Development of bilateral diffuse pulmonary arteriovenous fistula after Fontan procedure: is there nonhepatic factor?

The cause of pulmonary arteriovenous fistulas after a cavopulmonary anastomosis is not clearly known, but the previous studies regarding their causes have been focused mainly on the hepatic factors. We report two unusual Fontan cases with multiple bilateral pulmonary arteriovenous fistulas. One of them died of progressive hypoxia, and the other underwent multiple coiling. Our cases suggest that potentially lethal diffuse pulmonary arteriovenous fistulas may develop bilaterally in spite of balanced hepatic effluent flow to pulmonary circulation through factors other than a hepatic factor.