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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 - Clin Mol Hepatol (2014)

Bottom Line: 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.

View Article: PubMed Central - PubMed

Affiliation: Department of Surgery, Keimyung University School of Medicine, Daegu, Korea.

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.

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Preoperative contrast-enhanced transesophageal echocardiography (TEE) with agitated saline bubble revealed the existence of a pulmonary arteriovenous shunt. A. Opacification of the right atrium (RA) and right ventricle (RV), with microbubbles being observed after injecting microbubbles. B. Delayed opacification of the left atrium (LA) and left ventricle (LV) was found five cycles later. TEE, transesophageal echocardiography, RA, right atrium, RV, right ventricle, LA, left atrium, LV, left ventricle.
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Figure 2: Preoperative contrast-enhanced transesophageal echocardiography (TEE) with agitated saline bubble revealed the existence of a pulmonary arteriovenous shunt. A. Opacification of the right atrium (RA) and right ventricle (RV), with microbubbles being observed after injecting microbubbles. B. Delayed opacification of the left atrium (LA) and left ventricle (LV) was found five cycles later. TEE, transesophageal echocardiography, RA, right atrium, RV, right ventricle, LA, left atrium, LV, left ventricle.

Mentions: A 16 years-old female was born with biliary atresia, and she underwent porto-enterostomy (Kasai's procedure) on the 55th day after birth. She had intermittent attacks of cholangitis during her childhood. She had been pretty good and developed normally until the age of 15 years. Although she had hepatosplenomegaly, there was no overt symptom. However, she had progressed liver failure aggressively for 6 months before LT. In addition, she was on a bed ridden status because of dyspnea for last 2 months before LT. Both fingers showed clubbing (Fig. 1), ankles were swollen and lips were cyanotic, those were suspected as secondary changes of chronic respiratory insufficiency. Her chest X-ray showed no abnormal findings, except elevated left diaphragm due to splenomegaly. However, her partial pressure of arterial oxygen and arterial oxygen saturation was markedly decreased (PaO2 54.5 mmHg, O2sat 84.2%). Her hepatic and renal profiles were as follows: total bilirubin 42.3 mg/dL, albumin 2.5 g/dL, AST 241 U/L, ALT 93 U/L, INR 2.39, ammonia 2.54 µg/mL and creatinine 0.5 mg/dL. Her Child-Turcotte-Pugh classification was C and MELD (Model of End-Stage Liver Disease) score was 30. The transesophageal echocardiography (TEE) with agitated saline bubble showed an extracardiac right to left shunt, which suggested intrapulmonary arterio-venous shunt (Fig. 2).


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 - Clin Mol Hepatol (2014)

Preoperative contrast-enhanced transesophageal echocardiography (TEE) with agitated saline bubble revealed the existence of a pulmonary arteriovenous shunt. A. Opacification of the right atrium (RA) and right ventricle (RV), with microbubbles being observed after injecting microbubbles. B. Delayed opacification of the left atrium (LA) and left ventricle (LV) was found five cycles later. TEE, transesophageal echocardiography, RA, right atrium, RV, right ventricle, LA, left atrium, LV, left ventricle.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC3992334&req=5

Figure 2: Preoperative contrast-enhanced transesophageal echocardiography (TEE) with agitated saline bubble revealed the existence of a pulmonary arteriovenous shunt. A. Opacification of the right atrium (RA) and right ventricle (RV), with microbubbles being observed after injecting microbubbles. B. Delayed opacification of the left atrium (LA) and left ventricle (LV) was found five cycles later. TEE, transesophageal echocardiography, RA, right atrium, RV, right ventricle, LA, left atrium, LV, left ventricle.
Mentions: A 16 years-old female was born with biliary atresia, and she underwent porto-enterostomy (Kasai's procedure) on the 55th day after birth. She had intermittent attacks of cholangitis during her childhood. She had been pretty good and developed normally until the age of 15 years. Although she had hepatosplenomegaly, there was no overt symptom. However, she had progressed liver failure aggressively for 6 months before LT. In addition, she was on a bed ridden status because of dyspnea for last 2 months before LT. Both fingers showed clubbing (Fig. 1), ankles were swollen and lips were cyanotic, those were suspected as secondary changes of chronic respiratory insufficiency. Her chest X-ray showed no abnormal findings, except elevated left diaphragm due to splenomegaly. However, her partial pressure of arterial oxygen and arterial oxygen saturation was markedly decreased (PaO2 54.5 mmHg, O2sat 84.2%). Her hepatic and renal profiles were as follows: total bilirubin 42.3 mg/dL, albumin 2.5 g/dL, AST 241 U/L, ALT 93 U/L, INR 2.39, ammonia 2.54 µg/mL and creatinine 0.5 mg/dL. Her Child-Turcotte-Pugh classification was C and MELD (Model of End-Stage Liver Disease) score was 30. The transesophageal echocardiography (TEE) with agitated saline bubble showed an extracardiac right to left shunt, which suggested intrapulmonary arterio-venous shunt (Fig. 2).

Bottom Line: 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.

View Article: PubMed Central - PubMed

Affiliation: Department of Surgery, Keimyung University School of Medicine, Daegu, Korea.

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.

Show MeSH
Related in: MedlinePlus