Unilateral pulmonary vein atresia: Diagnosis and treatment

The clinical, hemodynamic, angiographic and pathologic features of unilateral pulmonary vein atresia in a 16 month old boy are described. The relevant findings were symptoms and signs of influenza, roentgenographic evidence of unilateral diffuse interstitial edema, prominent interlobar fissures and pleural effusion, absence of abnormal cardiac structure and prominent unilateral bronchial circulation. Angiocardiography was necessary for a definitive diagnosis and revealed the lack of a site of entry for venous blood from the right lung into the circulation. The patient was treated successfully by a right pneumonectomy. The histologic hallmark in this case was extensive intimal fibrosis of the pulmonary veins.