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An Infant with Abernethy Malformation Associated with Heterotaxy and Pulmonary Hypertension

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Author:
No author available
Journal Title:
Chinese Medical Journal
Issue:
18
DOI:
10.4103/0366-6999.213978
Key Word:
Heterotaxy Syndrome;Portosystemic Shunt;Pulmonary Hypertension

Abstract: 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.

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