Portal Hypertension VII: Proceedings of the 7th Baveno Consensus Workshop: Personalized Care in Portal Hypertension

Preface
Thomas D. Boyer, MD (1943–2018)—A Tribute
Memories of Prof Andrew K. Burroughs, MD (1953–2014)
Tribute to Roberto J. Groszmann
Tribute to Prof Luigi Pagliaro (1931–2020)
Contents
Contributors
Part I: Introductory Lectures
	1: Introduction: Baveno I to Baveno VII … and Beyond
		Baveno I to VI
			Attendance at the Baveno Workshops
			Publications Derived from the Baveno Workshops
			Impact of the Baveno Consensus on the Medical Literature
			Validation of the Baveno Definitions and Recommendations
			Beyond Baveno VII, the Baveno Cooperation
		The Baveno I–VII Workshops Were a Concerted Effort of the Following
			Speakers and Chairpersons
		References
	2: New Concepts in Risk Stratification
		Introduction
		Risk Prediction and Probabilistic Thinking
		Diagnosis as a Risk Prediction Problem
		Decision Thresholds and Risk Stratification
		Risk Prediction and the Interpretation of Treatment Effects in Randomized Controlled Trials
		Conclusion
		References
	3: Clinical Stages and Ordinal Outcomes in Portal Hypertension
		Background
		Definition and Incidence of Decompensation
		Clinical Stages of Cirrhosis
		Ordinal Outcomes
		Examples of Application of an Ordinal Outcome in Compensated Cirrhosis
		Ordinal Outcomes for Randomized Clinical Trials in Cirrhosis
		Sample Size Estimation with Ordinal Compared to Binary Outcomes
		Using Ordinal Outcomes in Portal Hypertension
		Conclusions
		References
	4: Lifestyle and Genetic Modifiers of Liver Disease Progression
		Lifestyle Modifiers of Liver Disease Progression
			Unhealthy Lifestyle
				Alcohol
				Obesity
				Malnutrition, Sarcopenia, and Frailty
				Cigarette Smoke
			Healthy Lifestyle—Protective Factors
				Physical Activity and Exercise
				Coffee Consumption and Mediterranean Diet
		Genetic Modifiers of Progression of cACLD
			Individual Genetic Variants
				PNPA3 and HSD17B13
				SERPINA1/Alpha-1 Antitrypsin Deficiency
				NOD2
				NR1H4/FXR
			Conclusions and Outlook
		References
Part II: HVPG as a Gold Standard
	5: HVPG as a Gold Standard: Accuracy Is Essential
		Procedure Technique
		Pressure Measurements and Data Recording
		Technical Aspects
		Diagnosis of Clinically Significant Portal Hypertension (CSPH) and Prediction of Main Outcomes in Patients with Different Etiologies of Cirrhosis
		Variceal Hemorrhage
		Hepatocellular Carcinoma (HCC)
		Survival
		Assessment of HVPG in Patients Receiving NSBBs for Prevention of Variceal Hemorrhage and Decompensation
		HVPG Predicts Risk of Decompensation and Mortality after Hepatic and Non-hepatic Surgery
			Patients with Cirrhosis and HCC: Candidates for Hepatic Resection
			Patients with Cirrhosis Who Undergo Extrahepatic Surgery
		PPG in the Setting of Tips
			PPG Measurement
			Anatomic Location for PPG Measurement
			Optimal PPG Threshold for Portal Hypertensive Bleeding/Ascites
				PPG Thresholds in Overshunting Adverse Events
		References
	6: HVPG as a Gold Standard: Consensus Statements of Panel 1
		Descriptions of HVPG Measurement
		Diagnosis of CSPH in Patients with Cirrhosis
		Inclusion of HVPG Assessment in Trial Design
		Assessment of Surgical Risks
		Portal Pressure Gradient (PPG) in the Setting of TIPS
		Research Agenda
Part III: Noninvasive Tools for cACLD and Portal Hypertension
	7: Results of the Baveno VII Questionnaire on the Use of Noninvasive Tools for cACLD and Portal Hypertension
		Introduction
			Concept and Cutoffs of cACLD
			Use of NITs to Diagnose CSPH
			Triage of Patients for Screening Endoscopy
			Use of NITs to Monitor cACLD
			Use of Spleen Stiffness
			Emerging NITs in the Above-Mentioned Settings
	8: Compensated Advanced Chronic Liver Disease (cACLD)
		From Baveno VI to Baveno VII
		From Histological Staging to a Noninvasive Clinical Staging of Chronic Liver Disease
		Excluding cACLD (LSM <10 kPa)
		Detecting Highly Suggestive cACLD (LSM >15 kPa)
		Suggestive cACLD (Grey Zone/LSM ≥10 to <15 kPa)
		Other Elastography Techniques
		Summary
		References
	9: Noninvasive Detection of Clinically Significant Portal Hypertension with Liver Elastography
		From Baveno VI to Baveno VII
		Ruling in CSPH
		Ruling Out CSPH
		Other Elastographic Techniques
		Summary
		References
	10: Varices and Screening Endoscopy
		The Baveno VI Criteria
		Validation of Baveno VI Criteria
		Impact of Etiology on the Performance of Baveno VI Criteria
		Can Baveno VI Criteria Be Expanded?
		Other Elastography Methods and Baveno VI Criteria
		Methods Beyond Baveno VI and Expanded Baveno VI Criteria
		Summary and Conclusions
		References
	11: Liver Elastography for Prognostication and Monitoring Patients With Compensated Advanced Chronic Liver Disease
		Introduction
		Liver Stiffness by Transient Elastography as a Prognostic Tool
		How to Monitor Patients with Chronic Liver Disease Using Liver Stiffness
		The Clinical Relevance of Changes in Liver Stiffness in Patients with Chronic Liver Disease
		References
	12: Spleen Stiffness
		Rationale for the Use of Spleen Stiffness for Portal Hypertension
			SSM Using Transient Elastography
			SSM Using Other Ultrasound Elastography Methods
			SSM Using Magnetic Resonance Elastography
			SSM for the Prediction of Liver-Related Events, Mortality, and Response to Therapy
		Summary
		References
	13: Emerging Non-invasive Markers: Imaging, Blood, and Liver Clearance Tests
		Emerging Non-invasive Methods as a Surrogate for HVPG Measurement
			Imaging Markers
				Contrast-Enhanced Ultrasound
				Computed Tomography
				Magnetic Resonance Imaging
					Haemodynamic Measures
					Structural and Architectural Changes
					Dynamic Contrast-Enhanced MRI (DCE-MRI)
					Combination MRI Markers
					Magnetic Resonance Elastography (MRE)
			Serum Markers
		Non-invasive Methods for Assessment of HVPG Response
		Conclusion
		References
	14: Noninvasive Surrogates for cACLD, CSPH, Varices: Consensus Statements of Panel 2
		Definition of Compensated Advanced Chronic Liver Disease (cACLD)
		Criteria to Identify cACLD
		Outcome and Prognosis
		How to Monitor?
		Diagnosis of CSPH in Patients with cACLD
		Varices and Screening Endoscopy in Patients That cannot Be Treated With NSSB
		Spleen Stiffness
		Research Agenda
Part IV: New Scenarios 1: Introductory Lectures—Progression and Regression of Cirrhosis
	15: Progression and Regression of Cirrhosis: The Histologic Perspective
		Introduction
		Pathology of Cirrhosis—The Main Histologic Elements
		Pathogenesis of Cirrhosis—The Vascular Theory
		Hepatic Fibrosis—A New Classification
		Regression
		Discussion
		References
	16: Liver Fibrosis and Its Regression in the Context of Portal Hypertension
		Introduction
		Etiology-Driven Fibrosis and Cirrhosis: A Key for Understanding Cirrhotic Portal Hypertension
		Is Fibrosis Regression Different in Different CLDs?
		References
	17: Angiogenesis and Progression of ACLD
		Sinusoidal Architecture and Functional Components
			Liver Vascular Structure
			The Sinusoid Microenvironment
			Sinusoidal Endothelial Cell Functions
			Hepatic Stellate Cell Functions
		Relationship of Angiogenesis and Angiocrine Signaling During Liver Health and Regeneration
			Overview of Angiogenesis: Friend or Foe?
			Modulators of Angiogenesis and Regeneration: Angiocrine Signals from Sinusoidal Endothelial Cells
			Relationship of Sinusoidal Endothelial Cells and Hepatic Stellate Cells during Angiogenesis and Regeneration
		Endothelial Cell Dysfunction: Implications for ACLD and Portal Hypertension
			Decrease Vasodilators
			Increased Vasoconstrictors
			Pathogenic Shift in the Sinusoid Microenvironment
		Pathological Angiogenesis and Ancillary Pathogenic Modalities
			Driver of Pathological Angiogenesis: Hepatic Stellate Cell Activation
			Driver of Pathological Angiogenesis: Hypoxia
			Driver of Pathological Angiogenesis: Inflammation
		Clinical Manifestations and Measures of ACLD
			Portal Hypertension
			Transient Elastography
			Magnetic Resonance Elastography
		Conclusion
		References
	18: Drugs to Modify Liver Fibrosis Progression and Regression
		Introduction
		Basics of Liver Fibrosis Progression and Regression
		Antifibrotic Drugs Targeting HSCs
			Activation of HSC Pathways
			Inhibition of HSC Pathways
		Antifibrotic Dugs Targeting the ECM
			Collagen Synthesis Inhibition
			Targeting MMPs and TIMPs
			Inhibition of Collagen Cross-Linking
			Inhibiting Collagen Contraction
		Antifibrotic Drugs Targeting Inflammation and Oxidative Stress
			Anti-Inflammatory Strategies
			Antioxidants
			Anticoagulants
			Apoptosis Signal-Regulating Kinase 1 (ASK1) Inhibitor
			Pan-Caspase Inhibitor
			Cholesterol-Lowering Drugs
			Type-2 Diabetes Drugs
		New Strategies: Antifibrotic Cell Therapy
		References
Part V: New Scenarios 2: Management of ACLD after Removal of the Primary Etiological Factor
	19: Therapies for Alcohol-Related Liver Disease and for Non-Alcoholic Fatty Liver Disease
		Alcohol-Related Liver Disease
			Natural History
			Impact of Alcohol Abstinence on the Natural History of ALD
			Management of Patients with ALD who Fail to Maintain Abstinence from Alcohol
			Potential Impact of Novel Pharmacological Therapies for ALD Currently in Clinical Development
			Conclusions and Future Perspectives
		Non-Alcoholic Fatty Liver Disease
			Natural History
			Hepatic Decompensation in Patients with Compensated NAFLD-ACLD
				Prognosis of Decompensated NAFLD-ACLD
			Specifics of PHT in NAFLD
			Potential Impact of Therapies for NAFLD
				Weight Loss in NAFLD
				Histology and Clinical Events as Endpoints in Clinical Trials
				HVPG
				Varices
				Hepatic Decompensation in Patients With Compensated NASH-ACLD
				Outcomes in Trials for Decompensated NAFLD-ACLD
			Management of NAFLD Patients With ACLD Who Achieve Weight Loss/on Pharmacological Therapies
				Monitoring the Evolution of Liver Disease in Patients Who Achieved Weight Loss/on Pharmacological Therapies
					Invasive Methods
					Non-Invasive Methods
				Screening for Varices in Patients Who Achieved Weight Loss/on Pharmacological Therapies
				General Considerations
		References
	20: Management of ACLD After HBV-Suppression and HCV-Cure
		Hepatitis B
			Impact of HBV-Suppression on the Course of ACLD
			Monitoring the Evolution of Liver Disease in Patients With HBV-Suppression
			Screening for Varices and Risk Stratification in Patients With HBV-Suppression
		Hepatitis C
			Impact of HCV-Eradication on the Course of ACLD
			Co-Factors Modifying the Course of ACLD After HCV-Eradication and Adjunctive Therapies
			Pooled Analysis on the Evolution of PH and NIT After HCV-Eradication
			Screening for Varices and Risk Stratification After HCV-Eradication
			Implications for Patient Management After HCV-Eradication
		References
	21: Management of ACLD After Removal/Suppression of the Etiological Factor: Consensus Statements of Panel 3
		Research Agenda
Part VI: New Scenarios 3: Impact of Non-etiological Novel Therapies in the Course of Cirrhosis
	22: Results of the Baveno VII Questionnaire on the “Impact of Non-etiological Therapies in the Course of Cirrhosis”
		Use of Statins
		Use of Anticoagulants
		Addressing the Gut-Liver Axis
	23: Statins in Compensated and Decompensated Cirrhosis: Approaching the Bedside
		General Underlying Mechanisms of Statins
		Statins in Cardiovascular Diseases and Interaction with Liver Disease
		Adverse Effects and Hepatotoxicity
		Distinct and Common Mechanisms of Statins in Liver Diseases
		Summary/Conclusion
		References
	24: Anticoagulation for Portal Vein Thrombosis in Cirrhosis: An Evidence-Based Approach to When and How
		Prevalence and Natural History
		Impact of PVT in Cirrhosis
		Anticoagulation for PVT in Cirrhosis
			Goals and Efficacy
			Anticoagulation Duration
			Type of Anticoagulation
			Safety of Anticoagulation
		References
	25: Novel Approaches and Disease Modifiers to Alter the Course of Cirrhotic Portal Hypertension
		Introduction
		Future Developments: Non-etiological-Disease Modifiers
			Targeting Regression and Progression of Cirrhosis: Basic Science and Preclinical Insights in Regard to Liver Fibrosis and Angiogenesis
			Targeting the Active Dynamic Component of PHT: Basic Science and Preclinical Insights
			Multi-Targeted Intervention Translated to the Patient Level: Clinical Insights–Ready for Prime Time?
				Statins
				Albumin
				Aspirin
				Anticoagulation
				Rifaximin
				Faecal Microbiota Transplantation (FMT) and Gut Liver Axis
		Expanding the Horizon in Portal Pressure Measurement: EUS-Guided Portal Pressure Gradient (EUS-PPG) Measurement
		References
	26: Targeting the Gut Microbiome in Cirrhosis
		Introduction
		Methods to Study the Microbiome
		Approaches to Target the Microbiome
		Gut Microbiome in Cirrhosis
		References
	27: Impact of Non-etiological Novel Therapies in the Course of Cirrhosis: Consensus Statements of Panel 4
		Research Agenda
Part VII: Clinical Settings 1: Preventing (First) Decompensation
	28: Prevention of First Decompensation: Questionnaire
		Introduction
		Defining Consensus
		The Questionnaire
		Analysis of Responses
			Definition of Clinical Decompensation
			Prevention of the First Decompensation of Cirrhosis
			Use of HVPG Monitoring
		Summary
		References
	29: Definition of First Decompensation in Cirrhosis
		Introduction
		Definition of First Decompensation in Compensated Cirrhosis
			Inclusion of Subclinical (or Not Clinically Detectable) Forms of the Major Complications of Cirrhosis
			Jaundice
			Complications That Require Classical Decompensation (Ascites) by Definition
			Complications That Can Follow Other Pathophysiological Pathways
		New Entities in the Natural History of Cirrhosis
		Concluding Remarks
		References
	30: Evaluation of the Impact of the Sole Presence of Infection (Without Accompanying Decompensation) in the Natural History of Compensated Cirrhosis
		Introduction
		The Role of Systemic Inflammation in Compensated Cirrhosis
			Pathophysiological Background: Systemic Inflammation
			Systemic Inflammation in Compensated Cirrhosis
		The Impact of Bacterial Infections (BI) on Compensated Cirrhosis
			Pathophysiological Background of BI in Compensated Cirrhosis
			Epidemiology of BI in Compensated Cirrhosis
			Definitions of Severity of BI
			Location of BI and Type of Microorganism in Compensated Cirrhosis
			Factors Associated With BI in Compensated Cirrhosis
			Impact of BI on the Natural History of Compensated Cirrhosis
			Criticism to the Available Literature and Further Directions
		References
	31: Evaluation of the Role of Jaundice in the Definition of Decompensation in the Compensated Patients
		References
	32: Evaluation of the Role of Minimal Perihepatic Ascites, Minimal Hepatic Encephalopathy, and Bleeding Due to Portal Hypertensive Gastroenteropathy in the Definition of Decompensation
		Introduction
		Minimal Hepatic Encephalopathy
		Minimal Perihepatic Ascites
		Bleeding from Portal Hypertensive Gastroenteropathy
		References
	33: Evaluation of the Role of Sarcopenia in the Definition of Decompensation of the Compensated Patient
		Introduction
		Pathophysiological Background: Potential Role of Sarcopenia as a Driver of Decompensation
		Modalities to Evaluate Muscle Mass and Frailty in Patients with Cirrhosis
		Sarcopenia in Compensated Liver Disease
			Sarcopenia in Chronic Liver Disease
			Sarcopenia in Compensated Cirrhosis
		Frailty in Compensated Liver Disease
		Criticism to the Available Literature and Further Directions
		References
	34: β-Blockers to Prevent Decompensation of Cirrhosis in Compensated Patients With Clinically Significant Portal Hypertension
		Progression of Portal Hypertension in Compensated Cirrhosis and Risk of Decompensation
		Patients With Compensated Cirrhosis and CSPH: An Ideal Target Population for NSBBs
		The Hemodynamic Effects of NSBBs Influence Clinical Outcomes
		NSBBs to Prevent Decompensation in Patents With Compensated Cirrhosis
		Carvedilol, the β-Blocker of Choice Iin Patients With Compensated Cirrhosis
		Conclusions
		References
	35: Evaluation of the Effect of CSPH, Reduction of HVPG, and Other Factors Predicting the First Decompensation in Cirrhosis
		Stages of Cirrhosis and Clinically Significant Portal Hypertension
		Hepatic Venous Pressure Gradient in Predicting Decompensation
			Baseline HVPG as a Marker of Risk of Decompensation
			HVPG Response as a Marker of Risk of Decompensation
		The Role of Other Factors in Predicting Decompensation
		Conclusions
		References
	36: Preventing (First) Decompensation: Consensus Statements of Panel 5
		Research Agenda
Part VIII: Clinical Settings 2: Acute Variceal Bleeding
	37: General Management of Acute Variceal Bleeding
		Orotracheal Intubation in AVB
		Pharmacological Treatment
		Bacterial Infections
		Nutrition
		Use of Proton Pump Inhibitors (PPI) in AVB
		References
	38: Risk Stratification and Prognostic Factors in Variceal Bleeding
		2021
		Conclusion
		References
	39: Endoscopic Management: Classic and New Therapies
		Endoscopic Therapy for Acute Esophageal Variceal Bleeding
		Esophageal Varices: Endoscopic Band Ligation
		Gastric Varices
			Endoscopic Approach to GOV-1: Band or Glue
			Endoscopic Approach to GOV-2 and IGV
			Endoscopic Ultrasound (EUS)-Guided Injection Therapies
		Summary
		References
	40: Preemptive TIPS (p-TIPS)
		References
	41: Management of Refractory Variceal Bleeding
		Introduction
		Definition of Refractory Bleeding (Unchanged from Baveno VI)
		Bridge Treatment: Balloon Tamponade or Self-Expandable Metal Stents (SEMS)
		Salvage TIPS
			Which Class of Stents Should We Use: Covered/Uncovered?
			Is Salvage TIPS a Futile Procedure for Patients in the Most Severe Conditions?
			Embolization During Salvage TIPS Placement
			Is There Any Therapeutic Alternative in Case of Rebleeding After Salvage TIPS?
			What Place for Balloon-Occluded Retrograde Transvenous Obliteration (BRTO) in Case of Refractory Bleeding?
		Conclusion
		References
	42: Hepatic Encephalopathy and Acute Variceal Bleeding
		Prevalence and Prognosis of HE in Cirrhotic Patients with Gastrointestinal Bleeding
		Treatment and Prevention of HE in Patients with Gastrointestinal Bleeding
			Treatment of HE
			Prevention of HE
		The Question of TIPS in the Setting of Gastrointestinal Bleeding in Cirrhotic Patients with HE or History of HE
			Is HE a Contra-Indication to TIPS?
				Salvage TIPS
				Pre-Emptive TIPS
				TIPS in Case of Failure of Secondary Prophylaxis
			Which TIPS Should Be Used?
			Treatment of HE after TIPS
		Conclusion
		References
	43: Management of Coagulation in Acute Variceal Bleeding
		Coagulation in Cirrhosis
		Assessment of Hemostasis in Cirrhosis
		Correction of Coagulation Parameters in Acute Variceal Bleeding
			Fresh Frozen Plasma
			Platelet’s Transfusion
			Fibrinogen
			Recombinant Activated Factor VII
			Tranexamic Acid
		Conclusion
		References
	44: Gastric Varices and Ectopic Varices
		Introduction
		Hemodynamic Features of GV
		Management of Acute Gastric Variceal Bleeding
		Ectopic Varices
		Summary
		References
	45: Special Settings: Acute Variceal Bleeding and Portal Vein Thrombosis in Cirrhosis
		Introduction
		The Impact of PVT on the Prognosis of Patients with Cirrhosis and AVB
		Management of AVB in Patients with Cirrhosis and PVT
		The Anticoagulation Therapy in Patients with AVB and PVT
		Conclusion
		References
	46: Clinical Settings 2: Acute Variceal Bleeding—Consensus Statements of Panel 6
		Research Agenda
Part IX: Clinical Settings 3: Preventing Further Decompensation
	47: Concept of Further Decompensation and Recompensation
		Further Decompensation
			Pathophysiology of Decompensation and Further Decompensation
		Definition of Decompensation and Further Decompensation
			Outcomes After Presentation with Ascites
			Outcomes After Presentation With Variceal Bleeding
		Recompensation
		References
	48: Prevention of Variceal Bleeding and Rebleeding
		Introduction
		Prevention of First Variceal Bleeding in Patients With Ascites
		Prevention of Recurrent Variceal Bleeding
		Type of NSBB Recommended in the Prevention of Variceal Bleeding/Rebleeding
		Safety of NSBB/Carvedilol in Patients With Ascites
		Patients with Intolerance or Failure of NSBB
		Role of TIPS
			Primary Prophylaxis of Variceal Bleeding in Decompensated Patients
			Secondary Prophylaxis of Variceal Bleeding and Prevention of Further Decompensation
		Prevention of Bleeding/Rebleeding: Is it Time for a Personalized Approach?
		Summary and Conclusions
		References
	49: Prevention of Further Decompensation in Patients With Ascites
		Ascites and Further Decompensation After Ascites as the Index Decompensating Event
		Classification of Ascites
		Changing Paradigm in Treating Ascites: From Ascites Control to Prevention of Further Decompensation
			Ascites Control by Diuretics and Paracentesis
			Etiologic Treatment
			Betablocker Therapy to Ameliorate PH
			Antibiotics
			TIPS
			Albumin
		Conclusions
		References
	50: The Impact of Sarcopenia, Frailty, and Malnutrition on Further Decompensation
		Introduction
		Definitions and Assessment of Sarcopenia, Frailty, and Malnutrition
		Pathophysiology of Frailty, Sarcopenia, and Malnutrition in Cirrhosis and Cirrhosis Progression
		Sarcopenia, Frailty, and Mortality
		Sarcopenia, Frailty, and Further Decompensation
		Sarcopenia and Transjugular Intrahepatic Portosystemic Shunts (TIPS)
		Effects of Treatment of Sarcopenia, Frailty, Malnutrition on Further Decompensation
		Future Directions
		References
	51: Preventing Further Decompensation: Consensus Statements of Panel 7
		Definition of “Further Decompensation”
		Preventing Further Decompensation in Patients with Ascites
		Preventing Recurrent Variceal Hemorrhage (Secondary Prophylaxis)
		Preventing Recurrent Bleeding from Portal Hypertensive Gastropathy (PHG)
		Role of Infections in Decompensated Cirrhosis
		The Role of Sarcopenia and Frailty in Further Decompensation
		Definition of Cirrhosis Recompensation
		Research Agenda
			Further Decompensation and re-Compensation
			NSBB and Further Decompensation
			TIPS and Further Decompensation
			Sarcopenia, Frailty and Nutrition, and Further Decompensation
Part X: Vascular Liver Disorders 1: Splanchnic Vein Thrombosis
	52: Portal Vein Thrombosis: Anticoagulation Vs. Interventional Radiology
		Introduction
		Diagnosis
		Determination of the Presence/Absence of Cirrhosis
		Etiology
		Acute Portal Vein Thrombosis
			Management
				Anticoagulation
				Thrombolysis and Interventional Vascular Procedures
		Chronic Portal Vein Thrombosis
			Management
				Portal Hypertension Complications
				Anticoagulation
				Portal Vein Recanalization
		References
	53: Staging of Portal Vein Thrombosis: Recurrent Thrombosis and Prognostic Factors for Recurrence in Non-Cirrhotic Non-Tumoral Portal Vein Thrombosis (PVT)
		Introduction
		Staging/Classification of Portal Vein Thrombosis
		Recurrent Thrombosis and Prognostic Factors for Recurrence in Non-Cirrhotic Non-Tumoral PVT
			Recurrent Deep Vein Thrombosis (DVT)
			Recurrence of Non-Cirrhotic PVT
		Conclusion
		References
	54: Myeloproliferative Neoplasms and Splanchnic Vein Thrombosis
		Introduction
		Should we Still Use the Good Old Diagnostic Tools?
		Molecular Markers
		MPN Diagnosis in the Context of SVT
		Treatment of MPN in the Context of SVT
		Conclusion
		References
	55: Splanchnic Vein Thrombosis: Consensus Statements of Panel 8
		Aetiological Work-Up in Primary Thrombosis of the Portal Venous System or Hepatic Venous Outflow Tract
		Budd–Chiari Syndrome—Definition
		Budd–Chiari Syndrome—Diagnosis
		Budd–Chiari Syndrome—Management
		Portal Vein Thrombosis and Portal Cavernoma in the Absence of Cirrhosis—Definition
		Portal Vein Thrombosis and Portal Cavernoma in the Absence of Cirrhosis—Diagnosis
		Portal Vein Thrombosis and Portal Cavernoma in the Absence of Cirrhosis—Management
		Recent Portal Vein Thrombosis in the Absence of Cirrhosis—Management
		Past Portal Vein Thrombosis or Cavernoma in the Absence of Cirrhosis—Management
		Treatment of Portal Hypertension in EHPVO
		Research Agenda
			Budd–Chiari Syndrome
			Portal Vein Thrombosis Without Cirrhosis
Part XI: Vascular Liver Disorders 2: Other Issues in Vascular Liver Disorders
	56: Porto-Sinusoidal Vascular Disorder
		Introduction
		Definition
		Epidemiology
		Associated Conditions
			Drug Exposure
			Immunological Disorders
			Thrombophilia
			Infections
			Hereditary and Genetic Disorders
		Clinical Presentation
			PSVD With Portal Hypertension
		PSVD Without Portal Hypertension
		Histopathological Findings
		Imaging
		Elastography
		Hepatic Venous Pressure Gradient Measurement and Hepatic Venography
		Additional Considerations
			Focal Liver Lesions
			Pregnancy
			Non-Hepatic Abdominal Surgery
		Management
			Anticoagulation
			Treatment of Portal Hypertension
		Current and Future Perspectives in Translational and Clinical Research
			Translational
			Clinical
		Conclusions
		References
	57: Anticoagulation in Splanchnic Vein Thrombosis With and Without Underlying Liver Disease
		Introduction
		Budd–Chiari Syndrome
		Portal Vein Thrombosis in Absence of Cirrhosis
			Recent Portal Vein Thrombosis
			Extrahepatic Portal Vein Obstruction (Chronic Portal Vein Thrombosis)
		Portal Vein Thrombosis in Cirrhosis
		References
	58: Medical Approach to Fontan Patients
		Introduction
		Pathophysiology and Natural History
		Natural History of Fontan-Associated Liver Disease
		Diagnosis and Staging of Fontan-Associated Liver Disease
			Liver Biopsy
			Liver Function Tests and Serological Biomarkers
			Abdominal Imaging
			Elastography
			Hepatic Hemodynamics
		Hepatic Complications of Fontan-Associated Liver Disease
			Ascites
			Esophageal Varices
			Hepatic Encephalopathy
			Hepatic Nodules and Hepatocellular Carcinoma
		Medical Management of Fontan-Associated Liver Disease
			Strategies of Prevention and Amelioration of Liver Damage
			Risk Stratification of Advanced FALD and Screening Strategies
		Heart and Liver Transplantation
		References
	59: Other Issues in Vascular Liver Disorders: Consensus Statements of Panel 9
		Use of Anticoagulants in Non-Cirrhotic Vascular Liver Diseases
		Anticoagulation and Portal Vein Thrombosis (PVT) in Cirrhosis
		Porto-Sinusoidal Vascular Disorder (PSVD)
		Diagnosis of PSVD
		Management of PSVD
		Research Agenda
			Anticoagulation in PVT in Cirrhosis
			PSVD
		Reference