pah exercise study

Conditions: Pulmonary Arterial Hypertension (PAH); Exercise Therapy

Summary: 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.


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

Keywords: pah pulmonary arterial hypertension sotatercept exercise