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

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.

Immediate extubation following pediatric liver transplantation

“…Excluding reoperations, only one recipient in the IE cohort was reintubated on postoperative day (POD) 7 for hypoxemia in the setting of refractory very severe hepatopulmonary syndrome. 12 Of the 23 recipients with delayed extubated (DE), 20 were not immediately extubated due to their clinical status, 2 had failed extubation attempts in the OR, and 1 did not have documentation of why IE did not occur. These recipients remained intubated for an average of 4.9 days (±6.7 days) and a median of 2 days (range 1-21 days).…”

Rescue venovenous extracorporeal membrane oxygenation for the deterioration of acute respiratory distress syndrome in pediatric liver transplantation

“…ECMO is an intervention utilized for severe cardiac or respiratory failure refractory to conventional therapy that allows extra time for definitive diagnosis, treatment, and recovery. Even though no clear evidence has established whether VV‐ECMO improves the outcomes in pediatric acute respiratory failure, an increasing number of cases treated with VV‐ECMO for severe respiratory failure before and after LT have been reported with favorable outcomes 9–17 . The majority of those cases developed respiratory failure, necessitating ECMO after LT, whereas some cases required ECMO prior to LT, in which LT was performed on ECMO.…”

“…Even though no clear evidence has established whether VV-ECMO improves the outcomes in pediatric acute respiratory failure, an increasing number of cases treated with VV-ECMO for severe respiratory failure before and after LT have been reported with favorable outcomes. [9][10][11][12][13][14][15][16][17] The majority of those cases developed respiratory failure, necessitating ECMO after LT, whereas some cases required ECMO prior to LT, in which LT was performed on ECMO. However, a pediatric case with respiratory failure requiring intraoperative commencement of ECMO during LT has never been reported to date.…”

Extracorporeal Membrane Oxygenation After Liver Transplant in Children: A Review of the ELSO Registry

“…3 Although the results of adult patients undergoing LT and requiring ECMO have been documented in previous studies, 4,5 data regarding children are predominantly confined to case reports and small case series. [6][7][8][9] The aim of this study was to determine in-hospital mortality in children requiring ECMO after LT. We also sought to identify pre-and on-ECMO factors associated with in-hospital mortality in this same population of patients. Peripheral venous cannulation in children undergoing ECMO after LT presents significant challenges due to the risk of hepatic vein obstruction and graft edema caused by improper venous cannula positioning.…”

“…3 Although the results of adult patients undergoing LT and requiring ECMO have been documented in previous studies, 4,5 data regarding children are predominantly confined to case reports and small case series. 6–9…”