Background: Patients with complex congenital heart defects palliated by connecting the systemic veins directly to the pulmonary circulation are known to develop hepatopulmonary syndrome (HPS). Although rare, HPS can develop following the Fontan operation.
Objective: To present and analyse the CT findings of HPS in patients with a Fontan circulation.
Materials and methods: From May to December 2005, six patients with HPS following the completion of a Fontan circulation were evaluated. CT findings were reviewed and were compared with angiographic findings.
Results: All six patients showed unilateral involvement. All patients except one had inferior vena cava (IVC) interruption with azygos continuation. CT scans showed abnormal vascular dilatation in one lung, and properly demonstrated the anatomy causing the hepatic venous blood to flow preferentially into one lung. These CT findings correlated well with the angiography findings.
Conclusion: HPS that develops after the Fontan procedure is typically unilateral and is often associated with IVC interruption and azygos or hemiazygos continuation. CT demonstrates dilatation of pulmonary vessels in the affected lung and may be able to demonstrate the underlying anatomical cause for the predilection of hepatic venous flow to the contralateral lung.