Pulmonary venoocclusive disease: case report and review of the literature

G. D. Shackelford, E. J. Sacks, J. D. Mullins, W. H. McAlister

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Abstract

A fatal case of pulmonary venoocclusive disease in a 4-year-old boy is reported, with emphasis on the radiologic features of this condition. The disease is characterized by fibrous intimal obliteration of the lumina of small pulmonary veins and venules leading to severe secondary pulmonary arterial hypertension. The clinical picture is usually indistinguishable from that of other forms of pulmonary arterial hypertension, but radiologic features of postcapillary pulmonary hypertension, in conjunction with a normal pulmonary wedge pressure, are suggestive of pulmonary venoocclusive disease. The diagnosis is definitively established by lung biopsy.

Original languageEnglish
Pages (from-to)643-648
Number of pages6
JournalAmerican Journal of Roentgenology
Volume128
Issue number4
DOIs
StatePublished - 1977

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