Hiroko Tanaka

Diffuse pulmonary arteriovenous fistulae secondary to patent ductus venosus

We report on a 10-year-old girl in whom a patent ductus venosus caused diffuse pulmonary arteriovenous fistulae which were cured after surgical closure of the ductus. A 4-year-old Japanese girl developed hyperthyroidism in December 1995. In March 2000, systemic lupus erythematosus (SLE) was diagnosed with findings of photosensitivity, thrombocytopenia, and positive autoantibodies. Remission of SLE was obtained by administration of methylprednisolone. The patient developed exercise intolerance in May 2000, followed by the appearance of cyanosis of the lips and digital clubbing. Arterial blood gases revealed a PaO2 of 56.6 mm Hg and a PaCO2 of 30.1 mm Hg. Perfusion scintigraphy of the lung using Tc-macroagglutinated albumin demonstrated the presence of significant right-to-left shunting. Pulmonary arteriography showed a pattern indicating diffuse pulmonary arteriovenous fistulae (D-PAVF) (Fig. 1). Mean pulmonary arterial pressure was 9 mm Hg. Laboratory examinations showed mild hepatic dysfunction (AST 57 U/l,