RecruitingNCT05462574

Right Ventricle Lipid in Pulmonary Arterial Hypertension (PAH)

Clinical and Mechanistic Understanding of Right Ventricular Steatosis in Pulmonary Arterial Hypertension (PAH)

Sponsor

Vanderbilt University Medical Center

Enrollment

75 participants

Start Date

Jan 17, 2023

Study Type

OBSERVATIONAL

Conditions

Idiopathic Pulmonary Arterial Hypertension Heritable Pulmonary Arterial Hypertension Pulmonary Arterial Hypertension Associated With Connective Tissue Disease

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.

Eligibility

Min Age: 18 Years

Inclusion Criteria6

≥ 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 Criteria6

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