pulmonary veno-occlusive disease

pulmonary veno-occlusive disease

An idiopathic condition of children/young adults that causes progressive fibrous obliteration of veins, resulting in severe postcapillary pulmonary hypertension Etiology Uncertain, possibly linked to BM transplantation, chemotherapy–eg, carmustine, malignancy, viral infection Clinical Nodular zones of congestion, edema, hemorrhage, hemosiderosis Radiology CXR reveal prominent interstitial markings, Kerley B lines, pulmonary artery dilatation Management Temporary response may occur with vasodilators, nifedipine, α-adrenergic blocker, prazosin Prognosis Poor, most die in 2 yrs