Valley Health System
This prospective open-label matched-control pilot study evaluates whether inhaled treprostinil improves exercise pulmonary vascular resistance and functional outcomes in adults with interstitial lung disease or connective tissue disease and exercise-induced pulmonary hypertension. Participants will receive inhaled treprostinil plus standard care or standard care alone and will be followed for 6 months, with an optional extension to 12 months.
1.1 Purpose/Specific Aims This pilot study aims to determine whether early treatment of exercise-induced pulmonary hypertension with inhaled Treprostinil can improve pulmonary vascular hemodynamics, preserve cardiopulmonary reserve, and delay progression to overt resting pulmonary hypertension in patients with interstitial lung disease (ILD) or connective tissue disease (CTD). A. Objectives Primary Objective: • To evaluate the effect of inhaled treprostinil on exercise pulmonary vascular resistance (PVR) at 6 months in patients with PH-ILD or PH-CTD exercise-induced pulmonary hypertension.…
Inclusion Criteria: * Age ≥18 years * ILD diagnosis or CTD diagnosis * WHO Functional Class II-III * Resting RHC: * mPAP ≤20 mmHg * PVR ≤2 WU * PAWP ≤15 mmHg * Exercise-induced pulmonary hypertension on iCPET * Able to provide informed consent Exclusion Criteria: * Resting pulmonary hypertension * Significant left heart disease * CTEPH * Acute PE within 6 months * Current PAH-targeted therapy * Pregnancy or breastfeeding * Severe renal or hepatic impairment * Inability to perform exercise testing * Contraindication to RHC * Treprostinil hypersensitivity
Description: * Initial dose 18 mcg four times daily * Titrated to 54-72 mcg four times daily as tolerated Standard disease-specific therapy continues
Standard management of ILD/CTD without treprostinil.
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