PAH Exercise Study

Remotely-Monitored Exercise to Enhance Functional Outcomes in Patients With Pulmonary Arterial Hypertension (PAH) Initiating Sotatercept Therapy: A Single-site Feasibility Study

Ten patients with PAH who are stable and eligible to initiate sotatercept therapy will participate in a 26 week study that consists of a 24-week intervention period where patients will receive complimentary sotatercept as prescribed, plus a tailored, progressive home exercise program with wrist-worn fitness tracker and oxygen saturation monitoring.

Study Overview

• Status: Recruiting
• Conditions: Exercise Therapy; Pulmonary Arterial Hypertension (PAH)
• Intervention / Treatment: Other: Home exercise program

• Study Type: Interventional
• Enrollment (Estimated): 10
• Phase: Phase 3

Contacts and Locations

• Study Contact: Name: Claire E Child, DPT, MPH; Phone Number: 206-616-8601; Email: cechild@uw.edu

Study Locations

• United States
  • Washington
    • Seattle, Washington, United States, 98195
      • Recruiting
      • University of Washington
      • Contact: Claire E Child, DPT, MPH; Phone Number: 206-616-8601; Email: pah-study@uw.edu

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: Adult; Older Adult
• Accepts Healthy Volunteers: No

Description

Inclusion Criteria:

• Age ≥ 18 years
• Documented diagnostic right heart catheterization (RHC) at any time prior to screening confirming the diagnosis of WHO PAH Group 1 in any of the following subtypes: Idiopathic PAH, Heritable PAH, Drug/toxin-induced PAH, PAH associated with CTD, PAH associated with simple, congenital systemic-to-pulmonary shunts at least 1 year following repair
• Symptomatic PH classified as WHO FC II or III
• Baseline RHC performed during the Screening Period documenting a minimum PVR of ≥ 5 WU and a pulmonary capillary wedge (PCWP) or left ventricular end-diastolic pressure of ≤ 15 mmHg
• Receiving stable background therapy for PAH for >90 days and will continue receiving throughout trial. Background treatments may consist of monotherapy, double therapy, or triple therapy with currently available medications for PAH.
• Initiation of Sotatercept is clinically indicated
• Willing and able to participate in a remotely-monitored home exercise program for 24 weeks
• Ability to adhere to study visit schedule and understand and comply with all protocol requirements
• Ability to understand and provide written informed consent

Exclusion Criteria:

• Diagnosis of PH WHO Groups 2, 3, 4, or 5
• Diagnosis of the following PAH Group 1 subtypes: human immunodeficiency virus (HIV)-associated PAH, PAH associated with portal hypertension, schistosomiasis-associated PAH and pulmonary veno-occlusive disease
• Hemoglobin at screening above gender-specific ULN
• Baseline platelet count < 50,000/mm3 (< 50.0 × 109/L) at screening
• Uncontrolled systemic hypertension as evidenced by sitting systolic BP > 160 mmHg or sitting diastolic BP > 100 mmHg during screening visit after a period of rest; Baseline systolic BP < 90 mmHg at screening
• Pregnant or breastfeeding females
• Clinical laboratory liver and kidney function tests outside of normal range
• Currently enrolled in or have completed any other investigational product study within 30 days for small-molecule drugs or within 5 half-lives for biologics prior to the date of signed informed consent
• Prior exposure to sotatercept (ACE-011) or luspatercept (ACE-536) or known allergic reaction to either one
• History of full pneumonectomy
• Initiation of a structured exercise program within 90 days prior or planned initiation during the study
• Known history of portal hypertension or chronic liver disease, including hepatitis B and/or hepatitis C (with evidence of recent infection and/or active virus replication), defined as mild to severe hepatic impairment (Child-Pugh Class A-C).
• Cardiac related history: History of restrictive, constrictive, or congestive cardiomyopathy; History of atrial septostomy within 180 days prior to the screening visit; ECG with Fridericia's corrected QT interval (QTcF) > 500 ms during the Screening Period; Personal or family history of long QT syndrome (LQTS) or sudden cardiac death; Left ventricular ejection fraction (LVEF) < 45% on historical ECHO within 12 months prior to the screening visit; Any symptomatic coronary disease events (prior myocardial infarction, percutaneous coronary intervention, coronary artery bypass graft surgery, or cardiac anginal chest pain) within 6 months prior to the screening visit. Note: Anginal pain can be ignored as an exclusion criterion if coronary angiography shows no obstructions
• Cerebrovascular accident within 3 months prior to the screening visit
• Acutely decompensated heart failure within 14 days prior to the screening visit, as per investigator assessment
• Significant (≥ 2+ regurgitation) mitral regurgitation or aortic regurgitation valvular disease
• Received intravenous inotropes (e.g., dobutamine, dopamine, norepinephrine, vasopressin) within 30 days prior to the screening visit

Study Plan

How is the study designed?

Design Details

• Primary Purpose: Treatment
• Allocation: N/A
• Interventional Model: Single Group Assignment
• Masking: None (Open Label)

Number of Arms

1

Arms and Interventions

Participant Group / Arm	Intervention / Treatment
Experimental: Exercise Arm; Patients with PAH on sotatercept plus a tailored, progressive home exercise program with wrist-worn fitness tracker and oxygen saturation monitoring	Other: Home exercise program; A tailored, progressive home exercise program with wrist-worn fitness tracker and oxygen saturation monitoring

What is the study measuring?

Primary Outcome Measures

Outcome Measure	Measure Description	Time Frame
Recruitment rate		18 months
Adherence and Completion Rate		18 months
Study-related adverse events (SAE) rate		18 months
Measurement fidelity for the innovative 'remote Six Minute Walk Test (6MWT)'	Patient self-administered via the digital fitness platform with remote monitoring at two interim assessment timepoints	18 months

Secondary Outcome Measures

Outcome Measure	Measure Description	Time Frame
Change from baseline in Six Minute Walk Test (6MWT) distance	Via Six Minute Walk Test administered during baseline and final in-person study visits	24 weeks
Change from baseline in Calculated Cardiac Effort (CEC) during 6MWT	Cardiac Effort to be calculated from Six Minute Walk Test administered during baseline and final in-person study visits	24 weeks
Change from baseline in nT-proBNP	Via bloodwork during baseline and final in-person study visits	24 weeks
Average weekly accelerometry minutes of moderate-to-vigorous physical activity (MVPA)		24 weeks
Health-related quality of life (QoL)	As measured by the PAH-specific survey PAH-SYMPACT®	24 weeks

Collaborators and Investigators

• Sponsor: University of Washington
• Investigators: Principal Investigator: Mary Beth Brown, PT, PhD, University of Washington

Study record dates

Study Major Dates

• Study Start (Actual): April 15, 2025
• Primary Completion (Estimated): September 30, 2026
• Study Completion (Estimated): September 30, 2026

Study Registration Dates

• First Submitted: April 15, 2025
• First Submitted That Met QC Criteria: April 15, 2025
• First Posted (Actual): April 23, 2025

Study Record Updates

• Last Update Posted (Actual): April 23, 2025
• Last Update Submitted That Met QC Criteria: April 15, 2025
• Last Verified: April 1, 2025

More Information

Terms related to this study

• Keywords: exercise; PAH; pulmonary arterial hypertension; sotatercept
• Additional Relevant MeSH Terms: Vascular Diseases; Cardiovascular Diseases; Respiratory Tract Diseases; Lung Diseases; Hypertension, Pulmonary; Pulmonary Arterial Hypertension; Hypertension; Familial Primary Pulmonary Hypertension
• Other Study ID Numbers: STUDY00021808 (Other Identifier: OHSU Knight Cancer Institute)

Plan for Individual participant data (IPD)

• Plan to Share Individual Participant Data (IPD)?: NO

Drug and device information, study documents

• Studies a U.S. FDA-regulated drug product: Yes
• Studies a U.S. FDA-regulated device product: No
• product manufactured in and exported from the U.S.: No