Recruiting
Right Ventricle Lipid in Pulmonary Arterial Hypertension (PAH)
The investigators propose to study the relationship between right ventricle (RV) steatosis and RV function, exercise capacity, and outcomes in humans with pulmonary arterial hypertension (PAH) and to identify potential drivers of lipid accumulation.
ClinicalTrials.gov IDNCT05462574
PhaseNot Applicable
Enrollment75
SponsorVanderbilt University Medical Center
Age18 Years
SexALL
Conditions studied
Idiopathic Pulmonary Arterial Hypertension, Heritable Pulmonary Arterial Hypertension, Pulmonary Arterial Hypertension Associated With Connective Tissue Disease
About this study
The investigators propose to study the relationship between right ventricle (RV) steatosis and RV function, exercise capacity, and outcomes in humans with pulmonary arterial hypertension (PAH) and to identify potential drivers of lipid accumulation.
Interventions
- Other: No Intervention — No Intervention
Primary outcomes
- Change in Right Ventricular (RV) Ejection Fraction (Baseline to 36 months)
- Change in Right Ventricular (RV) Lipid Content (Baseline to 36 months)
- Identification of metabolic markers (dihyroxybutyrate, acetylputriscene, hydroxystearate and glucuronate) in the peripheral circulation and coronary sinus. (Baseline to 36 months)
- Ratio of BMPR2 isoform B/A. (Baseline to 36 months)
- Change in skeletal muscle lipid content. (Baseline to 36 months)
Eligibility information
Inclusion criteria:
* ≥ 18 years old
* Diagnosed with idiopathic, heritable, connective tissue disease-associated PAH, associated pulmonary arterial hypertension (PAH), or drug-or toxin-associated PAH according to World Health Organization (WHO) consensus recommendations.
* Stable PAH-specific medication regimen for three months prior to enrollment. Adjustments in IV prostacyclin for side effect management are allowed. Diuretic adjustments are permitted.
* WHO Functional Class I-III
* Ambulatory
* Able to have an MRI/MRS, perform a 6MWD test, and cardiopulmonary exercise test
Exclusion criteria:
* Pregnancy
* Diagnosis of PAH etiology other than idiopathic, heritable, connective tissue disease - associated PAH or associated with drugs and toxins
* WHO Functional class IV heart failure
* Requirement for continuous oxygen
* Unable to have an MRI/MRS, perform a 6MWD test, or cardiopulmonary exercise test.
* Patients with implanted/embedded ferromagnetic material that would preclude cardiac MRI
Study locations
- Vanderbilt University Medical Center, Nashville, Tennessee 37232 United States
Before you participate: Varda Clinical is an independent discovery tool, not the study sponsor or a medical provider. Eligibility can only be determined by the official study team. Discuss potential risks and benefits with a qualified healthcare professional.
Source: ClinicalTrials.gov. Record last refreshed by Varda Clinical: 2026-09-27.