TY - JOUR T1 - Impact of the Revised Hemodynamic Definition on the Diagnosis of Pulmonary Hypertension in Patients with Systemic Sclerosis JF - European Respiratory Journal JO - Eur Respir J DO - 10.1183/13993003.00586-2019 SP - 1900586 AU - Sara Jaafar AU - Scott Visovatti AU - Amber Young AU - Suiyuan Huang AU - Paul Cronin AU - Dharshan Vummidi AU - Vallerie McLaughlin AU - Dinesh Khanna Y1 - 2019/01/01 UR - http://erj.ersjournals.com/content/early/2019/05/30/13993003.00586-2019.abstract N2 - Introduction Pulmonary arterial hypertension (PAH) is one of the leading causes of mortality in scleroderma-spectrum disorders (SSc). We explored the impact of the updated hemodynamic definition of pulmonary hypertension (PH), as proposed by the 6th World Symposium on Pulmonary Hypertension.Methods In this single center retrospective analysis, patients with SSc who had right heart catheterizations (RHCs) were included. We compared the prior PH definition, which defined PH as mPAP≥25 mmHg and further classified into pre-capillary PH [PAH and PH due to lung diseases], post-capillary PH, and combined pre- and post-capillary PH. For the updated definition, we classified PH as mPAP>20 mmHg and further classified them into different groups defined above. We validated our findings in the DETECT cohort.Results Between 2005 and March 2019, 268 RHCs were performed in single center cohort. Using the prior definition, 137 (51%) were diagnosed with PH, with 89 classified as pre-capillary PH (56 with PAH; 33 with PH-lung diseases), 29 as post-capillary PH, and 19 as combined PH. When the updated definition was applied to the cohort, 7 of 131 (5%) with no PH were reclassified to pre-capillary PH (PAH [N=1], PH-lung diseases [N=3]) and post-capillary PH (N=3). In those with mPAP of 21–24 mmHg, no left heart or lung disease, 1 of 28 (4%) in our cohort and 4 of 36 (11%) in the DETECT cohort were reclassified as PAH.Conclusion The updated PH definition does not appear to have a significant impact on the diagnosis of PH in 2 different screening cohorts.FootnotesThis manuscript has recently been accepted for publication in the European Respiratory Journal. It is published here in its accepted form prior to copyediting and typesetting by our production team. After these production processes are complete and the authors have approved the resulting proofs, the article will move to the latest issue of the ERJ online. Please open or download the PDF to view this article.Conflict of interest: Dr. Jaafar has nothing to disclose.Conflict of interest: Dr. Visovatti has nothing to disclose.Conflict of interest: Dr. Young has nothing to disclose.Conflict of interest: Dr. Huang has nothing to disclose.Conflict of interest: Dr. Cronin has nothing to disclose.Conflict of interest: Dr. Vummidi has nothing to disclose.Conflict of interest: Dr. Mclaughlin reports grants and personal fees from Actelion, grants and personal fees from Acceleron, grants and personal fees from Arena, grants and personal fees from Bayer, personal fees from Caremark, grants from Gilead, grants from Sonovie, and perosnal fees from United Therapeutics.Conflict of interest: Dr. Khanna reports personal fees from Actelion, personal fees from Astra Zeneca, grants and personal fees from Bayer, grants and personal fees from BMS, grants and personal fees from Boehringer-Ingelheim, personal fees from Chemomab, personal fees from Civi Biopharma, personal fees from Corbus, personal fees from CSL Behring, personal fees from Cytori, personal fees from Eicos, personal fees from EMD Serono, grants and personal fees from Genetech/Roche, personal fees from GSK, personal fees from Horizon, grants from NIH NIAID, grants from NIH NIAMS, grants from Pfizer, grants and personal fees from Sanofi-Aventis/Genzy, personal fees from UCB Pharma, during the conduct of the study; personal fees from Astra Zeneca, outside the submitted work. ER -