J Korean Soc Neonatol.  2008 Nov;15(2):176-182.

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

Affiliations
  • 1Department of Pediatrics, Seoul National University College of Medicine, Seoul, Korea. neona@plaza.ac.kr
  • 2Department of Radiology, Seoul National University College of Medicine, Seoul, Korea.

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.

Keyword

Congenital intrahepatic portosystemic shunt; Heart failure; Ventricular septal defect; Coil embolization

MeSH Terms

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
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