bayer, personal fees from Gilead, personal fees from GSK, personal fees from MSD, personal fees from Novartis, personal fees from Roche, personal fees from Sanofi, grants from Boehringer Ingelheim, grants from Roche, personal fees from Promedior, personal fees from Celgene, personal fees from Galapagos, outside the submitted work.
Conflict of interest: Dr. Prevot reports personal fees from Actelion, Bayer, Boehringer Ingelheim, GSK, Roche, outside the submitted work.
Conflict of interest: Dr. BOURLIER reports personal fees from NOVARTIS, outside the submitted work.
Conflict of interest: Pr Chaouat reports grants from DGOS, during the conduct of the study; personal fees and non-financial support from Actelion Pharmaceuticals, personal fees and non-financial support from Bayer, personal fees and non-financial support from MSD, and non-financial support from GSK.
Conflict of interest: Dr. Savale reports grants, personal fees and non-financial support from Actelion Pharmaceuticals, grants, personal fees and non-financial support from Bayer, grants and personal fees from Pfizer, grants, personal fees and non-financial support from GlaxoSmithKline, grants, personal fees and non-financial support from MSD, outside the submitted work.
Conflict of interest: Dr. Jais reports personal fees and non-financial support from Actelion Pharmaceuticals, grants and personal fees from Bayer, grants, personal fees and non-financial support from MSD, grants, personal fees and non-financial support from GSK, outside the submitted work.
Conflict of interest: Dr. Jevnikar has nothing to disclose.
Conflict of interest: Dr. Traclet reports personal fees from Actelion Pharmaceuticals, personal fees from Boehringer Ingelheim, personal fees from Roche, outside the submitted work.
Conflict of interest: Dr. de Groote has nothing to disclose.
Conflict of interest: Dr. Simonneau reports grants, personal fees and non-financial support from Actelion Pharmaceuticals, grants, personal fees and non-financial support from Bayer, grants and personal fees from Pfizer, grants, personal fees and non-financial support from GlaxoSmithKline, grants, personal fees and non-financial support from MSD, outside the submitted work; .
Eric Hachulla
Conflict of interest: Dr. MONTANI reports grants and personal fees from Actelion, grants and personal fees from Bayer, personal fees from BMS,personal fees from GSK, personal fees from MSD, personal fees from Pfizer, outside the submitted work.
Conflict of interest: Dr. Humbert reports personal fees from Actelion Pharmaceuticals Ltd, grants and personal fees from Bayer, grants and personal fees from GSK, personal fees from Pfizer, during the conduct of the study; personal fees from Novartis, outside the submitted work.
Conflict of interest: Dr. Sitbon reports grants, personal fees and non-financial support from Actelion Pharmaceuticals, grants, personal fees and non-financial support from Bayer, grants and personal fees from Pfizer, grants, personal fees and non-financial support from GlaxoSmithKline, grants, personal fees and non-financial support from MSD, outside the submitted work.
Conflict of interest: Dr. Claire DAUPHIN has nothing to disclose.
Conflict of interest: Dr. Mouthon has nothing to disclose.
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