A Study of RVT-1201 in Patients With Pulmonary Arterial Hypertension (ELEVATE 1)

Key Inclusion Criteria: * Symptomatic PAH belonging to one of the following types: * Idiopathic * Heritable * Drug- or toxin- induced * Associated with one of the following: connective tissue disease or congenital heart disease * World Health Organization (WHO) Functional Class (FC) II or III * PAH diagnosed by right heart cardiac catheterization prior to Screening * Receiving standard of care treatment for PAH with oral monotherapy or dual therapy for at least 12 weeks prior to Screening at a dose which has been stable for at least 8 weeks prior to Screening * If on a diuretic, dose must be stable for at least 4 weeks prior to Screening, with no changes anticipated during study participation * 6-Minute Walk Distance (6MWD) between 150 and 500 meters at Screening and Baseline visits * Plasma N-terminal pro B-type natriuretic peptide (NT-proBNP) level ≥ 300 pg/mL at Screening * Ability and willingness to give written informed consent and to comply with the requirements of the study Key Exclusion Criteria: * PAH associated with human immunodeficiency virus (HIV) infection, portal hypertension or schistosomiasis * Other types of pulmonary hypertension (PH): * Pulmonary hypertension due to left heart disease (WHO PH Group 2) * Pulmonary hypertension due to lung diseases and/or hypoxia (WHO PH Group 3) * Chronic thromboembolic pulmonary hypertension (WHO PH Group 4) * Pulmonary hypertension with unclear multifactorial mechanisms (WHO PH Group 5) * Hospitalization for pulmonary hypertension within 12 weeks of screening * Cardiopulmonary rehabilitation program based on exercise (planned, or started ≤ 12 weeks prior to Screening) * Prostanoid or prostacyclin receptor agonist therapy within 12 weeks of screening * Evidence of left-sided heart disease * If Pulmonary function tests were done prior to screening, Pulmonary function tests demonstrate obstructive or restrictive lung disease * Use of telotristat (Xermelo®) within the last 6 months * Use of any investigational drug within 30 days or five half-lives (whichever is longer) prior to Screening, or 90 days if an investigational drug for PAH * Have uncontrolled atrial fibrillation (AFib) or other uncontrolled arrhythmias * Body mass index (BMI) \>45 kg/m2 * Women of childbearing potential who are pregnant, planning to become pregnant, or lactating or female/male patients unwilling to use effective contraception