consensus report
Austrian consensus on the definition and treatment of portal hypertension and its complications (Billroth II)
7
1 3
Treatment for high-risk patients with failure of
medical/endoscopic therapy in the prophylaxis
of rebleeding
1. TIPS implantation is recommended for selected high-
risk patients, in whom medical and endoscopic treat-
ment fails to prevent rebleeding (at least twice) (II-2).
2. These patients should be considered for liver trans-
plantation (III).
Portal hypertensive gastropathy (PHG), gastric
antral vascular ectasia (GAVE)
Definitions
1. PHG is defined as a macroscopically visible mosaic
like pattern of the gastric mucosa. The prevalence of
PHG in portal hypertension varies between 20 % and
80 %. PHG should be differentiated into PHG with and
without signs of bleeding [
29
]. Severe PHG is charac-
terized by red marks and associated with a higher risk
of bleeding [
30
].
2. GAVE is a distinct clinical, endoscopic, and histo-
pathologic entity endoscopically
characterized by
aggregates of red spots arranged in linear pattern or
diffuse lesions (in this case they have to be confirmed
by biopsy of the antrum). GAVE can be seen with other
conditions than cirrhosis of the liver. The prevalence
of GAVE in cirrhosis is low (about 2 %) [
31
].
3. The incidence of acute PHG bleeding is low (less than
3 % at 3 years).
4. The incidence of chronic PHG bleeding is around 10–
15 % at 3 years.
5. The lesions may change over time (fluctuate, worsen,
or improve).
Treatment of acute PHG bleeding
1. Vasoactive drugs are used with a high success rate
(70–100 %) in uncontrolled studies. Controlled studies
are lacking but patients are managed in a similar way
as acute variceal bleedings in many centers (III).
2. Emergency transjugular intrahepatic porto-systemic
shunt (TIPS; or shunt surgery in rare cases) should be
regarded as rescue treatments in cases of failure of va-
soactive drugs (III).
Treatment of chronic PHG bleeding
1. Nonselective beta-blockers (II-1), and if needed iron,
are the first-choice treatment.
2. Beta-blockers and isosorbide-5-mononitrate, as well
as other medical treatments (i.e., carvedilol, long-
acting somatostatin analogues), should be evaluated.
3. Treatment should be continued indefinitely (III).
4. TIPS is a rescue therapy in PHG-associated bleedings,
which could respond to a decrease in HVPG (II-3).
APC might be an additional therapeutic option for pa-
tients with refractory bleeding from PHG (III).
Treatment of GAVE bleeding
1. Argon plasma coagulation is an effective therapy in
patients with mild to moderate bleedings (II-3).
2. Multiple sessions are usually needed to control acute
bleeding or to stop chronic blood loss, but no con-
trolled studies are available (III).
Gastric varices
Definitions
1. The classification of Sarin et al. should be used [
32
];
it distinguishes gastroesophageal varices type 1 and 2
(GOV 1 and 2) and isolated gastric varices 1 and 2 (IGV
1 and 2), which occur in 5–33 % of patients with portal
hypertension.
2. GOV2 and IGV1: the presence of red signs, large size,
and Child Class B or C should be considered as risk
factors for bleeding (Fig.
1
).
3. GOV2, GOV1, and IGV1 are at the highest risk of bleed-
ing (bleeding risk 55–78 %), which has a high mortality
rate [
32
,
33
].
For therapy of bleeding gastric varices, the
following approaches can be used
1. Cyanoacrylate glue injection is the most effective
therapy for acute fundal variceal bleeding [
34
–
37
] (I).
Fig. 1
Gastroesophageal varices
Gastro-oesophageal varices (GOV)
GOV 1
GOV 2
IGV 1
IGV 2
8
Austrian consensus on the definition and treatment of portal hypertension and its complications (Billroth II)
consensus report
1 3
2. One single injection should consist of 0.5 ml cyano-
acrylate and 0.5 ml lipiodol. Not more than 1.0 ml of
the cyanoacrylate/lipodol mixture should be injected
at each time to minimize the risk of embolization [
38
]
(II-2).
3. Endoscopic variceal sclerotherapy is no alternative
(bleeding control of 60–100 % but rebleeding rate of
up to 90 %) [
39
] (II-3).
4. Vasoactive drugs could be used in combination with
other treatments (III).
5. Balloon tamponade can be used as a bridge in case of
failure to control bleeding (III).
6. Endoscopic band ligation is not an established thera-
py for bleeding varices GOV2, IGV 1 + 2 due to a lower
rate of hemostasis and a higher rebleedings rate com-
pared to cyanoacrylate [
34
,
36
] (I); GOV1 are consid-
ered extensions of esophageal varices and should be
treated accordingly [
33
] (III).
7.
Balloon-occluded retrograde transvenous oblitera-
tion (B-RTO) is a treatment option for gastric varices
mostly used in Japan [
40
–
42
]. It shows promising re-
sults there but the efficacy in Caucasians populations
has not adequately been documented.
8. TIPS (rarely surgery) is indicated as rescue therapy
(“emergency TIPS”: bleeding control rate up to 90 %)
[
43
] for patients not responding to medical and en-
doscopic therapy [
44
] (II-3). TIPS might be a good
first-line treatment option also for high-risk patients
with gastric variceal bleeding (GOV2, IGV1 + 2; “early
TIPS”) but has not been studied specifically in this
patient population. Surgical devascularization is a
rescue therapy in case of failure of medical and endo-
scopical treatment to prevent rebleeding in patients
in whom neither a TIPS can be implanted nor shunt
surgery can be performed.
For long-term therapy of fundal varices, there are
no established and prospectively evaluated thera-
pies. Potential candidate therapies include
1. Long-term cyanoacrylate glue application
2. TIPS (rarely shunt surgery in patients with very good
liver function)
3. Medical therapy with NSBB (propranolol, carvedilol)
and/or ISMN
Management of ascites
Diagnostic approach in patients with ascites
1. Ascites should be graded according to the International
Ascites Club guidelines into uncomplicated (grade 1:
only visible on ultrasound; grade 2: moderate ascites;
grade 3: massive ascites), and refractory ascites (not
responsive or intolerant to diuretic therapy even after
paracentesis) [
2
].
2. All patients presenting with ascites for the first time,
with recurrence of ascites or deterioration of ascites,
need investigation of their ascites. Substitution of co-
agulation factors or platelets is not indicated even in
patients with severe coagulopathy, because paracen-
tesis rarely leads to serious bleeding complications
[
1
,
45
] (I).
3. Investigation of ascites should
include at least the de-
termination of the ascitic neutrophil count, protein
concentration, and serum-ascites albumin gradient
(I).
4. Additionally, aerobic and anaerobic blood culture
bottles should be inoculated with ascitic fluid for bac-
teriologic diagnosis of SBP (I).
Therapy of uncomplicated ascites
1. For initial therapy of patients with mild ascites (grade
1), sodium restriction (90 mmol NaCl/day, corre-
sponding to 5.2 g NaCl/day) is recommended(III).
2. In patients with moderate ascites (grade 2), the initial
therapy consists of sodium restriction and diuretic
therapy (I).
3. Diuretic therapy should be started with spironolac-
tone (100–200 mg). In case of insufficient ascites con-
trol or lack of effectiveness (< 1–2 kg weight reduction
within 7 days), furosemide should be added. The daily
dose of 400 mg spironolactone and 160 mg furose-
mide should not be exceeded. Alternatively, butizide
in combination with spironolactone can be used in-
stead of or in combination with furosemide (I).
4. In patients with tense ascites (grade 3), paracentesis
is the treatment of choice and should be followed by
diuretic therapy. Total paracentesis should be carried
out as a single procedure, even when a large volume of
ascites is present (I).
5. Plasma volume expansion using albumin is recom-
mended in all patients undergoing paracentesis,
especially if more than 5 L of ascites have been re-
moved; for prevention of hypovolemia and circulatory
dysfunction. Albumin at a dose of 8 g/L of ascites re-
moved should be administered (I).
6. Patients responsive to diuretics should primarily be
treated with sodium restriction and diuretics and
should not undergo serial paracentesis.
7. In patients with hyponatremia< 120 mmol/L (or mM),
diuretic therapy should be halted, since at these levels
diuretics are ineffective and worsen hyponatremia.
Substitution of concentrated NaCl solutions is not in-
dicated (II-1).
8. Patients with moderate to severe ascites should be
considered for liver transplantation.
9.
The administration of nonsteroidal antiinflamma-
tory drugs (NSAID) in patients with decompensated
cirrhosis and ascites can lead to renal failure and
therefore should be avoided [
46
]. The same is true for
angiotensin-converting enzyme inhibitors [
47
,
48
] (I).