Key result
Plexiform lesions and veno-occlusive patterns are linked to worse prognosis in primary pulmonary hypertension.
Why the study?
Several distinct histopathologic patterns of pulmonary vascular disease in primary pulmonary hypertension had not been fully characterized in a large patient cohort.
Observational (n=58)
Yes
Primary pulmonary hypertension presents with distinct histopathologic patterns (plexiform, thrombotic, veno-occlusive) that are associated with differing prognoses.
Histopathologic subtyping may refine prognostic stratification in pulmonary hypertension; leaves open need for prospective validation before clinical adoption.
Qualitative and quantitative studies were performed on pulmonary blood vessels in lung tissue obtained by biopsy, pneumonectomy, or autopsy from 58 patients in the Registry of Primary Pulmonary Hypertension sponsored by the Heart, Lung, and Blood Institute of the National Institutes of Health. In 49 patients (84%), the hypertensive vascular disease involved predominantly or exclusively muscular pulmonary arteries and arterioles. In each of these 49 patients, pulmonary artery medial hypertrophy was observed, and in 48 patients, it was also associated with intimal or luminal lesions. On the basis of the predominant histopathologic features, 25 of the 48 patients were classified as having pulmonary arteriopathy with plexiform lesions characterized by a combination of concentric laminar intimal fibrosis, eccentric intimal fibrosis, and plexiform lesions; in nine of these 25, recanalized thrombi were also present. Pulmonary arteriopathy with thrombotic lesions, defined by the presence of both eccentric intimal fibrosis and recanalized thrombi but without plexiform lesions, was observed in 19 patients. Intimal fibrosis, either concentric or eccentric, without plexiform or thrombotic lesions was found in four patients. Among the remaining nine patients in the Registry, pulmonary veno-occlusive disease was present in seven and chronic pulmonary venous hypertension in one. Pulmonary blood vessels were microscopically normal in a lung biopsy specimen from another patient. In general, patients with plexiform lesions and those with veno-occlusive disease had a much poorer prognosis than patients with thrombotic lesions. The present study shows the existence of several distinct histopathologic patterns of pulmonary vascular disease in individuals with primary pulmonary hypertension diagnosed by standardized clinical and laboratory criteria.
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Pietra et al. (1989) conducted an observational in Primary pulmonary hypertension (n=58). Histopathologic patterns was evaluated on Histopathologic patterns of pulmonary vascular disease. Histopathologic evaluation of 58 patients with primary pulmonary hypertension identified distinct vascular patterns, with plexiform lesions and veno-occlusive disease indicating a poorer prognosis.