Unilateral Pulmonary Vein Atresia with Absent Right Superior Vena Cava

ABSTRACT

A case of unilateral pulmonary vein atresia with absent right superior vena cava in 9 years-old boy is described with a discussion. Clinical findings were similar to other reports and consisted of hemoptysis, dyspnea and anemia. A chest X-rary revealed irregular vascular marking on left lung fiedld and increased interstitial marking on right upper lung field. The diagnosis could be confirmed with radionuclide perfusion study, MRI scan of heart and great vessels and angiography which demonstrated a small right pulmonary artery, pruned its peipheral branches, stasis of contrast material and nonvisualization of draining righ pulmonary veins. Pneumonectomy, patch angioplasty of diaphargm excision will be necessary.