Guideline sources The following summarized guidelines for the evaluation and management of portal hypertension are prepared by our editorial team based on guidelines from the Baveno VII Consensus Workshop (Baveno VII 2022), the European Society of Gastrointestinal Endoscopy (ESGE 2022), the American College of Gastroenterology (ACG 2022; 2016), the European Society of Cardiology (ESC/ERS 2022), the British Association for the Study of the Liver (BASL/BSG 2021), the American Association for the Study of Liver Diseases (AASLD 2021; 2017; 2014), the World Society of Emergency Surgery (WSES/AAST 2021), the Advancing Liver Therapeutic Approaches Consortium (ALTA 2021), the Society of Critical Care Medicine (SCCM 2020), the European Association for the Study of the Liver (EASL 2018; 2010), the European Society of Medical Oncology (ESMO 2018), the European Society for Vascular Surgery (ESVS 2017), the International Liver Transplantation Society (ILTS 2016), the British Society of Gastroenterology (BSG 2015), the American Society for Gastrointestinal Endoscopy (ASGE 2014), and the American College of Gastroenterology (ACG/AASLD 2007). 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18 19 20 Calculator Calculator Child-Pugh score for mortality in Mumtaz readmission risk score i https://web.pathway.md/diseases/reckuN9AFS9LRM5Wu 1/20 6/29/23, 11:20 PM Portal hypertension Pathway Guidelines 1. Screening and diagnosis Diagnostic criteria, clinical findings: As per ESMO 2018 guidelines, diagnose clinically important portal hypertension based on the presence of esophageal varices and/or splenomegaly with blood platelet counts of 100 10 cells/L. B As per AASLD 2017 guidelines, diagnose clinically significant portal hypertension based on the presence of gastroesophageal varices on endoscopy. E Diagnostic criteria, HPVG: As per Baveno VII 2022 guidelines, diagnose sinusoidal portal hypertension in the presence of hepatic venous pressure gradient > 5 mmHg. A As per ESMO 2018 guidelines, diagnose clinically important portal hypertension based on invasive measurement by the transjugular route (hepatic venous pressure gradient > 10 mmHg). B As per AASLD 2017 guidelines, measure hepatic venous pressure gradient as the gold standard method to assess the presence of clinically significant portal hypertension, defined as a hepatic venous pressure gradient 10 mmHg. E Diagnostic criteria, liver elastography: As per Baveno VII 2022 guidelines, rule out clinically significant portal hypertension in patients with compensated advanced chronic liver disease based on liver stiffness measurement 15 kPa (by transient elastography) and platelet count 150 10 /L. B Show 3 more As per AASLD 2017 guidelines, consider identifying clinically significant portal hypertension with noninvasive tests: liver stiffness 20-25 kPa, alone or combined with platelet count and spleen size. Diagnose clinically significant portal hypertension based on the presence of portosystemic collaterals on imaging. E Diagnostic criteria (spleen elastography): consider obtaining spleen stiffness measurement by transient elastography in compensated advanced chronic liver disease due to viral hepatitis (untreated HCV, untreated and treated HBV) to rule out (spleen stiffness < 21 kPa) or rule in (spleen stiffness > 50 kPa) clinically significant portal hypertension. C 2. Classification and risk stratification Prognosis: recognize that the presence of clinically significant portal hypertension, determined either by hepatic venous pressure gradient 10 mmHg or by clinical manifestations of portal hypertension, is associated with a higher risk of decompensation and mortality in patients with cirrhosis undergoing liver resection for HCC. A Show 5 more https://web.pathway.md/diseases/reckuN9AFS9LRM5Wu 2/20 6/29/23, 11:20 PM Portal hypertension Pathway 3. Diagnostic investigations HVPG measurement (technical considerations): prefer using an end-hole, compliant balloon occlusion catheter over a conventional straight catheter to reduce the random error of wedged hepatic vein pressure measurements. A Show 9 more HVPG measurement (considerations in various etiologies): obtain hepatic venous pressure gradient measurement as the gold-standard method to determine the presence of clinically significant portal hypertension (hepatic venous pressure gradient 10 mmHg) in patients with viral- and alcohol-related cirrhosis. A Show 4 more HVPG measurement (pre- and post-TIPS): obtain portal pressure gradient measurement before and after TIPS insertion. A Show 5 more Evaluation of variceal hemorrhage: As per ESGE 2022 guidelines: Obtain endoscopic evaluation within 12 hours from the time of presentation in patients with suspected variceal hemorrhage, provided the patient has been hemodynamically resuscitated. B Administer IV erythromycin 250 mg, if not contraindicated, 30-120 minutes before gastrointestinal endoscopy in patients with suspected acute variceal hemorrhage. A As per EASL 2018 guidelines: Perform upper gastrointestinal endoscopy within the first 12 hours after admission once hemodynamic stability has been achieved, to ascertain the cause of hemorrhage and to provide endoscopic therapy. B Consider administering pre-endoscopy erythromycin (250 mg IV 30-120 minutes before), in the absence of contraindications (QT prolongation), to facilitate the procedure. B As per AASLD 2017 guidelines, perform upper gastrointestinal endoscopy within 12 hours of admission, and once the patient is hemodynamically stable, in patients presenting with acute esophageal variceal bleeding. E As per BSG 2015 guidelines, obtain endoscopy in unstable patients with severe acute upper gastrointestinal bleeding immediately after resuscitation. B Obtain endoscopy within 24 hours of admission in all other patients with upper gastrointestinal bleeding. B As per ASGE 2014 guidelines: Perform urgent endoscopy within 12 hours of admission in patients with suspected acute variceal hemorrhage. B Consider intubating patients before endoscopy to prevent aspiration during the procedure, especially in patients with encephalopathy. C Evaluation of ascites (initial evaluation): Obtain the following as part of the initial evaluation of patients with ascites: history and physical examination abdominal ultrasound https://web.pathway.md/diseases/reckuN9AFS9LRM5Wu 3/20 6/29/23, 11:20 PM Portal hypertension Pathway laboratory assessment of liver and renal functions, serum and urine electrolytes ascitic fluid analysis. Use contrast media cautiously and implement appropriate preventive measures for renal impairment, B although the use of contrast media does not appear to be associated with an increased risk of renal impairment in patients with ascites and preserved renal function. B Evaluation of ascites, diagnostic paracentesis: As per AASLD 2021 guidelines, perform diagnostic paracentesis in all patients with new- onset ascites accessible for sampling. E Show 5 more As per BASL/BSG 2021 guidelines, perform diagnostic paracentesis in all patients with new- onset ascites. B Show 6 more As per EASL 2018 guidelines, perform diagnostic paracentesis in all patients with new-onset grade 2 or 3 ascites, and in hospitalized patients with worsening ascites or any complication of cirrhosis. B Show 6 more Evaluation of spontaneous bacterial peritonitis: As per AASLD 2021 guidelines, perform diagnostic abdominal paracentesis to rule out SBP in patients with ascites due to cirrhosis emergently admitted to the hospital, even in the absence of symptoms/signs of infection. E Show 3 more As per BSG 2021 guidelines, perform diagnostic paracentesis without a delay to rule out SBP in all patients with cirrhosis and ascites on hospital admission. B Show 3 more As per EASL 2018 guidelines, obtain blood cultures in all patients with suspected SBP before antibiotic treatment initiation. B Show 6 more Evaluation of hepatopulmonary syndrome: As per EASL 2018 guidelines, evaluate patients with the hallmarks of chronic liver disease for hepatopulmonary syndrome in the presence of tachypnea and polypnea, digital clubbing, and/or cyanosis. B Show 3 more As per ILTS 2016 guidelines, obtain screening for hepatopulmonary syndrome, especially in liver transplant candidates, since its clinical presentation may be variable, adversely influencing the quality of life and survival. B Show 5 more Evaluation of portopulmonary hypertension: As per EASL 2018 guidelines: Obtain Doppler TTE to screen for portopulmonary hypertension in patients deemed potential recipients for TIPS or liver transplantation. Perform right heart catheterization in patients with a positive screening echocardiogram. B Obtain repeated echocardiography in patients with portopulmonary hypertension listed for transplantation. B https://web.pathway.md/diseases/reckuN9AFS9LRM5Wu 4/20 6/29/23, 11:20 PM Portal hypertension Pathway As per ILTS 2016 guidelines, obtain Doppler transthoracic echocardiography to screen for portopulmonary hypertension in patients with portal hypertension being candidates for TIPS insertion or liver transplantation. B Obtain repeated screening for portopulmonary hypertension in patients with portal hypertension while waiting for liver transplantation. B Show 6 more As per AASLD 2014 guidelines, exclude portopulmonary hypertension in liver transplant candidates by routine echocardiography. Perform right heart cardiac catheterization in patients with RV systolic pressure 45 mmHg. B Evaluation for pulmonary hypertension: Consider obtaining diagnostic tests (echocardiography, BNP/N-terminal pro-BNP, pulmonary function tests, and/or cardiopulmonary exercise testing) to screen for pulmonary arterial hypertension in symptomatic patients with portal hypertension. C Obtain echocardiography in patients with liver disease or portal hypertension with signs or symptoms suggestive of pulmonary hypertension, and as a screening tool in patients evaluated for liver transplantation or transjugular portosystemic shunt placement. B Evaluation for mesenteric vein thrombosis: evaluate for mesenteric vein thrombosis in patients with newly diagnosed portal hypertension. B 4. Medical management Management of the etiological factor: Remove/suppress the primary etiological factor to meaningfully decrease the hepatic venous pressure gradient in most patients and substantially reduce the risk of hepatic decompensation, including: achieve sustained virological response in patients with HCV infection suppress HBV in the absence of hepatitis D virus coinfection achieve long-term abstinence from alcohol in patients with ALD. A Show 3 more Primary prevention of gastroesophageal varices: As per AASLD 2017 guidelines, insufficient evidence to support the use of nonselective - blockers for the prevention of varices formation. I As per ACG 2007 guidelines, do not use nonselective -blockers to prevent the development of varices in patients with cirrhosis. D Primary prevention of variceal hemorrhage: As per ESGE 2022 guidelines: Initiate nonselective -blockers (preferably carvedilol), if not contraindicated, to prevent the development of variceal bleeding in patients with compensated advanced chronic liver disease and clinically significant portal hypertension (hepatic venous pressure gradient > 10 mmHg and/or liver stiffness by transient elastography > 25 kPa). B Perform endoscopic band ligation as the endoscopic prophylactic treatment of choice in patients unable to receive nonselective -blocker therapy with high-risk esophageal varices on screening upper gastrointestinal endoscopy. Repeat endoscopic band ligation every 2-4 https://web.pathway.md/diseases/reckuN9AFS9LRM5Wu 5/20 6/29/23, 11:20 PM Portal hypertension Pathway weeks until variceal eradication is achieved. Obtain surveillance upper gastrointestinal endoscopy every 3-6 months in the first year following eradication. B As per ALTA 2021 guidelines, insufficient evidence to recommend preoperative TIPS creation for the prevention of bleeding complications or the need for blood transfusion during or after invasive nontransplant surgical procedures in patients with portal hypertension requiring nontransplant surgery. I As per EASL 2018 guidelines, initiate primary prophylaxis upon detection of high-risk varices - small varices with red signs, medium or large varices irrespective of Child-Pugh classification, or small varices in Child-Pugh C patients - in patients with decompensated cirrhosis A , ascites, or an acute intercurrent condition. B Show 7 more As per AASLD 2017 guidelines, initiate nonselective -blockers for the prevention of first variceal hemorrhage in patients with small varices at high risk of bleeding (red wale sign or red spot). E Show 5 more As per BSG 2015 guidelines, initiate primary prophylaxis in patients with grade I varices and red signs or grade 2-3 varices, irrespective of the severity of the liver disease. A Show 6 more As per ASGE 2014 guidelines, perform endoscopic variceal ligation in patients with large esophageal varices unable to tolerate or having contraindications to nonselective -blockers. B Show 3 more Secondary prevention of variceal hemorrhage: As per ESGE 2022 guidelines: Perform follow-up endoscopic band ligations at 1- to 4-weekly intervals to eradicate esophageal varices (secondary prophylaxis) in patients undergone endoscopic band ligation for acute esophageal variceal hemorrhage. B Initiate nonselective -blockers (propranolol or carvedilol) in combination with endoscopic therapy for secondary prophylaxis in esophageal variceal hemorrhage in patients with advanced chronic liver disease. A As per EASL 2018 guidelines, attempt reinitiating nonselective -blockers after recovery from bleeding. B Manage the bleeding risk by expeditious endoscopic band ligation if nonselective -blocker intolerance or contraindications persist. B Show 2 more As per AASLD 2017 guidelines, initiate nonselective -blockers combined with endoscopic variceal ligation as first-line therapy for the prevention of rebleeding in patients with a history of variceal hemorrhage. E Show 4 more As per BSG 2015 guidelines, initiate nonselective -blockers (propranolol or nadolol) and variceal band ligation combination therapy for secondary prophylaxis in patients with variceal hemorrhage. A Show 9 more As per ASGE 2014 guidelines: https://web.pathway.md/diseases/reckuN9AFS9LRM5Wu 6/20 6/29/23, 11:20 PM Portal hypertension Pathway Repeat endoscopy with endoscopic variceal ligation after treatment of the acute episode of variceal hemorrhage until varices have been eradicated. B Consider repeating endoscopy at 1- to 8-week intervals for endoscopic variceal ligation for secondary prophylaxis. C Management of portal hypertensive gastropahy: initiate nonselective -blockers and iron supplementation and/or administer blood transfusion when indicated as first-line therapy for chronic hemorrhage from portal hypertension gastropathy. A Show 2 more Management of variceal hemorrhage, vasoactive agents: As per ESGE 2022 guidelines, administer vasoactive agents (terlipressin, octreotide, or somatostatin) initiated at the time of presentation in patients with suspected acute variceal bleeding and continue for a duration of up to 5 days. A As per EASL 2018 guidelines, administer vasoactive drugs as soon as acute variceal bleeding is suspected and before endoscopy. Administer terlipressin, somatostatin, or octreotide as accepted options. Administer therapy for 3-5 days in patients with acute variceal bleeding. A As per AASLD 2017 guidelines, administer vasoactive drugs (somatostatin, octreotide, vasopressin, or terlipressin) as soon as variceal hemorrhage is suspected. E Show 3 more As per BSG 2015 guidelines, administer vasoconstrictors, such as terlipressin or somatostatin, in all patients with variceal hemorrhage, starting as soon as variceal bleeding is suspected and continuing until hemostasis is achieved or for up to 5 days. Consider administering octreotide if terlipressin or somatostatin is unavailable. A As per ASGE 2014 guidelines, administer octreotide in patients with suspected variceal hemorrhage and continue for 3-5 days after endoscopy if variceal hemorrhage is confirmed. B Management of variceal hemorrhage (intravenous fluids): initiate prompt volume replacement with colloids and/or crystalloids to restore and maintain hemodynamic stability. B Do not use starch for volume replacement. A Management of variceal hemorrhage, antibiotic prophylaxis: As per ESGE 2022 guidelines, administer antibiotic prophylaxis with ceftriaxone 1 g/day for up to 7 days, or in accordance with local antibiotic resistance and patient allergies, in all patients with advanced chronic liver disease presenting with acute variceal hemorrhage. A As per EASL 2018 guidelines, administer antibiotic prophylaxis in patients with cirrhosis with acute gastrointestinal bleeding to reduce the incidence of infections and improve control of bleeding and survival. Initiate treatment on the presentation of bleeding and continue for up to 7 days. A Administer ceftriaxone 1 g/24 hours as the first choice in patients with decompensated cirrhosis, already on quinolone prophylaxis, and in hospital settings with a high prevalence of quinolone-resistant bacterial infections. Administer oral quinolones, such as norfloxacin 400 mg BID, in the remaining patients. A As per AASLD 2017 guidelines, administer short-term antibiotic prophylaxis in patients with cirrhosis and gastrointestinal bleeding. Administer ceftriaxone (1 g IV once daily) as the antibiotic of choice for prophylaxis, and complete a maximum of 7 days of treatment. https://web.pathway.md/diseases/reckuN9AFS9LRM5Wu 7/20 6/29/23, 11:20 PM Portal hypertension Pathway Consider discontinuing antibiotics when hemorrhage has resolved and vasoactive drugs are discontinued. E As per BSG 2015 guidelines, administer antibiotics in all patients with suspected or confirmed variceal bleeding. A As per ASGE 2014 guidelines, administer prophylactic antibiotics for 7 days in patients with cirrhosis presenting with variceal hemorrhage. A Management of variceal hemorrhage, blood product transfusion: As per ESGE 2022 guidelines, use a restrictive RBC transfusion strategy, with a Hgb threshold of 70 g/L prompting RBC transfusion in hemodynamically stable patients with acute upper gastrointestinal hemorrhage and no history of CVD. Set a post-transfusion target Hgb of 70-90 g/L. B As per EASL 2018 guidelines, use a restrictive transfusion strategy in most patients with a Hgb threshold for transfusion of 7 g/dL and a target range of 7-9 g/dL. A As per AASLD 2017 guidelines, administer packed RBC transfusion when the Hgb level reaches a threshold of around 7 g/dL, with the goal of maintaining Hgb 7-9 g/dL. E As per BSG 2015 guidelines, administer blood, platelets, and clotting factors in patients with massive bleeding in line with local protocols. B Show 7 more Management of variceal hemorrhage, endoscopic variceal ligation: As per ESGE 2022 guidelines, perform endoscopic band ligation for the treatment of acute esophageal variceal hemorrhage. A As per EASL 2018 guidelines: Perform variceal ligation within the same procedure when acute variceal bleeding is confirmed by endoscopy. A Combine vasoactive drugs and endoscopic ligation as the first therapeutic option in acute variceal bleeding. A As per AASLD 2017 guidelines, perform endoscopic variceal ligation if a variceal source for gastrointestinal bleeding is confirmed/suspected. E Show 2 more As per BSG 2015 guidelines, perform variceal band ligation as the preferred method for endoscopic treatment. A As per ASGE 2014 guidelines, perform endoscopic variceal ligation in patients with acute variceal hemorrhage or in patients with varices and stigmata of recent hemorrhage. B Management of variceal hemorrhage, endoscopic sclerotherapy: As per AASLD 2017 guidelines, consider administering cyanoacrylate glue injection as an option if TIPS or balloon-occluded retrograde transvenous obliteration is not technically feasible, recognizing that it is not approved for the treatment of gastric varices in the United States, and should be performed only in centers with expertise. E As per ASGE 2014 guidelines, consider performing endoscopic sclerotherapy if endoscopic variceal ligation is technically difficult to perform. C Management of variceal hemorrhage, TIPS: https://web.pathway.md/diseases/reckuN9AFS9LRM5Wu 8/20 6/29/23, 11:20 PM Portal hypertension Pathway As per ESGE 2022 guidelines: Consider performing preemptive TIPS placement within 72 hours (preferably within 24 hours) in patients at high risk for recurrent esophageal variceal bleeding following successful endoscopic hemostasis (Child-Pugh C 13 or Child-Pugh B > 7 with active esophageal variceal hemorrhage at the time of endoscopy despite vasoactive agents, or hepatic venous pressure gradient > 20 mmHg). B Consider performing urgent rescue TIPS placement (where available) in patients with persistent esophageal variceal bleeding despite vasoactive pharmacological and endoscopic hemostasis therapy. B As per ALTA 2021 guidelines, perform preemptive TIPS creation in patients with acute variceal hemorrhage successfully banded but meeting high-risk criteria for rebleeding (Child- Turcotte-Pugh class C with 10-13 points or Child-Turcotte-Pugh class B > 7 points with active bleeding at endoscopy). Perform TIPS within 72 hours of admission in patients without contraindications to TIPS. B Show 4 more As per EASL 2018 guidelines: Consider performing early preemptive covered TIPS placement within 24-72 hours in selected high-risk patients, such as with Child class C with a score < 14. B Perform TIPS placement as the rescue therapy of choice in patients with persistent bleeding or early rebleeding despite treatment with vasoactive drugs plus variceal ligation, and prophylactic antibiotics. A As per AASLD 2017 guidelines, perform an early preemptive TIPS placement within 72 hours of endoscopy/endoscopic variceal ligation in patients at high risk of treatment failure or rebleeding, in the absence of contraindications for TIPS. E Show 4 more As per BSG 2015 guidelines, consider performing early covered TIPS (< 72 hours after index variceal bleed), after satisfactory hemostasis and depending on local resources, in selected patients with Child's B cirrhosis and active bleeding or Child's C cirrhosis with Child's score < 14. C As per ASGE 2014 guidelines, perform TIPS creation in patients failed combined endoscopic and pharmacological therapies. B Management of variceal hemorrhage (balloon-occluded retrograde transvenous obliteration): perform TIPS placement or balloon-occluded retrograde transvenous obliteration as first-line treatments in the prevention of rebleeding in patients recovered from type 2 gastroesophageal or type 1 isolated gastric variceal hemorrhage. E Management of variceal hemorrhage, balloon tamponade: As per EASL 2018 guidelines, perform balloon tamponade in patients with uncontrolled bleeding but with a prerequisite of expertise and as a temporary "bridge" until definitive treatment can be instituted and for a maximum of 24 hours. B Consider placing removable, covered, and self-expanding esophageal stents as an alternative to balloon tamponade. B As per ASGE 2014 guidelines, consider performing balloon tamponade when initial endoscopic therapy fails to control acute variceal hemorrhage, to temporarily control bleeding until more definitive therapy can be performed. C https://web.pathway.md/diseases/reckuN9AFS9LRM5Wu 9/20 6/29/23, 11:20 PM Portal hypertension Pathway Management of variceal hemorrhage (Sengstaken-Blakemore tube): insert a Sengstaken- Blakemore tube if bleeding is difficult to control, until further endoscopic treatment, TIPS, or surgery is performed depending on local resources and expertise. B Management of variceal hemorrhage, management of gastric hemorrhage: As per ESGE 2022 guidelines, administer cyanoacrylate injection for acute gastric (cardiofundal) variceal (gastroesophageal varices type 2, isolated gastric varices type 1) hemorrhage. A Show 2 more As per ALTA 2021 guidelines: Decide on the initial management of patients with bleeding gastric-fundal varices based on center expertise. Consider performing variceal obliteration/embolization with or without TIPS in patients with bleeding gastric-fundal varices if unable to be managed endoscopically. B Perform variceal obliteration with or without TIPS creation in patients with rebleeding gastric-fundal varices after endoscopic therapy. B As per EASL 2018 guidelines, treat acute gastric variceal hemorrhage medically similar to esophageal variceal hemorrhage. Use cyanoacrylate as the endoscopic hemostatic treatment for cardiofundal varices (gastroesophageal varices type 2 or isolated gastric varices type 1). B Show 2 more As per BSG 2015 guidelines, treat patients with gastroesophageal varices type 1 as for patients with esophageal varices. B Show 4 more As per ASGE 2014 guidelines: Consider performing gastric variceal obturation with a cyanoacrylate-based compound for the treatment of acute gastric variceal hemorrhage at centers familiar with this technique. Otherwise, consider performing endoscopic variceal ligation. C Perform alternative interventions when gastric variceal hemorrhage cannot be controlled with endoscopic and pharmacological therapy. B Management of variceal hemorrhage (management of anorectal hemorrhage): consider administering IV fluids, blood transfusion if necessary, correction of coagulopathy, and optimal medication for portal hypertension in patients with anorectal varices and mild bleeding. C Show 2 more Management of ascites, salt restriction: As per AASLD 2021 guidelines, advise moderate sodium restriction (2 g or 90 mmol/day) in patients with cirrhosis and grade 2 ascites. E As per BASL/BSG 2021 guidelines: Advise moderate salt restriction with daily salt intake of 5-6.5 g (87-113 mmol sodium), meaning no added salt diet with avoidance of precooked meals, in patients with cirrhosis and ascites. B Provide nutritional counseling on the sodium content in the diet in patients with cirrhosis and ascites. B https://web.pathway.md/diseases/reckuN9AFS9LRM5Wu 10/20 6/29/23, 11:20 PM Portal hypertension Pathway As per EASL 2018 guidelines, advise moderate restriction of sodium intake (80-120 mmol/day, corresponding to 4.6-6.9 g of salt), meaning no added salt diet with avoidance of pre-prepared meals, in patients with moderate, uncomplicated ascites. A Show 2 more Management of ascites, diuretics: As per AASLD 2021 guidelines, initiate diuretics (spironolactone with or without furosemide), along with moderate sodium restriction (2 g or 90 mmol/day), as first-line therapy in patients with cirrhosis and grade 2 ascites. E Show 2 more As per BASL/BSG 2021 guidelines, consider initiating spironolactone monotherapy (starting dose 100 mg, increased to 400 mg) in patients with the first presentation of moderate ascites. B Show 6 more As per EASL 2018 guidelines, initiate spironolactone (starting at 100 mg/day PO, with stepwise increases every 72 hours to a maximum of 400 mg/day if there is no response to lower doses) in patients with a first episode of grade 2 ascites. A Show 8 more Management of ascites, IV albumin: As per AASLD 2021 guidelines: Consider administering human albumin solution (20-40 g/week) in patients with severe muscle cramps. E Insufficient evidence to support the routine long-term infusion of human albumin solution in patients with cirrhosis and diuretic-responsive ascites. I As per BASL/BSG 2021 guidelines: Administer albumin (20% or 25% solution) after paracentesis of > 5 L is completed at a dose of 8 g albumin/L of ascites removed. A Consider administering albumin (20% or 25% solution) after paracentesis of < 5 L at a dose of 8 g albumin/L of ascites removed in patients with acute-on-chronic liver failure or high risk of post-paracentesis AKI. C Management of ascites (correction of hyponatremia): obtain monitoring and advise water restriction in patients with mild hyponatremia (126-135 mEq/L) without symptoms. E Show 6 more Management of ascites, therapeutic paracentesis: As per AASLD 2021 guidelines, perform large-volume paracentesis as first-line therapy in patients with grade 3 ascites. Advise sodium restriction and initiate diuretics after paracentesis. E As per BASL/BSG 2021 guidelines: Consider using ultrasound guidance when available during large-volume paracentesis to reduce the risk of adverse events. C Do not obtain routine measurement of the PT and platelet count before therapeutic or diagnostic paracentesis and infusion of blood products. D https://web.pathway.md/diseases/reckuN9AFS9LRM5Wu 11/20 6/29/23, 11:20 PM Portal hypertension Pathway As per EASL 2018 guidelines, perform large-volume paracentesis, removing ascites completely in a single session, as first-line therapy in patients with large ascites (grade 3 ascites), followed by plasma volume expansion to prevent post-paracentesis circulatory dysfunction. A Show 4 more Management of ascites, TIPS: As per BASL/BSG 2021 guidelines: Consider performing TIPS placement in patients with refractory ascites. B Be cautious when considering TIPS placement in patients with: age > 70 years serum bilirubin > 50 mcmol/L platelet count < 75 10 /L model for end-stage liver disease score 18 current hepatic encephalopathy active infection or hepatorenal syndrome. B As per EASL 2018 guidelines, diagnose refractory ascites based on the assessment of the response of ascites to diuretic therapy and salt restriction in stable patients without associated complications, such as bleeding or infection, after ascertaining patient compliance with treatment. B Show 6 more Management of ascites, implantable peritoneal pump: As per BASL/BSG 2021 guidelines, consider placing an automated low-flow ascites pump only in special circumstances with robust arrangements of clinical governance, audit or research. C As per EASL 2018 guidelines, consider placing an implantable peritoneal pump in experienced centers in patients with refractory ascites not amenable to transhepatic intrajugular portosystemic shunt insertion. Obtain close monitoring because of the high risk of adverse events including renal dysfunction and technical difficulties. B Management of ascites, liver transplantation: As per AASLD 2021 guidelines, consider referring patients with grade 2 or 3 ascites for evaluation for liver transplantation. E As per EASL 2018 guidelines, evaluate patients with refractory ascites or hydrothorax for liver transplantation. B Show 10 more As per AASLD 2014 guidelines, consider evaluating for liver transplantation once a patient with cirrhosis has experienced an index complication, such as ascites, hepatic encephalopathy, or variceal hemorrhage, or hepatocellular dysfunction results in a model for end-stage liver disease score 15. B Show 2 more Prevention of spontaneous bacterial peritonitis, primary prevention: As per Baveno VII 2022 guidelines, administer primary antibiotic prophylaxis in selected patients (gastrointestinal bleeding, Child-Pugh C cirrhosis with low-protein ascites) at high https://web.pathway.md/diseases/reckuN9AFS9LRM5Wu 12/20 6/29/23, 11:20 PM Portal hypertension Pathway risk of SBP. B As per AASLD 2021 guidelines: Administer antibiotic prophylaxis for SBP in patients with cirrhosis and upper gastrointestinal hemorrhage. Administer IV ceftriaxone 1 g/24 hours as the antibiotic of choice for a maximum of 7 days. E Consider administering primary prophylaxis for SBP in selected patients with cirrhosis and low protein (< 1.5 g/L) ascites and renal dysfunction (serum creatinine level > 1.2 mg/dL, BUN level > 25 mg/dL or serum sodium level < 130 mEq/L) or liver failure (Child-Turcotte- Pugh score > 9 and bilirubin > 3 mg/dL). E As per BASL/BSG 2021 guidelines: Administer prophylactic antibiotic treatment (chosen based on local data) to prevent the development of SBP in patients presenting with gastrointestinal bleeding and underlying ascites due to cirrhosis. A Administer primary prophylaxis for SBP in high-risk patients, as defined by an ascitic protein count < 1.5 g/dL, with a discussion of potential risks and benefits and existing uncertainties with patients. B As per ACG/AASLD 2007 guidelines, administer short-term (maximum 7 days) antibiotic prophylaxis to prevent infectious complications, including SBP, in any patient with cirrhosis and gastrointestinal hemorrhage. A Show 2 more Prevention of spontaneous bacterial peritonitis, secondary prevention: As per Baveno VII 2022 guidelines: Administer secondary antibiotic prophylaxis in patients with previous SBP. A Administer secondary antibiotic prophylaxis in patients with previous SBP. A As per AASLD 2021 guidelines, initiate long-term prophylaxis with daily norfloxacin in patients recovered from an episode of SBP. Consider initiating oral ciprofloxacin if norfloxacin is unavailable. E As per BASL/BSG 2021 guidelines, consider initiating norfloxacin (400 mg once daily), ciprofloxacin (500 mg once daily, PO), or co-trimoxazole (800 mg sulfamethoxazole and 160 mg TMP daily, PO) to prevent further episodes of SBP in patients recovered from an episode of SBP. C As per EASL 2018 guidelines: Initiate prophylactic norfloxacin (400 mg/day, PO) in patients recovered from an episode of SBP, also to prevent hepatorenal syndrome-AKI. A Insufficient evidence to support the use of rifaximin as an alternative to norfloxacin for secondary prophylaxis of SBP. I Management of spontaneous bacterial peritonitis, IV albumin: As per Baveno VII 2022 guidelines, administer short-term albumin in patients with SBP. A As per AASLD 2021 guidelines, administer IV albumin in addition to antibiotics (1.5 g/kg on day 1 and 1 g/kg on day 3) in patients with SBP, especially in the presence of AKI and/or jaundice at the time of SBP diagnosis. E https://web.pathway.md/diseases/reckuN9AFS9LRM5Wu 13/20 6/29/23, 11:20 PM Portal hypertension Pathway As per BASL/BSG 2021 guidelines, administer albumin (1.5 g/kg infusion within 6 hours of diagnosis followed by 1 g/kg on day 3) in patients with SBP and increased or rising serum creatinine. B As per EASL 2018 guidelines, administer albumin (1.5 g/kg at diagnosis and 1 g/kg on day 3) in patients with SBP. A Management of spontaneous bacterial peritonitis, antibiotic therapy: As per AASLD 2021 guidelines, initiate empiric IV antibiotics in all patients with an ascites/pleural fluid polymorphonuclear count > 250/mm . E Show 3 more As per BASL/BSG 2021 guidelines: Initiate immediate empirical antibiotic therapy with due consideration of the context of SBP (community-acquired or healthcare-associated), the severity of infection, and local bacterial resistance profile. Guide the choice of an antibiotic agent by local resistance patterns and protocols. B Consider performing a second diagnostic paracentesis at 48 hours from the start of treatment to check the efficacy of antibiotic therapy in patients with apparent inadequate response or if secondary bacterial peritonitis is suspected. C As per EASL 2018 guidelines, initiate empiric IV antibiotics immediately after the diagnosis of SBP. B Show 6 more Management of hepatorenal syndrome, pharmacotherapy: As per ACG 2022 guidelines, consider initiating terlipressin C or norepinephrine to improve renal function in hospitalized patients with cirrhosis and hepatorenal syndrome-AKI without high-grade acute-on-chronic liver failure or major cardiopulmonary or vascular disease. C Landmark trials: CONFIRM In adult patients with type 1 hepatorenal syndrome, terlipressin was superior to placebo with respect to verified reversal of hepatorenal syndrome. Florence Wong et al. N Engl J Med. 2021 Mar 4. As per Baveno VII 2022 guidelines: Administer short-term albumin in patients with AKI > stage 1A B and combined with terlipressin in patients with hepatorenal syndrome-AKI. B Administer short-term albumin in patients with AKI > stage 1A B and combined with terlipressin in patients with hepatorenal syndrome-AKI. B As per AASLD 2021 guidelines, decide on the management (including initiation of vasoconstrictor therapy and RRT) by a multidisciplinary team including specialists in hepatology, nephrology, critical care, and transplant surgery, if possible, given the complexity of patients with suspected hepatorenal syndrome-AKI. E Show 5 more https://web.pathway.md/diseases/reckuN9AFS9LRM5Wu 14/20 6/29/23, 11:20 PM Portal hypertension Pathway As per SCCM 2020 guidelines, initiate vasopressors in critically ill patients with acute-on- chronic liver failure developing hepatorenal syndrome. B As per EASL 2018 guidelines, initiate vasoconstrictors and albumin expeditiously in all patients meeting the current definition of AKI-hepatorenal syndrome stage > 1A. B Show 10 more As per EASL 2018 guidelines, discontinue diuretics and/or -blockers and other drugs that could be associated with AKI, such as vasodilators, NSAIDs, and nephrotoxic drugs. B Show 2 more As per AASLD 2017 guidelines, initiate nonselective -blockers combined with endoscopic variceal ligation as first-line therapy for the prevention of rebleeding in patients with a history of variceal hemorrhage. E Show 4 more As per EASL 2010 guidelines, discontinue all diuretics at the initial evaluation and diagnosis of hepatorenal syndrome. A Show 3 more Management of hepatorenal syndrome, renal replacement therapy: As per AASLD 2021 guidelines: Initiate RRT in candidates for liver transplantation with worsening renal function or electrolyte disturbances or increasing volume overload unresponsive to vasoconstrictor therapy. E Initiate RRT with a clear endpoint in mind in patients not being candidates for liver transplantation. E As per EASL 2018 guidelines, decide on initiating RRT based on the individual severity of illness. B Management of hepatorenal syndrome (TIPS): Insufficient evidence to recommend TIPS placement in patients with hepatorenal syndrome- AKI. I Consider placing a TIPS in selected patients with hepatorenal syndrome outside the criteria of AKI. C Management of hepatorenal syndrome, therapeutic paracentesis: As per EASL 2018 guidelines, administer albumin with therapeutic paracentesis, even when a low volume of ascetic fluid is removed, in patients with AKI and tense ascites. B As per EASL 2010 guidelines, consider performing large-volume paracentesis with albumin in patients with tense ascites, despite the paucity of data on the use of paracentesis in patients with hepatorenal syndrome. B Management of hepatopulmonary syndrome: As per SCCM 2020 guidelines, provide supportive care with supplemental oxygen in the management of patients with hepatopulmonary syndrome, pending possible liver transplantation. E As per EASL 2018 guidelines: Initiate long-term oxygen therapy in patients with hepatopulmonary syndrome and severe hypoxemia, recognizing that there is insufficient evidence regarding its effectiveness, https://web.pathway.md/diseases/reckuN9AFS9LRM5Wu 15/20 6/29/23, 11:20 PM