Histopathology of Primary Pulmonary Hypertension

By definition, the clinical diagnosis of primary pulmonary hypertension (PPH) can be made only after any secondary cause of pulmonary hypertension is ruled out. In the past, pathologic confirmation of the clinical diagnosis was based on demonstrating the presence of the so-called plexogenic pulmonary arteriopathy in the absence of any congenital cardiac left-to-right shunt. It is now recognized that there are no specific histopathologic lesions of PPH and that the diagnosis of this disease requires the close integration of clinical symptoms, laboratory investigations, and histopathologic findings. The purpose of this presentation was to describe the types of histopathologic lesions that can produce PPH. Table 1Types of Pulmonary Angiopathy in Five Series of Patients With PPH 1970 3 Wagenvoort CA Wagenvoort N Primary pulmonary hypertension: a pathologic study of the lung vessels in 156 clinically diagnosed cases. Circulation. 1970; 42: 1163-1184 Crossref Google Scholar 1980 22 Wagenvoort CA Lung biopsy specimens in the evaluation of pulmonary vascular disease. Chest. 1980; 77: 614-625 Crossref PubMed Scopus (136) Google Scholar 1985 1 Bjornsson J Edwards WD Kay JM Rich S Kernis J Schloo B et al. Primary pulmonary hypertension: a histopathologic study of 80 cases. Mayo Clin Proc. 1985; 60: 16-25 Abstract Full Text Full Text PDF PubMed Scopus (203) Google Scholar 1989 2 Pietra GG Edwards WD Kay JM et al. Histopathology of pulmonary hypertension: a qualitative and quantitative study of pulmonary blood vessels from 58 patients in National Heart, Lung, and Blood Institute, Primary Pulmonary Hypertension Registry. Circulation. 1989; 80: 1198-1206 Crossref PubMed Scopus (424) Google Scholar 1990 12 Burke AP Farb A Virmani R The pathology of primary pulmonary hypertension. Modem Pathol. 1991; 4: 269-282 PubMed Google Scholar No. Patients 156 40 80 58 94 Arteriopathy (%) PPA 98 (63) 14 (35) 22 (28) 29 (50) 52 (55) TPA 31 (20) 12 (30) 45 (56) 19 (33) 11 (12) IMH 12 (8) … 3 (4) 1 (2) 6 * Includes patients with medial hypertrophy and intimal fibrosis. (6) Venopathy (%) PVOD 5 (3) 2 (5) 5 (6) 7 (12) 25 (26) CVH 5 (3) 10 (25) 2 (3) 1 (2) … Others (%) † Includes patients with normal pulmonary vessels, hypoxic arteriopathy, pulmonary arteritis, etc. 5 (3) 2 (5) 3 (4) 1 (2) … * Includes patients with medial hypertrophy and intimal fibrosis. † Includes patients with normal pulmonary vessels, hypoxic arteriopathy, pulmonary arteritis, etc. Open table in a new tab