We then assessed the clinical correlation of these five deregulated ECM proteins with mean pulmonary arterial pressure (mPAP), pulmonary vascular resistance (PVR) and cardiac index (CI), as well as N-terminal prohormone of brain natriuretic peptide (NT-proBNP levels) in the PAH participants. The gene discussed is NPPB; the disease is pulmonary arterial hypertension.