Extracorporeal membrane oxygenation as rescue therapy in a pediatric liver transplant recipient with very severe hepatopulmonary syndrome.
hepatopulmonary syndrome
liver transplantation
pediatric liver transplantation
pediatric transplantation
Journal
Pediatric transplantation
ISSN: 1399-3046
Titre abrégé: Pediatr Transplant
Pays: Denmark
ID NLM: 9802574
Informations de publication
Date de publication:
Mar 2022
Mar 2022
Historique:
received:
10
08
2021
accepted:
22
10
2021
pubmed:
7
11
2021
medline:
17
3
2022
entrez:
6
11
2021
Statut:
ppublish
Résumé
In children with cirrhosis, the prevalence of HPS ranges from 3% to 20%, resulting in impaired gas exchange due to alterations in pulmonary microvasculature. LT is the gold-standard cure for cirrhosis complicated by HPS and should ideally be performed prior to the development of severe HPS due to increased risk for post-transplant hypoxia, right heart failure, and outflow obstruction. We present a case of a 13-year-old man, who underwent pediatric LT for severe HPS complicated by postoperative respiratory collapse, requiring a 92-day course of veno-venous ECMO. Post-transplant, despite BiPAP, inhaled nitric oxide and isoproterenol infusion, he remained hypoxic postoperatively and acutely decompensated on postoperative day 25, requiring veno-venous ECMO. After 84 days on ECMO, a persistent large splenorenal shunt was identified that was embolized by interventional radiology, and 8 days after shunt embolization and ASD closure, he was successfully weaned off ECMO. This case describes the longest known duration of ECMO in a pediatric LT recipient and a unique improvement in hypoxemia following a portosystemic shunt closure. ECMO presents a heroic rescue measure for pediatric LT recipients with HPS that develops acute respiratory failure postoperatively refractory to alternative measures.
Sections du résumé
BACKGROUND
BACKGROUND
In children with cirrhosis, the prevalence of HPS ranges from 3% to 20%, resulting in impaired gas exchange due to alterations in pulmonary microvasculature. LT is the gold-standard cure for cirrhosis complicated by HPS and should ideally be performed prior to the development of severe HPS due to increased risk for post-transplant hypoxia, right heart failure, and outflow obstruction.
METHODS
METHODS
We present a case of a 13-year-old man, who underwent pediatric LT for severe HPS complicated by postoperative respiratory collapse, requiring a 92-day course of veno-venous ECMO.
RESULTS
RESULTS
Post-transplant, despite BiPAP, inhaled nitric oxide and isoproterenol infusion, he remained hypoxic postoperatively and acutely decompensated on postoperative day 25, requiring veno-venous ECMO. After 84 days on ECMO, a persistent large splenorenal shunt was identified that was embolized by interventional radiology, and 8 days after shunt embolization and ASD closure, he was successfully weaned off ECMO.
CONCLUSIONS
CONCLUSIONS
This case describes the longest known duration of ECMO in a pediatric LT recipient and a unique improvement in hypoxemia following a portosystemic shunt closure. ECMO presents a heroic rescue measure for pediatric LT recipients with HPS that develops acute respiratory failure postoperatively refractory to alternative measures.
Types de publication
Case Reports
Langues
eng
Sous-ensembles de citation
IM
Pagination
e14185Subventions
Organisme : NCATS NIH HHS
ID : TL1 TR002533
Pays : United States
Organisme : NCATS NIH HHS
ID : TL1 TR002533
Pays : United States
Informations de copyright
© 2021 Wiley Periodicals LLC.
Références
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