Rapid onset of pulmonary arteriovenous malformations after cavopulmonary anastomosis

The reported incidence of pulmonary arteriovenous malformations after superior cavopulmonary anastamosis in patients with heterotaxia syndrome is 18%–21%. The manifestation is usually in young children and the onset is gradual. We report an unusual case of pulmonary arteriovenous malformations developing within 72 hours of bilateral superior cavopulmonary anastamosis (Kawashima procedure) in an adolescent with heterotaxia syndrome.