Severe hepatopulmonary syndrome with hypoxemia refractory to liver transplant: Recovery after 67 days of ECMO support

Abstract

Hepatopulmonary syndrome (HPS) is a complication of end stage liver disease (ESLD) and is manifested by severe hypoxemia, which usually responds to liver transplantation (LT). As compared to patients undergoing LT for other etiologies, patients with HPS present an increased risk of postoperative morbidity and mortality. There is no effective treatment for patients whose hypoxemia does not respond to LT. This subset of patients is at a highly increased risk of death. There are very few reports on the use of extracorporeal membrane oxygenation (ECMO) in this setting with rapid response. However, there is no prior report of ECMO utilization for longer than 4 weeks. We present the case of a 17 year-old male patient who underwent LT for ESLD secondary to chronic portal vein thrombosis and HPS. He received a liver from a deceased donor and presented with severe HPS after LT, requiring ECMO support for 67 days. The patient was discharged home and is breathing in ambient air. He is currently asymptomatic and has a normal liver function.

Extracorporeal Membrane Oxygenation use on Liver Transplantation: An Integrative Review

“…Included in these studies were eleven case reports, 11,13,21-25 two case series 19,26 and two original articles, 2,6 in which the main indications were severe respiratory syndromes and cardiovascular conditions. Among the studies in this scenario, six addressed remnant hepatopulmonary syndrome 11,19,[23][24][25][26] and six dealt with secondary respiratory distress syndrome. 6,19,[21][22][23]26 ECMO was also present in scenarios of cardiovascular failure when the use of medications, mechanical ventilation, and other conventional treatments proved ineffective; one reported two cases of thrombus, 19 one addressed severe pulmonary embolism and cardiac arrest, 26 one discussed cardiogenic shock, 26 one portal vein thrombosis 25 and one pulmonary congestion.…”

“…Among the studies in this scenario, six addressed remnant hepatopulmonary syndrome 11,19,[23][24][25][26] and six dealt with secondary respiratory distress syndrome. 6,19,[21][22][23]26 ECMO was also present in scenarios of cardiovascular failure when the use of medications, mechanical ventilation, and other conventional treatments proved ineffective; one reported two cases of thrombus, 19 one addressed severe pulmonary embolism and cardiac arrest, 26 one discussed cardiogenic shock, 26 one portal vein thrombosis 25 and one pulmonary congestion. 27 Furthermore, its use was also frequent as a treatment protocol for septic shock, established in 3 of the 14 articles analyzed; 2,13,22 however, these scenarios were generally accompanied by respiratory insufficiencies.…”

The Edge of Unknown: Postoperative Critical Care in Liver Transplantation

“…Although the syndrome was first described in 1884, besides LT, there is still not an effective treatment [ 106 ]. Intraoperative strategies to improve oxygenation include the use of methylene blue and extracorporeal membrane oxygenation (ECMO) [ 107 , 108 ].…”

Excellent outcomes with liver transplantation in hepatopulmonary syndrome across pre-transplant PaO2 spectrum

“…Severe postoperative hypoxemia in HPS patients tends to occur early in the first 24 hours after liver transplantation and is a known significant contributor to the majority of reported deaths in this setting 25,26 27 . However, treatment algorithms have been developed for the treatment of refractory hypoxemia in HPS, which include approaches such as trendelenberg or prone patient positioning, inhalational nitric oxide or epoprostenol, intravenous methylene blue, ventilatory modifications, embolization of lower lobe pulmonary vessels and in some cases extracorporeal membrane oxygenation (ECMO) 25,[28][29][30][31][32] .…”

“…There have been several reported case reports involving the use of ECMO in severe refractory hypoxemia associated with both pre-and post-transplant HPS cases [28][29][30][31][32] . Veno-venous ECMO has been mainly utilized in profoundly hypoxemic HPS patients unresponsive to other therapies where heart function is considered adequate.…”