NCT05462574

Brief Summary

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.

Trial Health

77
On Track

Trial Health Score

Automated assessment based on enrollment pace, timeline, and geographic reach

Enrollment
75

participants targeted

Target at P50-P75 for all trials

Timeline
17mo left

Started Jan 2023

Longer than P75 for all trials

Geographic Reach
1 country

1 active site

Status
recruiting

Health score is calculated from publicly available data and should be used for screening purposes only.

Trial Relationships

Click on a node to explore related trials.

Study Timeline

Key milestones and dates

Study Progress70%
Jan 2023Sep 2027

First Submitted

Initial submission to the registry

July 14, 2022

Completed
4 days until next milestone

First Posted

Study publicly available on registry

July 18, 2022

Completed
6 months until next milestone

Study Start

First participant enrolled

January 17, 2023

Completed
4.7 years until next milestone

Primary Completion

Last participant's last visit for primary outcome

September 30, 2027

Expected
Same day until next milestone

Study Completion

Last participant's last visit for all outcomes

September 30, 2027

Last Updated

May 16, 2025

Status Verified

May 1, 2025

Enrollment Period

4.7 years

First QC Date

July 14, 2022

Last Update Submit

May 13, 2025

Conditions

Idiopathic Pulmonary Arterial HypertensionHeritable Pulmonary Arterial HypertensionPulmonary Arterial Hypertension Associated With Connective Tissue Disease

Outcome Measures

Primary Outcomes (5)

  • Change in Right Ventricular (RV) Ejection Fraction

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

    Baseline to 36 months

  • Change in Right Ventricular (RV) Lipid Content

    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.

    Baseline to 36 months

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

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

    Baseline to 36 months

  • Ratio of BMPR2 isoform B/A.

    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.

    Baseline to 36 months

  • Change in skeletal muscle lipid content.

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

    Baseline to 36 months

Study Arms (1)

Participants with Pulmonary Arterial Hypertension (PAH)

Participants with heritable, idiopathic, and scleroderma associated PAH.

Other: No Intervention

Interventions

No InterventionOTHER

No Intervention

Participants with Pulmonary Arterial Hypertension (PAH)

Eligibility Criteria

Age18 Years+
Sexall
Healthy VolunteersNo
Age GroupsAdult (18-64), Older Adult (65+)
Sampling MethodNon-Probability Sample
Study Population

Patients with PAH will be recruited from the Center for Pulmonary Vascular Disease (CPVD) at Vanderbilt University Medical Center.

You may qualify if:

  • ≥ 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

You may not qualify if:

  • 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

Contact the study team to confirm eligibility.

Sponsors & Collaborators

Study Sites (1)

Vanderbilt University Medical Center

Nashville, Tennessee, 37232, United States

RECRUITING

MeSH Terms

Conditions

Familial Primary Pulmonary Hypertension

Condition Hierarchy (Ancestors)

Hypertension, PulmonaryLung DiseasesRespiratory Tract Diseases

Study Officials

  • Evan Brittain, MD, MSCI

    Vanderbilt University Medical Center

    PRINCIPAL INVESTIGATOR

Central Study Contacts

Natasha Billard

CONTACT

(434) 851-3306natasha.billard.1@vumc.org

Evan Brittain, MD, MSCI

CONTACT

(615) 322-4382evan.brittain@vumc.org

Study Design

Study Type
observational
Observational Model
COHORT
Time Perspective
PROSPECTIVE
Sponsor Type
OTHER
Responsible Party
PRINCIPAL INVESTIGATOR
PI Title
Associate Professor of Medicine

Study Record Dates

First Submitted

July 14, 2022

First Posted

July 18, 2022

Study Start

January 17, 2023

Primary Completion (Estimated)

September 30, 2027

Study Completion (Estimated)

September 30, 2027

Last Updated

May 16, 2025

Record last verified: 2025-05

Locations

US(1)