A 12-year-old boy with polysplenia and single ventricle experienced recurrent episodes of pneumonia, hemoptysis, and pulmonary hypertension. Unilateral pulmonary vein obstruction was diagnosed, and a left pneumonectomy was performed. Microscopy of the resected specimen revealed pulmonary veno-occlusive disease in the small pulmonary venules, and old arteritis in the small pulmonary arteries.
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