Recruiting

Pulmonary Arterial Hypertension

Sponsor:

Vanderbilt University Medical Center

Code:

NCT05462574

Conditions

Idiopathic Pulmonary Arterial Hypertension

Heritable Pulmonary Arterial Hypertension

Pulmonary Arterial Hypertension Associated With Connective Tissue Disease

Eligibility Criteria

Sex: All

Age: 18+

Healthy Volunteers: Not accepted

Interventions

No Intervention

Study Details

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.

Conditions

Idiopathic Pulmonary Arterial Hypertension

Heritable Pulmonary Arterial Hypertension

Pulmonary Arterial Hypertension Associated With Connective Tissue Disease

Study ID

NCT05462574

Start date

Jan 17, 2023

Status verified date

May, 2026

Completion date

Sep 30, 2027

Anticipated

Primary completion date

Sep 30, 2027

Anticipated

Eligibility Criteria

Eligibility Criteria

Sex: All

Age: 18+

Healthy Volunteers: Not accepted

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 Design

Enrollment

75 participants

Anticipated

Interventions and Outcome Measures

Arms

Participants with Pulmonary Arterial Hypertension (PAH)

Participants with heritable, idiopathic, and scleroderma associated PAH.

Interventions

No Intervention

No Intervention

Primary outcome measure

  • Change in Right Ventricular (RV) Ejection Fraction [ Time Frame: Baseline to 36 months ]
  • Change in Right Ventricular (RV) Lipid Content [ Time Frame: Baseline to 36 months ]
  • Identification of metabolic markers (dihyroxybutyrate, acetylputriscene, hydroxystearate and glucuronate) in the peripheral circulation and coronary sinus. [ Time Frame: Baseline to 36 months ]
  • Ratio of BMPR2 isoform B/A. [ Time Frame: Baseline to 36 months ]
  • Change in skeletal muscle lipid content. [ Time Frame: Baseline to 36 months ]

Central Contacts and Locations

Central contacts

Natasha Billard

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

Evan Brittain, MD, MSCI

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

Locations

Vanderbilt University Medical Center

Recruiting

Nashville, Tennessee, United States, 37232

More Information

Sponsor

Vanderbilt University Medical Center

Last update posted

May 29, 2026

Last verified

May, 2026

Trial information was received from ClinicalTrials.gov and was last updated on 2026-09-09. This information was provided to ClinicalTrials.gov by Vanderbilt University Medical Center on 2026-05-29.