A Case of Pulmonary Arteriovenous Fistula in a Patient with Rendu-Osler-Weber Disease with Severe Anemia with the Hemoglbin Level of 3.4g/dl

Abstract

A 70-year-old man who had been aware of recurrent bouts of nasal bleeding since around 50 years of age was pointed out a tumorous shadow in the left middle lung field on a chest x-ray examination at a medical checkup 6 years ealier, but he let it alone without any treatment. Recently he was seen at the Department of Cardiovascular Diseases in our hospital because of dyspnea, when blood analysis revealed severe anemia with the Hb level of 3.4g/dl due to recurrent bouts of nasal bleeding. In addition, an increase in size of the tumorous shadow and bilateral pleural effusion which might be caused by right heart insufficiency were demonstrated. Echocardiography showed a high right atrial pressure of 50 mmHg. Three-dimensional-CT scan reveled an about 6-cm sized pulmonary arteriovenous fistula at the left upper lobe. Accordingly the patient was transferred to our surgical department for the purpose of surgery. His family members were confirmed also to have recurrent bouts of nasal bleeding, and Rendu-Osler-Weber disease (ROW) was strongly suspected. Pulmonary arteriovenous fistula associated with ROW was thus diagnosed. At surgery, left upper lobectomy was performed under left posterolateral thoracotomy. His anemia showing the Hb level of 3.4 g/dl before the operation was improved up to 11.2g/dl after the operation when he was discharged. The SaO2 level was also improved from 93％ before the operation to 97％ (room air) after the operation. The patient was discharged on the seventh hospital day.