Pulmonary Arterial Hypertension
Pulmonary Arterial Hypertension Updated: 10/12/2022 © Jun Wang, MD, PhD Definition Formerly called primary pulmonary hypertension Resting mPAP ≥ 20 mm Hg, pulmonary capillary wedge pressure (PCWP) < 15 mm Hg, and PVR above 3 Wood units Absence of more prevalent causes of pulmonary hypertension such as left heart disease (group 2), chronic lung disease (group 3), or venous thromboembolism (group 4) General features Precapillary pulmonary hypertension Heterogenous in pathogenesis More common in women More common in white population Schistosomiasis likely the most common cause worldwide Most cases in the US are idiopathic Prognosis depends on etiology, severity and treatment Subclassification Idiopathic PAH Heritable PAH: BMPR2, ALK2 etc Drug- and toxin-induced PAH PAH associated with: o Connective tissue disease o HIV infection o Portal hypertension o Congenital heart disease o Schistosomiasis PAH long-term responders to calcium ch