PRIMARY PULMONARY-HYPERTENSION - INSIGHTS INTO PATHOGENESIS FROM EPIDEMIOLOGY

Primary pulmonary hypertension (PPH) is a rare disease that affects yo ung people predominantly of female gender. Early epidemiologic studies have shown that the diagnosis is usually made 1 to 2 years after symp toms onset, and the mean survival is reduced to 2 to 3 years thereafte r. New insights into the pathogenesis of PPH by epidemiologic studies may be obtained through the utilization of informatic technologies cou pled to a clear definition of the disease. Early stages of precapillar y pulmonary hypertension could be identified through screening tests l ike echocardiography in populations with higher incidence, such as fam ilial PPH and the conditions associated with pulmonary hypertension. T hese latter conditions are hemodynamically and pathologically similar to the primary form, and they can give insight into several possible a spects of the pathogenesis of PPH, Prospective registries are very use ful in coordinating the collection of epidemiologic data, and new tech nologies, such as informatics, may improve the management and the cont inuous updating of the databases.