Choi Young Hun, Lee Whal, Cheon Jung-Eun, Kim Woo Sun, Kim In-One, Park Jae Hyung, Yeon Kyung Mo
Radiology Department, Seoul National University Hospital, Chong-No-Gu, Seoul, Korea.
Pediatr Radiol. 2009 Apr;39(4):336-42. doi: 10.1007/s00247-008-1120-6. Epub 2009 Feb 3.
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.
To present and analyse the CT findings of HPS in patients with a Fontan circulation.
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.
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.
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.
通过将体循环静脉直接连接到肺循环来缓解复杂先天性心脏缺陷的患者已知会发生肝肺综合征（HPS）。虽然罕见，但HPS可在Fontan手术后发生。
呈现并分析Fontan循环患者HPS的CT表现。
2005年5月至12月，对6例完成Fontan循环后发生HPS的患者进行评估。回顾CT表现并与血管造影结果进行比较。
所有6例患者均表现为单侧受累。除1例患者外，所有患者均存在下腔静脉（IVC）中断并奇静脉延续。CT扫描显示一侧肺血管异常扩张，并正确显示了导致肝静脉血优先流入一侧肺的解剖结构。这些CT表现与血管造影结果密切相关。
Fontan手术后发生的HPS通常为单侧，且常与IVC中断及奇静脉或半奇静脉延续有关。CT可显示患侧肺血管扩张，并可能能够显示肝静脉血流偏向对侧肺的潜在解剖学原因。
