PAH Exercise Study

University of Washington

Status and phase

Enrolling

Phase 3

Conditions

Exercise Therapy

Pulmonary Arterial Hypertension (PAH)

Treatments

Other: Home exercise program

Study type

Interventional

Funder types

Other

Identifiers

NCT06941441

STUDY00021808 (Other Identifier)

Details and patient eligibility

About

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.

Enrollment

10 estimated patients

Sex

All

Ages

18+ years old

Volunteers

No Healthy Volunteers

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