Pulmonary Vascular Occlusive Disease Presenting as Sudden Death

We report a case of pulmonary vascular occlusive disease (also termed pulmonary veno occlusive disease) which caused the sudden death of an eleven-year-old girl. The post-mortem findings are described and the current theories of aetiology and pathogenesis of this unusual condition are reviewed.

The clinical picture is characterized by pulmonary hypertension with marked increase of pulmonary arterial pressure but often normal or only slightly elevated wedge pressure. The pulmonary vasculature, especially the veins, show progressive fibrotic occlusion. The aetiology is unknown but an infective cause is considered most likely. Definitive diagnosis usually depends on an open lung biopsy or, more commonly, autopsy examination.