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Journal of the Korean Society of Neonatology 2008;15(2):176-182.
Published online November 1, 2008.
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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