The reported incidence of pulmonary arteriovenous malformations after super
ior cavopulmonary anastamosis in patients with heterotaxia syndrome is 18%-
21%. The manifestation is usually in young children and the onset is gradua
l. We report an unusual case of pulmonary arteriovenous malformations devel
oping within 72 hours of bilateral superior cavopulmonary anastamosis (Kawa
shima procedure) in an adolescent with heterotaxia syndrome. (C) 1999 by Th
e Society of Thoracic Surgeons.