Abstract
Guidelines recommend to periodically evaluate PAH severity with a panel of variables. Prognostic variables and cut-off values are currently based on expert opinion.
Aims:The prospective multicentre EFORT study aimed to (i) determine transplant-free survival in a cohort of incident idiopathic, heritable or drug-induced PAH patients and (ii) identify prognostic variables and cut-off values using a dynamic model.
Methods:NYHA functional class (FC), 6-min walk distance (6MWD), BNP or NT-proBNP level, and hemodynamic variables were collected at diagnosis, 3-4 months after each treatment initiation and once a year. Patients were censored at the time of transplantation or after a 60-month follow-up. Cox proportional hazard model with time-dependent covariates was used to characterise relevant variables, and logistic regression and multivariate adaptive regression splines analysis were performed to identify cut-off values.
Results:146 incident patients (67% females; 53±18 year-old) were included between 01/2012 and 12/2013. Transplant-free survival rates were 97%, 90%, 83% and 71% at 1, 2, 3 and 5 years, respectively. Time-dependent values for FC, 6MWD, cardiac index (CI) and BNP/NT-proBNP levels predicted transplant-free survival. With NYHA FC I-II, cut-off values were 400 m for 6MWD, 2.4L/min/m² for CI, 150 ng/L for BNP, 700 ng/L for NT-proBNP. Achieving at least 2 of these targets at any time during follow-up decreased the risk of death or lung transplantation with Hazard Ratios of 0.23 for 2, 0.10 for 3 and 0.04 for 4 targets achieved.
Conclusion:The prospective EFORT study has robustly identified PAH prognostic variables and their cut-off values.
Footnotes
Cite this article as: European Respiratory Journal 2019; 54: Suppl. 63, OA497.
This is an ERS International Congress abstract. No full-text version is available. Further material to accompany this abstract may be available atwww.ers-education.org(ERS member access only).
- Copyright ©the authors 2019