An Infant with Abernethy Malformation Associated with Heterotaxy and Pulmonary Hypertension

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 Abernethy 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.