[Primary pulmonary hypertension]

Primary pulmonary hypertension is a progressive disease of an unknown etiology which attacks mainly young people. Metabolic disturbances of biologically active substances produced in the endotheliocytes of pulmonary vessels play the leading role in pathogenesis of the disease. They result in structural changes of the vessels wall. In spite of the variety of the morphologic changes the clinical picture of the disease is generally common. A great variety of invasive and noninvasive investigations is necessary for verification of the diagnosis. Chemotherapy fails to stop the disease progress, though the main parameters of central hemodynamics are improved under the influence of vasodilators. Foreign investigators believe heart-lung transplantation to be radical treatment of patients suffering from primary pulmonary hypertension.