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 Portal hypertension Pathway tolerance, cost-effectiveness, compliance, and effects on survival rates. B Insufficient evidence to support the use of medical therapy or TIPS placement for the treatment of patients with hepatopulmonary syndrome. I As per ILTS 2016 guidelines, recognize that aside from supplemental oxygen (rest, exercise, and sleep) no medical therapies are definitively established or approved by the FDA for hepatopulmonary syndrome. B Show 14 more Management of portopulmonary hypertension: As per SCCM 2020 guidelines, consider treating portopulmonary hypertension with agents approved for pulmonary arterial hypertension in patients with mean pulmonary artery pressure > 35 mmHg. C As per EASL 2018 guidelines, consider offering therapies approved for primary pulmonary arterial hypertension in patients with portopulmonary hypertension to improve exercise tolerance and hemodynamics. Be cautious when using endothelin antagonists because of concerns over hepatic impairment. B Show 2 more As per ILTS 2016 guidelines, consider offering agents approved for pulmonary artery hypertension to improve hemodynamics and exercise capacity in patients with portopulmonary hypertension. B Show 6 more Antithrombotic therapy: do not discourage the use of aspirin in patients with cirrhosis and an approved indication for aspirin therapy as it may reduce the risk of HCC, liver-related complications, and mortality. D Show 2 more Statin therapy: Encourage the use of statins in patients with cirrhosis and an approved indication for statin therapy as these agents may decrease portal pressure A and improve overall survival. B Initiate statins at a lower dose, such as simvastatin at the maximum dose of 20 mg/day, in patients with Child-Pugh B cirrhosis, A and restrictively in patients with Child-Pugh C cirrhosis. B Monitor closely for muscle and liver toxicity. A 5. Specific circumstances Pregnant patients: Obtain screening upper endoscopy for esophageal varices in the second trimester in pregnant patients with suspected portal hypertension. B Initiate treatment with -blockers and/or offer band ligation in pregnant patients with large esophageal varices. B Patients with pulmonary arterial hypertension: obtain abdominal ultrasound to screen for portal hypertension in patients with pulmonary arterial hypertension. B Show 4 more https://web.pathway.md/diseases/reckuN9AFS9LRM5Wu 16/20 6/29/23, 11:20 PM Portal hypertension Pathway 6. Follow-up and surveillance Serial liver elastography: repeat an index liver stiffness measurement 10 kPa in fasting conditions as soon as feasible or complement with an established serum marker of fibrosis (FIB-4 2.67, enhance liver fibrosis test 9.8, FibroTest 0.58 for alcohol-related/viral liver disease, FibroTest 0.48 for non-alcoholic fatty liver disease) since transient elastography can lead to false-positive results. B Show 2 more Surveillance for gastroesophageal varices: As per Baveno VII 2022 guidelines: Obtain endoscopic surveillance for variceal screening in patients with compensated cirrhosis with liver stiffness measurement 20 kPa or platelet count 150 10 L and not eligible for nonselective -blocker therapy (contraindication/intolerance) for the prevention of decompensation. A Obtain annual transient elastography and platelet count measurement to follow-up patients avoiding screening endoscopy. Perform screening endoscopy if liver stiffness measurement increases ( 20 kPa) or platelet count declines ( 150 10 L). B As per EASL 2018 guidelines, perform screening upper gastrointestinal endoscopy for gastroesophageal varices, if not previously diagnosed and treated, in patients with decompensated cirrhosis. B Show 2 more As per AASLD 2017 guidelines, perform screening endoscopy for the diagnosis of gastroesophageal varices when the diagnosis of cirrhosis is made. E Show 4 more As per BSG 2015 guidelines, perform screening endoscopy in all patients with cirrhosis at the time of diagnosis. A Show 3 more As per ASGE 2014 guidelines, perform screening endoscopy in all patients with a diagnosis of cirrhosis, to assess for esophageal and gastric varices. A Show 3 more As per AASLD 2007 guidelines, perform screening upper gastrointestinal endoscopy when a diagnosis of cirrhosis is made in order to assess for the presence of esophageal and/or gastric varices. B Show 2 more Clinical findings Symptoms Past medical history Abdominal discomfort Gastroesophageal varices Abdominal pain Gastrointestinal bleeding Hematemesis HCC Hematochezia Hepatic encephalopathy Melena Hepatopulmonary syndrome https://web.pathway.md/diseases/reckuN9AFS9LRM5Wu 17/20 6/29/23, 11:20 PM Portal hypertension Pathway Hepatorenal syndrome Vascular exam Liver cirrhosis Cruveilhier-baumgarten bruit Mesenteric vein thrombosis Portal vein thrombosis Integument exam Portopulmonary hypertension Rectal varices Caput medusae Splenic vein thrombosis Hematological findings Abdominal exam Anemia Abdominal distension blood platelet count Ascites Diagnostic procedures Splenomegaly hepatic venous pressure gradient Lab findings Hypoalbuminemia Iron deficiency Imaging findings liver stiffness Studies 2019 AVB-TIPS In patients with advanced cirrhosis and acute variceal bleeding who had been treated with vasoactive drugs plus endoscopic therapy, early TIPS was superior to standard care with respect to death or liver transplantation. Yong Lv et al. Lancet Gastroenterol Hepatol. 2019 Aug. 2010 Early TIPS for variceal hemorrhage In patients with cirrhosis and acute variceal bleeding who had been treated with vasoactive drugs plus endoscopic therapy, early TIPS implantation was superior to vasoactive drugs and endoscopic band ligation with respect to freedom from rebleeding or failure to control bleeding. Garcia-Pagan JC et al. N Engl J Med. 2010 Jun 24. References 1. Guruprasad P Aithal, Naaventhan Palaniyappan, Louise China et al. Guidelines on the management of ascites in cirrhosis. Gut. 2021 Jan;70 1 9 29. Open 2. Roberto de Franchis, Jaime Bosch, Guadalupe Garcia-Tsao et al. Baveno VII Renewing consensus in portal hypertension. J Hepatol. 2022 Apr;76 4 959 974. Open 3. Garcia-Tsao G, Abraldes JG, Berzigotti A et al. Portal hypertensive bleeding in cirrhosis: Risk stratification, diagnosis, and management: 2016 practice guidance by the American Association for the study of liver diseases. Hepatology. 2017 Jan;65 1 310 335. Open https://web.pathway.md/diseases/reckuN9AFS9LRM5Wu 18/20 6/29/23, 11:20 PM Portal hypertension Pathway 4. European Association for the Study of the Liver. EASL Clinical Practice Guidelines for the management of patients with decompensated cirrhosis. J Hepatol. 2018 Aug;69 2 406 460. Open 5. Tram T Tran, Joseph Ahn, Nancy S Reau. ACG Clinical Guideline: Liver Disease and Pregnancy. Am J Gastroenterol. 2016 Feb;111 2 176 94; quiz 196. Open 6. Scott W Biggins, Paulo Angeli, Guadalupe Garcia-Tsao et al. Diagnosis, Evaluation, and Management of Ascites, Spontaneous Bacterial Peritonitis and Hepatorenal Syndrome: 2021 Practice Guidance by the American Association for the Study of Liver Diseases. Hepatology. 2021 Aug;74 2 1014 1048. Open 7. Joo Ha Hwang, Amandeep K Shergill, Ruben D Acosta et al. The role of endoscopy in the management of variceal hemorrhage. Gastrointest Endosc. 2014 Aug;80 2 221 7. Open 8. Dhiraj Tripathi, Adrian J Stanley, Peter C Hayes et al. U.K. guidelines on the management of variceal haemorrhage in cirrhotic patients. Gut. 2015 Nov;64 11 1680 704. Open 9. Ian M Gralnek, Marine Camus Duboc, Juan Carlos Garcia-Pagan et al. Endoscopic diagnosis and management of esophagogastric variceal hemorrhage: European Society of Gastrointestinal Endoscopy ESGE Guideline. Endoscopy. 2022 Sep 29. Open 10. Jasmohan S Bajaj, Jacqueline G O'Leary, Jennifer C Lai et al. Acute-on-Chronic Liver Failure Clinical Guidelines. Am J Gastroenterol. 2022 Jan 10. Open 11. Garcia-Tsao G, Sanyal AJ, Grace ND et al. Prevention and management of gastroesophageal varices and variceal hemorrhage in cirrhosis. Hepatology. 2007 Sep;46 3 922 38. Open 12. European Association for the Study of the Liver. EASL clinical practice guidelines on the management of ascites, spontaneous bacterial peritonitis, and hepatorenal syndrome in cirrhosis. J Hepatol. 2010 Sep;53 3 397 417. Open 13. Marc Humbert, Gabor Kovacs, Marius M Hoeper et al. 2022 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Heart J. 2022 Oct 11;43 38 3618 3731. Open 14. Rahul Nanchal, Ram Subramanian, Constantine J Karvellas et al. Guidelines for the Management of Adult Acute and Acute-on-Chronic Liver Failure in the ICU Cardiovascular, Endocrine, Hematologic, Pulmonary and Renal Considerations: Executive Summary. Crit Care Med. 2020 Mar;48 3 415 419. Open 15. Michael J Krowka, Michael B Fallon, Steven M Kawut et al. International Liver Transplant Society Practice Guidelines: Diagnosis and Management of Hepatopulmonary Syndrome and Portopulmonary Hypertension. Transplantation. 2016 Jul;100 7 1440 52. Open 16. Antonio Tarasconi, Gennaro Perrone, Justin Davies et al. Anorectal emergencies: WSES AAST guidelines. World J Emerg Surg. 2021 Sep 16;16 1 48. Open 17. Paul Martin, Andrea DiMartini, Sandy Feng et al. Evaluation for liver transplantation in adults: 2013 practice guideline by the American Association for the Study of Liver Diseases and the American Society of Transplantation. Hepatology. 2014 Mar;59 3 1144 65. Open 18. M Bj rck, M Koelemay, S Acosta et al. Editor's Choice Management of the Diseases of Mesenteric Arteries and Veins: Clinical Practice Guidelines of the European Society of Vascular Surgery ESVS . Eur J Vasc Endovasc Surg. 2017 Apr;53 4 460 510. Open 19. Justin R Boike, Bartley G Thornburg, Sumeet K Asrani et al. North American Practice-Based Recommendations for Transjugular Intrahepatic Portosystemic Shunts in Portal Hypertension. Clin Gastroenterol Hepatol. 2021 Jul 15;S1542 3565 21 00749 7. Open https://web.pathway.md/diseases/reckuN9AFS9LRM5Wu 19/20 6/29/23, 11:20 PM Portal hypertension Pathway 20. A Vogel, A Cervantes, I Chau et al. Hepatocellular carcinoma: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up. Ann Oncol. 2018 Oct 1;29 Suppl 4):iv238-iv255. Open 21. de Franchis R, Baveno VI Faculty. Expanding consensus in portal hypertension: Report of the Baveno VI Consensus Workshop: Stratifying risk and individualizing care for portal hypertension. J Hepatol. 2015 Sep;63 3 743 52. Open 22. Expert Panels on Interventional Radiology and Vascular Imaging, Jason W Pinchot, Sanjeeva P Kalva et al. ACR Appropriateness Criteria Radiologic Management of Portal Hypertension. J Am Coll Radiol. 2021 May;18 5S S153 S173. Open 23. Rahul Nanchal, Ram Subramanian, Waleed Alhazzani et al. Guidelines for the Management of Adult Acute and Acute-on-Chronic Liver Failure in the ICU Neurology, Peri-Transplant Medicine, Infectious Disease, and Gastroenterology Considerations. Crit Care Med. 2023 May 1;51 5 657 676. Open https://web.pathway.md/diseases/reckuN9AFS9LRM5Wu 20/20