Outcome of liver transplantation for hepatopulmonary syndrome: a Eurotransplant experience.

Hepatopulmonary syndrome (HPS) is a pulmonary vascular complication of liver disease that affects up to 30% of patients with cirrhosis [1]. Intrapulmonary vascular dilatations and shunts result in gas exchange abnormalities, ranging from elevated alveolar–arterial oxygen gradients with no hypoxaemia to very severe hypoxaemia [1, 2]. Currently, liver transplantation (LT) is the only treatment option [3]. The Model for End-Stage Liver Disease (MELD) is a scoring system for assessing liver disease severity that has been validated to predict the 3-month waiting list mortality and is used by Eurotransplant for prioritising allocation of liver transplants [4]. However, this score poorly predicts overall and post-transplant survival, and does not take into account complications that affect outcomes independent of liver disease severity
Copyright ©ERS 2019
https://doi.org/10.1183/13993003.01096-2018 Eur Respir J 2019; 53: 1801096
[5]. Hypoxaemia in HPS is generally progressive and mortality is highest in advanced stages [6, 7]. In this sense, a standard exception (SE) policy has been established to prioritise patients with severe HPS (arterial oxygen tension (PaO2) <60 mmHg), as their severity of illness is not properly reflected by the MELD score. In the pre-SE MELD era, FALLON et al. [1] reported that HPS is associated with a doubled risk of mortality compared to patients without HPS. In 2014, GOLDBERG et al. [5] reviewed SE LT outcomes in HPS patients in the USA and found that LT candidates with SE for HPS had decreased pre-transplantation mortality and superior overall survival compared to non-HPS patients. The European outcomes for patients with SE for HPS have never been explored. In this retrospective study, we analysed overall, pre-transplant and post-transplant survival in LT candidates with SE for HPS within Eurotransplant, and determined whether the intent of the exception policy is being met.