Improved severe hepatopulmonary syndrome after liver transplantation in an adolescent with end-stage liver disease secondary to biliary atresia.
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.
Department of Surgery, Keimyung University School of Medicine, Daegu, Korea.
Tae Jun
TJ
Park
Department of Surgery, Keimyung University School of Medicine, Daegu, Korea.
Keun Soo
KS
Ahn
Department of Surgery, Keimyung University School of Medicine, Daegu, Korea.
Yong Hoon
YH
Kim
Department of Internal Medicine, Keimyung University School of Medicine, Daegu, Korea.
Hyungseop
H
Kim
Department of Surgery, Keimyung University School of Medicine, Daegu, Korea.
Ui Jun
UJ
Park
Department of Surgery, Keimyung University School of Medicine, Daegu, Korea.
Hyoung Tae
HT
Kim
Department of Surgery, Keimyung University School of Medicine, Daegu, Korea.
Won Hyun
WH
Cho
Department of Surgery, Keimyung University School of Medicine, Daegu, Korea.
Woo-Hyun
WH
Park
Department of Surgery, Keimyung University School of Medicine, Daegu, Korea.
Koo Jeong
KJ
Kang
D000293:Adolescent; D001164:Arteriovenous Fistula / Q000209:etiology; D001656:Biliary Atresia / Q000175:diagnosis* / Q000209:etiology; D003490:Cyanosis / Q000150:complications; D004417:Dyspnea / Q000150:complications; D017548:Echocardiography, Transesophageal; D058625:End Stage Liver Disease / Q000150:complications / Q000601:surgery*; D005260:Female; D006499:Hepatic Artery / Q000002:abnormalities; D020065:Hepatopulmonary Syndrome / Q000175:diagnosis* / Q000000981:diagnostic imaging; D006801:Humans; D000860:Hypoxia; D016031:Liver Transplantation; D010005:Osteoarthropathy, Secondary Hypertrophic / Q000150:complications
Biliary atresia; Hepatopulmonary syndrome; Hypoxemia; Liver transplantation