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.
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