Improved Survival After Liver Transplantation in Patients with Hepatopulmonary Syndrome

Abstract

Hepatopulmonary syndrome (HPS) is present in 10-32% of chronic liver disease patients, carries a poor prognosis and is treatable by liver transplantation (LT).Previous reports have shown high LT mortality in HPS and severe HPS (arterial oxygen (PaO 2 ) ≤50 mmHg). We reviewed outcomes in HPS patients who received LT between 2002 and 2008 at two transplant centers supported by a dedicated HPS clinic. We assessed mortality, complications and gas exchange in 21 HPS patients (mean age 51 years, MELD score 14), including 11/21 (52%) with severe HPS and 5/21 (24%) with living donor LT (median follow-up 20.2 months after LT). Overall mortality was 1/21 (5%); mortality in severe HPS was 1/11 (9%). Peritransplant hypoxemic respiratory failure occurred in 5/21 (24%), biliary complications in 8/21 (38%) and bleeding or vascular complications in 6/21 (29%). Oxygenation improved in all 19 patients in whom PaO 2 or SaO 2 were recorded. PaO 2 increased from 52.2 ± 13.2 to 90.3 ± 11.5 mmHg (room air) (p < 0.0001) (12 patients); a higher baseline macroaggregated albumin shunt fraction predicted a lower rate of postoperative improvement (p = 0.045) (7 patients). Liver transplant survival in HPS and severe HPS was higher than previously demonstrated. Severity of HPS should not be the basis for transplant refusal.

Proposed Management Algorithm for Severe Hypoxemia After Liver Transplantation in the Hepatopulmonary Syndrome

“…Monsel, et al described successful use of ECLS for 13 days pre‐ and 5 days posttransplant in a 51 year‐old man with alcoholic cirrhosis, ARDS and intrapulmonary shunting 45. As a supportive therapy designed to function as a bridge to recovery, ECLS is theoretically well suited to patients with HPS, given that shunt reversal and corresponding improvement in gas exchange occurs in nearly 100% of LT survivors 9, 46. Given the known complications of prolonged ventilation, including ventilator‐associated pneumonia and lung injury, early initiation of ECLS would be preferable, as it could both reduce ventilation requirements and mitigate end‐organ hypoxia 47.…”

“…Accordingly, our algorithmic approach seeks to rapidly guide clinicians through various therapeutic approaches, in order that ECLS is considered early in patients who are unresponsive to other therapies. However, timing of improvement in HPS‐related hypoxemia is highly variable between patients and difficult to predict 9. Given this and the high risk of complications and mortality associated with prolonged ECLS in adults 44, 48, ECLS is suggested as a “last resort” approach, as above.…”

“…This disease is associated with progressive hypoxemia and a high mortality 3, 5. Although liver transplantation (LT) is curative in HPS, these patients have an elevated postoperative complication rate 6, 7, 8, 9. In particular, “severe posttransplant hypoxemia,” defined as a need for 100% inspired oxygen (FiO 2 ) to maintain a saturation of ≥85% (out of proportion to any other concurrent lung process) 10, has been identified as a major complication leading to prolonged ICU stay and death in this population 6, 9, 10, 11, 12.…”

“…Although liver transplantation (LT) is curative in HPS, these patients have an elevated postoperative complication rate 6, 7, 8, 9. In particular, “severe posttransplant hypoxemia,” defined as a need for 100% inspired oxygen (FiO 2 ) to maintain a saturation of ≥85% (out of proportion to any other concurrent lung process) 10, has been identified as a major complication leading to prolonged ICU stay and death in this population 6, 9, 10, 11, 12. Although survivors have a complete normalization of gas exchange over time, severe posttransplant hypoxemia occurs in 6–21% of HPS patients, carries a mortality of 45%, and accounts for the majority of peri‐operative deaths in this population 10.…”

“…Although survivors have a complete normalization of gas exchange over time, severe posttransplant hypoxemia occurs in 6–21% of HPS patients, carries a mortality of 45%, and accounts for the majority of peri‐operative deaths in this population 10. A variety of strategies to attempt to manage this complication have been described in the literature, but these have never been reviewed and summarized and are used inconsistently, which has led to calls for a systematic approach 6, 9, 10, 13. We sought to review available evidence in order to develop a practical clinical management algorithm for severe posttransplant hypoxemia in HPS.…”