Introduction
Abernethy malformation, also known as a congenital portal shunt or congenital portal vein loss, results from malformation of the visceral venous system1. The disease was identified in 1793 by John Abernethy in an autopsy of a female infant; it was named Abernethy malformation in 19972,3. Two types of shunt have been defined: Type I is characterized by complete shunting of portal vein blood into the vena cava and is accompanied by congenital portal vein loss; Type II is characterized by an intact portal vein with an extrahepatic connection to the other side (the vena cava)4. Patients with Abernethy malformation have symptoms such as hematochezia, hematemesis, and abnormal liver function5,6. It may be associated with malformations such as hepatic nodules, musculoskeletal abnormalities, and congenital heart diseases1,4,7-9.
Although other congenital heart deformities have been described in the literature, we believe the following is the first reported case of Abernethy malformations with major aortopulmonary collateral arteries (MAPCAs), an unusual cardiac malformation in which systemic blood drains into pulmonary circulation via several collateral vessels. This article also discussed the characteristics of congenital heart disease in patients with Abernethy malformation.