Primary Pulmonary Arterial Hypertension

Primary Pulmonary Arterial Hypertension (PPAH) is a rare and progressive intrinsic disease of the pulmonary vascular smooth muscle and endothelial cells. PPAH is generated from a combination of abnormalities interacting at the cellular and molecular level. Since the ability of blood circulating through the lungs to pick up oxygen decreases, the patient experiences shortness of breath, fatigue, chest pain, dizzy spells, fainting, and even death if undiagnosed or untreated. Common abnormalities that can trigger PPAH involve endothelial cell dysfunction that disrupt a balance between vasoconstrictors and vasodilators, mutations in BMP/TGF-β protein structure that lead to cellular dysfunctions, and the interaction of Serotonin molecules with transmembrane proteins that generate cell signaling to constrict and divide cells. All molecular abnormalities result in the enhancement of Ca2+ and K+ concentration in the cytosol of the cell. The elevated concentration of both ions is the main reason why the pulmonary arterial smooth muscle cells constrict and continuously divide. Consequently, the lumen of the Pulmonary Artery decreases in size and increases in resistance, causing blood pressure to rise. Recent clinical trials have made progress in identifying treatment through oral, inhaled, subcutaneous, and intravenous medication that attacks the different abnormalities involved in the development of the disease