[Primary pulmonary arterial hypertension. Current considerations]

Primary pulmonary hypertension (PPH) is a rare, progressive and fatal condition. Because of nonspecific signs and symptoms, patients diagnosed with PPH are often present with a relatively advanced disease. Significant advances have been made in recent years. A revised nomenclature has been proposed, the pathobiology is now better defined, new risk factors have been identified, and the newer therapeutic strategies appear to be changing the natural history and prognosis of this disease.