Consensus report

12

    Austrian consensus on the definition and treatment of portal hypertension and its complications (Billroth II)

consensus report

1 3

ment modalities are comparatively safe methods. The risk 

of arterial hypotension is higher with intermittent extra-

corporeal treatment employing large volume depletion 

and should be avoided in hemodynamically impaired 

patients. Up to date anticoagulation such as citrate antico-

agulation is also safe in patients with advanced stage liver 

disease and should preferably be used.

Liver transplantation

Liver transplantation is the treatment of choice for suit-

able candidates with HRS. Patients should be given high 

priority on the waiting list. This relates to type-1 as well 

as type-2 HRS (I).

Simultaneous liver kidney transplantation is not 

routinely recommended and may only be indicated in 

patients with prolonged HRS undergoing renal replace-

ment therapy since recovery of renal function is unusual 

in these patients [

80

].

Prevention of HRS

Long term treatment with norfloxacin (400  mg/day) in 

patients with end stage liver disease has been demon-

strated to reduce the recurrence of HRS and improved 

survival [

81

] (I).

Short term and long term administration of Pent-

oxifylline was shown to have beneficial effects on renal 

function in patients with severe alcoholic hepatitis and 

advanced cirrhosis [

82

, 

83

].

Management of vascular liver disease

Noncirrhotic portal hypertension can be caused by vas-

cular disorders of the liver, which encompass a hetero-

geneous group of diseases with portal hypertension 

but without liver cirrhosis. Vascular liver disorders can 

sometimes occur in liver cirrhosis—especially Budd-

Chiari syndrome.

The three main vascular liver disorders associated 

with portal hypertension are portal vein thrombosis 

(PVT), sinusoidal obstruction syndrome (SOS), and 

Budd-Chiari syndrome (BCS), where the affected vascu-

lar compartments are the portal- and splanchnic veins, 

the sinusoids and the hepatic outflow tract, respectively.

Congenital vascular malformations and hereditary 

hemorrhagic telangiectasia are rare vascular liver diseases 

that can also be associated with portal hypertension.

Epidemiology

1.   The lifetime risk of PVT is estimated to be 1 % [

84

, 

85

]. 

Chronic portal vein thrombosis is found in 10–25 % of 

patients with nonmalignant liver cirrhosis. Although 

data on the epidemiology of acute portal vein thrombo-

sis are lacking, acute extrahepatic portal vein thrombo-

sis accounts for up to 30 % of variceal bleeds [

86

] (IIb).

2.  SOS is the most frequent cause of portal hyperten-

sion in patients receiving high dose chemotherapy 

especially during “conditioning” of patients for bone 

marrow transplantation. The incidence of SOS is up to 

10 % in a large cohort study of European bone marrow 

transplant recipients. SOS has also been associated 

with drugs listed in Table 

1

 and plant alkaloids (IIb).   

3.   All forms of hepatic venous outflow tract obstruction 

are referred to as Budd-Chiari syndrome, indepen-

dent of the level or cause of obstruction [

87

, 

88

]. The 

mean age-standardized incidence and prevalence 

of BCS in a Swedish cohort was estimated to be 0.8 

per million per year and 1.4 per million, respectively 

[

89

]. The main causes of primary BCS are prothrom-

botic conditions, which have been identified in 77 % 

of patients with BCS. Myeloproliferative disorders are 

single most important prothrombotic condition iden-

tified in 39 % of patients with BCS [

90

]. Additional pro-

thrombotic risk factors associated with BCS and PVT 

are listed in Table 

1

 (IIb).

Pathophysiology

1.   JAK2 positive myeloproliferative disorders have been 

reported in 20 to 35 % of patients with acute PVT  

[

91

–

93

]. Other causes of thrombophilia frequently 

identified in patients with portal vein thrombosis in-

clude protein S and protein C deficiency, antithrombin 

III deficiency, paroxysmal nocturnal hemoglobinuria.

2.   Accepted risk factors for SOS are toxic conditioning 

regimens for bone marrow transplantation especially 

regimens including cyclophosphamide in combina-

tion with either busulfan (especially when given oral-

ly) or total body irradiation or regimens that include 

Table 1.  Risk factors for PVT and BCS

Risk factor

PVT (%)

BCS (%)

Myeloproliferative disease

30–40

40–50

Atypical

14

25–35

Classic

17

10–25

Antiphospholipid syndrome

6–19

4–25

PNH

0–2

0–4

Beçet disease

0–31

0–33

Faktor V Leiden

6–32

6–32

Prothormbin gene-mutation

14–40

5–7

Protein C deficiency

0–26

10–30

Protein S deficiency

2–30

7–20

AT III deficiency

0–26

0–23

Plasminogen deficiency

0–6

0–4

Recent pregnancy

6–40

6–12

Hormonal contraception

12

6–60

Hyperhomocysteinemia

12–22

37

MTHFR genotype 677TT

11–50

12–22

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