Pulmonary hypertension and right ventricular failure. Part VI. Classification and pathomorphology of primary pulmonary arterial hypertension.

In a series of articles the authors discuss literature data concerning epidemiology of pulmonary hypertension (PH), its modern classification; peculiarities of its pathogenesis and treatment in various diseases and conditions. In the sixth communication they present classification of PH accepted at the Third World PH Symposium (venice, Italy, 2003). This classification abandons terms "primary" and "secondary" PH. Primary PH which is now recommended to be called "idiopathic pulmonary arterial hypertension" is grouped in one category with familial cases of PH, PH associated with administration of anorexigens, collagen vascular disease, congenital systemic to pulmonary shunts, portal hypertension and hyperthyroidism, as well as with pulmonary veno-occlusive disease and and pulmonary capillary hemangiomatosis, taking into consideration similarity of histopathological changes of vascular tree, pathophysiology and therapeutic approaches in these forms of PH.