| Popis: |
Purpose Determine whether treatment with oral PAH-targeted therapy is associated with functional or hemodynamic improvement in patients with PH-ILD. Methods We conducted a retrospective review of 1507 consented patients with PH from the University of Chicago PH Registry. Exclusion criteria included: use of inhaled treprostinil, use of PAH-targeted therapy prior to registry enrollment, and enrollment in PH-related clinical trials. Data analyzed included demographics, ILD classification, PAH-targeted therapy, functional data, hemodynamics, and NT-proBNP before and after initiation of treatment. Data were analyzed using paired t-test, or related-samples Wilcoxon signed rank test. Results Of 37 patientsincluded, 27 (73%) received treatment with one PAH-targeted therapy and 9 (24%) received dual therapy. At baseline, median NT-proBNP was 1,498 ng/dL [675-3,208], mean pulmonary artery pressure (mPAP) was 45 ± 11 mmHg, and pulmonary vascular resistance (PVR) of 9±4 Wood units. In patients with measurements both before and after treatment with PAH-targetedtherapy, there was a decrease in PVR (n=13, 8 vs 5 WU, pConclusions In this study, use of PAH-targeted therapy in patients with PH-ILD was associated with statistically significant and clinically meaningful improvements in NT-proBNP and pulmonary hemodynamics. |
