Right Ventricle Lipid in Pulmonary Arterial Hypertension (PAH) Study

This observational study is looking at how fat (lipid) in the right side of your heart (right ventricle, or RV) is related to how well your heart works and your ability to exercise if you have pulmonary arterial hypertension (PAH). Researchers want to understand why fat builds up in the RV and how it affects people with PAH, specifically those with idiopathic (unknown cause), heritable (runs in families), or connective tissue disease-associated PAH. The study will measure changes in your RV's pumping ability (ejection fraction) and fat content over 36 months, and look for specific markers in your blood. You may be able to join if you are 18 or older, have a diagnosis of PAH, and have been on a stable PAH medication for at least three months. The study aims to understand the connections between RV fat, heart function, and exercise capacity.

Study design
This is an observational study with a planned enrollment of 75 participants. It does not involve any new interventions or treatments.
What's involved
Participants will undergo a 6-minute walk test, cardiopulmonary exercise testing, and will be followed for clinical events. A subgroup will have repeat MRS (Magnetic Resonance Spectroscopy) scans at four different times over three years.
Compensation
Not stated in the trial record.
Follow-up
Participants will be followed for up to 36 months to measure changes in heart function and lipid content.

AI-generated from the public study record. Only the study team can confirm whether you're eligible — confirm details with them before making decisions.

NCT05462574

Right Ventricle Lipid in Pulmonary Arterial Hypertension (PAH)

Recruiting
Not specifiedAges 18+Observational
Vanderbilt University Medical Center
~75 participants
Updated 2026-05-29 on ClinicalTrials.gov
What's tested:No Intervention

At a glance

Recruiting sites
1 of 1 listed site is recruiting right now
RecruitingSuspended, closed, or not yet open
What they're measuring
Change in Right Ventricular (RV) Ejection Fraction
Measured over Baseline to 36 months
+4 more outcomes measured
Idiopathic Pulmonary Arterial Hypertension
Heritable Pulmonary Arterial Hypertension
Pulmonary Arterial Hypertension Associated With Connective Tissue Disease

NCT05462574

Where you'd take part

This study runs at 1 site. They're the same protocol — you choose where, and that choice sets who your contact draft is addressed to.

  • Vanderbilt University Medical Center

    Nashville, Tennesseeno site contact published

    Recruiting

Sites open and close at different times, so the status above is per site — it can differ from the study's overall status.

  • Evan Brittain, MD, MSCI · PRINCIPAL_INVESTIGATOR · Vanderbilt University Medical Center

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Eligibility criteria

Inclusion

≥ 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

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
  • Change in Right Ventricular (RV) Ejection FractionBaseline to 36 months

    Change in RV ejection fraction will be measured by cardiac MRI.

  • Change in Right Ventricular (RV) Lipid ContentBaseline to 36 months

    Change in RV lipid content will be measured by cardiac proton magnetic resonance spectroscopy (MRS). Lipid content is expressed as a percent of the voxel occupied by lipid.

  • Identification of metabolic markers (dihyroxybutyrate, acetylputriscene, hydroxystearate and glucuronate) in the peripheral circulation and coronary sinus.Baseline to 36 months

    Metabolite markers will be measured by ultrahigh performance liquid chromatography and mass spectrometry.

  • Ratio of BMPR2 isoform B/A.Baseline to 36 months

    BMPR2 isoforms A and B and wild type gene expression will be measured by real-time polymerase chain reaction (PCR) and validated by measuring protein content using Western blot test.

  • Change in skeletal muscle lipid content.Baseline to 36 months

    Change in skeletal muscle lipid content will be measured by skeletal muscle proton MRS. Lipid content is expressed as percent triglyceride (%TG)