Clin Mol Hepatol.  2014 Mar;20(1):76-80. 10.3350/cmh.2014.20.1.76.

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

• Park TJ
• Ahn KS
• Kim YH
• Kim H
• Park UJ
• Kim HT
• Cho WH
• Park WH
• Kang KJ

• KMID: 1737602
• DOI: http://doi.org/10.3350/cmh.2014.20.1.76

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.

Keyword

Hepatopulmonary syndrome; Liver transplantation; Biliary atresia; Hypoxemia

MeSH Terms

Adolescent
Anoxia
Arteriovenous Fistula/etiology
Biliary Atresia/*diagnosis/etiology
Cyanosis/complications
Dyspnea/complications
Echocardiography, Transesophageal
End Stage Liver Disease/complications/*surgery
Female
Hepatic Artery/abnormalities
Hepatopulmonary Syndrome/*diagnosis/ultrasonography
Humans
*Liver Transplantation
Osteoarthropathy, Secondary Hypertrophic/complications