Extracorporeal membrane oxygenation as rescue therapy in a pediatric liver transplant recipient with very severe hepatopulmonary syndrome

Pediatr Transplant. 2022 Mar;26(2):e14185. doi: 10.1111/petr.14185. Epub 2021 Nov 6.

Abstract

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: 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: 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: 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.

Keywords: hepatopulmonary syndrome; liver transplantation; pediatric liver transplantation; pediatric transplantation.

Publication types

  • Case Reports

MeSH terms

  • Adolescent
  • Extracorporeal Membrane Oxygenation*
  • Hepatopulmonary Syndrome / surgery
  • Hepatopulmonary Syndrome / therapy*
  • Humans
  • Liver Transplantation*
  • Male
  • Postoperative Complications / therapy*