Baveno 7

Seminar
Baveno VII – Renewing consensus in portal hypertension

Roberto de Franchis1,*, Jaime Bosch2,3, Guadalupe Garcia-Tsao4,5, Thomas Reiberger6,7,
Cristina Ripoll 8, on behalf of the Baveno VII Faculty§
Summary
To expand on the work of previous meetings, a virtual Baveno VII workshop was organised for October Keywords: Cirrhosis; diagnosis;
decompensation; treatment;
2021. Among patients with compensated cirrhosis or compensated advanced chronic liver disease
recommendations.
(cACLD – defined at the Baveno VI conference), the presence or absence of clinically significant portal
hypertension (CSPH) is associated with differing outcomes, including risk of death, and different diag- Received 18 November 2021;
received in revised form 10
nostic and therapeutic needs. Accordingly, the Baveno VII workshop was entitled “Personalized Care for
December 2021; accepted 17
Portal Hypertension”. The main fields of discussion were the relevance and indications for measuring the December 2021; available online
hepatic venous pressure gradient as a gold standard, the use of non-invasive tools for the diagnosis of 30 December 2021
cACLD and CSPH, the impact of aetiological and non-aetiological therapies on the course of cirrhosis, the
prevention of the first episode of decompensation, the management of an acute bleeding episode, the
prevention of further decompensation, as well as the diagnosis and management of splanchnic vein
thrombosis and other vascular disorders of the liver. For each of these 9 topics, a thorough review of the
medical literature was performed, and a series of consensus statements/recommendations were dis-
cussed and agreed upon. A summary of the most important conclusions/recommendations derived from
the workshop is reported here. The statements are classified as unchanged, changed, and new in relation
to Baveno VI.
© 2021 European Association for the Study of the Liver. Published by Elsevier B.V. All rights reserved.
1
Department of Biomedical and
Introduction Clinical Sciences, University of
Portal hypertension is a major consequence of ings were successful and produced consensus rec- Milan, Italy; 2Department of
Visceral Surgery and Medicine,
cirrhosis and is responsible for its most severe ommendations that referred mostly to the Inselspital, Bern University
complications, including ascites, bleeding from management of varices and variceal haemorrhage. Hospital, University of Bern,
gastro-oesophageal varices and encephalopathy. To continue and expand on the work of previous Switzerland; 3Instituts
d’Investigacions Biomèdiques
The evaluation of diagnostic tools and the design meetings, a Baveno VII workshop was planned for
August Pi i Sunyer (IDIBAPS) and
and conduct of high-quality clinical trials for the March 20-21, 2020. This would also include rec- CIBERehd, University of
treatment of portal hypertension and its compli- ommendations on other complications of cirrhosis Barcelona, Spain; 4Yale
cations have always been difficult. Awareness of and portal hypertension besides variceal haemor- University , New Haven, USA;
5
VA-CT Healthcare System, West
these difficulties has led to the organisation of a rhage. However, the COVID-19 pandemic and the Haven, USA; 6Division of
series of consensus meetings. The first one was consequent lockdown forced the organisers to Gastroenterology and
organised by Andrew Burroughs in Groningen, the postpone the workshop until the end of October Hepatology, Department of
Medicine III, Medical University
Netherlands in 1986.1 After Groningen, other 2021 and to change the format from a face-to-face of Vienna, Vienna, Austria;
meetings followed, in Baveno, Italy in 1990 (Baveno to a virtual meeting. Despite these limitations, 7
Vienna Hepatic Hemodynamic
I)2 and in 1995 (Baveno II)3,4; in Milan, Italy in many of the experts responsible for the major recent Lab, Medical University of
Vienna, Vienna, Austria;
19925; in Reston, the United States,6 in 1996; in achievements in the field of portal hypertension and 8
Internal Medicine IV,
Stresa, Italy, in 2000 (Baveno III)7,8; in Baveno in its complications participated in the workshop. Universitätsklinikum Jena,
2005 (Baveno IV)9,10; in Atlanta, the United States Many of them had attended the previous meetings. Friedrich Schiller University,
in 200711; in Stresa in 2010 (Baveno V)12,13; and in Importantly, following the spirit of the Baveno Jena, Germany
§
Baveno in 2015 (Baveno VI).14,15 meetings, the Baveno Cooperation was formed in The members of the Baveno
VII Faculty are given before
The aims of these meetings were to develop 2016 with the aim of expanding the scope of such the references
definitions of key events in portal hypertension, to meetings towards the continuous collaboration of
* Corresponding author.
review the existing evidence on the natural history, experts in portal hypertension and to the estab-
Address: Department of
the diagnosis, and the therapeutic modalities of lishment of a continuous, high-quality research Biomedical and Clinical Sci-
portal hypertension, and to issue evidence-based agenda. In 2019, the European Association for the ences, University of Milan,
recommendations for the conduct of clinical trials Study of the Liver (EASL) endorsed the Baveno Milan, Italy.
and the management of patients. All these meet- Cooperation as an official EASL consortium. https://doi.org/
10.1016/j.jhep.2021.12.022
Journal of Hepatology 2022 vol. 76 j 959–974

Seminar
Patients with cirrhosis transition through different prognostic 1.6. To properly reflect portal venous pressure, WHVP requires a
stages, the main ones being the compensated and decom- stabilisation time. Recording of WHVP requires a minimum
pensated stages. Transition from the compensated to the of 1 minute, with particular attention to stability during the
decompensated stage is clinically marked by the development of last 20-30 seconds. WHVP should be recorded in triplicate.
complications such as ascites, variceal haemorrhage and overt (D.1) (New)
hepatic encephalopathy. Because “cirrhosis” implies a patho- 1.7. The wedged to free hepatic vein pressure gradient has su-
logical (invasive) diagnosis, at the Baveno VI conference, the perior clinical prognostic value than wedged to right atrial
concept of compensated advanced chronic liver disease (cACLD) pressure gradient and should be used as the standard ref-
was put forward based on non-invasive tests (NITs) that would erence.(B.1) Right atrial pressure can be measured to rule out
predict the development of complications of cirrhosis. Among a post-hepatic component of portal hypertension.
patients with compensated cirrhosis or cACLD, at least two (B.1) (New)
different stages have been identified based on the presence or 1.8. Free hepatic vein pressure must be measured in the hepatic
absence of clinically significant portal hypertension (CSPH). The vein within 2-3 cm of its confluence with the inferior vena
various disease stages are associated with differing outcomes, cava (IVC). IVC pressure should be measured as an internal
including risk of death, and therefore patients in different stages control, at the level of the hepatic vein ostium. If the free
have different diagnostic and therapeutic needs. Accordingly, the hepatic vein pressure is more than 2 mmHg above IVC
Baveno VII workshop was entitled “Personalized Care for Portal pressures, the presence of a hepatic vein outflow obstruc-
Hypertension”. The main fields of discussion were the relevance tion should be ruled out by injecting a small amount of
and indications for measuring the hepatic venous pressure contrast medium. (A.1) (New)
gradient (HVPG) as a gold standard, the use of non-invasive tools
for the diagnosis of cACLD and CSPH, the impact of aetiological
and non-aetiological therapies on the course of cirrhosis, the Diagnosis of CSPH in patients with cirrhosis
prevention of the first episode of decompensation, the man- 1.9. HVPG values >5 mmHg indicate sinusoidal portal hyperten-
agement of an acute bleeding episode, the prevention of further sion. (A.1) (Unchanged)
decompensation, as well as the diagnosis and management of 1.10. In patients with viral- and alcohol-related cirrhosis, HVPG
splanchnic vein thrombosis and other vascular disorders of the measurement is the gold-standard method to determine
liver. For each of these 9 topics, a thorough review of the medical the presence of “clinically significant portal hypertension”
literature was performed, and a series of consensus statements/ (CSPH), which is defined as an HVPG > −10 mmHg.
recommendations were discussed and agreed upon. Whenever (A.1) (Changed)
applicable, the level of existing evidence was evaluated, and the 1.11. In patients with primary biliary cholangitis, there may be
recommendations were ranked according to the GRADE sys- an additional pre-sinusoidal component of portal hyper-
tem,16 according to which the scientific evidence was graded tension that cannot be assessed by HVPG.(B.1) As such,
from A (high) to D (very low). The strength of the recommen- in these patients, HVPG may underestimate the preva-
dations was graded 1 (strong) and 2 (weak). The presentations lence and severity of PH. (B.1) (New)
made during the workshop are reported ‘in extenso’ in the 1.12. In patients with non-alcoholic steatohepatitis (NASH)-
Baveno VII proceedings book.17 A summary of the most impor- related cirrhosis, although an HVPG > −10 mmHg remains
tant conclusions/recommendations derived from the workshop strongly associated with the presence of clinical signs
is reported here. The statements are classified as unchanged, of portal hypertension, these signs can also be present in a
changed, and new in relation to Baveno VI. small proportion of patients with HVPG values <10 mmHg.
(C.2) (New)
1) HVPG as a gold standard 1.13. In patients with chronic liver disease and clinical signs of
Description of HVPG measurement portal hypertension (gastro-oesophageal varices, ascites,
1.1. The use of an end-hole, compliant balloon occlusion catheter portosystemic collateral vessels) but with HVPG <10 mmHg,
reduces the random error of wedged hepatic vein pressure porto-sinusoidal vascular disorder (PSVD) must be ruled
(WHVP) measurements and is preferred over the use of a out. (B.1) (New)
conventional straight catheter. (A.1) (New) 1.14. In alcohol-related or viral cirrhosis, a decrease in HVPG in
1.2. A small volume of contrast medium should be injected when response to non-selective beta-blockers (NSBBs) is associ-
the occlusion balloon is inflated to confirm a satisfactory ated with a significant reduction in the risk of variceal
occluded position and to exclude the presence of hepatic bleeding or of other decompensating events.
venous-to-venous communications. (A.1) (New) (A.1) (Changed)
1.3. Hepatic venous-to-venous communications may result in
underestimation of the WHVP and must be reported.
(A.1) (New) Inclusion of HVPG assessment in trial design
1.4. Deep sedation during liver haemodynamic measurement 1.15. HVPG measurements should be encouraged in clinical trials
may cause inaccurate HVPG values.(B.1) If light sedation is investigating novel therapies but are not essential if portal
required, low dose midazolam (0.02 mg/kg) does not hypertension-associated endpoints are well defined.
modify the HVPG and is acceptable. (B.1) (New) (B.1) (Unchanged)
1.5. Slow speed (up to 7.5 mm/s) permanent tracings of pres- 1.16. In viral, alcohol-related, and reasonably in NASH-related
sures, recorded either on paper or electronically, are rec- cirrhosis, HVPG response assessment is recommended as
ommended. Digital, on-screen, readings are much less a surrogate endpoint in phase II clinical trials where a low
accurate and should not be used. (A.1) (New) rate of events is expected. (D.2) (Changed)
960 Journal of Hepatology 2022 vol. 76 j 959–974

1.17. Test-retest reliability of HVPG measurement is excellent correlation between the haemodynamic outcomes of TIPS
but influenced by the stage of liver disease (lower in and the clinical response of ascites.
decompensated patients) and its aetiology (higher in pa- Investigate the optimal PPG increase (in the context of TIPS
tients with alcohol-related disease). This should be taken reduction) needed to ameliorate adverse events related to
into consideration when designing clinical trials based on over-shunting.
HVPG assessment. (C.1) (New)
2) Non-invasive tools for cACLD and portal

Assessment of surgical risks hypertension
1.18. The presence of CSPH, determined either by HVPG > −10 Definition of cACLD
mmHg or by clinical manifestations of portal hypertension, 2.1 The use of elastography in clinical practice has enabled the
is associated with a higher risk of decompensation and early identification of patients with untreated/active chronic
mortality in patients with cirrhosis undergoing liver liver disease at risk of having CSPH and consequently, at risk
resection for hepatocellular carcinoma (HCC). (A.1) (New) of decompensation and liver-related death. (A.1) (Changed)
1.19. In candidates for non-hepatic abdominal surgery, a HVPG 2.2 The term “compensated advanced chronic liver disease
>
−16 mmHg is associated with an increased risk of short- (cACLD)” had been proposed to reflect the continuum of
term mortality after surgery. (C.1) (New) severe fibrosis and cirrhosis in patients with ongoing
chronic liver disease. A pragmatic definition of cACLD based
on liver stiffness measurement (LSM) is aimed at stratifying
PPG in the setting of TIPS the risk of CSPH and decompensation at point of care, irre-
1.20. Portal pressure gradient (PPG) should be measured before spective of histological stage or the ability of LSM to identify
and after transjugular intrahepatic portosystemic shunt these stages. (B.1) (Changed)
(TIPS) insertion. (A.1) (New) 2.3 Currently, both terms “cACLD” and “compensated cirrhosis”
1.21. Anatomic locations for post-TIPS PPG measurement should are acceptable, but not interchangeable. (B.1) (Changed)
include the main portal vein and the IVC (at the shunt
outflow). (B.1) (New)
1.22. The immediate post-TIPS PPG may be influenced by Criteria to identify cACLD
various factors, such as general anaesthesia, use of vaso- 2.4 LSM values by transient elastography (TE) <10 kPa in the
active agents or haemodynamic instability and therefore absence of other known clinical/imaging signs rule out
immediate post-TIPS PPG may not represent long-term cACLD; values between 10 and 15 kPa are suggestive of
PPG.(B.1) PPG measurements in haemodynamically sta- cACLD; values >15 kPa are highly suggestive of cACLD.
ble, non-sedated patients better reflect post-TIPS PPG (B.1) (Changed)
values and are recommended.(B.1) (New) 2.5 Patients with chronic liver disease and an LSM <10 kPa by TE
1.23. In patients with variceal bleeding undergoing TIPS, reduc- have a negligible 3-year risk (<−1%) of decompensation and
tion of absolute PPG to <12 mmHg is associated with near liver-related death. (A.1) (New)
complete protection from portal hypertensive bleeding and 2.6 Patients with cACLD should be referred to a liver disease
is the preferred target for haemodynamic success. (A.1) A specialist for further work-up. (B.1) (Changed)
relative reduction of PPG, by at least 50% from pre-TIPS 2.7 Invasive methods (liver biopsy, HVPG) can be used for
baseline, may also be useful. (B.2) (New) further work-up in an individualised manner at referral
1.24. PPG re-measurement is indicated to evaluate the need for centres. (B.1) (Changed)
TIPS revision if there is clinical or Doppler-ultrasonographic
suspicion of TIPS dysfunction. (B.1) (New)
Outcome and prognosis
Research agenda 2.8 LSM (irrespective of the technique used for its measurement)
Further evaluate the usefulness, safety, and accuracy of direct holds prognostic information in cACLD, both at index
portal pressure measurement by endoscopic ultrasound. investigation and during follow-up. (A.1) (New)
Further investigate the prognostic role of HVPG and define 2.9 A rule of 5 for LSM by TE (10-15-20-25 kPa) should be used to
specific cut-offs in patients with NASH-cirrhosis. denote progressively higher relative risks of decompensation
Confirm the utility of HVPG-guided therapy in randomised and liver-related death independently of the aetiology of
clinical trials. chronic liver disease. (B.1) (New)
Further investigate the prognostic role of HVPG in patients
undergoing extrahepatic surgery in prospective cohorts that
should compare HVPG with non-invasive tests. How to monitor
Evaluate test-retest HVPG reliability at an individual level and 2.10 Patients with LSM values 7-10 kPa and ongoing liver injury
examine factors that determine variability. should be monitored on a case-by-case basis for changes
Evaluate portocaval- vs. porto-atrial-measured PPG and clin- indicating progression to cACLD. (C.2) (New)
ical outcomes after TIPS (e.g., rebleeding). 2.11 TE can lead to false positive results, therefore an index LSM
Determine the optimal PPG decrease required to medically >
−10 kPa should be repeated in fasting conditions as soon as
control recurrent/refractory ascites. Further investigate the feasible or complemented with an established serum
Journal of Hepatology 2022 vol. 76 j 959–974 961

Seminar
marker of fibrosis (fibrosis-4 > −2.67, enhance liver fibrosis 2.22 In patients who are not candidates for NSBBs (contraindi-
test >
−9.8, FibroTest >
−0.58 for alcohol-related/viral liver dis- cation/intolerance) and in whom endoscopy would be
ease, FibroTest >−0.48 for non-alcoholic fatty liver disease). required according to the Baveno VI criteria (LSM by TE > −20
9
(B.2) (New) −150x10 L), SSM <
kPa or platelet count < −40 kPa by TE can be
2.12 In patients with cACLD, LSM could be repeated every 12 used to identify those at low probability of high-risk varices,
months to monitor changes. (B.2) (New) in whom endoscopy can be avoided. (C.2) (New)
2.13 A clinically significant decrease in LSM, which is associated
with substantially reduced risk of decompensation and
liver-related death, can be defined as a decrease in LSM of Research agenda
>
−20% associated with LSM <20 kPa or any decrease to a LSM Define risk of decompensation associated with different LSM
<10 kPa. (C.2) (New) cut-offs in different aetiologies of cACLD.
Validate and refine non-invasive tools for CSPH in patients
with NASH.
Diagnosis of CSPH in patients with cACLD Evaluate the diagnostic value of LSM for CSPH in aetiologies
2.14 Although the concept of CSPH is HVPG-driven, non-invasive other than viral/alcohol/NASH.
tests are sufficiently accurate to identify CSPH in clinical Establish whether sex and age require specific calibration of
practice. (A.1) (New) NITs for CSPH.
2.15 LSM by TE < −15 kPa plus platelet count >
9
−150x10 /L rules out Validate circulating biomarkers for prediction of decompen-
CSPH (sensitivity and negative predictive value >90%) in sation in all aetiologies.
patients with cACLD. (B.2) (New) Validate LSM thresholds for CSPH, high-risk varices and
2.16 In patients with virus- and/or alcohol-related cACLD and decompensation obtained from devices other than TE.
non-obese (BMI <30 kg/m2) NASH-related cACLD, a LSM Validate what constitutes a clinically significant improvement
value by TE of − >25 kPa is sufficient to rule in CSPH (speci- or worsening of LSM in all aetiologies.
ficity and positive predictive value >90%), defining the group Validate SSM in non-viral aetiologies.
of patients at risk of endoscopic signs of portal hypertension Evaluate emerging methods to diagnose CSPH and determine
and at higher risk of decompensation. (B.1) (Changed) response to NSBBs, such as contrast-enhanced ultrasound-
2.17 In patients with virus- and/or alcohol-related and non- based methods (SHAPE), MRI methods, and the combination
obese NASH-related cACLD with LSM values <25 kPa, the of elastography, novel imaging methods and tests addressing
ANTICIPATE model can be used to predict the risk of CSPH. liver function.
Based on this model, patients with LSM values between 20-
25 kPa and platelet count <150x109/L or LSM values be- 3) Management of ACLD after removal/suppression of
tween 15-20 kPa and platelet count <110x109/L have a CSPH the primary aetiological factor
risk of at least 60%. (B.2) (New) 3.1 Removal/suppression of the primary aetiological factor in-
2.18 In patients with NASH-related cACLD, the ANTICIPATE- cludes sustained virological response (SVR) in patients with
NASH model (including LSM, platelet count and BMI) may HCV infection, HBV suppression in the absence of HDV co-
be used to predict the risk of CSPH, but further validation is infection in patients with chronic HBV infection, and long-
needed. (C.2) (New) term abstinence from alcohol in patients with alcohol-
related liver disease. (A.1) (New)
3.2 The definition and impact of the removal/suppression of the
Varices and screening endoscopy in patients that cannot be primary aetiological factor in other ACLDs is less well
treated with NSSBs established. (A.1) (New)
2.19 Patients with compensated cirrhosis who are not candidates 3.3 Overweight/obesity, diabetes, and alcohol consumption are
for initiating NSBBs (contraindication/intolerance) for the important contributors to liver disease progression even
prevention of decompensation should undergo an endoscopy after removal/suppression of the primary aetiological factor
for variceal screening if LSM by TE is >
−20 kPa or platelet count is and should be addressed. (A.1) (Changed)
<150x109L. (A.1) (New)
− 3.4 Removal/suppression of the primary aetiological factor leads
2.20 Patients avoiding screening endoscopy can be followed up to potentially meaningful decreases in HVPG in most patients
by yearly repetition of TE and platelet count. If LSM in- and substantially reduces the risk of hepatic decompensa-
9 tion. (A.1) (Changed)
creases (>−20 kPa) or platelet count declines (< −150x10 L),
these patients should undergo screening endoscopy (Fig. 1). 3.5 Absence/resolution of CSPH following removal/suppression
(D.1) (Unchanged) of the primary aetiological factor prevents hepatic decom-
pensation. (B.1) (Changed)
3.6 The optimal percent/absolute decrease in HVPG associated
Spleen stiffness with a reduction in hepatic decompensation following the
2.21 Spleen stiffness measurement (SSM) by TE can be used in removal/suppression of the primary aetiological factor in
cACLD due to viral hepatitis (untreated HCV; untreated and patients with cACLD and CSPH has yet to be established.
treated HBV) to rule out and rule in CSPH (SSM <21 kPa and (B.1) (New)
SSM >50 kPa, respectively). Validation of the best cut-off 3.7 In the absence of co-factors, patients with HCV-induced
using a 100 Hz specific TE-probe, as well as using point- cACLD who achieve SVR and show consistent post-
shear wave elastography and 2D-shear wave elastography treatment improvements with LSM values of <12 kPa and
is needed. (B.2) (New) PLT >150x109/L can be discharged from portal hypertension

Liver decompensation and liver-related death
+Plat >150, Baveno VI-avoid endoscopy
+Plat ≥150, exclude CSPH
5 kPa 10 kPa 15 kPa 20 kPa 25 kPa
Normal
Exclude cACLD Assume cACLD
Assume CSPH:
HCV, HBV, ALD
Non-obese NASH
Fig. 1. Algorithm for the non-invasive determination of cACLD and CSPH. ALD, alcohol-related liver disease; cACLD, compensated advanced chronic liver
disease; CSPH, clinically significant portal hypertension; NASH, non-alcoholic steatohepatitis.
surveillance (LSM and endoscopy), as they do not have CSPH long-term data on the risk of hepatic decompensation (and
and are at negligible risk of hepatic decompensation. In these more specifically, variceal bleeding) and its evolution over
patients, HCC surveillance should continue until further data time in patients with cACLD.
is available. (B.1) (New)
3.8 The Baveno VI criteria (i.e., LSM <20 kPa and PLT >150x109/L)
4) Impact of non-aetiological therapies
can be used to rule out high-risk varices in patients with
4.1 The use of statins should be encouraged in patients with
HCV- and HBV-induced cACLD who achieved SVR and viral
cirrhosis and an approved indication for statins since these
suppression, respectively. (B.1) (New)
agents may decrease portal pressure (A.1) and improve
3.9 Patients with cACLD on NSBB therapy with no evident CSPH
overall survival. (B.1) (Changed)
(LSM <25 kPa) after removal/suppression of the primary
4.2 In patients with Child-Pugh B and C cirrhosis, statins should
aetiological factor, should be considered for repeat endos-
be used at a lower dose (simvastatin at max. 20 mg/d) and
copy, preferably after 1–2 years. In the absence of varices,
patients should be followed closely for muscle and liver
NSBB therapy can be discontinued. (C.2) (New)
toxicity.(A.1) In Child-Pugh C cirrhosis the benefit of statins
has not been proven yet and their use should be more
restrictive. (D.1) (Changed)
Research agenda
4.3 The use of aspirin should not be discouraged in patients with
Define the impact of the removal/suppression of primary
cirrhosis and an approved indication for aspirin, since it may
aetiological factors (particularly non-alcoholic fatty liver
reduce the risk of HCC, liver-related complications, and
disease) other than HCV/HBV infection and alcohol-related
death. (B.2) (New)
liver disease in cACLD.
4.4 Long-term albumin administration may reduce complica-
Identify factors responsible for liver disease progression
tions of cirrhosis and improve transplant-free survival in
despite removal/suppression of the primary aetio-
patients with uncomplicated ascites, but a formal recom-
logical factor.
mendation cannot be given until further data become
Establish the optimal percent/absolute decrease in HVPG
available. (B.2) (New)
associated with a reduction in hepatic decompensation
4.5 Short-term albumin administration is indicated for sponta-
following the removal/suppression of the primary aetio-
neous bacterial peritonitis (SBP) (A.1), acute kidney injury
logical factor in patients with cACLD and CSPH.
(AKI) >stage 1A (C.1), large-volume paracentesis (A.1) and
Evaluate the diagnostic ability of NITs for monitoring disease
combined with terlipressin for hepatorenal syndrome (HRS)-
regression and determining the presence of CSPH after
AKI. (B.1) (New)
removal/suppression of a non-viral primary aetio-
4.6 Primary antibiotic prophylaxis is recommended in selected
logical factor.
patients (i.e., gastrointestinal [GI] haemorrhage, Child-Pugh C
Evaluate and validate other non-invasive risk
cirrhosis with low protein ascites) at high risk of SBP.
stratification algorithms (e.g., LSM/VITRO [von Willebrand
(B.1) (New)
factor antigen to platelet ratio] and SSM) in patients in
4.7 Secondary antibiotic prophylaxis is indicated in patients with
whom the primary aetiological factor has been
previous SBP. (A.1) (New)
removed/suppressed.
4.8 Rifaximin is indicated for the secondary prophylaxis of he-
Establish estimates for the regression of varices after removal/
patic encephalopathy. (A.1) (New)
suppression of the primary aetiological factor and collect

Seminar
4.9 Rifaximin should be considered for prophylaxis of overt he- serum ascites albumin gradient [>1.1 g/dl]), overt hepatic
patic encephalopathy in patients with previous overt hepatic encephalopathy (West Haven grade > −II) and variceal
encephalopathy undergoing elective TIPS. (B.2) (New) bleeding. (B.1) (New)
4.10 Rifaximin is not indicated beyond these indications, 5.5 Other relevant liver-related events in compensated cirrhosis
including primary or secondary prophylaxis of SBP. are the development of superimposed liver injury (see
(C.1) (New) statement 5.12)/ACLF and HCC. (B1) (New)
4.11 Anticoagulation should not be discouraged in patients with 5.6 Insufficient data are available regarding whether a minimal
cirrhosis and an approved indication for anticoagulation, amount of ascites only detected in imaging procedures,
since anticoagulation may reduce liver-related outcomes in minimal hepatic encephalopathy, and occult bleeding from
patients with and without portal vein thrombosis (PVT) and portal hypertensive gastroenteropathy (PHG) can be
may improve overall survival. (B.1) (Changed) considered as decompensation. (D.1) (New)
4.12 Direct-acting oral anticoagulants (DOACs) are as safe and 5.7 Limited data suggest that jaundice alone (in non-cholestatic
effective for the prevention of cardiovascular events in pa- aetiologies) may be the first manifestation of cirrhosis in a
tients with Child-Pugh A/B cirrhosis as in those without minority of patients; however, its definition, whether it
cirrhosis (B.2) DOACs are not recommended in patients should be considered true first decompensation or if it re-
with Child-Pugh C cirrhosis outside study protocols. flects superimposed liver injury/ACLF in compensated
(B.2) (New) cirrhosis requires further research. (D.1) (New)
5.8 Non-hepatic comorbidities are frequent in patients with
compensated cirrhosis, can adversely impact prognosis, and
Research agenda should be specifically dealt with. (A.1) (Changed)
The gut microbiome can be targeted by several means 5.9 There is insufficient data to draw definitive conclusions on
including pre-, pro-, syn- and post-biotics, diet, faecal the impact of sarcopenia and frailty on the natural history of
microbiota transplantation, phage therapy, drugs, bio- compensated cirrhosis. (D.1) (New)
engineered bacteria, and antibiotics. Interventional trials 5.10 Bacterial infections are frequent in compensated patients
are needed to assess the functional mechanisms and clinical with CSPH, can lead to decompensation (ascites, variceal
outcomes associated with such therapies. bleeding, hepatic encephalopathy) and, consequently,
The composition of the gut microbiome (e.g., high relative adversely affect natural history. (B.1) (New)
abundance of Enterobacteriaceae) in various body fluids 5.11 There is insufficient data as to whether infections are
(stool, saliva, blood, bile, intestinal mucosa, skin) is associ- frequent in compensated cirrhosis without CSPH and
ated with severity of cirrhosis, complications, and presence whether they may impact prognosis per se. (D.1) (New)
of organ failures and acute-on-chronic liver failure (ACLF). 5.12 Superimposed liver damage, such as (acute) alcoholic hep-
Components of the gut microbiome should be explored for atitis, acute viral hepatitis (HEV, HAV), HBV flares or drug-
biomarkers to inform stage of disease (diagnostic), and to induced liver injury can precipitate decompensation.
predict the risk of progression (prognostic), the likelihood (A.1) (New)
of benefitting from an intervention (predictive) and the 5.13 Other factors such as HCC and major surgery can precipitate
efficacy of an intervention. decompensation of cirrhosis in patients with CSPH.
Faecal microbiota transplant (by enema or by the oral route) (B.1) (New)
seems to be safe in patients with cirrhosis and hepatic en- 5.14 Treatment with NSBBs (propranolol, nadolol or carvedi-
cephalopathy but efficacy studies are pending. lol*) should be considered for the prevention of decom-
Antifibrotic strategies including targeting the farnesoid X re- pensation in patients with CSPH. (B.1) (New) *In contrast
ceptor pathway, the renin-angiotensin system, and angio- with the traditional NSBBs (i.e. propranolol and nadolol),
genesis should be further explored in cirrhosis and carvedilol has intrinsic anti-alpha adrenergic vasodilatory
portal hypertension. effects that contribute to its greater portal pressure
reducing effect.
5.15 Carvedilol is the preferred NSBB in compensated cirrhosis,
5) Prevention of (first) decompensation since it is more effective at reducing HVPG (A.1), has a
5.1 Compensated cirrhosis is defined by the absence of present or tendency towards greater benefit in preventing decom-
past complications of cirrhosis. The transition from pensation and towards better tolerance than traditional
compensated to decompensated cirrhosis leads to an NSBBs and has been demonstrated to improve survival (B.1)
increased mortality risk. (A.1) (New) compared to no active therapy in compensated patients
5.2 Compensated cirrhosis can be divided into 2 stages, based on with CSPH. (Changed)
the absence or presence of CSPH. Patients with CSPH are at 5.16 The decision to treat with NSBBs should be taken when
increased risk of decompensation. The goal of treatment in clinically indicated, independent of the possibility of
compensated cirrhosis is to prevent complications that measuring HVPG. (B.2) (Unchanged)
define decompensation. (A.1) (Changed) 5.17 Patients with compensated cirrhosis who are on NSBBs for
5.3 Prevention of decompensation is especially relevant in the prevention of decompensation do not need a screening
compensated patients with CSPH and/or oesophageal or endoscopy for the detection of varices since endoscopy will
gastric varices due to their higher risk of developing not change management. (B.2) (New)
decompensation. (B.1) (New) 5.18 There is no evidence that endoscopic therapies such as
5.4 The events that define decompensation in a compensated endoscopic band ligation or glue might prevent ascites or
patient are overt ascites (or pleural effusion with increased hepatic encephalopathy. (D.1) (New)

5.19 In compensated patients with high-risk varices who have 6.5 In suspected variceal bleeding, vasoactive drugs (terli-
contraindications or intolerance to NSBBs, endoscopic band pressin, somatostatin, octreotide) should be started as soon
ligation is recommended to prevent first variceal bleeding. as possible and continued for 2-5 days. (A.1) (Changed)
(A.1) (Changed) 6.6 Hyponatremia has been described in patients on terli-
5.20 There is no indication at present to use NSBBs in patients pressin, especially in patients with preserved liver function.
without CSPH. (A.1) (Unchanged) Therefore, sodium levels should be monitored.
5.21 Although a single study suggested that cyanoacrylate in- (B.1) (Unchanged)
jection is more effective than propranolol in preventing first 6.7 Antibiotic prophylaxis is an integral part of therapy for
bleeding in patients with large type 2 gastro-oesophageal patients with cirrhosis presenting with upper gastrointes-
varices or isolated type 1 gastric varices, there were no tinal bleeding and should be instituted from admission.
differences in survival. However, NSBBs are indicated in (A.1) (Unchanged)
these patients to prevent decompensation.(B.1) Further 6.8 The risk of bacterial infection and mortality are very low in
studies are required in these patients using new therapeutic patients with Child-Pugh A cirrhosis, but more prospective
approaches in addition to NSBBs. (D.1) (Changed) studies are still needed to assess whether antibiotic pro-
5.22 There is no indication at present for balloon-occluded phylaxis can be avoided in this subgroup of patients.
retrograde (antegrade) transvenous obliteration (BRTO or (B.2) (Unchanged)
BATO) or TIPS in primary prophylaxis of gastric variceal 6.9 Intravenous ceftriaxone 1 g/24 h should be considered in
bleeding in compensated patients. (D.1) (New) patients with advanced cirrhosis (A.1) in hospital settings
with high prevalence of quinolone-resistant bacterial in-
fections and in patients on previous quinolone prophylaxis,
Research agenda and should always be in accordance with local resistance
Competing risks from comorbidities should be considered in patterns and antimicrobial policies. (D.2) (Changed)
future studies in compensated cirrhosis. 6.10 Malnutrition increases the risk of adverse outcomes in
Determine the impact of early detection and treatment patients with cirrhosis and acute variceal bleeding (AVB) and
of comorbidities. oral nutrition should be started as soon as possible.
Determine the impact of sarcopenia and frailty (and of its (D.2) (New)
treatment) on prognosis and mortality of patients with 6.11 Airway manipulation, including use of a nasogastric tube,
compensated cirrhosis. should be performed with caution because of the risk of
Determine the prognostic significance of the sole presence of pulmonary infection. (D.2) (New)
minimal ascites only detected in imaging procedures, mini- 6.12 Proton pump inhibitors, when started before endoscopy,
mal hepatic encephalopathy, and chronic bleeding from PHG. should be stopped immediately after the procedure unless
Determine the prognostic significance of the sole presence of there is a strict indication to continue them. (D.2) (New)
jaundice in compensated cirrhosis, and its definition. 6.13 Six-week mortality should be the primary endpoint for
Evaluate the role of statins in preventing decompensation. studies on the treatment of AVB. (D.1) (Unchanged)
Determine the impact of sole bacterial infection and non- 6.14 Five-day treatment failure is defined either by absence of
bacterial infections on the natural history of compen- control of bleeding or by rebleeding within the first 5 days.
sated cirrhosis. (D.1) (Changed)
Determine the impact of vaccination (pneumococcal, hae- 6.15 Child-Pugh class C, the updated model for end-stage liver
mophilus, influenza, coronavirus) on the natural history of disease (MELD) score, and failure to achieve primary hae-
compensated cirrhosis. mostasis are the variables most consistently found to pre-
Evaluate the prevention of bacterial infections in patients with dict 6-week mortality. (B.2) (Unchanged)
CSPH and its impact on the incidence of decompensation. 6.16 Child-Pugh and MELD scores are currently the most utilised
Identify factors predicting which infections will give rise to severity scoring systems. (D.2) (Unchanged)
decompensation and/or worsen prognosis. 6.17 Following haemodynamic resuscitation, patients with sus-
pected AVB should undergo upper endoscopy within 12 h of
presentation (B.1). If the patient is unstable, endoscopy
6) Acute variceal bleeding should be performed as soon as safely possible.
6.1 The goal of resuscitation is to preserve tissue perfusion. (D.1) (Changed)
Volume restitution should be initiated to restore and 6.18 The availability of an on-call GI endoscopist proficient in
maintain haemodynamic stability. (D.2) (Unchanged) endoscopic haemostasis and on-call support staff with
6.2 Packed red blood cell transfusions should be performed technical expertise in the usage of endoscopic devices,
conservatively, with a target haemoglobin level between enabling performance of endoscopy on a 24/7 basis, is
7-8 g/dl, although transfusion policy in individual patients recommended. Trainees performing the procedure must
should also consider other factors such as cardiovascular always be closely supervised by the GI endoscopist.
disorders, age, haemodynamic status and ongoing (D.1) (Changed)
bleeding. (A.1) (Unchanged) 6.19 In the absence of contraindications (QT prolongation), pre-
6.3 Intubation is recommended before endoscopy in patients endoscopy infusion of erythromycin (250 mg IV 30-120
with altered consciousness and those actively vomiting minutes before endoscopy) should be considered.
blood. (D.1) (New) (B.1) (Unchanged)
6.4 Extubation should be performed as quickly as possible after 6.20 Patients with AVB should be managed in intensive or in-
endoscopy. (D.2) (New) termediate care units. (D.1) (Unchanged)

Seminar
6.21 Ligation is the recommended form of endoscopic therapy haemostatic status of patients with advanced liver diseases.
for acute oesophageal variceal bleeding. (A.1) (Unchanged) (B.1) (Changed)
6.22 Endoscopic therapy with tissue adhesives (e.g. N-butyl- 6.36 In the AVB episode, transfusion of fresh frozen plasma is not
cyanoacrylate/thrombin) is recommended for acute recommended as it will not correct coagulopathy and may
bleeding from isolated gastric varices (A.1) and type 2 lead to volume overload and worsening of portal hyper-
gastro-oesophageal varices that extend beyond the cardia. tension. (B.1) (New)
(D.2) (Unchanged) 6.37 In the setting of AVB, there is no evidence that platelet
6.23 Endoscopic variceal ligation (EVL) or tissue adhesive can be count and fibrinogen levels are correlated with the risk of
used in bleeding from type 1 gastro-oesophageal varices. failure to control bleeding or rebleeding. However, in case
(D.1) (Unchanged) of failure to control bleeding, the decision to correct the
6.24 Based on current evidence, haemostatic powder cannot be haemostatic abnormalities should be considered on a case-
recommended as first-line endoscopic therapy for AVB. by-case basis. (D.2) (New)
(D.1) (New) 6.38 Recombinant factor VIIa and tranexamic acid are not rec-
6.25 Endoscopic therapy (argon plasma coagulation, radio- ommended in AVB. (A.1) (New)
frequency ablation or band ligation for PHG and gastric 6.39 In patients with AVB who are on anticoagulants, these
antral vascular ectasia) may be used for local treatment of should be temporarily discontinued until the haemorrhage
PHG bleeding. (C.2) (New) is under control. Length of discontinuation should be indi-
6.26 All patients with AVB should undergo abdominal imaging, vidualised based on the strength of the indication for
preferably contrast-enhanced cross-sectional imaging (CT anticoagulation. (D.2) (New)
or MRI) to exclude splanchnic vein thrombosis, HCC and to 6.40 In patients with GOV2, type 1 isolated gastric varices, and
map portosystemic collaterals in order to guide treatment. ectopic varices, BRTO could be considered as an alternative
(D.1) (New) to endoscopic treatment or TIPS, provided it is feasible (type
6.27 Pre-emptive TIPS with polytetrafluoroethylene (PTFE)- and diameter of shunt) and local expertise is available, as it
covered stents within 72 h (ideally <24 h) is indicated has been shown to be safe and effective. (D.2) (New)
in patients bleeding from oesophageal varices and type 1/ 6.41 Either endovascular or endoscopic treatment should be
2 gastro-oesophageal varices who meet any of the considered in patients with ectopic varices. (D.1) (New)
following criteria: Child-Pugh class C <14 points or Child- 6.42 TIPS may be combined with embolisation to control
Pugh class B >7 with active bleeding at initial endoscopy bleeding or to reduce the risk of recurrent variceal bleeding
or HVPG >20 mmHg at the time of haemorrhage. from gastric or ectopic varices, particularly in cases when,
(A.1) (Changed) despite a decrease in portosystemic pressure gradient,
6.28 In patients fulfilling the criteria for pre-emptive TIPS, ACLF, portal flow remains diverted to collaterals. (D.2) (New)
hepatic encephalopathy at admission and hyper- 6.43 In patients with cirrhosis and PVT, management of AVB
bilirubinemia at admission should not be considered con- should be performed according to the guidelines for pa-
traindications. (B.1) (New) tients without PVT, when possible. (D.1) (New)
6.29 In refractory variceal bleeding, balloon tamponade or self-
expandable metal stents (SEMS) should be used as a
bridge therapy to a more definite treatment such as PTFE- Research agenda
covered TIPS. SEMS are as efficacious as balloon tampo- Determine the role of vasoactive drugs and antibiotics in
nade and are a safer option. (B.1) (Changed) Child-Pugh A patients.
6.30 Failure to control variceal bleeding despite combined Identify an optimal shorter time frame limit for vasoactive
pharmacological and endoscopic therapy is best managed drug therapy?
by salvage PTFE-covered TIPS. (B.1) (Changed) Define active bleeding at endoscopy, and assess its subjectivity
6.31 TIPS may be futile in patients with Child-Pugh >−14 cirrhosis, and prognostic value.
or with a MELD score >30 and lactate >12 mmol/L, unless Identify the clinical role of non-invasive markers of por-
liver transplantation is envisioned in the short-term.(B.1) tal pressure.
The decision to perform TIPS in such patients should be Determine the role of haemostatic powder in acute and re-
taken on a case-by-case basis. (D.1) (New) fractory variceal bleeding.
6.32 In patients with AVB and hepatic encephalopathy, bouts of Determine the role of thrombin in gastric variceal bleeding.
hepatic encephalopathy should be treated with lactulose Assess pre-emptive TIPS in patients with gastric varices.
(oral or enemas). (D.1) (New) Define the optimal management of patients not fulfilling the
6.33 In patients presenting with AVB, rapid removal of blood high-risk criteria used for pre-emptive TIPS.
from the gastrointestinal tract (lactulose oral or enemas) Determine the cost-effectiveness of SEMS.
should be used to prevent hepatic encephalopathy. Develop alternatives to Blakemore/Linton as they are in
(B.1) (New) short supply.
6.34 Variceal bleeding is due to portal hypertension, and the aim Determine the role of global haemostasis tests, such as
of the treatment should be focused on lowering portal viscoelastic tests and thrombin generation assays, to assess
pressure rather than correcting coagulation abnormalities. and correct haemostasis abnormalities in decompensated
(B.1) (New) cirrhosis and AVB (using clinical endpoints).
6.35 Conventional coagulation tests, namely, prothrombin time/ Determine the potential role of prothrombin complex con-
international normalised ratio (PT/INR) and activated par- centrates, fibrinogen, or cryoprecipitate in bleeding patients
tial thromboplastin time, do not accurately reflect the with cirrhosis.

Evaluate whether there is any relation between low platelet or mean arterial pressure <65 mmHg) and/or HRS-AKI.(B.1)
count (up to which level?) or fibrinogen and the risk of Once blood pressure returns to baseline and/or HRS-AKI re-
variceal bleeding, failure to control bleeding, or bleeding solves, NSBBs can be re-initiated or re-titrated.(B.1) If a pa-
after endoscopic band ligation. tient remains intolerant to NSBBs, EVL is then recommended
Identify patients that will benefit from variceal embolisation to prevent variceal haemorrhage. (B.1) (Changed)
during TIPS.
Determine the role of endoscopic ultrasound-guided therapy
with tissue adhesive with or without coils. Preventing recurrent variceal haemorrhage
Determine the impact of PVT on the prognosis of cirrhotic (secondary prophylaxis)
patients with AVB. 7.8 First-line therapy for the prevention of recurrent variceal
Identify the optimal duration of vasoactive therapy in cirrhotic haemorrhage is the combination of traditional NSBBs or
patients with PVT and AVB. carvedilol and EVL. (A.1) (Changed)
Determine the role of pre-emptive TIPS in cirrhotic patients 7.9 TIPS is the treatment of choice in patients who rebleed
with PVT presenting with AVB. despite traditional NSBBs or carvedilol and EVL.
Establish the optimal management of AVB in patients with (B.1) (Unchanged)
cirrhosis and PVT, including management of anticoagulation 7.10 In patients who cannot get/tolerate EVL or carvedilol or
and timing of endoscopic/invasive procedures. traditional NSBBs, any of these therapies can be maintained
alone (A1) and TIPS should be considered in patients with
recurrent ascites. (B.1) (Changed)
7) Prevention of further decompensation 7.11 In patients who bleed despite adherence to traditional
Definition of “further decompensation” NSBBs or carvedilol as primary prophylaxis, the combination
7.1 Further decompensation in cirrhosis represents a prognostic of traditional NSBBs or carvedilol and EVL is recommended,
stage associated with an even higher mortality than that and TIPS should be considered in those with recurrent as-
associated with first decompensation. Specific events that cites. (B.1) (New)
define further decompensation are any of the following:
(B.1) (New)
a) Development of a second portal hypertension-driven Preventing recurrent bleeding from PHG
decompensating event (ascites, variceal haemorrhage or 7.12 PHG and portal hypertension-associated gastric or small
hepatic encephalopathy) and/or jaundice; intestinal polypoid lesions have to be distinguished from
b) Development of recurrent variceal bleeding, recurrent gastric antral vascular ectasia because treatments are
ascites (requirement of − >3 large-volume paracenteses different. (B.1) (Changed)
within 1 year), recurrent encephalopathy, development of 7.13 NSBBs are the first-line therapy for preventing recurrent
SBP and/or HRS-AKI; bleeding from PHG. (A.1) (Unchanged)
c) In patients presenting with bleeding alone, development 7.14 Endoscopic therapy (e.g., argon plasma coagulation or
of ascites, encephalopathy, or jaundice after recovery from hemospray) may be used to treat recurrent bleeding from
bleeding but not if these events occur around the time PHG. (D.1) (New)
of bleeding. 7.15 TIPS should be considered for transfusion-dependent PHG
despite traditional NSBBs or carvedilol and endoscopic ther-
apy. (C.1) (Changed)
Preventing further decompensation in patients with ascites
7.2 Patients with decompensated cirrhosis should be considered
for liver transplantation. (A.1) (New) Role of infections in decompensated cirrhosis
7.3 Patients with ascites who are not on traditional NSBBs (i.e., 7.16 Bacterial infections are common in patients with decom-
propranolol or nadolol) or carvedilol should undergo pensated cirrhosis and may cause further decompensation.
screening endoscopy. (B.1) (New) (A.1) (New)
7.4 TIPS should be considered in patients with recurrent ascites 7.17 In all patients hospitalised with decompensation, bacterial
(requirement of > −3 large-volume paracenteses within 1 infections should be ruled out. The minimal work-up for
year) irrespective of the presence or absence of varices or infections should include diagnostic paracentesis, chest X-
history of variceal haemorrhage. (A.1) (New) ray, cultures of blood, ascites and urine, and skin examina-
7.5 In patients with ascites and low-risk varices (small [<5 mm], tion. (A.1) (New)
no red signs, not Child-Pugh C), traditional NSBBs or carve- 7.18 Patients with bacterial infections should be promptly
dilol may be used to prevent first variceal haemorrhage. treated with antibiotics. The empirical antibiotic treatment
(B.2) (Changed) should be tailored to local epidemiology, risk factors for
7.6 In patients with ascites and high-risk varices (large varices multidrug-resistant bacteria and severity of infection.(A.1) If
[>
−5 mm]), or red spot signs, or Child-Pugh C), prevention of no response to antibiotics is observed, consider viral and
first variceal haemorrhage is indicated, with traditional fungal infections. (C.1) (Changed)
NSBBs or carvedilol being preferred over EVL.
(B.1) (Changed)
7.7 In patients with ascites, traditional NSBBs or carvedilol The role of sarcopenia and frailty in further decompensation
should be dose-reduced or discontinued in case of persis- 7.19 Frailty, malnutrition, and sarcopenia have an impact on
tently low blood pressure (systolic blood pressure <90 mmHg survival in patients with decompensated cirrhosis. They

Seminar
should be evaluated with available standardised tools. traditional NSBBs/carvedilol therapy and determine whether
(B.1) (New) dose reduction (vs. discontinuation) is safe.
7.20 All patients with decompensated cirrhosis should receive Determine the impact of NSBB discontinuation on the natural
nutrition consultation and be advised regarding the benefits history of decompensated cirrhosis.
of regular exercise. (B.1) (New) Assess the benefit of carvedilol over traditional NSBBs in sec-
7.21 While sarcopenia improves in some patients after TIPS, pre- ondary prophylaxis of variceal haemorrhage.
procedural sarcopenia has also been associated with poor
outcomes (e.g., encephalopathy, slower resolution of ascites) TIPS and further decompensation
and a higher mortality. Therefore, sarcopenia by itself should Assess the benefit of TIPS for secondary prophylaxis in patients
not be an indication for TIPS. (C.2) (New) with NSBB intolerance/non-response and ascites that do not
meet the strict criteria for recurrent ascites.
Establish whether TIPS placement past the 72 h pre-emptive
Definition of cirrhosis recompensation TIPS window is still beneficial.
7.22 The concept of recompensation implies that there is at Evaluate the haemodynamic and non-haemodynamic effects
least partial regression of the structural and functional of NSBBs in patients after TIPS.
changes of cirrhosis after removal of the aetiology of cirrhosis.
(A.1) (New)
7.23 Clinically, the definition of “recompensation” is based on Sarcopenia, frailty and nutrition and further decompensation
expert consensus and requires fulfilment of all the following Determine the impact of nutritional interventions on the
criteria: (C.2) (New) natural history of decompensation.
a. Removal/suppression/cure of the primary aetiology of Determine the impact of therapies targeting sarcopenia and
cirrhosis (viral elimination for hepatitis C, sustained viral frailty on the natural history of decompensation.
suppression for hepatitis B, sustained alcohol abstinence Define the role of sarcopenia in the selection of patients
for alcohol-induced cirrhosis); for TIPS.
b. Resolution of ascites (off diuretics), encephalopathy (off
lactulose/rifaximin) and absence of recurrent variceal
haemorrhage (for at least 12 months); 8) Splanchnic vein thrombosis
c. Stable improvement of liver function tests (albumin, Aetiological work-up in primary thrombosis of the portal
INR, bilirubin). venous system or hepatic venous outflow tract
7.24 Because CSPH may persist despite recompensation, NSBBs 8.1 For patients with primary thrombosis of the splanchnic veins
should not be discontinued unless CSPH resolves. in the absence of cirrhosis, close collaboration with sub-
(B.1) (New) specialists is recommended for a complete work-up that
7.25 Resolution of ascites (while on diuretics or after TIPS) and/ considers prothrombotic factors and systemic diseases.
or lack of recurrent variceal haemorrhage (while on tradi- (A.1) (Changed)
tional NSBBs + EVL or carvedilol + EVL or after TIPS) without 8.2 Various combinations of risk factors for thrombosis can be
removal/suppression/cure of the primary aetiologic factor present, so that identification of 1 risk factor does not deter
and without improvement in liver synthetic function, is not from a complete work-up. (A.1) (New)
evidence of recompensation. (B.1) (New) 8.3 In all adult patients, myeloproliferative neoplasia (MPN)
should be searched for by testing for the V617F JAK2 muta-
tion in peripheral blood. (A.1) (Unchanged)
Research agenda 8.4 In patients with no detectable JAK2 V617F mutation, consider
Further decompensation and recompensation additional investigations for MPN, including somatic calre-
Investigate the effect of time to further decompensation ticulin and JAK2-exon12 mutations, and next-generation
on prognosis. sequencing. (A.1) (Changed)
Obtain data to support the suggested concept of cirrhosis 8.5 In all adult patients with primary thrombosis of the
recompensation, particularly on the timeframe necessary to splanchnic veins without an MPN driver mutation, bone
consider a patient truly recompensated. marrow biopsy should be discussed in collaboration with
Evaluate the association between recompensation and reso- haematologists to rule out MPN, irrespective of blood cell
lution of CSPH. counts. Bone marrow biopsy should be considered particu-
Determine the impact of aetiological therapy other than larly in patients without major risk factors for thrombosis.
alcohol abstinence and antiviral therapy on recompensation. (B.2) (Changed)
NSBBs and further decompensation

Prospective studies should assess if NSBB treatment prevents Budd-Chiari syndrome – definition
further (non-rebleeding) decompensation in decom- 8.6 Budd-Chiari syndrome (BCS) is the consequence of an
pensated patients. obstruction to the hepatic venous outflow. Obstruction can
Prospective studies should assess if HVPG-guided (traditional be located from the level of the small hepatic veins to the
NSBB/carvedilol) therapy is more efficient to prevent further level of the entrance of the IVC into the right atrium.
decompensation over non-HVPG-guided strategies. (A.1) (Unchanged)
Identify optimal blood pressure cut-offs (mean arterial pres- 8.7 BCS is the preferred designation for any primary hepatic
sure/systolic arterial pressure) to define safe use of venous outflow tract obstruction. (D.1) (New)

8.8 BCS is considered secondary when the mechanism for 8.24 The BCS-TIPS prognostic index score can be used to predict
venous obstruction is an extrinsic compression, for example outcome in patients in whom TIPS insertion is being
by a benign or malignant tumour. BCS is considered primary considered. (B.1) (Changed)
otherwise. (A.1) (Changed) 8.25 Liver transplantation should be considered in patients
with uncontrolled clinical manifestation despite a
stepwise approach, or in patients with a high BCS-TIPS
Budd-Chiari syndrome – diagnosis prognostic index score (>7) before TIPS placement.
8.9 BCS presentation and manifestations are extremely diverse, (C.1) (Changed)
so that the diagnosis must be considered in any patient with 8.26 In patients with BCS presenting as acute liver failure, urgent
acute, acute-on-chronic, or chronic liver disease. liver transplantation should be considered. Emergency TIPS
(A.1) (Changed) should be performed, if possible, independently of listing
8.10 BCS is diagnosed by the demonstration of an obstruction of for liver transplantation. (C.1) (New)
the venous lumen, or by the presence of hepatic vein col-
laterals together with the absence of patent hepatic veins.
(A.1) (Unchanged) PVT and portal cavernoma in the absence of cirrhosis
8.11 Liver biopsy should not be performed to diagnose BCS when – definition
vascular imaging demonstrates obstruction of the hepatic 8.27 PVT is characterised by the presence of a thrombus in the
venous outflow tract. (B.1) (Unchanged) portal vein trunk or its branches. Portal cavernoma is a
8.12 Liver biopsy is necessary to diagnose BCS if obstruction of network of porto-portal collaterals which develops as a
the small hepatic veins is not seen on imaging. consequence of prior portal vein obstruction. (D.1)
(B.1) (Changed) Obstruction leading to cavernoma is mostly related to
8.13 In patients with BCS, hepatic nodules are frequent and most thrombosis in adults, but less likely so in children and
often benign. However, HCC may occur and therefore pa- young adults. (B.1) (Changed)
tients should be monitored with periodic imaging and 8.28 Imaging tools should be used to distinguish PVT from the
alpha-fetoprotein measurements. (B.1) (Changed) extravascular compression of the venous lumen by a
8.14 A 6-month interval can be proposed for periodic imaging. neighbouring space-occupying formation. (D.1) (New)
(C.1) (New) 8.29 Cirrhosis and/or malignancy should be ruled out and other
8.15 It is still unclear which of ultrasound or MRI should be used underlying liver diseases (e.g., PSVD or other chronic liver
for periodical screening. (C.1) (New) disease) should be investigated. (D.1) (Changed)
8.16 Patients developing nodules should be referred to centres
experienced in managing BCS. (D.1) (Unchanged)
8.17 Characterisation of the nodule may first include MRI using PVT and portal cavernoma in the absence of cirrhosis
hepatobiliary contrast agents.(C.1) Biopsy of the lesion is – diagnosis
indicated for a definitive diagnosis of HCC. (C.1) (New) 8.30 For diagnosis of PVT or cavernoma, Doppler ultrasound, CT
or MR angiography should demonstrate solid intraluminal
material not showing enhancement after injection of
Budd-Chiari syndrome – management vascular contrast agents (for PVT) or a network of porto-
8.18 Management of BCS should be undertaken using a stepwise portal collaterals (for cavernoma). (B.1) If diagnosed by
approach including anticoagulation, angioplasty/stent/ Doppler ultrasound, confirmation with contrast-enhanced
thrombectomy/thrombolysis, TIPS and orthotopic liver CT or MR angiography is needed. (D.1) (Changed)
transplantation, at experienced centres. (B.1) (Unchanged) 8.31 A standardised documentation (as proposed in Table 1) of
8.19 Long-term anticoagulation should be given to all patients initial site, extent/degree of luminal obstruction, and chro-
with primary BCS. (B.1) (Changed) nicity of clot formation is required to enable subsequent
8.20 Because of the increased risk of heparin-induced throm- evaluation of the spontaneous course and/or response to
bocytopenia, the use of unfractionated heparin is generally treatment. (D.1) (New)
not recommended and may only be reserved for special 8.32 PVT and portal cavernoma in adults are frequently associ-
situations (e.g., glomerular filtration rate <30 ml/min, ated with > −1 risk factor(s) for thrombosis, which may be
pending invasive procedures). (D.2) (New) occult at presentation and should be investigated.
8.21 Stenoses that are amenable to percutaneous angioplasty/ (B.1) (Unchanged)
stenting (short length stenoses) should be actively looked 8.33 In patients with PVT following abdominal surgery or pancre-
for and treated accordingly. (B.1) (Unchanged) atitis, invasive procedures (e.g., bone marrow biopsy and liver
8.22 TIPS insertion should be attempted by operators with spe- biopsy) should be discussed on an individual basis considering
cific experience in BCS when angioplasty/stenting/throm- the expected low diagnostic yield in such populations and the
bectomy/thrombolysis is not feasible, and when the patient risk of morbidity associated with these procedures.
does not improve on medical therapy including anticoag- (C.2) (New)
ulants. (B.1) (Unchanged) 8.34 If the liver is dysmorphic on imaging or liver tests
8.23 Consider improvement as a combination of several of the are persistently abnormal, liver biopsy and HVPG mea-
following outcomes: decreasing rate of ascites formation, surement are recommended to rule out cirrhosis or
decreasing serum bilirubin, serum creatinine and INR when PSVD.(B.1) Liver stiffness by TE may be useful to exclude
elevated (or increasing factor V in patients receiving cirrhosis although precise cut-offs cannot be proposed yet.
vitamin K antagonists). (D.1) (New) (C.2) (Changed)

Seminar
Table 1. Recommended standardised nomenclature for the description of portal vein thrombosis and portal cavernoma in both the clinical and
research setting.18
Feature Definition
Time course
Recent Portal vein thrombosis presumed to be present for <6
months
Chronic Portal vein thrombosis present or persistent for >6 months
Percent occlusion of main portal vein
Completely occlusive No persistent lumen
Partially occlusive Clot obstructing >50% of original vessel lumen
Minimally occlusive Clot obstructing <50% of original vessel lumen
Cavernous transformation Gross porto-portal collaterals without original PV seen
Response to treatment or interval change
Progressive Thrombus increases in size or progresses to more complete
occlusion
Stable No appreciable change in size or occlusion
Regressive Thrombus decreases in size or degree of occlusion
PVT and portal cavernoma in the absence of cirrhosis the superior mesenteric vein. Therefore, a multidisci-
- management plinary approach with early image-guided intervention,
8.35 In the absence of cirrhosis, recent PVT rarely resolves spon- thrombolysis and surgical intervention should be consid-
taneously. Therefore, at diagnosis, anticoagulation should be ered in referral centres. (C.2) (New)
started immediately at a therapeutic dosage. (B.1) (Changed)
8.36 Because of the increased risk of heparin-induced throm-
bocytopenia, the use of unfractionated heparin is not Past PVT or cavernoma in the absence of cirrhosis
generally recommended and may only be reserved for – management
special situations (e.g. glomerular filtration rate <30 ml/min, 8.45 In patients with past PVT or cavernoma, including those
pending invasive procedures). (D.2) (New) with incomplete resolution of recent PVT at 6 months, long-
8.37 As a primary treatment option for recent PVT in the absence term anticoagulation is recommended in patients with a
of cirrhosis, start with low-molecular-weight heparin permanent underlying prothrombotic state (B.1) and
(LMWH) and switch to vitamin K antagonists when possi- should also be considered in patients without an underlying
ble. (B.1) (Changed) DOACs can be considered the primary prothrombotic state. (B.2) (New)
option in selected cases in the absence of so-called “triple 8.46 No data are available to recommend or discourage anti-
positive” anti-phospholipid syndrome, although data are coagulation in childhood-onset past PVT or cavernoma in the
limited. (C.2) (New) absence of an underlying prothrombotic state. (C.1) (New)
8.47 In patients with past PVT or cavernoma not yet receiving
anticoagulants, anticoagulation should be started after
Recent PVT in the absence of cirrhosis – management adequate prophylaxis for portal hypertensive bleeding has
8.38 Anticoagulation should be given for at least 6 months in all been initiated in patients with high-risk varices.
patients with recent PVT in the absence of cirrhosis. (C.2) (Changed)
(B.1) (Unchanged) 8.48 Mesenteric-left portal vein bypass (Meso-Rex operation)
8.39 After 6 months, long-term anticoagulation is recommended should be considered in all children with complications of
in patients with a permanent underlying prothrombotic portal cavernoma, and these patients should be referred to
state (B.1) and should also be considered in patients centres with experience in treating this condition.
without an underlying prothrombotic state. (B.2) (New) (B.1) (Unchanged)
8.40 If anticoagulation is discontinued, D-dimers <500 ng/ml 1 8.49 Patients with refractory complications of PVT or cavernoma
month after discontinuation may be used to predict a low should be referred to expert centres to consider percuta-
risk of recurrence. (C.2) (New) neous recanalisation of the portal vein or other vascular
8.41 In patients without cirrhosis who do not develop compli- interventional procedures. (C.1) (New)
cations of recent PVT despite the absence of portal vein
recanalisation, interventions other than anticoagulation are
not required. (B.2) (Changed) Treatment of portal hypertension in extrahepatic portal
8.42 A follow-up contrast-enhanced CT scan should be per- vein obstruction
formed 6 months after recent PVT. (C.1) (New) 8.50 There is insufficient data on whether beta-blockers or endo-
8.43 Because of the risk of recurrence of splanchnic vein scopic therapy should be preferred for primary prophylaxis of
thrombosis, patients need to be followed up, irrespective of portal hypertension-related bleeding in patients with past
the discontinuation of anticoagulation. (C.1) (New) PVT or cavernoma. Guidelines for cirrhosis should be applied.
8.44 The risk of intestinal infarction and organ failure is (C.2) (Changed)
increased in patients with recent PVT and (i) persistent 8.51 Oesophageal variceal band ligation can be performed safely
severe abdominal pain despite anticoagulation therapy, without withdrawing vitamin K antagonists. (C.2) (New)
(ii) bloody diarrhoea, (iii) lactic acidosis, (iv) bowel 8.52 All patients in whom thrombosis has not been recanalised
loop distention, or (v) occlusion of second order radicles of should be screened for gastroesophageal varices within 6

months of the acute episode. In the absence of varices, (>50%) thrombosis of the portal vein trunk with or without
endoscopy should be repeated at 12 months and 2 years extension to the superior mesenteric vein, or (ii) symp-
thereafter. (B.1) (Unchanged) tomatic PVT, independently of the extension, or (iii) PVT in
8.53 In patients with acute portal hypertension-related bleeding, potential candidates for liver transplantation, indepen-
recommendations for patients with cirrhosis may be dently of the degree of occlusion and extension. (C.2) (New)
applied. (D.1) (Changed) 9.6 In potential liver transplant candidates, the goal of anti-
8.54 Based on recommendations for cirrhosis, the combination coagulation is to prevent re-thrombosis or progression of
of NSBBs and band ligation is recommended for secondary thrombosis to facilitate adequate portal anastomosis in liver
prophylaxis. (D.1) (New) transplantation and reduce post-transplant morbidity and
mortality. (C.1) (Changed)
9.7 Anticoagulation should be considered in patients with
Research agenda cirrhosis and minimally occlusive (<50%) thrombosis of the
Budd-Chiari syndrome: portal vein trunk that (i) progresses on short-term follow-
Determine risk factors for HCC in patients with BCS. up (1-3 months) or (ii) compromises the superior mesen-
Identify the best approach for the non-invasive diagnosis of teric vein. (C.2) (New)
HCC in patients with BCS. 9.8 Anticoagulation should be (i) maintained until portal vein
Evaluate criteria for short-term (8 days) evolution that pre- recanalisation or for a minimum of 6 months, (ii) continued
dict a good mid/long-term outcome (i.e., criteria for after recanalisation in patients awaiting liver transplantation,
“treatment response”) in patients with BCS. and (iii) considered after recanalisation in all others, while
balancing the benefits of preventing recurrence and
increasing survival with the risk of bleeding. (C.1) (New)
PVT without cirrhosis 9.9 Patients with low platelet count (e.g., <50 x109/L) are at
Identify predictors of development, progression, and sponta- higher risk of PVT, but also of bleeding complications on
neous resolution of PVT. anticoagulation, and should be assessed on a case-by-case
Determine the influence of beta-blockers on the natural his- basis. (C.2) (Changed)
tory of PVT. 9.10 TIPS is recommended in patients with thrombosis of the
Determine the effect of early recanalisation using interven- portal vein trunk without recanalisation on anticoagulation,
tional radiology or TIPS vs. fibrinolytic agents and/or anti- especially in patients listed for liver transplantation.
coagulants in patients with recent PVT. (C.2) (New)
Determine the efficacy of anticoagulation on recanalisation 9.11 Anticoagulation is preferably initiated with LMWH and
and on prevention of progression of PVT in children/young maintained with either LMWH, vitamin K antagonists or
adults with PVT. DOACs. Advantages of LMWH are that its use is based on
Evaluate the pathophysiology and management of cytopenia solid data. Vitamin K antagonists carry challenges with re-
in patients with non-cirrhotic portal hypertension. gard to INR monitoring in patients with cirrhosis. Advan-
tages of DOACs are that they are easier to use but less data
are available. (C.1) (Changed)
9) Other issues in vascular liver disorders 9.12 Currently available data suggest that there are no major
Use of anticoagulants in non-cirrhotic vascular liver diseases safety concerns regarding the use of DOACs in patients with
9.1 LMWH and vitamin K antagonists are widely accepted and Child-Pugh class A cirrhosis. Due to the possibility of accu-
used to treat primary thrombosis of the portal venous system mulation, DOACs should be used with caution in patients
or hepatic venous outflow tract. (A.1) (Unchanged) with Child-Pugh class B cirrhosis, as well as in patients with
9.2 There are no major concerns regarding the safety of DOACs creatinine clearance below 30 ml/min. The use of DOACs in
in patients with non-cirrhotic vascular liver diseases, as those with Child-Pugh class C cirrhosis is not recommended
long as liver function is preserved. DOACs should be used outside study protocols. (B.2) (New)
with caution in patients with impaired liver function 9.13 DOACs likely have different safety-efficacy profiles in pa-
(equivalent to Child-Pugh class B), as well as in patients tients with cirrhosis, although at the moment no recom-
with creatinine clearance below 30 ml/min. The use of mendation can be made in favour of a specific DOAC in this
DOACs in patients with severe liver dysfunction (equivalent setting. (D.2) (New)
to Child-Pugh C) is not recommended outside study pro-
tocols. (C.2) (New)
Porto-sinusoidal vascular disorder
Anticoagulation and PVT in cirrhosis 9.14 PSVD is a broad clinico-pathological entity encompassing
9.3 Screening for PVT is recommended in all patients who are non-cirrhotic portal fibrosis, idiopathic portal hypertension or
potential liver transplant candidates, at the time of non-cirrhotic intrahepatic portal hypertension, and various
screening for HCC. (D.2) (Changed) overlapping histological patterns including nodular regener-
9.4 Occurrence of PVT in the presence of HCC does not directly ative hyperplasia, obliterative portal venopathy, hepatoportal
imply vascular malignant invasion, but further imaging is sclerosis, incomplete septal cirrhosis. (B.1) (New)
recommended (CT scan and/or MRI and/or contrast- 9.15 The absence of portal hypertension does not rule out PSVD.
enhanced ultrasound). (D.2) (Changed) The presence of common causes of liver disease (e.g., viral
9.5 Anticoagulation is recommended in patients with cirrhosis hepatitis, excessive alcohol consumption, metabolic syn-
and (i) recent (<6 months) completely or partially occlusive drome, etc.) does not rule out PSVD, and both can coexist.

Seminar
The presence of PVT does not rule out PSVD, and both can Management according to cirrhosis guidelines is recom-
coexist. (B.1) (New) mended. (D.2) (New)
9.16 PSVD should be considered in the following situations: (i) 9.25 A contrast-enhanced CT scan is suggested at diagnosis of
signs of portal hypertension contrasting with atypical fea- PSVD in order to assess the anatomy/patency of the portal
tures of cirrhosis (e.g., HVPG <10 mmHg; liver stiffness venous system and potential portosystemic collaterals.
measurement <10 kPa; smooth liver surface and no atrophy (D.2) (New)
of segment IV; hepatic vein-to-vein communications; 9.26 Screening for PVT in patients with PSVD: there is no data on
although none of these features is considered pathogno- the best screening method and interval.(D.2) (New)
monic for PSVD); or (ii) liver blood test abnormalities or Doppler ultrasound every 6 months is suggested in patients
portal hypertension in a patient with a condition known to with PSVD and features of portal hypertension.(C.1) (New)
be associated with PSVD (Table S1); or (iii) unexplained liver In case of abdominal pain, Doppler ultrasound or cross-
blood test abnormalities even without signs of portal hy- sectional imaging should be performed to rule out
pertension. (B.1) (New) splanchnic vein thrombosis. (B.1) (New)
9.27 No recommendation can be made regarding anticoagulation
therapy to prevent the development of PVT in PSVD.
Diagnosis of PSVD (D.2) (New)
9.17 PSVD can be observed in the absence of clinical, laboratory 9.28 In those patients developing PVT, anticoagulant therapy
or imaging features of portal hypertension. (B.1) (New) should be started according to recommendations for non-
9.18 A liver biopsy specimen of adequate size (>20 mm) and of cirrhotic PVT. (C.1) (New)
minimal fragmentation – or otherwise considered adequate 9.29 TIPS can be considered to treat severe complications of
for interpretation by an expert pathologist – is required for portal hypertension. Underlying/associated conditions,
the diagnosis of PSVD. (C.1) (New). which negatively impact post-TIPS outcome, must be taken
9.19 Diagnosis of PSVD requires the exclusion of cirrhosis and of into account in making individual decisions regarding TIPS
other causes of portal hypertension (B.1), together with 1 of insertion. (C.2) (New)
the following 3 criteria (C.2): (i) at least 1 feature specific for 9.30 Liver transplantation is an option in selected patients
portal hypertension; or (ii) at least 1 histologic lesion spe- with PSVD and severe or refractory complications of
cific for PSVD; or (iii) at least 1 feature not specific for portal portal hypertension or with advanced liver dysfunction.
hypertension together with at least 1 histologic lesion Indications should be discussed in expert centres.
compatible although not specific for PSVD (Table 2). (New) (D.2) (New)
Research agenda
Management of PSVD Anticoagulation in PVT in cirrhosis
9.20 Once the diagnosis of PSVD is made, patients should be Assess the safety and efficacy of each DOAC in patients
screened for associated immunological diseases, pro- with cirrhosis.
thrombotic or genetic disorders and exposure to drugs/ Identify indicators associated with a favourable outcome in
toxins (Table S1). (D.2) (New) patients with cirrhosis and PVT treated with anticoagulants.
9.21 Endoscopic screening for gastro-oesophageal varices is Develop stopping rules for long-term anticoagulant treatment
required at diagnosis of PSVD. (C.1) (New) in patients with cirrhosis and PVT.
9.22 The non-invasive Baveno VII criteria for screening of oeso- Evaluate the advantages and disadvantages of prophylactic vs.
phageal varices used in patients with cirrhosis cannot be full dose anticoagulation in patients with cirrhosis and PVT.
applied to patients with PSVD. (B.1) (New) Define response to treatment in patients with cirrhosis
9.23 During follow-up, the frequency of endoscopic screening for and PVT.
varices has not yet been defined. Management according to
cirrhosis guidelines is recommended, except for stopping
rules. (D.2) (New) PSVD
9.24 There is insufficient data on which therapy should be Evaluate the natural history of PSVD without por-
preferred for portal hypertension prophylaxis in PSVD. tal hypertension.
Table 2. Criteria in the definition of porto-sinusoidal vascular disorder (adapted from19).

Feature of portal hypertension Histological lesions suggestive of porto-sinusoidal vascular disorder
assessed by an expert pathologist
Specific Gastric, oesophageal, or ectopic varices Obliterative portal venopathy (thickening of vessel wall, occlusion of
Portal hypertensive bleeding the lumen, vanishing of portal veins)
Portosystemic collaterals at imaging Nodular regenerative hyperplasia
Incomplete septal fibrosis (also called incomplete septal cirrhosis);
this latter feature can only be assessed on liver explants and not on
liver biopsies
Not specific Ascites Portal tract abnormalities (multiplication, dilatation of arteries, per-
Platelet count <150,000/mm3 iportal vascular channels, aberrant vessels)
Spleen size >
−13 cm in the largest axis Architectural disturbance: irregular distribution of the portal tracts
and central veins
Non-zonal sinusoidal dilatation
Mild perisinusoidal fibrosis

Develop improved non-invasive methods to screen for PSVD Scientific Societies: EASL (European Association for the Study of
(e.g., cross-sectional imaging, SSM). the Liver), b) national scientific societies: AASLD (American As-
Evaluate the prophylaxis for PVT in patients with PSVD and sociation for the Study of Liver Disease); AEEH (Spanish Associ-
signs of portal hypertension. ation for the Study of the Liver), AFEF (French Association for the
Evaluate the incidence and predictors of development of PVT Study of the Liver), AIGO (Italian Association of Hospital Gas-
in patients with PSVD and assess the efficacy of anti- troenterologists and Endoscopists); AISF (Italian Association for
coagulation in this setting. the Study of the Liver); CIBERehd (Spanish network of biomed-
ical investigation in liver and digestive diseases), ÖGGH (Austrian
Society for Gastroenterology and Hepatology), SASL (Swiss As-
Other issues sociation for the Study of the Liver), SIGE ( Italian Society of
Besides the supporting data and consensus recommendations for Gastroenterology), c) International Research Groups; Decision,
the 9 Baveno sessions, 7 lectures were given at Baveno VII. The Galaxy, Microb-Predict. JB is supported by the Stiftung für Leb-
topics of these lectures were: ‘New concepts of risk stratifica- erkrankheiten, Bern (Switzerland).
tion’, ‘Clinical stages and ordinal outcomes in portal hyperten-
sion’, ‘Lifestyle and genetic modifiers of liver disease Conflict of interest
progression’, Parenchymal extinction lesions (PELS) in progres- None relating to this manuscript. JB has been a consultant for Zydus,
sion. Can they regress?’, ’Fibrogenesis and regression of fibrosis’, Surrozen and Actelion. TR is a consultant for and/or receives
’Angiogenesis and progression of advanced chronic liver disease research support from Abbvie, Bayer, Boehringer-Ingelheim,
(ACLD)’, and ’Drugs to modify liver fibrosis progression and Gilead, Intercept, MSD, Philips, Pliant Pharmaceuticals, Roche,
regression’. The 9 Baveno sessions and the 7 lectures will be and Siemens. RdF, CR and GGT have no disclosures to report.
summarised in the Baveno VII proceedings book.17 The Baveno Please refer to the accompanying ICMJE disclosure forms for
VII consensus workshop was followed by a paediatric satellite further details.
meeting entitled ‘Primary prophylaxis of variceal haemorrhage,
complexities in the development of evidence-based approaches Authors’ contributions
in paediatrics’. The whole Baveno Faculty contributed to the development of the
Readers interested in examining the evolution of the recom- consensus statements. Roberto de Franchis drafted the text of the
mendation on cirrhosis and portal hypertension can refer to the paper together with Jaime Bosch, Guadalupe Garcia-Tsao,
Baveno I-VI reports.2–4,7–10,12–14 Thomas Reiberger and Cristina Ripoll.
Use of the definitions and adherence to the recommendations
in future studies is encouraged to provide further validation. The Baveno VII Faculty
topics listed in the research agenda reflect the opinions of the The following were members of the Baveno VI Scienti-
experts about the areas where new information is most needed. fic Committee:
Roberto de Franchis [Milan, Italy (Honorary President)], Jaime
Abbreviations Bosch [Bern, Switzerland (Chair)] Guadalupe Garcia-Tsao [West
ACLF, acute-on-chronic liver failure; AKI, acute kidney injury; Haven, USA (Vice-Chair)], Thomas Reiberger [Vienna, Austria
AVB, acute variceal bleeding; BATO, balloon-occluded antegrade (Scientific Secretary)], Cristina Ripoll [Jena, Germany (Scientific
transvenous obliteration; BCS, Budd-Chiari syndrome; BRTO, Secretary)], Juan G Abraldes (Edmonton, Canada), Agustin Albil-
balloon-occluded retrograde transvenous obliteration; cACLD, los (Madrid, Spain), Annalisa Berzigotti (Bern, Switzerland),
compensated advanced chronic liver disease; CSPH, clinically Gennaro D’Amico (Palermo, Italy), Andrea De Gottardi (Lugano,
significant portal hypertension; DOAC, direct-acting oral antico- Switzerland), Alessandra Dell’Era (Milan, Italy), Juan Carlos Gar-
agulants; EVL, endoscopic variceal ligation; HCC, hepatocellular cia-Pagàn (Barcelona, Spain), Joan Genescà (Barcelona, Spain),
carcinoma; HRS, hepatorenal syndrome; HVPG, hepatic vein Aleksander Krag (Odense, Denmark), Wim Laleman (Leuven,
pressure gradient; ICU, intensive care unit; INR, international Belgium), Vincenzo La Mura (Milan, Italy), Dominique Thabut
normalised ratio; IVC, inferior vena cava; LMWH, low-molecular- (Paris, France), Jonel Trebicka (Frankfurt, Germany), Emmanouil
weight heparin; LSM, liver stiffness measurement; MELD, model Tsochatzis (London, UK), Dominique Valla (Paris, France), Candid
for end-stage liver disease; MPN, myeloproliferative neoplasm; Villanueva (Barcelona Spain).
MR, magnetic resonance; NASH, non-alcoholic steatohepatitis; The following chaired sessions or lectures:
NIT(s), non-invasive test(s); NSBB(s), non-selective beta Agustin Albillos (Madrid, Spain), Annalisa Berzigotti (Bern,
blocker(s); PHG, portal hypertensive gastropathy; PPG, portal Switzerland), Jaime Bosch (Barcelona, Spain), Roberto de Fran-
pressure gradient; PSVD, porto-sinusoidal vascular disorder; chis (Milan, Italy), Andrea De Gottardi (Lugano, Switzerland),
PTFE, polytetrafluoroethylene; PVT, portal vein thrombosis; SBP, Hector Ferral (Evanston, USA),Juan Carlos Garcia-Pagàn (Barce-
spontaneous bacterial peritonitis; SEMS, self-expanding metal lona, Spain), Guadalupe Garcia-Tsao (West Haven, USA), Joan
stent; SSM, spleen stiffness measurement; SVR, sustained viro- Genescà (Barcelona, Spain), Virginia Hernandez-Gea (Barcelona,
logical response; TE, transient elastography; TIPS, transjugular Spain), Wim Laleman (Leuven, Belgium), Mattias Mandorfer
intrahepatic portosystemic shunt; VNT, varices needing treat- (Vienna, Austria), David Patch (London, UK), Pierre Emmanuel
ment; WHVP, wedged hepatic venous pressure. Rautou (Paris, France), Thomas Reiberger (Vienna, Austria),
Cristina Ripoll (Jena, Germany), Shiv K Sarin (New Delhi, India),
Financial support Dominique Thabut (Paris, France), Jonel Trebicka (Frankfurt,
The Baveno VII Consensus workshop was endorsed and sup- Germany), Emmanouil Tsochatzis (London, UK), Dominique Valla
ported with unrestricted grants by the following: a) International (Paris, France).

Seminar
The following participated in the presentations and the dis- (Cluj-Napoca, Romania), Marika Rudler (Paris, France) ,Filippo
cussions as panellists in the consensus sessions: Schepis, (Modena, Italy), Marco Senzolo (Padua, Italy), Akash
Anna Baiges (Barcelona, Spain), Jasmohan Bajaj (Richmond, Shukla (Mumbai, India), Puneeta Tandon (Edmonton, Canada),
USA), Rafael Bañares (Madrid, Spain), Marta Barrufet (Barcelona, Luis Tellez (Madrid, Spain), Maja Thiele (Odense, Denmark),
Spain), Lina Benajiba (Paris, France), Christophe Bureau (Tou- Dhiraj Tripathi (Birmingham, UK), Laura Turco (Bologna, Italy),
louse, France), Vincenza Calvaruso (Palermo, Italy), Andres Car- Fanny Turon (Barcelona, Spain), Candid Villanueva (Barcelona
denas (Barcelona, Spain), Alessandra Dell’Era (Milan, Italy), Spain), Hitoshi Yoshiji (Nara, Japan).
Angels Escorsell (Barcelona, Spain), Jonathan Fallowfield (Edin- The following gave review lectures:
burgh, UK), Sven Francque (Antwerp, Belgium), Ron Gaba (Chi- Juan G Abraldes (Edmonton, Canada), Annalisa Berzigotti
cago, USA), Susana Gomes Rodrigues (Bern, Switzerland), (Bern, Switzerland), Gennaro D’Amico (Palermo, Italy), Jordi
Guogong Han (Xi’an, China), Jidong Jia (Beijing, China), Jean Gracia-Sancho (Barcelona, Spain), Massimo Pinzani (London,
Jacques Kiladjian (Paris, France), Aleksander Krag (Odense, UK), Vijay Shah (Rochester, USA), Ian Wanless (Halifax, Canada).
Denmark), Vincenzo La Mura (Milan, Italy), Sabela Lens (Barce-
lona, Spain), Xuefeng Luo (Chengdu, China), Sarwa Darwish Supplementary data
Murad (Rotterdam, The Netherlands), Valerie Paradis (Clichy, Supplementary data to this article can be found online at https://
France), Salvatore Piano (Padua, Italy), Aurelie Plessier (Clichy, doi.org/10.1016/j.jhep.2021.12.022.
France), Massimo Primignani (Milan, Italy), Bogdan Procopet
References [11] Garcia-Tsao G, Bosch J, Groszmann R. Portal hypertension and variceal

[1] Burroughs AK, editor. Methodology and review of clinical trials in portal bleeding, unresolved issues. Summary of an American Association for the
hypertension. Amsterdam, New York, Oxford: Excerpta Medical Congress study of liver disease and of the European Association for the Study of the
Service N 763; 1987. Liver single-topic conference. Hepatology 2008;47:1764–1772.
[2] de Franchis R, Pascal JP, Ancona E, Burroughs AK, Henderson JM, Fleig W, [12] de Franchis R, on behalf of the Baveno V Faculty. Revising consensus in
et al. Definitions, methodology and therapeutic strategies in portal hy- portal hypertension: report of the Baveno V consensus workshop on
pertension. A consensus development workshop. J Hepatol methodology of diagnosis and therapy in portal hypertension. J Hepatol
1992;15:256–261. 2010;53:762–768.
[3] de Franchis R. Developing consensus in portal hypertension. J Hepatol [13] de Franchis R, editor. Portal Hypertension V. Proceedings of the Vth
1996;25:390–394. Baveno international consensus workshop. Oxford, UK: Wiley-Black-
[4] de Franchis R, editor. Portal Hypertension II. Proceedings of the second well; 2011.
Baveno international consensus workshop on definitions, methodology [14] de Franchis R, on behalf of the Baveno VI Faculty. Expanding consensus in
and therapeutic strategies. Oxford: Blackwell Science; 1996. portal hypertension: report of the Baveno VI consensus workshop:
[5] Spina GP, Arcidiacono R, Bosch J, Pagliaro L, Burroughs AK, stratifying risk and individualizing care for portal hypertension. J Hepatol
Santambrogio R, et al. Gastric endoscopic features in portal hypertension: 2015;63:743–752.
final report of a consensus conference. J Hepatol 1994;21:461–467. [15] de Franchis R, editor. Portal Hypertension VI. Proceedings of the VIth
[6] Grace ND, Groszmann RJ, Garcia-Tsao G, Burroughs AK, Pagliaro L, Baveno consensus workshop: stratifying risk and individualizing care.
Makuch RW, et al. Portal hypertension and variceal bleeding: an AASLD New York, USA: Springer; 2016.
single topic symposium. Hepatology 1998;28:868–880. [16] GRADE working group. Grading quality of evidence and strength of rec-
[7] de Franchis R. Updating consensus in portal hypertension: report of ommendations. BMJ 2004;328:1490–1497.
the Baveno III consensus workshop on definitions, methodology and thera- [17] de Franchis R, editor. Portal Hypertension VII. Proceedings of the VIIth
peutic strategies in portal hypertension. J Hepatol 2000;33:846–852. Baveno consensus workshop: Personalized care in portal hypertension.
[8] de Franchis R, editor. Portal Hypertension III. Proceedings of the IIIrd New York, USA: Springer; 2022.
Baveno International Consensus Workshop on Definitions, Methodology [18] Northup PG, Garcia-Pagan JC, Garcia-Tsao G, Intagliata NM, Superina RA,
and Therapeutic Strategies. Oxford, UK: Blackwell Science; 2001. Roberts LN, et al. Vascular liver disorders, portal vein thrombosis, and
[9] de Franchis R. Evolving consensus in portal hypertension. Report of the procedural bleeding in patients with liver disease: 2020 practice guidance
Baveno IV consensus workshop on methodology of diagnosis and therapy by the American association for the study of liver diseases. Hepatology
in portal hypertension. J Hepatol 2005;43:167–176. 2021;73:366–413.
[10] de Franchis R, editor. Portal Hypertension IV. Proceedings of the IVth [19] De Gottardi A, Rautou PE, Schouten J, Rubbia-Brandt L, Leebeek F, Trebicka J,
Baveno international consensus workshop on methodology of diagnosis VALDIG group. Porto-sinusoidal vascular disease: proposal and description
and treatment. Oxford, UK: Blackwell publishing; 2006. of a novel entity. Lancet Gastroenterol Hepatol 2019;4:399–411.

Baveno 7

Uploaded by

Copyright:

Available Formats

Baveno 7

Uploaded by

Document Information

Original Title

Copyright

Available Formats

Share this document

Share or Embed Document

Sharing Options

Did you find this document useful?

Is this content inappropriate?

Copyright:

Available Formats

Baveno 7

Uploaded by

Copyright:

Available Formats

Seminar

Baveno VII – Renewing consensus in portal hypertension

Journal of Hepatology 2022 vol. 76 j 959–974

960 Journal of Hepatology 2022 vol. 76 j 959–974

2) Non-invasive tools for cACLD and portal

Journal of Hepatology 2022 vol. 76 j 959–974 961

962 Journal of Hepatology 2022 vol. 76 j 959–974

+Plat >150, Baveno VI-avoid endoscopy

+Plat ≥150, exclude CSPH

5 kPa 10 kPa 15 kPa 20 kPa 25 kPa

Exclude cACLD Assume cACLD

Journal of Hepatology 2022 vol. 76 j 959–974 963

964 Journal of Hepatology 2022 vol. 76 j 959–974

Journal of Hepatology 2022 vol. 76 j 959–974 965

966 Journal of Hepatology 2022 vol. 76 j 959–974

Journal of Hepatology 2022 vol. 76 j 959–974 967

NSBBs and further decompensation

968 Journal of Hepatology 2022 vol. 76 j 959–974

Journal of Hepatology 2022 vol. 76 j 959–974 969

970 Journal of Hepatology 2022 vol. 76 j 959–974

Journal of Hepatology 2022 vol. 76 j 959–974 971

Table 2. Criteria in the deﬁnition of porto-sinusoidal vascular disorder (adapted from19).

972 Journal of Hepatology 2022 vol. 76 j 959–974

Journal of Hepatology 2022 vol. 76 j 959–974 973

References [11] Garcia-Tsao G, Bosch J, Groszmann R. Portal hypertension and variceal

974 Journal of Hepatology 2022 vol. 76 j 959–974

You might also like