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. |
Ji Youn Na, Eun Sun Kim, Sang Duk Kim, Ee Kyung Kim, Han Suk Kim, Jung Hwan Choi, Jeong Eun Chung, Jin Uuk Joung |
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. |
산전 초음파로 발견되고 출생 후 4회의 코일 색전술과 심 교정술로 치료된 심실 중격 결손을 동반한 선천성 간내 문맥전신성 단락 1례 |
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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. |
Key Words:
Congenital intrahepatic portosystemic shunt; Heart failure; Ventricular septal defect; Coil embolization |
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