Haemodynamics and serial risk assessment in systemic sclerosis associated pulmonary arterial hypertension

Abstract

The prognostic importance of follow-up haemodynamics and the validity of multidimensional risk assessment are not well established for systemic sclerosis (SSc)-associated pulmonary arterial hypertension (PAH).We assessed incident SSc-PAH patients to determine the association between clinical and haemodynamic variables at baseline and first follow-up right heart catheterisation (RHC) with transplant-free survival. RHC variables included cardiac index, stroke volume index (SVI), pulmonary arterial compliance and pulmonary vascular resistance. Risk assessment was performed according to the number of low-risk criteria: functional class I or II, 6-min walking distance (6MWD) >440 m, right atrial pressure <8 mmHg and cardiac index ≥2.5 L·min-1·m-2Transplant-free survival from diagnosis (n=513) was 87%, 55% and 35% at 1, 3 and 5 years, respectively. At baseline, 6MWD was the only independent predictor. A follow-up RHC was available for 353 patients (median interval 4.6 months, interquartile range 3.9-6.4 months). The 6MWD, functional class, cardiac index, SVI, pulmonary arterial compliance and pulmonary vascular resistance were independently associated with transplant-free survival at follow-up, with SVI performing better than other haemodynamic variables. 1-year outcomes were better with increasing number of low-risk criteria at baseline (area under the curve (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 at baseline in SSc-PAH.

Conflict of interest statement

Conflict of interest: J. Weatherald reports grants from the European Respiratory Society and the Canadian Thoracic Society, during the conduct of the study; personal fees and non-financial support from Actelion Pharmaceuticals and Bayer, personal fees from Novartis, grants from Canadian Vascular Network, outside the submitted work. Conflict of interest: A. Boucly reports non-financial support from GSK, Bayer and MSD, and personal fees and non-financial support from Actelion, outside the submitted work. Conflict of interest: D. Launay reports grants from Pfizer, during the conduct of the study; and personal fees from Actelion, grants and personal fees from GSK, outside the submitted work. Conflict of interest: V. Cottin reports personal fees for consultancy, lecturing and travel to medical meetings from Actelion and Roche, personal fees for development of educational presentations, consultancy, lecturing and travel to medical meetings from Boehringer Ingelheim, personal fees for consultancy from Bayer and GSK, personal fees for adjudication committee work from Gilead, personal fees consultancy and travel to medical meetings from MSD, personal fees for consultancy and lecturing from Novartis and Sanofi, institutional grants from Boehringer Ingelheim and Roche, personal fees for chairing a DSMB from Promedior amd Celgene, and personal fees for consultancy and chairing a DSMB from Galapagos, outside the submitted work. Conflict of interest: G. Prévôt reports personal fees for consultancy, lecturing, development of educational presentations and travel to medical meetings from Actelion, Bayer, Boehringer Ingelheim, GSK and Roche, outside the submitted work. Conflict of interest: D. Bourlier reports personal fees from Novartis, outside the submitted work. Conflict of interest: C. Dauphin has nothing to disclose. Conflict of interest: A. Chaouat reports grants from Direction Générale de l'Offre de Soins (DGOS), during the conduct of the study, personal fees and non-financial support from Actelion Pharmaceuticals, Bayer and MSD, and non-financial support from GSK, outside the submitted work. Conflict of interest: L. Savale reports grants, personal fees and non-financial support from Actelion Pharmaceuticals, Bayer, GlaxoSmithKline and MSD and grants and personal fees from Pfizer, outside the submitted work. Conflict of interest: X. Jaïs reports personal fees and non-financial support from Actelion Pharmaceuticals, grants and personal fees from Bayer, and grants, personal fees and non-financial support from MSD and GSK, outside the submitted work. Conflict of interest: M. Jevnikar has nothing to disclose. Conflict of interest: J. Traclet reports personal fees from Actelion Pharmaceuticals, Boehringer Ingelheim and Roche, outside the submitted work. Conflict of interest: P. De Groote has nothing to disclose. Conflict of interest: G. Simonneau reports grants, personal fees and non-financial support from Actelion Pharmaceuticals, Bayer, GlaxoSmithKline and MSD and grants and personal fees from Pfizer, outside the submitted work. Conflict of interest: E. Hachulla has nothing to disclose. Conflict of interest: L. Mouthon has nothing to disclose. Conflict of interest: D. Montani reports grants and personal fees from Actelion and Bayer, and personal fees from BMS, GSK, MSD and Pfizer, outside the submitted work. Conflict of interest: M. Humbert has relationships with drug companies including Actelion, Bayer, GSK, Novartis and Pfizer. In addition to being investigator in trials involving these companies, relationships include consultancy service and membership of scientific advisory boards. Conflict of interest: O. Sitbon reports grants, personal fees and non-financial support from Actelion Pharmaceuticals, Bayer, GlaxoSmithKline and MSD, and grants and personal fees from Pfizer, outside the submitted work.

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