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

If the patient is asymptomatic, no treatment is recommended—observation only. 1

Initial Assessment and Risk Stratification

The finding of decreased liver attenuation on contrast CT in an HHT patient represents hepatic vascular malformations (VMs), which occur in 67-74% of HHT patients on multislice CT. 1 However, only 8% of patients with liver involvement become symptomatic, making the distinction between asymptomatic and symptomatic disease critical for management decisions. 1

Key Clinical Evaluation Steps

• Assess for symptoms of complications: high-output cardiac failure (most common), portal hypertension (ascites, variceal bleeding), biliary ischemia/cholangitis, or mesenteric angina 1
• Obtain baseline echocardiography to evaluate cardiac output and assess for high-output state, particularly if liver VMs appear severe on imaging 1
• Check liver biochemistry: Asymptomatic elevations in alkaline phosphatase and gamma-glutamyl transpeptidase (3-10 times normal) are common due to bile duct ischemia but have little clinical significance in the absence of symptoms 1
• Verify liver synthetic function is preserved (typically normal in HHT liver involvement) 1

Management Algorithm Based on Symptom Status

For Asymptomatic Patients (Majority)

No treatment is indicated. 1 The consensus guidelines are unequivocal on this point—asymptomatic liver involvement requires only monitoring, as liver synthetic function remains well preserved and most patients never develop complications. 1

• Avoid liver biopsy due to high bleeding risk given the prevalence of hepatic VMs (32-74% in HHT patients) 1
• Consider prophylactic measures for severe grade 4 liver VMs: ACE inhibitors or carvedilol to prevent cardiac remodeling, or beta-blockers if portal hypertension develops to prevent variceal bleeding 1

For Symptomatic Patients

Management is complication-specific and should involve consultation with an HHT expert center before any invasive intervention. 1

High-Output Cardiac Failure (Most Common Complication)

• Intensive medical therapy is first-line: correction of anemia, salt restriction, diuretics, antihypertensive agents, antiarrhythmic drugs, and digoxin as clinically indicated 1
• Obtain cardiology assessment and treatment before considering any invasive liver-directed therapy 1
• The majority of patients respond to medical management alone 1

Portal Hypertension Complications

• Treat variceal bleeding and ascites using standard cirrhosis protocols 1
• Avoid TIPS: it worsens the hyperdynamic circulatory state by increasing shunting and is contraindicated in HHT liver involvement 1

Biliary Ischemia/Cholangitis

• Treat with antibiotics (biliary stenting has no role) 1
• This complication carries a poor prognosis and may progress to "hepatic disintegration" (bile duct necrosis) 1

Invasive Treatment Options (Only After Medical Therapy Failure)

Transarterial Embolization

This is a palliative, temporizing, and risky procedure that should only be discussed in patients with high-output cardiac failure or mesenteric angina who are not transplant candidates. 1 Among 33 reported cases, four fatal outcomes occurred with significant complications in survivors, mandating extreme caution. 1 Cholangiopathy is a contraindication to embolization. 1

Liver Transplantation

Orthotopic liver transplantation is the only definitive curative option for hepatic VMs in HHT. 1

Indications for transplant:

• Intractable high-output cardiac failure despite intensive medical therapy 1
• Ischemic bile duct necrosis (urgent indication) 1
• Complicated portal hypertension 1

Pre-transplant requirements:

• Right heart catheterization is mandatory to exclude severe pulmonary hypertension (transplant may be allowed if pulmonary vascular resistance <240 dynes·sec·cm⁻⁵) 1
• Post-operative mortality is 7-10% with long-term survival of 82-92% 1

Critical caveat: Disease recurrence occurs in approximately 48% of patients by 15 years post-transplant, necessitating lifelong follow-up. 2

Bevacizumab (Emerging Option)

Bevacizumab has shown ability to reduce cardiac index in severe liver VMs, with complete response in 12% and partial response in 70% of 24 patients. 1 However, critical limitations include unpredictable efficacy, non-negligible toxicity, revascularization after drug withdrawal, and potential impairment of wound healing—problematic if emergency transplant becomes necessary. 1 This should be considered experimental and discussed only at expert centers.

Common Pitfalls to Avoid

• Do not perform liver biopsy in HHT patients—the bleeding risk is unacceptably high 1
• Do not use TIPS for portal hypertension—it worsens hemodynamics 1
• Do not pursue invasive treatment for asymptomatic patients—the natural history is benign in 92% of cases 1
• Do not assume cirrhosis—the nodular appearance with portal hypertension represents "pseudocirrhosis" from nodular regenerative hyperplasia, not true cirrhosis, and liver synthetic function remains preserved 1
• Consider focal nodular hyperplasia (FNH) first when evaluating liver masses in HHT patients, as prevalence is much higher than in the general population 1