Improved severe hepatopulmonary syndrome after liver transplantation in an adolescent with end-stage liver disease secondary to biliary atresia

Authors
Tae Jun ParkKeun Soo AhnYong Hoon KimHyungseop KimUi Jun ParkHyoung Tae KimWon Hyun ChoWoo-Hyun ParkKoo Jeong Kang
Department
Dept. of Surgery (외과학); Dept. of Internal Medicine (내과학)
Issue Date
2014
Citation
Clinical and Molecular Hepatology, Vol.20(1) : 76-80, 2014
ISSN
2287-2728
Abstract
Hepatopulmonary syndrome (HPS) is a serious complication of end-stage liver disease, which is characterized by hypoxia, intrapulmonary vascular dilatation, and liver cirrhosis. Liver transplantation (LT) is the only curative treatment modality for patients with HPS. However, morbidity and mortality after LT, especially in cases of severe HPS, remain high. This case report describes a patient with typical findings of an extracardiac pulmonary arteriovenous shunt on contrast-enhanced transesophageal echocardiography (TEE), and clubbing fingers, who had complete correction of HPS by deceased donor LT. The patient was a 16-year-old female who was born with biliary atresia and underwent porto-enterostomy on the 55th day after birth. She had been suffered from progressive liver failure with dyspnea, clubbing fingers, and cyanosis. Preoperative arterial blood gas analysis revealed severe hypoxia (arterial O2 tension of 54.5 mmHg and O2 saturation of 84.2%). Contrast-enhanced TEE revealed an extracardiac right-to-left shunt, which suggested an intrapulmonary arteriovenous shunt. The patient recovered successfully after LT, not only with respect to physical parameters but also for pychosocial activity, including school performance, during the 30-month follow-up period. (Clin Mol Hepatol 2014;20:76-80) Keywords: Hepatopulmonary syndrome; Liver transplantation; Biliary atresia; Hypoxemia
URI
http://kumel.medlib.dsmc.or.kr/handle/2015.oak/35473
Appears in Collections:
1. Journal Papers (연구논문) > 1. School of Medicine (의과대학) > Dept. of Internal Medicine (내과학)
1. Journal Papers (연구논문) > 1. School of Medicine (의과대학) > Dept. of Surgery (외과학)
Keimyung Author(s)
안근수; 김용훈; 박의준; 김형태; 조원현; 박우현; 강구정; 김형섭
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