Hormonal, Metabolic, and Signaling Interaction in Pulmonary Arterial Hypertension
Our hypothesis is that optimal treatment of the dysfunctional metabolic pathways which underlie PAH will improve pulmonary vascular function and consequences of the disease.
⁃ Project 1
⁃ Inclusion:
• Diagnosis of IPAH (idiopathic pulmonary arterial hypertension), HPAH (heritable pulmonary arterial hypertension), or APAH (associated pulmonary arterial hypertension), family members of affected persons
• Age 0-90, age 12-90 for skin biopsy
⁃ Exclusion:
• Other diagnosis
• Age greater than 90, age less than 12 or greater than 90 for skin biopsy
⁃ Project 2
⁃ Inclusion:
• Diagnosis of IPAH, HPAH, or APAH, family members of affected persons
• 0-90
• Subjects with reasonably easy access to clinic for blood collection and other testing
• Subject able to tolerate fasting state prior to sample collection and EndoPAT (endothelial function assessment) testing
⁃ Exclusion:
• Other diagnosis
• 0-90
• Subjects with difficulty reaching clinic for blood collection and other testing
• Subjects unable to tolerate fasting state
⁃ Project 3
⁃ Inclusion:
• Diagnosis of IPAH, HPAH, or APAH, family members of affected persons
• 7-90
⁃ Exclusion:
• Other diagnosis
• Age less than 7 or greater than 90
• \-