What is the recommended management for a patient with Hereditary Hemorrhagic Telangiectasia (HHT) and decreased liver attenuation on computed tomography (CT) with contrast?

Management of HHT with Decreased Liver Attenuation on CT

For patients with HHT and decreased liver attenuation on contrast CT, no treatment is recommended if asymptomatic; only monitoring is needed since liver synthetic function remains well preserved and only 8% develop symptoms. 1, 2

Understanding the Finding

Decreased liver attenuation on contrast CT represents hepatic vascular malformations, which are extremely common in HHT:

• 67-74% of HHT patients have liver involvement on multislice CT, making this an expected finding rather than an alarming one 1, 2
• The vast majority (92%) remain asymptomatic throughout their lives 1, 2
• Liver synthetic function is generally well preserved despite the vascular abnormalities 1

Initial Assessment Algorithm

Step 1: Determine Symptom Status

Asymptomatic patients:

• No treatment is indicated 1, 2
• Continue routine monitoring without invasive interventions 2
• Liver synthetic function testing to confirm preserved hepatic function 1

Symptomatic patients require complication-specific management:

Step 2: Identify the Specific Complication Type

The three main symptomatic presentations require different approaches:

High-output heart failure (most common complication):

• Intensive medical therapy is first-line: correction of anemia, salt restriction, diuretics, antihypertensive agents, antiarrhythmic agents, and digoxin as clinically indicated 1
• Most cases respond to medical management alone 1
• Echocardiography to assess cardiac function and measure cardiac index 3

Portal hypertension complications (varices, ascites):

• Treat using standard cirrhosis management protocols 1
• Critical pitfall: Avoid TIPS procedure - it worsens the hyperdynamic circulatory state by increasing shunting 1, 2

Biliary ischemia/cholangitis:

• Antibiotic therapy is the mainstay 1
• No role for biliary stenting 1
• This complication carries a poor prognosis 1

Invasive Treatment Considerations

Only for patients failing medical management with progressive clinical deterioration:

Transarterial Embolization

• Should be approached with extreme caution - significant complications and fatal outcomes have been reported 1, 2
• Consider only as a palliative, temporizing measure in non-transplant candidates with high-output cardiac failure 2
• Experience from 33 treated patients showed 4 fatal outcomes with significant complications in survivors 1

Liver Transplantation

• The only definitive curative option for hepatic vascular malformations in HHT 1, 2
• Indications: intractable high-output cardiac failure, ischemic bile duct necrosis, complicated portal hypertension 1, 2
• Post-operative mortality approximately 10% with favorable long-term results in most patients 1, 2
• Requires consultation with an HHT expert center before proceeding 2

Critical Pitfalls to Avoid

Never perform liver biopsy in HHT patients - extremely high bleeding risk due to the vascular malformations 1, 2, 4

Do not use TIPS for portal hypertension - worsens hemodynamics by increasing shunting 1, 2

Avoid treating asymptomatic liver involvement - no benefit and potential for harm from unnecessary interventions 1, 2, 4

Imaging Follow-up

• Doppler ultrasound is the preferred screening and monitoring modality due to accuracy, non-invasiveness, availability, and low cost 1, 2, 3
• Further imaging (CT, MRI, echocardiography) reserved for symptomatic patients or when assessing hemodynamic impact 1
• Multiphase CT can determine shunting patterns (arteriosystemic vs arterioportal) which correlate with symptom type 5, 6