Hepatopulmonary syndrome (HPS) is a serious pulmonary vascular complication that causes arterial hypoxemia in the setting of liver disease. HPS has a progressive course and is associated with a two-fold increased risk of mortality relative to cirrhotic patients without HPS. It primarily affects patients with portal hypertension. The key pathological features of HPS include intrapulmonary angiogenesis and vascular dilations (IPVDs). The prevalence of HPS varies widely due to inconsistent diagnostic criteria and a lack of standardized protocols. Despite advances in understanding its pathophysiology, no effective curative treatments for HPS exist. Liver transplantation remains the only definitive treatment, improving survival and altering the disease natural course. This review explores the pathophysiology, clinical features, and therapeutic strategies for HPS, highlighting recent advances in the literature.

Hepatopulmonary syndrome (HPS) is characterized by defects in oxygenation caused by intra-pulmonary vasodilation occurring because of chronic liver disease, portal hypertension, or congenital portosystemic shunts. Clinical implications of portal hypertension are very well-known, however, awareness of its effect on multiple organs such as the lungs are less known. The presence of HPS in chronic liver disease is associated with increased mortality. Medical therapies available for HPS have not been proven effective and definitive treatment for HPS is mainly liver transplantation (LT). LT improves mortality for patients with HPS drastically. This article provides a review on the definition, clinical presentation, diagnosis, and management of HPS.

Hepatopulmonary syndrome (HPS) is defined by the association of portal hypertension, increased alveolar-arterial oxygen gradient and intrapulmonary vascular dilations. Pathophysiological mechanisms of hypoxemia are characterized by ventilation-perfusion mismatch, oxygen diffusion limitation between alveolus and the centre of the dilated capillary, and right-to-left shunting. An excess of vasodilator molecules (like nitric monoxide) and proangiogenic factors (like VEGF) play an important role in the occurrence of HPS. Symptoms of HPS are not specific and dominated by a progressive dyspnea in upright position. Pulse oximetry is a simple non-invasive screening test but only detect the most severe forms of HPS. Medical treatment is disappointing and only liver transplantation may lead to resolution of HPS. Survival following liver transplantation is promising when hypoxemia is not severely decreased.

Hepatopulmonary syndrome (HPS) is a pulmonary complication observed in patients with chronic liver disease and/or portal hypertension, attributable to an intrapulmonary vascular dilatation that may induce severe hypoxemia. Microvascular dilation and angiogenesis in the lung have been identified as pathologic features that drive gas exchange abnormalities in experimental HPS. Pulse oximetry is a useful screening test for HPS, which can guide subsequent use of arterial blood gases. Contrast-enhanced echocardiography, perfusion lung scanning, and pulmonary arteriography are three currently used diagnostic imaging modalities that identify the presence of intrapulmonary vascular abnormalities. The presence of HPS increases mortality and impairs quality of life, but is reversible with liver transplantation. No medical therapy is established as effective for HPS. At the present time, liver transplantation is the only available treatment for HPS.

The hepatopulmonary syndrome (HPS) is defined as the triad of liver disease, intrapulmonary vascular dilatation, and abnormal gas exchange, and is found in 10-32% of patients with liver disease. Liver transplantation is the only known cure for HPS, but patients can develop severe posttransplant hypoxemia, defined as a need for 100% inspired oxygen to maintain a saturation of ≥85%. This complication is seen in 6-21% of patients and carries a 45% mortality. Its management requires the application of specific strategies targeting the underlying physiologic abnormalities in HPS, but awareness of these strategies and knowledge on their optimal use is limited. We reviewed existing literature to identify strategies that can be used for this complication, and developed a clinical management algorithm based on best evidence and expert opinion. Evidence was limited to case reports and case series, and we determined which treatments to include in the algorithm and their recommended sequence based on their relative likelihood of success, invasiveness, and risk. Recommended therapies include: Trendelenburg positioning, inhaled epoprostenol or nitric oxide, methylene blue, embolization of abnormal pulmonary vessels, and extracorporeal life support. Availability and use of this pragmatic algorithm may improve management of this complication, and will benefit from prospective validation.

Hepatopulmonary syndrome is a serious complication of liver disease. Type I hepatopulmonary syndrome is associated with diffuse dilatation of the pulmonary vasculature, leading to severe hypoxemia. Liver transplantation is the treatment of choice for this condition. There are limited options for those who are not candidates for liver transplantation. We present the case of a patient who presented with severe hypoxemia requiring FIO2 of 0.95 with PaO2 of 59 mm Hg. Workup revealed 33% intrapulmonary right-to-left shunt. A pulmonary angiogram showed diffuse dilatation of the pulmonary arteries, especially in the lower lobes. The patient was diagnosed with type I hepatopulmonary syndrome. He was not a candidate for liver transplantation. The patient underwent sequential coil embolizations of the lower lobe pulmonary arteries. He was discharged home on 2 L of supplemental oxygen. This case demonstrates that coil embolization of dilated pulmonary arteries is a potential palliative treatment for patients with diffuse type I hepatopulmonary syndrome.

Hepatopulmonary syndrome (HPS) is a serious vascular complication of liver disease that occurs in 5-32% of patients with cirrhosis. The presence of HPS markedly increases mortality. No effective medical therapies are currently available and liver transplantation is the only established treatment option for HPS. The definition and diagnosis of HPS are established by the presence of a triad of liver disease with intrapulmonary vascular dilation that causes abnormal arterial gas exchange. Experimental biliary cirrhosis induced by common bile duct ligation in the rat reproduces the pulmonary vascular and gas exchange abnormalities of human HPS and serves as a pertinent animal model. Pulmonary microvascular dilation and angiogenesis are two central pathogenic features that drive abnormal pulmonary gas exchange in experimental HPS, and thus might underlie HPS in humans. Defining the mechanisms involved in the microvascular alterations of HPS has the potential to lead to effective medical therapies. This Review focuses on the current understanding of the pathogenesis, clinical features and management of HPS.

Patients suffering from severe chronic liver disease, in particular cirrhosis, are at risk for pulmonary complications. The leading clinical symptom is shortness of breath, which can accompany the actual disease as indirect effect because of anemia, faint muscles or ascites. On the other hand, dyspnea can have multiple additive causes in case of accompanying cardial or pulmonary disease. The hepatopulmonary syndrome (HPS) and the portopulmonary hypertension (PoPH) belong to the most relevant pulmonary complications in liver cirrhosis. HPS appears to be more common than PoPH and the presence of either entity increases morbidity and mortality in patients with liver disease. The two diseases have to be strictly distinguished, as they have opposed histological and pathophysiological origin. While the HPS is a dilatative pulmonary- vascular disease, the PoPH is a constrictive or obliterative pulmonary-vascular disease in the context of a liver disease or a portal hypertension. Therefore, these diseases are separate entities also when it comes to diagnostics and therapy.

Although liver transplantation (LT) is the only effective treatment option for hepatopulmonary syndrome (HPS), the post-LT morbidity and mortality have been high for patients with severe HPS. We performed post-LT embolotherapy in a 10-year-old boy who had severe type I HPS preoperatively, but he failed to recover early from his hypoxemic symptoms after an LT. Multiple embolizations were then successfully performed on the major branches that formed the abnormal vascular structures. After the embolotherapy, the patient had symptomatic improvement and he was discharged without complications.

The hepatopulmonary syndrome is characterized as the triad of liver disease, pulmonary gas exchange abnormalities leading to arterial deoxygenation and evidence of intrapulmonary vascular dilatations. This review summarizes the pathological mechanisms leading to pulmonary vascular changes in hepatopulmonary syndrome. The role of the three currently used diagnostic imaging modalities of contrast-enhanced echocardiography, perfusion lung scanning and pulmonary arteriography that identify the presence of intrapulmonary vascular abnormalities are reviewed. Liver transplantation is considered to be the definitive treatment of hepatopulmonary syndrome with often successful reversal of hypoxemia, however other treatments have been trialed. This review further appraises the evidence for the use of pharmacological agents and the role of radiological interventions in hepatopulmonary syndrome.

The hepatopulmonary syndrome (HPS) is an important and often under-recognized vascular complication of cirrhosis and portal hypertension characterized by pulmonary vascular dilatation, which results in hypoxemia. This syndrome is identified in as many as 20% of patients who are evaluated for orthotopic liver transplantation (OLT), and it has recently been found to increase mortality in affected patients, particularly when hypoxemia is severe. Currently, OLT is the only therapy established to reverse intrapulmonary vasodilatation, although postoperative mortality is increased in patients with severe hypoxemia. No randomized controlled trials of pharmacologic therapies have been undertaken, but supplemental oxygen improves oxygenation. Data derived from case reports, small studies, and experimental models suggest that pharmacologic therapies may be effective. In cirrhotic patients with HPS, particularly those with moderate hypoxemia (PaO2 < 60 mmHg), OLT should be considered prior to the development of severe deoxygenation. Supplemental oxygen should be given to patients with a PaO2 < 60 mmHg or those with exercise oxygen desaturation. For those patients with mild hypoxemia or those who are not OLT candidates, a trial of pharmacologic treatment may be considered.

Few prognostic indicators and outcomes reported for patients who have hepatopulmonary syndrome can be consistently reproduced between institutions. The lack of practice recommendations based upon consistent and predictable outcomes creates uncertainty for physicians trying to make the best patient choices. With the scarcity of donor organs, these issues are of particular importance when transplantation in considered as a treatment option. Much uncertainty arises from the basic tasks of making an accurate diagnosis in patients who have symptoms suggestive of hepatopulmonary syndrome. This article focuses on problems that underlie diagnostic accuracy and uses this information to appraise critically outcomes literature.

Recent papers relevant to the preoperative evaluation and optimization of patients with severe liver disease will be discussed. The emphasis will be placed on cardiovascular, pulmonary, and renal complications. Other aspects such as preoperative management of hepatitis B and C, other infectious issues, and liver cancer will not be discussed because this rarely involves the anesthesiologist.

Dobutamine stress echocardiography has been the cornerstone of cardiac evaluation of liver transplant candidates. Combining liver transplantation with cardiac procedures has been shown to be feasible. While mild hepatopulmonary syndrome is well-tolerated, severe hepatopulmonary syndrome carries a fairly high mortality rate. New treatment modalities of severe portopulmonary hypertension have been introduced, and may have advantages over epoprostenol administration. Hepatic hydrothorax requires similar therapy to ascites [repeated thoracentesis or paracentesis, and transjugular intrahepatic portosystemic shunt (TIPS)], but refractory hydrothorax may require other interventions. Hepatorenal syndrome may improve by increasing renal blood flow through the use of vasoconstrictors (vasopressin, norepinephrine) in combination with albumin administration. Interventional radiologists can now change the flow through an established TIPS. Hepatic encephalopathy may result in some irreversible changes in the brain. It remains difficult to predict whether a patient with acute fulminant failure will recover spontaneously. Support devices that include hepatocytes show early promising results. The coagulation changes in living donors are incompletely understood. Finally, autonomic neuropathy as a complication of severe liver disease results in more hemodynamic instability.

Recent advances in preoperative evaluation and optimization are presented and discussed.

A 54-year-old Japanese woman with primary biliary cirrhosis (PBC) was admitted to our hospital due to hepatic coma and refractory pleural effusion. The physical examination revealed clubbed fingers and collateral veins. The patient had an increased alveolar-arterial oxygen gas tension difference. The levels of anti-mitochondrial antibody (AMA) and AMA M2 was 80 times normal. A technetium 99m-labeled macro-aggregated human albumin scintigram showed uptake in the spleen and the kidneys. A diagnosis of hepatopulmonary syndrome (HPS) was made. HPS may be overlooked because of the lack of symptoms. We conclude that closer attention should be paid to the occurrence of HPS.