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An Infant with Abernethy Malformation Associated with Heterotaxy and Pulmonary Hypertension

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 Ⅱ 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. 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 Ⅱ 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.
出处 《Chinese Medical Journal》 SCIE CAS CSCD 2017年第18期2257-2258,共2页 中华医学杂志(英文版)
关键词 Heterotaxy Syndrome Portosystemic Shunt Pulmonary Hypertension Heterotaxy Syndrome Portosystemic Shunt Pulmonary Hypertension
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