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.