Specificity of pulmonary vascular lesions in primary pulmonary hypertension. A reappraisal.

The type and distribution of pulmonary vascular lesions in 23 cases of primary pulmonary hypertension were reviewed. 15 cases were classified as plexogenic arteriopathy. The remaining cases displayed thromboembolic lesions. All cases showed a predominance of eccentric intimal lesions. In the plexogenic group, mixed concentric and eccentric intimal fibrosis coexisted. Therefore, in the present study, we propose an objective descriptive approach to the diagnosis of hypertensive pulmonary vascular disease, to correlate morphology and function.