Improved severe hepatopulmonary syndrome after liver transplantation in an adolescent with end-stage liver disease secondary to biliary atresia
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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 modalityfor patients with HPS. However, morbidity and mortality after LT, especially in cases of severe HPS, remain high. This casereport describes a patient with typical findings of an extracardiac pulmonary arteriovenous shunt on contrast-enhancedtransesophageal echocardiography (TEE), and clubbing fingers, who had complete correction of HPS by deceased donorLT. The patient was a 16-year-old female who was born with biliary atresia and underwent porto-enterostomy on the55th 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 saturationof 84.2%). Contrast-enhanced TEE revealed an extracardiac right-to-left shunt, which suggested an intrapulmonaryarteriovenous shunt. The patient recovered successfully after LT, not only with respect to physical parameters but alsofor pychosocial activity, including school performance, during the 30-month follow-up period.
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 modalityfor patients with HPS. However, morbidity and mortality after LT, especially in cases of severe HPS, remain high. This casereport describes a patient with typical findings of an extracardiac pulmonary arteriovenous shunt on contrast-enhancedtransesophageal echocardiography (TEE), and clubbing fingers, who had complete correction of HPS by deceased donorLT. The patient was a 16-year-old female who was born with biliary atresia and underwent porto-enterostomy on the55th 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 saturationof 84.2%). Contrast-enhanced TEE revealed an extracardiac right-to-left shunt, which suggested an intrapulmonaryarteriovenous shunt. The patient recovered successfully after LT, not only with respect to physical parameters but alsofor pychosocial activity, including school performance, during the 30-month follow-up period.
참고문헌 (12)
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