Abernethy
malformation withunusualcardiac malformation: case
report and literature review
Liyuan Xu1, Hongju Zhang1, Guowen
Liu1, Yunpeng Li2, Di
Li3, Ning Ma1,*
1 Department of Echocardiography, Beijing Children’s
Hospital, Capital Medical University, National Center for Children’s
Health, Beijing, 100045, China
2 Department of Emergency Surgery, Beijing Children’s
Hospital, Capital Medical University, National Center for Children’s
Health, Beijing, 100045, China
3 Department of Imaging Center, Beijing Children’s
Hospital, Capital Medical University, National Center for Children’s
Health, Beijing, 100045, China
* Corresponding author:
Department of Echocardiography, Beijing Children’s Hospital, Capital
Medical University, National Center for Children’s Health
No. 56 Nanlishi St, Xicheng District, Beijing, 100045, China
Tel.: +010-59616737
Email: echo_mn@163.com
Abstract: Abernethy malformation, also known as congenital
extrahepatic shunt, is a rare anomaly characterized by partial or
complete diversion of the portal blood into the systemic venous
circulation. The clinical manifestations of
Abernethy malformation during
childhood include neonatal cholestasis, failure to thrive, mental
retardation, and other congenital defects. We report a case of
Abernethy malformation Type Ⅱ in a
9-year-old boy whose left ventricle was slightly enlarged because of
several major aortopulmonary
collateral arteries but normal laboratory examinations five years
earlier. The characteristics of
congenital heart disease in patients with Abernethy malformation are
discussed. We propose that enlargement of the left ventricular with
systemic-pulmonary collateral
circulation should raise the suspicion of Abernethy malformation.
Keywords: Abernethy
malformation; echocardiography;
cardiac malformation; children; major aortopulmonary collateral arteries