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Two cases of familial pulmonary arteriovenous fistula are reported. Case 1: A 54-year-old woman was admitted with exertional dyspnea. An abnormal shadow on chest X-ray had been noted since the age of 37 years. Pulmonary angiography demonstrated bilateral pulmonary arteriovenous fistulas. Case 2: A 25-year-old man, the son of Case 1, was admitted for operation for the same disease. The mother and son both suffered from repeated epistaxis due to Rendu-Osler-Weber disease. In order to reserve pulmonary function, fistulectomy was performed in both cases, and their subsequent clinical courses were good.
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