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.
≥ 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
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
Patients with PAH will be recruited from the Center for Pulmonary Vascular Disease (CPVD) at Vanderbilt University Medical Center.
Cohort
Prospective
No Intervention
Other interventionNo Intervention
Change in Right Ventricular (RV) Ejection Fraction
Change in RV ejection fraction will be measured by cardiac MRI.
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.
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.
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.
Click on the lead sponsor to view all of their trials.
Vanderbilt University Medical Center
Lead sponsor
National Heart, Lung, and Blood Institute (NHLBI)
Collaborator
