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Pulmonary arteriovenous fistulas are seldom diagnosed in infancy because of few signs and symptoms in early life. The clinical picture is due to the right-to-left shunt and includes dyspnea, cyanosis, polycythemia, clubbing, and murmur over the fistula. The diagnosis can be suspected by demonstration of a typical density on chest X-ray. It can be established by angiocardiography. Because of the life-threatening complications resectional treatment is recommended already in infancy. A case is reported, diagnosed on the second day of life by angiocardiography and treated 3 months later by lobectomy.
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