Pulmonary arterial hypertension as a chief sign rows rare diseases

Aim: comparative analysis of roentgenological and morphological symptomatologys of such rare medical condition as primary idiopathic pulmonary hypertension, pulmonary vein-occlusion disease, pulmonary capillary hemangiomatosis with the chief sign - pulmonary arterial hypertension. Material and methods: x-ray and morphological data of 78 patients was analysed (49 female and 29 male; mean age: 33±4,2) Results: Several patterns of pulmonary vascularity changes were identified: patients had diffuse vascular abnormalities (oligemia and rapidly tapering vessels); patients had only peripheral vascular abnormalities, pulmonary vasculature pattern in the central lung zone was not modified; pulmonary peripheral vasculature remained intact in cases with peculiar chaotic pattern in the form of reticulation and looping. Pulmonary trunk and right descending branch of pulmonary artery dilation, moderate cardiomegaly. The series cases were characterised by the absence of manifestations of pulmonary vasculature and hypertension, which complicated life-time diagnosis of the condition. Morphological verification of the diagnosis was based on autopsy findings: presence both, surplus of thin-walled capillary-sized vessels that proliferated the adventitia of pulmonary arteries and veins, bronchioles and alveolus, and foci of the obliterated vessels with plexoform or tromboembolic types lesion.