PAH Exercise Study

Launched by UNIVERSITY OF WASHINGTON · Apr 15, 2025

Keywords

ClinConnect Summary

The PAH Exercise Study is a clinical trial aimed at exploring the effects of a medication called sotatercept combined with a home exercise program for patients with Pulmonary Arterial Hypertension (PAH). This study will last for 26 weeks and involves ten patients who are stable enough to start sotatercept therapy. During the trial, participants will receive the medication and follow a personalized exercise plan monitored through a fitness tracker. This plan is designed to help improve their overall health while keeping track of their oxygen levels.

To qualify for the study, participants must be at least 18 years old and have a confirmed diagnosis of certain types of PAH. They should also be experiencing moderate symptoms but have been stable on their current PAH treatment for at least 90 days. Those who are interested in participating should be willing to engage in the exercise program and attend regular study visits. It’s important to note that some individuals, such as those with specific heart or lung conditions, may not be eligible to join the study. If you think you might qualify or want more information, please speak with your healthcare provider.

Gender

ALL

Eligibility criteria

• 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