Complication Of Advanced Liver Disease Research Articles

Diagnosis and treatment of hepatorenal syndrome; a systematic review

Hepatorenal syndrome (HRS) is a severe form of functional renal failure associated with reduced renal blood flow, predominantly affecting patients with ascites and cirrhosis, showcasing normal kidney histology. This complication of advanced liver disease is marked by a grim prognosis, with survival typically spanning weeks or months. Diagnosis hinges on a blend of clinical and laboratory criteria, given the absence of definitive biomarkers. This systematic study sought to formulate guidelines for HRS diagnosis and treatment. Data, drawn from PubMed/Medline, Scopus databases, and Embase, encompassed English-language articles from 2010 to 2023, adhering to PRISMA guidelines. Among 457 studies, seven met eligibility criteria, employing surveys and national database analyses as prevalent methods. Current care standards involved albumin and terlipressin treatment, with continuous intravenous terlipressin administration proving equally effective and better-tolerated than boluses. Despite its 40-50% success rate, predictors included serum bilirubin and creatinine levels, blood pressure, and systemic inflammatory response syndrome presence. Liver transplant stands as the sole curative avenue, while alternative therapies like dopamine, noradrenaline, renal and liver replacement therapy, and transjugular intrahepatic portosystemic shunt are under evaluation. Ongoing research into advanced remedies is imperative, given current treatment limitations. Considerations such as antibiotic prophylaxis in severe cirrhosis with low protein ascitic fluid levels and volume expansion with albumin during spontaneous bacterial peritonitis and high-volume paracentesis further underscore the multifaceted approach required in managing HRS. This study advocates for continuous exploration of innovative therapies and emphasizes the pivotal role of liver transplant consideration for eligible patients.

The Management of Hepatic Encephalopathy from Ward to Domiciliary Care: Current Evidence and Gray Areas.

Hepatic encephalopathy (HE) is a common complication of advanced liver disease and acute liver failure. It is a condition that features several neuropsychiatric symptoms that affect mortality, morbidity and the quality of patients' and caregivers' lives. An HE diagnosis is generally an exclusion diagnosis. Once the patient is admitted to the hospital, clinical examination, blood tests and eventually neuroimaging should be performed with the aim of ruling out other causes of acute brain dysfunction. Moreover, HE is recognized using various precipitants that can potentially promote its onset, alone or in combination, and must be identified. Once the diagnostic process is complete, a correct treatment should be started. The anti-HE treatment is based on a combination of the correction of precipitants; non-absorbable antibiotics, such as rifaximin; and non-absorbable disaccharides. Once the patient is discharged from the hospital, specific anti-HE therapy should be maintained in order to prevent other HE episodes.

Outcomes among patients with hepatorenal syndrome based on hospital teaching and transplant status: Analysis of 159 845 hospitalizations.

Hepatorenal syndrome (HRS) is a life-threatening complication of advanced liver disease. This study aimed to examine the impact of hospital teaching/transplant status and availability of liver transplantation on survival among hospitalized patients with HRS in the United States. Patients with HRS were identified from the national inpatient sample 2016-2019. Information was collected regarding patient demographics, hospital characteristics, liver disease etiology, presence of liver disease decompensations, Elixhauser comorbidities, and interventions. Patients were classified as being treated at three hospital groups: non-teaching hospitals (NTHs), teaching non-transplant centers (TNTCs), and teaching transplant centers (TTCs). The relationship between hospital teaching/transplant status and in-hospital mortality and transplant-free mortality was examined using multivariable linear and logistic regression analysis. A total of 159,845 patients met the criteria for HRS. Of these, 24% were admitted to NTHs, 50.8% to TNTCs, and 25.2% to TTCs. Admission to a TTC was independently associated with a lower mortality risk compared to admission to non-TTCs (aOR = 0.75, 95% CI: 0.68-0.83, P <0.001). Patients at TTCs had a lower transplant-free mortality risk than those at NTHs (aOR = 0.75, 95% CI: 0.67-0.83, P < 0.001). There was no significant difference in all-cause or transplant-free mortality between TNTCs and NTHs. Patients with HRS admitted to TTCs have higher disease severity, but significantly improved outcomes compared to those admitted to NTHs. These data suggest opportunities for increased disease awareness and education among NTHs and support early referral for liver transplant evaluation among hospitalized patients with HRS.

The safety and efficacy of endoscopic ultrasound-guided portal pressure gradient measurement with concomitant endoscopic ultrasound-guided liver biopsy: a systematic review

IntroductionPortal hypertension (PH) is a complication of advanced liver disease. Traditionally, PH has been quantified using hepatic venous pressure gradient (HVPG) through an indirect transjugular approach requiring ionizing radiation exposure. Endoscopic ultrasound-guided porto-systemic pressure gradient (EUS-PPG) measurement is an emerging alternative, minimally invasive technique that provides direct portal pressure measurement. The aim of this systematic review is to evaluate the safety and efficacy of EUS-PPG measurement and concomitant EUS-guided liver biopsy (EUS-LB) in patients with chronic liver disease.MethodsThe preferred reporting items for systematic reviews and meta-analyses method was used. A PubMed, Medline, Web of Science, Google Scholar, and CINAHL search for terms “endoscopic ultrasound,” “EUS,” and “portal pressure gradient” was used to identify qualifying studies. Eligible studies included those which were published before 2022, reporting outcomes of EUS-PPG measurement, simultaneous EUS-LB if applicable, and adverse events rate. Risk of bias was assessed by Egger’s test. Results were synthesized using I2 to test heterogeneity.ResultsFour published studies including 147 patients met inclusion criteria, with mean age 59.6 years, 59% male. Indications for EUS-PPG measurement were history of chronic liver disease or suspected cirrhosis, viral hepatitis, alcohol associated liver disease, hepatic sinusoidal obstruction or Budd Chiari syndrome. The pooled technical success rate of EUS-PPG measurements was 98.61% with 95% confidence interval of 95.20% - 99.82%. A 25-gauge needle was used in 92% (135/147) of patients. EUS-PPG measurement was performed through a transgastric approach in all 147 (100%) patients using a compact manometer with pressure transducer and non-compressible tubing. The mean PPG was 10.07 (range 6.44 – 13.70) mmHg. Ninety-five patients underwent simultaneous EUS-LB using 19G needle with wet suction technique. Technical success rate of EUS-LB was 100% and specimen was adequate in 99% (94/95) patients to establish histological diagnosis. There were no major life-threatening complications of the EUS-PPG procedure. Predominant adverse events were abdominal pain 6.1% (9/147) and sore throat 5.4% (8/147).ConclusionEUS-PPG measurement is safe and useful in providing an assessment of portal pressure in patients with chronic liver disease. Future studies are needed to evaluate whether there is consistent correlation between EUS-PPG measurements and histologic fibrosis stage by liver biopsy.

Portal vein thrombosis in cirrhosis: A literature review.

Portal Vein Thrombosis (PVT), a common complication of advanced liver disease, is defined as an obstruction of the portal vein due to thrombus formation that can extend to the superior mesenteric and splenic veins. It was believed that PVT occurred predominantly due to prothrombotic potential. However, recent studies have shown that decreased blood flow related to portal hypertension appears to increase PVT risk as per Virchow's triad. It is well known that there is a higher incidence of PVTs in cirrhosis with a higher MELD and Child Pugh score. The controversy for management of PVTs in cirrhotics lies in the individualized assessment of risks versus benefits of anticoagulation, since these patients have a complex hemostatic profile with both bleeding and procoagulant propensities. In this review, we will systematically compile the etiology, pathophysiology, clinical features, and management of portal vein thrombosis in cirrhosis.

Gut Dysbiosis and Blood-Brain Barrier Alteration in Hepatic Encephalopathy: From Gut to Brain.

A common neuropsychiatric complication of advanced liver disease, hepatic encephalopathy (HE), impacts the quality of life and length of hospital stays. There is new evidence that gut microbiota plays a significant role in brain development and cerebral homeostasis. Microbiota metabolites are providing a new avenue of therapeutic options for several neurological-related disorders. For instance, the gut microbiota composition and blood-brain barrier (BBB) integrity are altered in HE in a variety of clinical and experimental studies. Furthermore, probiotics, prebiotics, antibiotics, and fecal microbiota transplantation have been shown to positively affect BBB integrity in disease models that are potentially extendable to HE by targeting gut microbiota. However, the mechanisms that underlie microbiota dysbiosis and its effects on the BBB are still unclear in HE. To this end, the aim of this review was to summarize the clinical and experimental evidence of gut dysbiosis and BBB disruption in HE and a possible mechanism.

Long-term renal outcomes comparison between patients with chronic kidney disease and hepatorenal syndrome after living donor liver transplantation.

Hepatorenal syndrome (HRS) is a disastrous renal complication of advanced liver disease with a poor prognosis. Restoring normal liver function through liver transplantation (LT) is a standardized treatment with favorable short-term survival. However, the long-term renal outcomes in patients with HRS receiving living donor LT (LDLT) are controversial. This study aimed to investigate the prognostic impact of LDLT in patients with HRS. We reviewed adult patients who underwent LDLT between July 2008 and September 2017. Recipients were classified into 1) HRS type 1 (HRS1, N = 11), 2) HRS type 2 (HRS2, N = 19), 3) non-HRS recipients with pre-existing chronic kidney disease (CKD, N = 43), and 4) matched normal renal function (N = 67). Postoperative complications and 30-day surgical mortality were comparable among the HRS1, HRS2, CKD, and normal renal function groups. The 5-year survival rate was >90% and estimated glomerular filtration rate (eGFR) transiently improved and peaked at 4 weeks post-transplantation in patients with HRS. However, renal function deteriorated and resulted in CKD stage ≥ III in 72.7% of HRS1 and 78.9% of HRS2 patients (eGFR <60 ml/min/1.73 m2). The incidence of developing CKD and end-stage renal disease (ESRD) was similar between the HRS1, HRS2, and CKD groups, but significantly higher than that in the normal renal function group (both P < 0.001). In multivariate logistic regression, pre-LDLT eGFR <46.4 ml/min/1.73 m2 predicted the development of post-LDLT CKD stage ≥ III in patients with HRS (AUC = 0.807, 95% CI = 0.617-0.997, P = 0.011). LDLT provides a significant survival benefit for patients with HRS. However, the risk of CKD stage ≥ III and ESRD among patients with HRS was similar to that in pre-transplant CKD recipients. An early preventative renal-sparing strategy in patients with HRS is recommended.

The progression of hepatorenal syndrome-acute kidney injury in acute alcohol-associated hepatitis: renal outcomes after liver transplant.

Hepatorenal syndrome-acute kidney injury (HRS-AKI) is a complication of advanced liver disease in patients with ascites and circulatory dysfunction. Little data remain on the relationship between HRS-AKI outcomes and different etiologies of liver disease post-liver transplant (LT). The primary aim was to evaluate the effect of HRS-AKI on renal outcomes in patients with acute alcohol-associated hepatitis (AAH) compared to chronic liver disease (CLD) after LT. The secondary aim was to evaluate the impact of acuity and chronicity of alcohol-associated liver disease in patients with HRS-AKI post-LT renal outcomes. A retrospective observational study of patients undergoing urgent inpatient liver transplant evaluation (LTE) for cirrhosis and AAH at single academic LT center between October 2017 and July 2021 was conducted. Patients with HRS-AKI were selected based on indication for LTE: acute AAHHRS or CLDHRS. CLDHRS was categorized by disease etiology: cirrhosis due to alcohol (A-CLDHRS) versus cirrhosis from other causes (O-CLDHRS). CLD patients without HRS-AKI were labeled CLDno HRS. A total of 210 subjects underwent LTE; 25% were evaluated for AAH and 75% were evaluated for CLD. Hepatorenal syndrome was more common in subjects evaluated for AAH (37/47) than CLD (104/163) (78.7 versus 63.8%, p = 0.04). For the primary outcome, AAHHRS subjects required ⩾30 days post-LT renal replacement therapy (RRT) more often than subjects with CLDHRS (p = 0.02) and CLDno HRS (p < 0.01). There was no significant difference in other forms of long-term renal outcomes including kidney transplant referral and kidney transplant among cohorts. In subgroup analysis, 30-days post-LT RRT was more common in AAHHRS than in A-CLDHRS (p = 0.08). Logistic regression showed that AAHHRS conferred a 20× and 3.3× odds of requiring ⩾30 days post-LT RRT compared to CLDno HRS and CLDHRS, respectively. Postoperative complications were similar across cohorts, but had a significant effect on 30-day renal outcome post-LT. Patients with AAH were more likely to develop HRS and require RRT pre- and post-LT at our center. The etiology of hepatic decompensation and postoperative complications affect renal recovery post-LT. The systemic inflammation of AAH in addition to conditions favoring renal hypoperfusion may contribute to the unfavorable outcomes of HRS-AKI after LT in this patient population.

Outcomes of Exercise Interventions in Patients With Advanced Liver Disease: A Systematic Review of Randomized Clinical Trials.

Frailty and sarcopenia are common complications of advanced liver disease. Owing to associated morbidity/mortality, there have been targeted efforts to prevent and/or improve both by enrolling these patients in focused exercise programs. This review systematically analyzes the data of randomized clinical trials (RCTs) on anthropometric, physical fitness, quality-of-life, and safety outcomes of exercise interventions in patients with advanced liver disease. Two authors independently searched trials on PubMed and EMBASE from inception up to November 18, 2021. A third independent arbitrator adjudicated all disagreements. We qualitatively summarized these outcomes as follows: (i) muscular fitness (maximal inspiratory/expiratory pressures, muscle size, muscle strength, and bioimpedance testing), (ii) cardiorespiratory fitness (cardiopulmonary exercise testing and 6-minute walk distance), (iii) quality of life, and (iv) others (safety or frailty indices). There were 11 RCTs (4 home-based interventions) with 358 participants. Interventions ranged from 8 to 14 weeks and included cycling, walking, resistance exercises, balance and coordination training, and respiratory exercises. All described outcomes compared preintervention with postintervention measurements. Nine studies showed statistically significant improvements in at least 1 physical fitness variable. Ten studies showed statistically significant improvements in at least 1 muscular fitness variable. Six studies showed statistically significant improvements in at least 1 quality-of-life variable. Attrition rates ranged from 5% to 36%, and adherence rates ranged very widely from 14% to 100%. Only 1 study reported frailty indices. Notably, no complications of portal hypertension were seen in intervention groups in the 9 studies that reported these data. A review of 11 RCTs with 358 participants with advanced liver disease demonstrates that exercise interventions can have favorable outcomes on muscular/cardiorespiratory fitness and quality of life. Although attrition and adherence varied, these interventions seem to be safe in patients with cirrhosis and are well tolerated.

Endoscopic Ultrasound-Guided Portal Pressure Gradient Measurement, Is It Ready for Prime Time?

Portal hypertension is a serious complication of advanced liver disease. Portal pressure gradient measurement accurately determines severity of portal hypertension, prognosis, and guides medical therapy. Conventionally, interventional radiology-guided hepatic venous portal gradient measures portal pressure gradient indirectly using specialized balloon catheter. Recent advances in endoscopy have paved the novel method of endoscopic ultrasound-guided direct portal pressure gradient measurement to evaluate portal hypertension with equivalent safety in both animal models and humans studies. With the expansion of Endo-Hepatology practice, a concept of “one stop shop” is becoming more popular which is a comprehensive endoscopic approach in the evaluation of chronic liver disease. During the same endoscopic procedure, patients could be evaluated for esophageal or gastric varices, portal hypertensive gastropathy, EUS-guided elastography, EUS-guided portal pressure measurement and EUS-guided liver biopsy when suspecting advanced liver disease or diagnosis remains uncertain. This article focuses on the overview of portal hypertension and EUS-guided interventions such as EUS-guided portal pressure measurement, EUS-guided elastography and EUS-guided liver biopsy in the evaluation of individuals with advanced liver disease.

A Case of Hepatic Encephalopathy Masquerading as Parkinsonism Unmasked by Classic Brain Imaging Findings: A Case Report

Abhinay Reddy Koppula, Dudekonda Sai Sandeep and Tekuru Yogesh Hepatic Encephalopathy (H.E), defined as a spectrum of neuropsychiatric abnormalities in patients with severe liver dysfunction. It is a most common complication of advanced liver disease with significant incidence and death rate [1].

Gut Microbes and Hepatic Encephalopathy: From the Old Concepts to New Perspectives.

Hepatic encephalopathy (HE) is a severe complication of advanced liver disease and acute liver failure. The clinical spectrum ranges from minor cognitive dysfunctions to lethargy, depressed consciousness, and coma and significantly impact the quality of life, morbidity, and mortality of the patients. It is commonly accepted that the gut milieu is essential for the development of HE; however, despite intensive research efforts, the pathogenesis of HE is still not fully elucidated. As our knowledge of gut microbiota moves from the pioneering era of culture-dependent studies, the connection between microbes, inflammation, and metabolic pathways in the pathogenesis of HE is becoming increasingly clear, providing exciting therapeutic perspectives. This review will critically examine the latest research findings on the role of gut microbes in the pathophysiological pathways underlying HE. Moreover, currently available therapeutic options and novel treatment strategies are discussed.

New Therapies of Liver Diseases: Hepatic Encephalopathy.

Hepatic encephalopathy (HE) is a common complication of advanced liver disease which has profound implications in terms of the patients’ ability to fulfil their family and social roles, to drive and to provide for themselves. Recurrent and persistent HE is still a serious management challenge, translating into a significant burden for patients and their families, health services and society at large. The past few years have been characterized by significantly more attention towards HE and its implications; its definition has been refined and a small number of new drugs/alternative management strategies have become available, while others are underway. In this narrative review we summarize them in a pragmatic and hopefully useful fashion.

Non-Invasive Predictors of Esophageal Varices in Patients with Cirrhosis of Liver- A Prospective Study

Background: Liver disease particularly chronic liver disease is one of the major public health problem, accounting for significant morbidity and mortality worldwide. Among the different complications, gastro-esophageal variceal bleeding is the deadliest complications of advanced liver disease and have been described in 50% of patients with liver cirrhosis. Aim: To identify the non-invasive predictors of esophageal varices in patients with cirrhosis of liver, so as to reduce number of unnecessary endoscopies. Methods: Ninety two patients with cirrhosis of liver irrespective of etiology were analysed prospectively from October 2019 to March 2020. Appropriate clinical, biochemical and ultrasonographic parameters were selected as variables to compare with esophageal varices. The data were assessed by univariate and multivariate analysis. Results: Univariate analysis of the studied parameters showed that decreased platelet count, albumin levels and platelet count/splenic diameter ratio and increased bilirubin levels, prothrombin time, splenic size, portal vein size were significantly associated with the presence of esophageal varices and their values correlated with increasing size of varices. On multivariate analysis of variables, the independent predictors for the presence of varices were platelet count/splenic size &lt;1433.1, splenic size&gt;115.75 mm. Conclusion: Platelet count/splenic size&lt;1433.1, Splenic size&gt;115.75 mm are independent noninvasive predictors of esophageal varices in patients with cirrhosis of liver

Role of renal Duplex ultrasonography in evaluation of hepatorenal syndrome

BackgroundHepatorenal syndrome is a potentially fatal complication of advanced liver disease. Markers for early diagnosis and identification of high-risk patients are lacking. Our aim was to evaluate the role of renal Duplex ultrasonography in the diagnosis and early prediction of hepatorenal syndrome. This study included 50 patients. Clinical assessment, liver function tests, hepatitis C virus antibody, kidney function tests, and abdominal and renal color Duplex ultrasound were done to all subjects.ResultsUnivariate regression analysis for hepatorenal syndrome showed a statistically significant positive correlation with the Model For End-Stage Liver Disease score (p-value <0.0001) and renal artery hilum resistivity index (p-value = 0.0017). Logistic multivariable regression analysis proved that the renal artery hilum resistivity index was an independent predictor of hepatorenal syndrome. Renal artery hilum resistivity index can be used as a predictor of hepatorenal syndrome with 100% sensitivity and 66.7% specificity with a cut-off value > 0.77.ConclusionThe renal resistive index could be a good predictor of hepatorenal syndrome.

Thrombocytopenia and Hemostatic Changes in Acute and Chronic Liver Disease: Pathophysiology, Clinical and Laboratory Features, and Management.

Thrombocytopenia, defined as a platelet count <150,000/μL, is the most common complication of advanced liver disease or cirrhosis with an incidence of up to 75%. A decrease in platelet count can be the first presenting sign and tends to be proportionally related to the severity of hepatic failure. The pathophysiology of thrombocytopenia in liver disease is multifactorial, including (i) splenomegaly and subsequently increased splenic sequestration of circulating platelets, (ii) reduced hepatic synthesis of thrombopoietin with missing stimulation both of megakaryocytopoiesis and thrombocytopoiesis, resulting in diminished platelet production and release from the bone marrow, and (iii) increased platelet destruction or consumption. Among these pathologies, the decrease in thrombopoietin synthesis has been identified as a central mechanism. Two newly licensed oral thrombopoietin mimetics/receptor agonists, avatrombopag and lusutrombopag, are now available for targeted treatment of thrombocytopenia in patients with advanced liver disease, who are undergoing invasive procedures. This review summarizes recent advances in the understanding of defective but at low level rebalanced hemostasis in stable cirrhosis, discusses clinical consequences and persistent controversial issues related to the inherent bleeding risk, and is focused on a risk-adapted management of thrombocytopenia in patients with chronic liver disease, including a restrictive transfusion regimen.

Hepatopulmonary Syndrome and Portopulmonary Hypertension: Current Status and Implications for Liver Transplantation.

Hepatopulmonary syndrome (HPS) and portopulmonary hypertension (PoPH) are both pulmonary vascular complications of advanced liver disease; however, these syndromes have distinct pathophysiology, clinical implications, and management. While both conditions are associated with portal hypertension, HPS results from diffuse pulmonary capillary vasodilation and PoPH results from vasoconstriction and vascular remodeling of pulmonary arteries. In HPS, no medical therapies clearly improve outcomes; however, patients have excellent post-LT outcomes with near uniform reversal of hypoxemia. In PoPH, several medical therapies used in idiopathic pulmonary hypertension have been shown improve pulmonary hemodynamics, symptoms, and potentially LT outcomes; however, further study is needed to determine best treatment regimens, long-term outcomes on medical therapy, and role of LT. While HPS results in severe hypoxemia that is usually reversible by LT, PoPH patients develop progressive pulmonary hypertension that may improve with medical therapy.

The gastrointestinal system in the critically ill cirrhotic patient.

ICU admissions due to complications of advanced liver disease continue to rise. Among indications for admission to the ICU in patients with cirrhosis, gastrointestinal issues such as bleeding are common. In patients in whom gastrointestinal issues are not the principal indication for ICU, gastrointestinal issues such as nutrition and ileus remain important concerns for generalized intensive care support. This review highlights current trends in management of gastrointestinal issues in patients with cirrhosis admitted to the ICU. General management of upper gastrointestinal bleeding remains largely unchanged. Improvements in interventional techniques have increased the options for difficult to control bleeding, these include the development of expandable esophageal stents and expanded experience with advanced interventional radiology techniques for the management of bleeding gastric varices. Frailty as an important prognostic marker in advanced liver disease and liver transplantation is the subject of several new studies and serves to highlight the importance of nutrition in the management of the critically ill cirrhotic patient. Gastrointestinal complications are frequent in the critically ill cirrhotic patient. Recognition and intervention in a timely manner may minimize morbidity and mortality and result in improved outcomes for this vulnerable population.

Clinical Implications of Thrombocytopenia for the Cirrhotic Patient.

Thrombocytopenia is a frequent complication in patients with cirrhosis. As many as 84% of patients with cirrhosis have thrombocytopenia, and it is an independent variable indicative of advanced disease and poor prognosis. Although there is great concern that it may aggravate bleeding during surgical procedures, there is limited evidence to inform decisions regarding the treatment of cirrhotic patients with thrombocytopenia undergoing invasive procedures. Finally, there is evidence that platelets play a significant role in liver regeneration. In this report, the clinical implications of thrombocytopenia in cirrhotic patients are reviewed. The utility of platelet counts in the prognosis of cirrhosis and relationship to complications of advanced liver disease, including portal hypertension, esophageal varices, and hepatocellular carcinoma. The impact of low platelet counts on bleeding complications during invasive procedures is outlined. Finally, the role of platelets and potential adverse impact in liver regeneration is reviewed.

Pulmonary manifestations of chronic liver disease: a comprehensive review.

Hepatopulmonary syndrome (HPS) and porto-pulmonary hypertension (PoPH) represent relatively common pulmonary vascular complications of advanced liver disease. Despite distinct differences in their pathogenetic background, both clinical states are characterized by impaired arterial oxygenation and limited functional status, and are associated with increased pre-transplantation mortality. Accumulation of ascitic fluid in the pleural cavity, known as hepatic hydrothorax (HH), is another frequent manifestation of decompensated cirrhosis, which may cause severe respiratory dysfunction, depending on the volume of the effusion, the rapidity of its development and its resistance to therapeutic measures. Orthotopic liver transplantation constitutes the only effective treatment able to resolve the pulmonary complications of liver disease. A prioritization policy for liver transplantation has evolved over the past years regarding advanced stages of HPS, yielding favorable outcomes regarding post-transplantation survival and HPS resolution. In contrast, severe PoPH is associated with poor post-transplantation survival. Hence, liver transplantation is recommended only for patients with PoPH and an acceptable reduction in pulmonary pressure values, after receiving PoPH-targeted vasodilating therapy. This review focuses on basic pathogenetic and diagnostic principles and discusses the current therapeutic approaches regarding HPS, PoPH, and HH.