CORE B: This Core supports the Projects in this application through three functions. These are: Function 1) to coordinate recruitment of PAH patients and controls, clinically evaluate enrollees and conduct sample collection;Function 2) establish and maintain a biospecimen repository and distribute its resources (serum, plasma, circulating cells, cultured fibroblasts, DNA, RNA, banked tissue samples) to Projects 1, 2 and 3 and to collaborators at Columbia and for other NIH funded investigators;Function 3) facilitate analysis of data, especially human data in Projects 1, 2 and 3. The idiopathic PAH patients will largely be drawn from our Center for Pulmonary Vascular Disease, a clinical center that has nearly 450 reference patients and has successfully participated in over 20 national clinical trials over the last decade. Controls will come from the Vanderbilt General Clinical Research Center bank ( and from within families. We use a national specimen and survey company for offsite collection of specimens. HPAH patients will also be recruited from Columbia University through an ongoing collaboration with Dr. Wendy Chung who runs the PH genetics component of the Columbia PH group. In Function 2, blood, urine, fibroblasts, cells, DNA, RNA and tissue will be generated, processed and stored in the laboratories of the Pulmonary Circulation Genomics Center, the Vanderbilt DNA Resource Core and the laboratories of the Pulmonary Division. Management of the entire collection will be done by dedicated personnel using the comprehensive REDCap data base. We have a history of over 2 thousand total subjects in our database, including the Progeny Software used for genetic and family data, and can move DNA, RNA, data and materials reliably and accurately. Function 3 is the analysis of data created largely in Projects 1 and 2. The development and design of experiments has been aided by our Epidemiologist and Statistician who will be the statistical coordinator for the entire PPG, including powering, sample size, estimates, outcome measures, data check and quality control, application of appropriate biostatlstical tests depending on data sets and hypotheses. Thus statistical support is fully integrated into the design, conduct and analysis.

Public Health Relevance

Pulmonary arterial hypertension (PAH) is elevated blood pressure in the lungs, which leads to right heart failure and death. No existing treatments are very effective. This Program Project Grant aims to develop new, more effective treatments based on interventions against the hormonal, metabolic, and signaling defects recently shown to form the molecular basis for disease. Core B supports these functions.

National Institute of Health (NIH)
National Heart, Lung, and Blood Institute (NHLBI)
Research Program Projects (P01)
Project #
Application #
Study Section
Special Emphasis Panel (ZHL1-CSR-Q)
Project Start
Project End
Budget Start
Budget End
Support Year
Fiscal Year
Total Cost
Indirect Cost
Vanderbilt University Medical Center
United States
Zip Code
Best, D Hunter; Sumner, Kelli L; Austin, Eric D et al. (2014) EIF2AK4 mutations in pulmonary capillary hemangiomatosis. Chest 145:231-6
Robbins, Ivan M; Hemnes, Anna R; Pugh, Meredith E et al. (2014) High prevalence of occult pulmonary venous hypertension revealed by fluid challenge in pulmonary hypertension. Circ Heart Fail 7:116-22
Brittain, Evan L; Hemnes, Anna R (2014) One generation's "junk" is another's treasure: the emerging role of microRNAs as therapeutic targets. J Heart Lung Transplant 33:233-4
Stearman, Robert S; Cornelius, Amber R; Lu, Xiao et al. (2014) Functional prostacyclin synthase promoter polymorphisms. Impact in pulmonary arterial hypertension. Am J Respir Crit Care Med 189:1110-20
Hemnes, Anna R; Brittain, Evan L; Trammell, Aaron W et al. (2014) Evidence for right ventricular lipotoxicity in heritable pulmonary arterial hypertension. Am J Respir Crit Care Med 189:325-34
Zhao, Min; Austin, Eric D; Hemnes, Anna R et al. (2014) An evidence-based knowledgebase of pulmonary arterial hypertension to identify genes and pathways relevant to pathogenesis. Mol Biosyst 10:732-40
Austin, Eric D; Loyd, James E (2014) The genetics of pulmonary arterial hypertension. Circ Res 115:189-202
Brittain, Evan L; Pugh, Meredith E; Wheeler, Lisa A et al. (2013) Prostanoids but not oral therapies improve right ventricular function in pulmonary arterial hypertension. JACC Heart Fail 1:300-7
Brittain, Evan L; Pugh, Meredith E; Wang, Li et al. (2013) Predictors of diastolic-to-wedge gradient in patients evaluated for pulmonary hypertension. PLoS One 8:e76461
Austin, Eric D; Loyd, James E (2013) Heritable forms of pulmonary arterial hypertension. Semin Respir Crit Care Med 34:568-80