From the Guidelines
For patients with arteriovenous malformation (AVM) associated liver dysfunction, a conservative management approach with close monitoring and supportive care is recommended, as invasive treatments are associated with significant morbidity and mortality. The primary goal is to manage symptoms and prevent complications, rather than attempting to cure the AVM. This approach is supported by the EASL clinical practice guidelines, which suggest that intensive medical treatment should be the initial approach for symptomatic liver VMs, with invasive treatments considered only for patients who fail to respond to medical therapy 1.
Key Recommendations
- Regular monitoring of liver function tests (LFTs) should be performed every 1-2 weeks initially, then adjusted based on trends.
- Avoid hepatotoxic medications and alcohol to prevent further liver damage.
- If LFTs are significantly elevated (e.g., ALT/AST >3x upper limit of normal), consider temporary discontinuation of any potentially hepatotoxic medications.
- Ursodeoxycholic acid at 13-15 mg/kg/day in divided doses may be prescribed to improve bile flow and potentially reduce liver enzyme elevations.
- In severe cases, consultation with a hepatologist is advised.
Invasive Treatments
Invasive treatments, such as transarterial embolization of liver VMs or orthotopic liver transplantation (OLT), are associated with significant risks and should be considered only for patients who fail to respond to intensive medical therapy. Transarterial embolization is a palliative, temporizing, and risky procedure, with a significant morbidity and 10% fatal complications 1. OLT is the only definitive curative option for hepatic VMs in HHT, but it is associated with a post-operative mortality of 7-10% and should be considered only for patients with intractable high-output cardiac failure, portal hypertension, or ischemic bile duct necrosis 1.
Special Considerations
- Right heart catheterization should always be performed in patients with HHT being evaluated for OLT, to exclude severe pulmonary hypertension.
- Bevacizumab, an angiogenesis inhibitor, has shown some promise in reducing the cardiac index in patients with severe liver VMs, but its use is associated with significant risks and should be carefully weighed 1.
From the FDA Drug Label
Ursodiol therapy has not been associated with liver damage. Abnormalities in liver enzymes have not been associated with Ursodiol therapy and, in fact, Ursodiol has been shown to decrease liver enzyme levels in liver disease.
The FDA drug label does not provide information on the association between Arteriovenous Malformation (AVM) and liver dysfunction, or the use of ursodeoxycholic acid (PO) in patients with AVM-associated liver dysfunction. 2
From the Research
Arteriovenous Malformation (AVM) Associated Liver Dysfunction
There are no research papers to assist in answering this question as the provided studies do not directly address the topic of Arteriovenous Malformation (AVM) associated liver dysfunction.
Liver Dysfunction in Various Conditions
However, the studies provide information on liver dysfunction in various conditions, including:
- Liver transplant recipients, where deranged liver function tests (LFTs) can range from mild and asymptomatic to severe and symptomatic elevations requiring expedited personalized assessment and management 3
- Statin use, where dose-dependent borderline elevations of liver function tests can occur over time, although these elevations are clinically and statistically insignificant 4
- COVID-19 infection, where LFT abnormalities are common and derangement increases as severity progresses, with the presence of deranged LFTs worsening clinical outcome and predicting in-hospital mortality 5
- Home parenteral nutrition, where the prevalence of deranged LFTs is 39%, with raised alkaline phosphatase being the most common abnormality, and length of small bowel of less than 100 cm being a significant independent variable for deranged LFTs 6
- Acute heart failure, where abnormal LFTs are associated with impaired clinical outcomes, with the combined elevation of ≥2 LFT panels at discharge being associated with long-term adverse outcomes 7
Key Findings
Key findings from the studies include:
- Deranged LFTs can be caused by various factors, including liver transplant, statin use, COVID-19 infection, home parenteral nutrition, and acute heart failure
- The pattern and severity of LFT derangement can vary depending on the underlying condition
- Abnormal LFTs can be associated with adverse outcomes, including mortality and morbidity
- Early evaluation and management of deranged LFTs are crucial to prevent long-term complications.