Priority Clinical Recommendations for LIVER VMs
November 1, 2006
1. The expert panel recommends that physicians screen patients with 1 or 2
HHT Curaçao criteria for liver VMs to better define the diagnosis of HHT
Clinical considerations: There are sensitive and low-risk screening tests for liver VMs,
however, given the high prevalence of liver VMs in patients with HHT and in the absence
of effective treatment, screening all patients with HHT is not warranted. However, the
panel feels that patients who meet only 1-2 criteria for HHT should be screened to obtain
a more definite assessment of the presence of HHT.
2. The expert panel recommends abdominal Doppler ultrasound as the initial
screening test for liver VMs, in patients with HHT.
*have evidence table comparing screening/diagnostic tests and safety of these
Clinical Considerations: Liver Doppler ultrasonography has the best combination of high
sensitivity and low-risk among screening tests for liver VMs in adults, though the expert
panel had concerns about differences in operator expertise. The expert panel agreed that
positive screening should be confirmed with abdominal spiral computed tomography
(CT).
3. The expert panel recommends that in patients with HHT and symptoms or
signs suggestive of liver VMs (specifically dyspnea on effort, orthopnea,
edema, ascites, variceal hemorrhage, abdominal pain, jaundice, liver bruit,
or abnormal liver enzymes), a Doppler ultrasound and/or an abdominal CT
be performed to confirm the diagnosis of liver VMs.
Clinical Considerations: Patients with symptomatic liver VMs have significant morbidity
and mortality and should be followed closely so that adequate specific therapy can be
initiated promptly. The panel felt that imaging studies (Doppler ultrasound, spiral CT or
magnetic resonance imaging) are sufficient to make the diagnosis of liver VMs.
However, the panel felt that the diagnosis of liver VMs in a symptomatic patient should
be followed by hemodynamic studies including right heart catheterization with
measurements of cardiac index and pulmonary pressures and hepatic vein catheterization
with measurement of hepatic venous pressure gradient to assess the severity of liver
involvement and the need for specific therapy.
4. The expert panel recommends that in patients with HHT with symptoms or
signs suggestive of liver VMs and/or with liver masses, liver biopsy be
precluded.
Clinical Considerations: The diagnosis of liver VMs is done on the basis of imaging
studies. Although liver biopsy findings in patients with liver involvement by HHT are
Priority Clinical Recommendations for LIVER VMs
November 1, 2006
quite characteristic, they may be misleading and the procedure may be associated with
higher complication rates compared to patients without liver VMs. Additionally, the
prevalence of focal nodular hyperplasia (FNH) is much higher in patients with liver VMs
than in the general population. FNH is a benign liver mass that can be characterized noninvasively, therefore in a patient with HHT and liver VMs in whom a liver mass is
discovered, biopsy or excision should not be performed.
5. The expert panel recommends that hepatic artery embolization/ligation be
avoided in patients with liver VMs.
*have evidence table containing case series of embolization
Clinical Considerations: Transcatheter embolotherapy has been demonstrated to be only
transiently effective in patients with liver VMs. Additionally, hepatic artery
embolization/ligation is associated with significant morbidity and mortality and has led to
the need for liver transplantation in a significant percentage of patients. The panel
recommends that this procedure be performed only after failure of medical therapy and
after ruling out the presence of portovenous shunting and only in a center with liver
transplant capability. The panel other invasive diagnostic/thereapeutic procedures such
as liver biopsy and endoscopic retrograde cholangiography should also be avoided
because of potential morbidity and mortality.
6. The expert panel recommends that referral for liver transplantation be
considered in patients with liver VMs that develop:
a. Cholangitis (right upper quadrant pain, fever, jaundice) in the
presence of liver cystic lesions
b. Intractable heart failure
c. Intractable portal hypertension
* have evidence table containing case series of liver transplant
Clinical considerations: Patients who develop biliary necrosis have the highest mortality,
particularly those who develop it in the setting of heart failure. In the United States, it has
been recommended that this complication be assigned a high priority analogous to posttransplant patients that develop bile duct necrosis. The panel also considered that
although most patients with heart failure or portal hypertension can be managed
medically, patients with liver VMs who are refractory to medical therapy should also be
referred for liver transplant evaluation.