Pulmonary veno-occlusive disease: antemortem diagnosis from roentgenographic and hemodynamic findings.

A 17-year-old boy with pulmonary veno-occlusive disease underwent Swan--Ganz catheterization. A normal pulmonary capillary wedge pressure was recorded in the presence of severe pulmonary arterial hypertension and roentgenographic evidence of pulmonary venous congestion. This triad of findings permitted an unequivocal diagnosis of pulmonary veno-occlusive disease, which was later confirmed at autopsy. The hemodynamics of this condition and of others included in the differential diagnosis are presented schematically.