Primary pulmonary hypertension: insights into pathogenesis from epidemiology

Primary pulmonary hypertension (PPH) is a rare disease that affects young people predominantly of female gender. Early epidemiologic studies have shown that the diagnosis is usually made 1 to 2 years after symptoms onset, and the mean survival is reduced to 2 to 3 years thereafter. New insights into the pathogenesis of PPH by epidemiologic studies may be obtained through the utilization of informatic technologies coupled to a clear definition of the disease. Early stages of precapillary pulmonary hypertension could be identified through screening tests like echocardiography in populations with higher incidence, such as familial PPH and the conditions associated with pulmonary hypertension. These latter conditions are hemodynamically and pathologically similar to the primary form, and they can give insight into several possible aspects of the pathogenesis of PPH. Prospective registries are very useful in coordinating the collection of epidemiologic data, and new technologies, such as informatics, may improve the management and the continuous updating of the databases.