[Vascular changes in the lung in a case of primary (idiopathic) pulmonary hypertension]

The authors present one lethal case of primary pulmonary hypertension (PPH) in a male of 40. The pathomorphological examination revealed very characteristic changes in the vessels of the lungs. Often slot-like canals were found, with proliferated myoepithelial cells, connecting the lumena of vessels with thickened walls with thin-wall vessels ("plexiform changes") or with vessels with sinusoidal-cavernous type ("angiomatoid formations"). Those vascular changes are in a strong contrast with the absence of inflammatory alterations in the lungs. The possibilities of developing of similar vascular changes in the lungs, leading to PPH are discussed in accordance with the up-to date literature data.