Extracorporeal Membrane Oxygenation in a Pediatric Patient with Hepatopulmonary Syndrome and Interrupted Inferior Vena Cava After Living Related Liver Donation

Abstract

Extracorporeal membrane oxygenation (ECMO) is used for cardiopulmonary dysfunction. Hepatopulmonary syndrome (HPS) occurs in the setting of liver failure and may cause hypoxemia. Previous reports have described the use of ECMO for HPS after liver transplant. Our patient is a 19-month-old female with biliary atresia, an interrupted inferior vena cava, and HPS on 8 liters per minute of high-flow oxygen. Following liver transplantation, her postoperative course was complicated by severe hypoxemia requiring ECMO. Due to her interrupted inferior vena cava, our standard bi-caval cannula could not be used. Hence, a 16-French double lumen venovenous right internal jugular to right atrial cannula was used to provide extracorporeal life support. She was decannulated after 17 days, remained intubated for 2 days, and weaned to room air over the next 3 weeks. This is the third pediatric liver transplant patient supported with ECMO identified in the literature, and the youngest and smallest of those reported. This approach to cannulation is unique because of the use of a double lumen venovenous cannula for HPS in a child, selected due to complex anatomy. Posttransplant ECMO may provide pediatric patients with HPS and posttransplant hypoxemia a period of support for their pulmonary remodeling and recovery from HPS.

The role of extracorporeal membrane oxygenation in adult liver transplant patients: A qualitative systematic review of literature

“…While the numbers were too small to draw any conclusions, we did not note any overt difference in mortality among HPS patients. To corroborate these findings, the limited studies among the pediatric population have revealed good outcomes 18–20 …”

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

“…reported, with ECMO duration ranging from 17-28 days. 4,7,16 Herein, we describe a pediatric liver transplant recipient with very severe HPS that experienced a delayed decompensation post-LT requiring ECMO support for 91 days with successful decannulation after embolization of a persistent splenorenal shunt. Research on the role of persistent portosystemic shunts following LT in refractory HPS is limited.…”

“…Use of ECMO in LT recipients with HPS has been described in the adult population with a demonstrated survival rate of 38%, 3 but the use of this therapy for complications of HPS in the pediatric LT population has not been well established 4‐7 …”

“…Use of ECMO in LT recipients with HPS has been described in the adult population with a demonstrated survival rate of 38%, 3 but the use of this therapy for complications of HPS in the pediatric LT population has not been well established. [4][5][6][7] We present a case pediatric LT for very severe HPS complicated by postoperative respiratory failure requiring ECMO for 91 days. To our knowledge, this is the longest course of ECMO for HPS in a pediatric patient post-LT documented with a successful outcome.…”

Extracorporeal Membrane Oxygenation in Pediatric Liver Transplantation: A Multicenter Linked Database Analysis and Systematic Review of the Literature

“…From 24 articles initially identified as potentially eligible, 16 studies were retrieved for data extraction after fulltext review (Figure 2). The studies included in the present systematic review were published between 2000 and 2020 and were conducted in the United States (n = 7), 8,9,[18][19][20][21][22] Asia (n = 5), 7,12,[23][24][25] Europe (n = 3), [4][5][6] and Canada (n = 1). 26 A total of 22 children that received ECMO on the day of transplant or later were included in the systematic review (Table 4).…”