An Infant with Abernethy Malformation Associated with Heterotaxy and Pulmonary Hypertension

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摘要Abernethy malformation (AM) is a rare congenital anomaly in which the splanchnic blood bypasses the liver and drains directly into the systemic veins.It was first described by Abemethy in 1793 and has two types.Type I is characterized by a complete portosystemic shunt and an absence of a portal vein (consists of superior mesenteric and splenic veins),while Type IⅡ exhibits partial portosystemic shunt and a hypoplastic portal vein.Type I is further subclassified into subtype a and b based on the absence or presence of a patent connection between the superior mesenteric vein and the splenic vein.

作者单位 Department of Echocardiography, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing 100045, China ^[1] Department of Radiology, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing 100045, China ^[2] Department of Pediatrics, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing 100045, China ^[3]

关键词 Heterotaxy Syndrome Portosystemic Shunt Pulmonary Hypertension

栏目名称

Clinical Challenge

DOI 10.4103/0366-6999.213978

发布时间 2017-10-27

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