Vascular Liver Disorders, Portal Vein Thrombosis, and Procedural Bleeding in Patients with Liver Disease

Patient Guideline Summary

Publication Date: November 19, 2020

Last Updated: March 3, 2023

Objective

This patient summary means to discuss key recommendations from the American Association for the Study of Liver Diseases (AASLD) for vascular liver disorders, portal vein thrombosis, and procedural bleeding in patients with liver disease.

Overview

This patient summary focuses on the management of blood vessel problems associated with the liver.

Preprocedural Interventions

Once the bleeding disorder is accurately diagnosed, blood factors and vitamin K can reduce the risk of bleeding during the procedure.

During surgery, surgical technique and blood transfusion further reduce the risk.

Portal Vein Thrombosis (PVT)

Diagnosis

In patients with PVT without cirrhosis, a full investigation for blood diseases or another causative condition is warranted, usually in consultation with a hematologist.

In patients with cirrhosis, appropriate contrast-enhanced imaging studies are mandatory to rule out liver cancer as the cause.

In liver transplant (LT) recipients, the presence of PVT at the time of transplant is associated with increased post-transplant mortality.

Treatment

Treatment decisions should be on a case-by-case basis.

In all patients with recent PVT and concern for intestinal ischemia (inadequate blood supply), immediate consultation with surgery, critical care, interventional radiology, and hematology is advised. Anticoagulation is essential, with the need for surgery in cases of intestinal infarction (tissue death due to interrupted blood flow).

In patients with recent PVT without cirrhosis, directed antithrombotic therapy should be considered in order to avoid intestinal ischemia and prevent the development of chronic PVT with portal hypertension (PHT).

Local or systemic thrombolytic therapy (treatment to dissolve blood clots) should be considered only in very selected cases of recent PVT in whom intestinal ischemia persists despite anticoagulation.

Surgical repair should be considered prior to transplant and for otherwise intractable bleeding or ascites. Such decisions are made as part of a multidisciplinary management process, including surgical and interventional radiology expertise.

Hepatic Vein Thrombosis: Budd-Chiari Syndrome (HVT/BCS)

HVT/BCS should be suspected in any patient with liver disease (recent or chronic) of unknown etiology. Suspicion should be even higher in a patient with a recognized prothrombotic (favors blood clotting) disorder.

Doppler ultrasound, performed by an experienced operator, is the first-line imaging study for HVT/BCS. MRI or CT can be used for diagnosis confirmation and interventional planning.

At the time of HVT/BCS diagnosis, a full thrombophilia (tendency to form blood clots) workup is recommended. Even when one causal factor is identified, additional factors should be investigated. Consultation with a hematologist is recommended.

Surveillance for hepatocellular cancer (HCC) in patients with chronic HVT/BCS is recommended as in the general cirrhosis population, with ultrasound every 6 months with or without alpha-fetoprotein (a cancer marker) determination.

A progressive “step-up” therapeutic strategy according to the clinical response from less to more invasive therapies is recommended for HVT/BCS. Early referral to tertiary care centers with expertise in this disorder is recommended.

All patients with HVT/BCS, even in the absence of a recognized prothrombotic disorder, should receive therapeutic anticoagulation.

Surgical shunting and liver transplantation are the treatments of choice for otherwise intractable HVT/BCS.

Sinusoidal Obstruction Syndrome (SOS)

SOS should be considered in patients in the appropriate clinical setting, typically 1-3 weeks after hematopoietic stem-cell transplantation (HSCT), with the onset of right upper quadrant pain, weight gain attributable to edema, hepatomegaly (enlarged liver), and ascites.

Ursodeoxycholic acid is recommended as prophylactic therapy for SOS in all patients undergoing allogeneic HSCT.

Defibrotide is the only FDA-approved treatment for SOS and is recommended for the treatment of moderate-to-severe SOS. The benefit of prophylaxis in high-risk cases is not established.

Liver Vascular Malformations (LVMs) and Hereditary Hemorrhagic Telangiectasia (LHHT)

LVMs associated with hereditary hemorrhagic telangiectasia (HHT) should be suspected in the presence of a liver bruit (noise produced by blood flow) and a CT scan or MRI with an enlarged hepatic artery and heterogeneous enhancement of the liver, particularly in the setting of a family or personal history of recurrent nosebleeds, heart failure, unspecified liver disease, or brain hemorrhage.

Asymptomatic LVMs do not warrant therapy or imaging surveillance.

Management of symptomatic LVMs consists of standard therapy of specific complications, including heart failure, portal hypertension, and biliary ischemia.

Symptomatic patients should be managed at a specialized center with a multidisciplinary team. In this setting, consideration for the use of bevacizumab and/or LT is warranted in non-responders to standard therapy.

Idiopathic Noncirrhotic Portal Hypertension (INCPH)

INCPH should be considered in any patient with evidence of portal hypertension but without cirrhosis or other known causes of non-cirrhotic portal hypertension.

A liver biopsy is required to exclude cirrhosis.

In patients with INCPH, underlying risk factors for venous thrombosis, immune disorders, and inherited disorders known to be associated with this condition should be routinely considered.

Hepatic Artery Aneurysms (HAAs) and Splenic Artery Aneurysms (SAAs)

Doppler ultrasound or CT scan should be used for the detection of HAAs or SAAs and multidetector CT angiography for characterization and treatment planning.

If an intervention is deemed necessary for patients with HAAs or SAAs, endovascular repair should be considered first using a multidisciplinary approach, and, if inappropriate, open repair.

For recently diagnosed HAAs or SAAs of less than 2 cm in size, early follow-up imaging (e.g., 3 and 12 months) should be performed to assess the growth rate. Any significant growth of an aneurysm on serial imaging should prompt consideration of intervention in consultation with other specialists.

Urgent intervention should be considered for symptomatic or complicated HAAs or SAAs.

In patients with pregnancy plans or LT candidates, elective interventions in patients with HAAs or SAAs should be considered. In other patients, the intervention has not been proven superior to expectant management.

Children

In children with congenital or acquired vascular diseases of the liver, early referral to centers with expertise in pediatric liver disease, hepatobiliary surgery, and LT is recommended.

In children with extrahepatic portal vein obstruction (EHPVO), evaluation for early intervention in the presymptomatic stage is recommended at a center with expertise in treating this disorder.

Abbreviations

6-MP: 6-mercaptopurine
ALD: Alcohol-Associated Liver Disease
ALFP: Acute Fatty Liver (ALF) Of Pregnancy
CT: Computed Tomography
EHPVO: Extrahepatic Portal Vein Obstruction
ERCP: Endoscopic Retrograde Cholangiopancreatography
GEV: Gastroesophageal Varices
HAAs:
Hepatic Artery Aneurysms
HCAS: Hepatocellular Adenomas
HCC: Hepatocellular Cancer
HELLP: Hemolysis, Elevated Liver Enzymes, Low Platelets Syndrome
HHT: Hereditary Hemorrhagic Telangiectasia
HSCT: Hematopoietic Stem Cell Transplantation
HVT/BCS: Hepatic Vein Thrombosis: Budd-Chiari Syndrome
ICP: Intrahepatic Cholestasis
INCPH: Idiopathic Noncirrhotic Portal Hypertension
LT: Liver Transplant
LVMs: Liver Vascular Malformations
MFM: Maternal-fetal Medicine
MPA: Mycophenolic Acid
MRCP: Magnetic Resonance Cholangiopancreatography
MRI: Magnetic Resonance Imaging
MTCT: Mother-to-child Transmission
NAFLD: Non-alcoholic Fatty Liver Disease
PBC: Primary Biliary Cholangitis
PHT: Portal Hypertension
PSC: Primary Sclerosing Cholangitis
PSC: Primary Sclerosing Cholangitis
PVT: Portal Vein Thrombosis
SAAs: Splenic Artery Aneuryms
SOS: Sinusoidal Obstruction Syndrome
US: Ultrasound
WD: Wilson Disease

Source Citation

Northup PG, Garcia-Pagan JC, Garcia-Tsao G, Intagliata NM, Superina RA, Roberts LN, Lisman T, Valla DC. Vascular Liver Disorders, Portal Vein Thrombosis, and Procedural Bleeding in Patients With Liver Disease: 2020 Practice Guidance by the American Association for the Study of Liver Diseases. Hepatology. 2021 Jan;73(1):366-413. doi: 10.1002/hep.31646. Epub 2021 Jan 20. PMID: 33219529.

Disclaimer

The information in this patient summary should not be used as a substitute for professional medical care or advice. Contact a health care provider if you have questions about your health.