Raevens, Sarah

Portopulmonary hypertension (POPH), pulmonary arterial hypertension that develops in the setting of portal hypertension, has long been of significant interest to the pulmonary, cardiology, and hepatology communities. Optimal management of POPH has been challenging to define due to a lack of evidence from clinical trials regarding pulmonary arterial hypertension therapies and uncertainty regarding the role of liver transplantation (LT). Initially, the high risk of intraoperative and early post-transplant death in predominantly untreated patients with POPH tempered consideration of LT. More recently, the observation that POPH can improve, and sometimes even resolve, following LT, has led to reconsideration of the role of LT in selected patients. The first International Liver Transplantation Society (ILTS) POPH and hepatopulmonary syndrome practice guideline was a multidisciplinary consensus of expert opinions based on available evidence. Since that publication, hemodynamic definitions, management approaches, and POPH MELD exception criteria have evolved, and there have been new randomized controlled trials in POPH as well as studies regarding long-term outcomes. In order to ensure the guidelines remained current and reflected recent evidence, the original writing committee of the 2016 guidelines, leaders of the ILTS Cardiovascular Special Interest Group, and colleagues active in POPH research were invited to participate in the writing committee. In this document, approved for publication by the ILTS executive council, we provide an update to the prior guidelines with expert recommendations to guide and advance POPH management. Recommendations in these guidelines are based on expert opinion and available evidence and were agreed upon by consensus.

Portopulmonary hypertension (POPH), a form of pulmonary artery hypertension, occurs because of portal hypertension. Pulmonary hemodynamics and right heart function are key entities that are considered when diagnosing and treating POPH. Medical management, combined with liver transplantation, offers a unique opportunity for POPH “cure”; however, transplant risk can be significant as it relates to the severity of POPH. In addition to recent POPH practice guidelines, continued efforts can be suggested to improve our understanding of prognostic factors, investigate newer medical treatments, and improve outcomes of POPH. Herein, we identify specific “unmet needs” that focus on expanding and complementing current POPH practice guidelines.

Patients with advanced chronic liver disease can develop specific pulmonary complications related or unrelated to pre-existing lung disease. The three major pulmonary complications in this patient population include hepatopulmonary syndrome (HPS), portopulmonary hypertension (PoPH), and hepatic hydrothorax (HH). These entities are most often revealed by increasing dyspnea together with signs of portal hypertension. The prevalence of these complications remains underestimated due to the lack of routine screening of the cirrhotic population. The pathophysiology of HH is better understood than that of HPS and PoPH. The clinical features, diagnosis, and therapeutic options of these pulmonary complications are extensively discussed in this chapter. Liver transplantation may offer a curative therapy in highly-selected cases and MELD exception points allow priority access to liver transplantation, thus avoiding potential deterioration while awaiting transplant and providing a better post liver transplant survival. The complexity of managing these pulmonary complications requires a multidisciplinary team approach, especially when liver transplantation is indicated.