A Case of Congenital Intrahepatic Portosystemic Shunt Associated with VSD Detected by Antenatal Sonography and Treated with Four Coil Embolizations and Open Heart Surgery after Birth.
- Author:
Ji Youn NA
1
;
Eun Sun KIM
;
Sang Duk KIM
;
Ee Kyung KIM
;
Han Suk KIM
;
Jung Hwan CHOI
;
Jeong Eun CHUNG
;
Jin Uuk JOUNG
Author Information
1. Department of Pediatrics, Seoul National University College of Medicine, Seoul, Korea. neona@plaza.ac.kr
- Publication Type:Case Report
- Keywords:
Congenital intrahepatic portosystemic shunt;
Heart failure;
Ventricular septal defect;
Coil embolization
- MeSH:
Early Diagnosis;
Heart;
Heart Failure;
Heart Septal Defects, Ventricular;
Hepatic Encephalopathy;
Humans;
Hyperammonemia;
Hypertension, Pulmonary;
Hypoglycemia;
Infant;
Jaundice;
Liver Cirrhosis;
Parturition;
Portasystemic Shunt, Surgical;
Thoracic Surgery
- From:Journal of the Korean Society of Neonatology
2008;15(2):176-182
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
A congenital portosystemic shunt is a very rare portosystemic vascular anomaly which leads to jaundice, hypoglycemia, hyperammonemia, liver cirrhosis, hepatic coma, and pulmonary hypertension. Anatomically, portosystemic shunts are divided into intra- and extra- hepatic shunts. Congenital intrahepatic portosystemic shunts are rare anomalies, and the early diagnosis is important to prevent hepatic encephalopathy and hypoglycemia. We report a case of an infant with symptoms of heart failure due to a congenital intrahepatic portosystemic shunt and a ventricular septal defect (VSD), which were treated successfully with four coil embolizations and open heart surgery for the VSD.
