Use of airway pressure release ventilation in a child with refractory hepatopulmonary syndrome after liver transplantation

HPS is a life‐threatening condition in patients with end‐stage liver disease, in which intrapulmonary vascular dilatations result in intrapulmonary shunts and hypoxemia. The only successful treatment is liver transplantation. Hypoxemia may be severe prior to transplantation; however, it can worsen o...

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Bibliographic Details
Published in:Pediatric transplantation 2013-05, Vol.17 (3), p.E81-E87
Main Authors: Jenkins, Julia K., Gebergzabher, Yodit D., Island, Eddie R., Habashi, Nader, Hauser, Gabriel J.
Format: Article
Language:English
Subjects:
anoxia
continuous positive airway pressure
Continuous Positive Airway Pressure - methods
Echocardiography
End Stage Liver Disease - complications
End Stage Liver Disease - therapy
Female
hepatopulmonary syndrome
Hepatopulmonary Syndrome - etiology
Hepatopulmonary Syndrome - therapy
Humans
Hypoxia - etiology
Hypoxia - therapy
Infant
liver transplantation
Liver Transplantation - adverse effects
Oxygen - metabolism
pediatrics
respiration, artificial
Treatment Outcome
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Summary:HPS is a life‐threatening condition in patients with end‐stage liver disease, in which intrapulmonary vascular dilatations result in intrapulmonary shunts and hypoxemia. The only successful treatment is liver transplantation. Hypoxemia may be severe prior to transplantation; however, it can worsen or become refractory after liver transplantation and result in increased post‐operative mortality. Here, we present the case of a 10‐month‐old female infant with progressive end‐stage liver disease and severe HPS, who developed refractory hypoxemia after a successful liver transplantation. After 19 days of unsuccessful attempts to reverse the hypoxemia using conventional mechanical ventilation and HFOV, the patient responded dramatically to APRV, with rapid improvement in her PaO2 and sharp decline in her OI. She was able to begin weaning from APRV two days later and was extubated within seven days. APRV was successful in treating refractory hypoxemia in this patient with severe HPS after liver transplantation, possibly by modifying distribution of pulmonary blood flow. Although we cannot rule out coincidental natural resolution of the HPS, APRV could be a useful rescue therapy in patients with HPS and refractory hypoxemia.
ISSN:1397-3142
1399-3046
DOI:10.1111/petr.12058