Effects of Parenteral Treprostinil on Functional Capacity, Hemodynamics, and Right Heart Function in Patients Referred to LTx with Pulmonary Fibrosis & Significant PH

Abstract

Purpose The use of pulmonary hypertension (PH) specific therapy in the setting of pulmonary fibrosis (PF) is controversial. Determine the effects of chronic parenteral treprostinil in patients with an advanced PH-PF phenotype. Methods and Materials Open label, prospective analysis of PF-PH patients referred for lung transplantation. PH was defined as mPA > 35mmHg. We compared 6MW distance, hemodynamics, oxygenation, and doppler echo (DE) before, and 12 weeks after parenteral treprostinil. Results 15 patients were included in the study with advanced PH phenotype: marked increase in PVR and significant right heart dysfunction at baseline. After 12 weeks of treprostinil, significant improvement in hemodynamics [RAP (9.5 + 3.4 vs. 6.0 + 3.7); mPA (47.8 vs. 38.9 + 13.4); CI (2.3 + 0.5 vs. 2.7 + 0.6); PVR (698 + 278 vs. 496 + 229); TPG (34.7 + 8.7 vs. 28.5 + 10.3); mvO2 (65 + 7.2 vs. 70.9 + 7.4); and SVI (29.2 + 6.7 vs. 33 + 7.3)]. Additional improvements were seen in DE [RV end-diastolic area (36.4+5.2 cm2 vs. 30.9+8.2), LV eccentricity index (1.7 + 0.6 vs. 1.3 + 0.5), TAPSE (1.6 + 0.5 vs. 1.9 + 0.2), 6MW (171 + 93 vs. 230+114). These changes occured without alteration in oxygenation, heart rate, or mean SBP. [ figure 1 ] Conclusions PH-specific therapy improved hemodynamics, right heart function, and functional capacity without a significant change in systemic oxygenation in an advanced PH phenotype in the setting of PF. Age years (SD) 63 (15) NYHA Class (n=15) 7/15 (47%) III 7/15 (47%) IV 8/15 (53%) Race Hispanic 8/15 (53%) Caucasian 4/15 (27%) Asian 2/15 (13%) Middle Eastren 1/15 (7%) PF Lung Subtype IPF 6 (40%) NSIPfib 3 (20%) HSP chronic 2 (13%) CPFE 4 (27%) Baseline Characteristics