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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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The preoperative pulmonary arteriovenous shunt had disappeared on follow-up contrast-enhanced TEE with preoperative agitated saline bubbles, on the 11th month after LT. A. Opacification of the RA and RV with microbubbles was observed after injecting microbubbles. B. Delayed opacification of the LA and LV was no longer observed after five cycles. TTE, transthoracic echocardiography, RA, right atrium, RV, right ventricle, LA, left atrium, LV, left ventricle.
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Figure 3: The preoperative pulmonary arteriovenous shunt had disappeared on follow-up contrast-enhanced TEE with preoperative agitated saline bubbles, on the 11th month after LT. A. Opacification of the RA and RV with microbubbles was observed after injecting microbubbles. B. Delayed opacification of the LA and LV was no longer observed after five cycles. TTE, transthoracic echocardiography, RA, right atrium, RV, right ventricle, LA, left atrium, LV, left ventricle.

Mentions: The partial pressure of arterial oxygen improved progressively during the postoperative follow up period and on postoperative month 11, the level was normalized (PaO2 118 mmHg). Follow-up transthoracic echocardiography (TTE) with agitated saline bubble on postoperative 11th months did not show pulmonary arteriovenous shunt any more (Fig. 3). Clubbing of both fingers also recovered to normal shape in postoperative month 11 (Fig. 1). The patient recovered successfully, not only physically, but also pychosocial activity, including school performance during the 48-months follow-up period.


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)

The preoperative pulmonary arteriovenous shunt had disappeared on follow-up contrast-enhanced TEE with preoperative agitated saline bubbles, on the 11th month after LT. A. Opacification of the RA and RV with microbubbles was observed after injecting microbubbles. B. Delayed opacification of the LA and LV was no longer observed after five cycles. TTE, transthoracic 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 3: The preoperative pulmonary arteriovenous shunt had disappeared on follow-up contrast-enhanced TEE with preoperative agitated saline bubbles, on the 11th month after LT. A. Opacification of the RA and RV with microbubbles was observed after injecting microbubbles. B. Delayed opacification of the LA and LV was no longer observed after five cycles. TTE, transthoracic echocardiography, RA, right atrium, RV, right ventricle, LA, left atrium, LV, left ventricle.
Mentions: The partial pressure of arterial oxygen improved progressively during the postoperative follow up period and on postoperative month 11, the level was normalized (PaO2 118 mmHg). Follow-up transthoracic echocardiography (TTE) with agitated saline bubble on postoperative 11th months did not show pulmonary arteriovenous shunt any more (Fig. 3). Clubbing of both fingers also recovered to normal shape in postoperative month 11 (Fig. 1). The patient recovered successfully, not only physically, but also pychosocial activity, including school performance during the 48-months follow-up period.

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