Last updated March 15, 2022

Portal Hypertensive Bleeding in Cirrhosis

Guidance statements

Risk Stratification

  • Cirrhosis should be described, analyzed, and managed in two distinct clinical stages, compensated and decompensated, defined by the presence or absence of overt clinical complications of cirrhosis (ascites, variceal hemorrhage (VH), and hepatic encephalopathy [HE]).
  • Patients with compensated cirrhosis should be substaged into those with mild PH and those with CSPH, an entity that predicts the development of more-advanced stages.
  • Patients with clinically significant portal hypertension (CSPH) are substaged into those with and without gastroesophageal varices (GEV).
  • Treatment of portal hypertension (PH) differs depending on the stage and substages of cirrhosis, because prognosis and mechanisms of disease (and therefore therapeutic targets) are different.
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Diagnosis and Monitoring

NONINVASIVE TESTS IN THE DIAGNOSIS OF CLINICALLY SIGNIFICANT PORTAL HYPERTENSION

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  • Hepatic venous pressure gradient (HVPG) measurement is the gold-standard method to assess the presence of CSPH, defined as an HVPG ≥10 mm Hg.
  • CSPH can be identified by noninvasive tests: liver stiffness (LS) > 20-25 kPa, alone or combined with platelet count and spleen size. The presence of portosystemic collaterals on imaging is sufficient to diagnose CSPH.
  • Patients with GEV on endoscopy have, by definition, CSPH.
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NONINVASIVE TESTS IN THE DIAGNOSIS OF GASTROESOPHAGEAL VARICES

  • Patients with an LS <20 kPa and platelet count >150,000/mm3 have a very low probability (<5%) of having high-risk varices, and esophagogastroduodenoscopy (EGD) can be circumvented.
  • In patients who do not meet these criteria, screening endoscopy for the diagnosis of GEV is recommended when the diagnosis of cirrhosis is made.
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MONITORING CHANGES IN HEPATIC VENOUS PRESSURE GRADIENT

  • Patients with compensated cirrhosis (CC) without varices on screening endoscopy should have endoscopy repeated every 2 years (with ongoing liver injury or associated conditions, such as obesity and alcohol use) or every 3 years (if liver injury is quiescent, e.g., after viral elimination, alcohol abstinence).
  • Patients with CC with small varices on screening endoscopy should have endoscopy repeated every year (with ongoing liver injury) or every 2 years (if liver injury is quiescent, e.g., after viral elimination, alcohol abstinence).
  • Patients with CC without varices or with small varices who develop decompensation should have a repeat endoscopy when this occurs.
  • Monitoring changes in HVPG should not be performed routinely (outside clinical trials). Noninvasive tests do not correlate well with changes in HVPG.
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Management

PATIENTS WITH COMPENSATED CIRRHOSIS AND MILD PORTAL HYPERTENSION

  • In patients in the earliest stage of compensated cirrhosis (patients with mild PH), the objective of treatment is to prevent development of CSPH/decompensation and perhaps even to achieve regression of cirrhosis.
  • Elimination of the etiologic agent is the current mainstay of therapy.
  • Drugs that act on portal flow, such as nonselective beta-blockers (NSBBs), will be mostly ineffective in this substage, given that the hyperdynamic circulatory state is not fully developed.
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PATIENTS WITH COMPENSATED CIRRHOSIS AND CLINICALLY SIGNIFICANT PORTAL HYPERTENSION, BUT WITHOUT GASTROESOPHAGEAL VARICES

  • In patients with cirrhosis and CSPH but without varices, the objective of treatment should no longer be to prevent varices, but to prevent clinical decompensation.
  • There is no evidence at present to recommend the use of NSBBs in preventing formation of varices.
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PATIENTS WITH COMPENSATED CIRRHOSIS AND GASTROESOPHAGEAL VARICES

Prevention of First Variceal Hemorrhage in Patients With Medium/Large Esophageal Varices
  • Either traditional NSBBs (propranolol, nadolol), carvedilol, or endoscopic variceal ligation (EVL) is recommended for the prevention of first VH (primary prophylaxis) in patients with medium or large varices.
  • Choice of treatment should be based on patient preference and characteristics.
  • Patients on NSBBs or carvedilol for primary prophylaxis do not require monitoring with serial EGD.
  • Combination therapy NSBB plus EVL is not recommended in this setting.
  • TIPS placement is not recommended in the prevention of first VH.
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Prevention of First Variceal Hemorrhage in Patients With Small Esophageal Varices
  • NSBB is the recommended therapy for patients with high-risk small esophageal varices (EV).
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PATIENTS PRESENTING WITH ACUTE ESOPHAGEAL VARICEAL HEMORRHAGE

  • Packed red blood cell (PRBC) transfusion should be done conservatively, starting to transfuse when the hemoglobin reaches a threshold of around 7 g/dL with the goal of maintaining it between 7 and 9 g/dL.
  • Short-term (maximum 7 days) antibiotic prophylaxis should be instituted in any patient with cirrhosis and gastrointestinal (GI) hemorrhage.
  • Intravenous ceftriaxone 1 g/24 h is the antibiotic of choice and should be used for a maximum of 7 days (consider discontinuing when hemorrhage has resolved and vasoactive drugs discontinued).
  • Vasoactive drugs (somatostatin [SMT] or its analogue, octreotide; somatostatin [VP] or its analogue, terlipressin) should be initiated as soon as VH is suspected.
  • EGD should be performed within 12 hours of admission and once the patient is hemodynamically stable.
  • If a variceal source is confirmed/suspected, EVL should be performed.
  • In patients at high risk of failure or rebleeding (Child-Turcotte-Pugh [CTP] class C cirrhosis or CTP class B with active bleeding on endoscopy) who have no contraindications for TIPS, an “early” (preemptive) TIPS within 72 hours from EGD/EVL may benefit selected patients.
  • For patients in whom an early TIPS is not performed, intravenous vasoactive drugs should be continued for 2-5 days and NSBBs initiated once vasoactive drugs are discontinued. Rescue transjugular intrahepatic portosystemic shunt (TIPS) is indicated in these patients if hemorrhage cannot be controlled or if bleeding recurs despite vasoactive drugs+EVL.
  • In patients in whom TIPS is performed successfully, intravenous vasoactive drugs can be discontinued.
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PATIENTS WHO HAVE RECOVERED FROM AN EPISODE OF ACUTE ESOPHAGEAL VARICEAL HEMORRHAGE

  • Combination of NSBB+EVL is first-line therapy in the prevention of rebleeding.
  • Patients who have a TIPS placed successfully during the acute episode do not require NSBBs or EVL.
  • TIPS is the recommended rescue therapy in patients who experience recurrent hemorrhage despite combination therapy NSBB+EVL.
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Gastric Varices

PREVENTION OF FIRST HEMORRHAGE FROM GASTRIC VARICES

  • For prevention of first VH from GOV2 or IGV1, NSBBs can be used, although the data are not as strong as for EV.
  • Prevention of first bleeding from GOV1 varices may follow the recommendations for EV.
  • Neither TIPS nor balloon occluded retrograde transvenous obliteration (BRTO) are recommended to prevent first hemorrhage in patients with fundal varices that have not bled.
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MANAGEMENT OF ACUTE HEMORRHAGE FROM GASTRIC VARICES

  • Patients with acute bleeding from gastric varices (GV) should be initially managed in a similar fashion to those bleeding from EV (using a restrictive transfusion policy, vasoactive drug infusion, and antibiotic prophylaxis).
  • In patients bleeding from GOV1 varices, either EVL (if technically feasible) or cyanoacrylate glue injection, if available, are the recommended endoscopic treatments.
  • TIPS is the treatment of choice in the control of bleeding from cardiofundal varices (GOV2 or IGV1).
  • Cyanoacrylate glue injection is an option for cases in which TIPS is not technically feasible, but it is not approved for treatment of GV in the United States and should be performed only in centers where the expertise is available.
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PREVENTION OF REBLEEDING

  • In patients who have recovered from a GOV1 hemorrhage, the combination of NSBBs and endoscopic variceal therapy (EVL or cyanoacrylate injection) is the first-line therapy to prevent rebleeding.
  • In patients who have recovered from GOV2 or isolated GV type 1 (IGV1) hemorrhage, TIPS or BRTO are first-line treatments in the prevention of rebleeding.
  • Cyanoacrylate glue injection is an option for cases in which TIPS or BRTO are not technically feasible, but it is not approved for the treatment of GV in the United States and should be performed only in centers where the expertise is available.
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Ectopic Varices

The management of ectopic varices requires a thorough knowledge of the vascular supply to the varices and a multidisciplinary approach. Options are ligation, cyanoacrylate injection, endosonographic coil placement, TIPS with or without embolization, and BRTO.
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Special Populations

PATIENTS WITH REFRACTORY ASCITES OR AFTER SPONTANEOUS BACTERIAL PERITONITIS

  • Refractory ascites and spontaneous bacterial peritonitis (SBP) are not absolute contraindications for treatment with NSBBs. In these patients, high doses of NSBBs (over 160 mg/day of propranolol or over 80 mg/day of nadolol) should be avoided, given that they might be associated with worse outcomes.
  • In patients with refractory ascites and severe circulatory dysfunction (systolic blood pressure <90 mm Hg, serum sodium <130 meq/L, or HRS), the dose of NSBBs should be decreased or the drug temporarily held. NSBBs might be reintroduced if circulatory dysfunction improves.
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PREVENTION OF REBLEEDING IN PATIENTS EXPERIENCING THE FIRST VARICEAL HEMORRHAGE WHILE ON PRIMARY PROPHYLAXIS WITH NON-SELECTIVE BETA-BLOCKERS OR ENDOSCOPIC VARICEAL LIGATION

  • Patients failing primary prophylaxis for VH may be treated with the combination of NSBBs and EVL or, alternatively, with TIPS. Randomized trials are required in this group of patients to clarify the best therapeutic strategy.
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PREVENTION AND TREATMENT OF VARICEAL HEMORRHAGE IN PATIENTS WITH HEPATOCELLULAR CARCINOMA

  • Prevention and treatment of acute VH in patients with hepatocellular carcinoma (HCC) should follow the same principles as those for patients without HCC.
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Recommendation Grading

Overview

Title

Portal Hypertensive Bleeding in Cirrhosis

Authoring Organization

Publication Month/Year

September 1, 2016

Document Type

Guideline

External Publication Status

Published

Country of Publication

US

Document Objectives

This guidance provides a data‐supported approach to risk stratification, diagnosis, and management of patients with cirrhosis and portal hypertension (PH).

Target Patient Population

Patients with cirrhosis and portal hypertension

Inclusion Criteria

Female, Male, Adolescent, Adult, Older adult

Health Care Settings

Ambulatory, Emergency care, Hospital, Operating and recovery room

Intended Users

Nurse, nurse practitioner, physician, physician assistant

Scope

Assessment and screening, Diagnosis, Management, Treatment

Diseases/Conditions (MeSH)

D008103 - Liver Cirrhosis, D006975 - Hypertension, Portal, D017082 - Portal Pressure, D004932 - Esophageal and Gastric Varices

Keywords

cirrhosis, varices, portal hypertension, Portal hypertensive bleeding