ABSTRACT
Primary pulmonary hypertension (PPH) is a progressive, fatal disease of unknown cause. It is characterized by elevation of pulmonary arterial pressure (PAP) and pulmonary vascular resistance (PVR), leading progressively to right heart failure and, eventually, death. Incidence of this disease is rare, occurring in one or two per million in the general population, and predominantly affects women. The time from diagnosis to right heart failure and death is short (median of 2.8 years) (1,2).
