Budd-Chiari Syndrome: Long term success via hepatic decompression using transjugular intrahepatic porto-systemic shunt
Budd-Chiari Syndrome: Long term success via hepatic decompression using transjugular intrahepatic porto-systemic shunt
Alexandra Zahn 0
Daniel Gotthardt 0
Karl Heinz Weiss 0
Gtz Richter 2
Jan Schmidt 1
Wolfgang Stremmel 0
Peter Sauer 0
0 Department of Gastroenterology, University Hospital Heidelberg , Im Neuenheimer Feld 410, 69120 Heidelberg , Germany
1 Department of Surgery, University Hospital Heidelberg , Im Neuenheimer Feld 110, 69120 Heidelberg , Germany
2 Department of Diagnostic Radiology, University Hospital Heidelberg , Im Neuenheimer Feld 110, 69120 Heidelberg , Germany
Background: Budd-Chiari syndrome (BCS) generally implies thrombosis of the hepatic veins and/or the intrahepatic or suprahepatic inferior vena cava. Treatment depends on the underlying cause, the anatomic location, the extent of the thrombotic process and the functional capacity of the liver. It can be divided into medical treatment including anticoagulation and thrombolysis, radiological procedures such as angioplasty and transjugular intrahepatic porto-systemic shunt (TIPS) and surgical interventions including orthotopic liver transplantation (OLT). Controlled trials or reports on larger cohorts are limited due to rare disease frequency. The aim of this study was to report our single centre long term results of patients with BCS receiving one of three treatment options i.e. medication only, TIPS or OLT on an individually based decision of our local expert group. Methods: 20 patients with acute, subacute or chronic BCS were treated between 1988 and 2008. Clinical records were analysed with respect to underlying disease, therapeutic interventions, complications and overall outcome. Results: 16 women and 4 men with a mean age of 34 12 years (range: 14-60 years) at time of diagnosis were included. Myeloproliferative disorders or a plasmatic coagulopathy were identified as underlying disease in 13 patients, in the other patients the cause of BCS remained unclear. 12 patients presented with an acute BCS, 8 with a subacute or chronic disease. 13 patients underwent TIPS, 4 patients OLT as initial therapy, 2 patients required only symptomatic therapy, and one patient died from liver failure before any specific treatment could be initiated. Eleven of 13 TIPS patients required 2.5 2.4 revisions (range: 0-8). One patient died from his underlying hematologic disease. The residual 12 patients still have stable liver function not requiring OLT. All 4 patients who underwent OLT as initial treatment, required re-OLT due to thrombembolic complications of the graft. Survival in the TIPS group was 92.3% and in the OLT group 75% during a median follow-up of 4 and 11.5 years, respectively. Conclusion: Our results confirm the role of TIPS in the management of patients with acute, subacute and chronic BCS. The limited number of patients with OLT does not allow to draw a meaningful conclusion. However, the underlying disease may generate major complications, a reason why OLT should be limited to patients who cannot be managed by TIPS.
Budd-Chiari syndrome (BCS) is a rare disorder defined as
a hepatic venous outflow obstruction at any level between
the hepatic veins and the right atrium  but generally
implies thrombosis of the hepatic veins and/or the
intrahepatic or suprahepatic inferior vena cava (IVC). Up to 50%
of all cases of BCS are due to chronic myeloproliferative
disorders like polycythemia vera (PV)  or
coagulopathies like factor V (Leiden) gene mutation [3-5]. The
clinical presentation is highly variable but may be categorized
as acute and perhaps fulminant hepatic failure, as subacute
without evidence of cirrhosis or as chronic with evidence
of portal hypertension and cirrhosis.
Treatment depends on the underlying cause, the
anatomic location, the extent of the thrombotic process and
the severity of liver disease. Treatment options can be
divided into medical treatment including anticoagulation
and thrombolysis [6-8], radiological procedures such as
angioplasty  and transjugular intrahepatic
porto-systemic shunt (TIPS) [10-14] and surgical procedures
including porto-systemic shunting (PSS) [15-17] and
orthotopic liver transplantation (OLT) [18,19].
Anticoagulation alone is unlikely to lead to sufficient
recanalization of occluded vessels, or development of adequate
collateral circulation. However, satisfactory long-term
survival with only medical therapy has been reported
[6,20]. Recent data failed to show a favorable impact of
PSS on survival [17,21], while TIPS has shown
encouraging results [11-13,22-25].
This may give rise to redefine the role of OLT which
may now be preserved for patients failing TIPS. The
present treatment recommendations of BCS [26,27] are
not based on randomized studies but on a small number
of retrospective studies [8,25,27] and one prospective
The aim of this single centre retrospective study was
to further enlarge the body of patients evaluated with
the intention to allow steady optimization of present
Patients admitted between 1988 and 2008 with a
primary diagnosis of BCS were enrolled into this
retrospective analysis. All available medical records especially
laboratory data, radiological imaging and procedures,
surgical interventions and discharge letters were
reviewed. All data at the time of diagnosis and of new
treatment were considered. Date of diagnosis was the
date of the first investigation when the criteria for
diagnosis were fulfilled. BCS was defined following the
European network for vascular disorders of the liver
(EnVie)  criteria and the last Baveno consensus based
on imaging showing an obstructed venous outflow tract
. Diagnosis of BCS was made by either Doppler
ultrasonography, magnetic resonance imaging or
Disease severity was defined as acute, subacute or
chronic. In contrast to the acute disease, the subacute
and chronic forms were assumed to be present for
several weeks to more than six months prior to clinical
Different from the last Baveno consensus 
treatment was only partly applied in a stepwise manner. On
the basis of radiological imaging and the severity of
clinical presentation a decision by an experienced
interdisciplinary team concerning adequate treatment was
reached. This could either be a medical treatment or a
prompt intervention. If patients did not improve on
medical therapy TIPS insertion was performed.
Patients who underwent OLT as initial therapy were
not candidates for TIPS. Two patients were transplanted
before TIPS had been introduced as a treatment option
of BCS, namely in 1988 and 1992. The other patients
had cirrhosis with signs of chronic liver failure in terms
of hepatic encephalopathy and high bilirubin levels so
that liver transplantation was considered essential in
Hematological evaluation of hypercoagulable state
To identify a hypercoagulable state as the underlying
etiology of BCS each patient received a comprehensive
hematological evaluation. The latter was performed
stepwise and included antiphospholipid antibodies,
homocysteine levels, testing for factor V (Leiden) and
prothrombin (20210) mutation, APC resistance, ATIII,
protein C and S levels. Patients in whom a
myeloproliferative disorder (MPD) like PV or essential
thrombocytosis was suspected received a bone marrow biopsy. As
our study begun in 1988, not all of our patients were
screened for the JAK2V617F mutation.
TIPS Technique and OLT
All TIPS were created using standard techniques [30-32]
by insertion of Palmaz stents (Johnson and Johnson
Interventional Systems, Warren, New Jersey), Wallstents
(Schneider, Minneapolis, Minnesota) or covered Viatorr
stents (GORE, Flagstaff, AZ). When a hepatic vein
remnant was not present the portal vein was punctured
directly from the IVC . After the TIPS procedure
patients underwent anticoagulation according to the
guidelines . Patients underwent control angiography
3 months after TIPS and in addition if shunt
dysfunction was suspected. An abdominal ultrasound was
performed every 6 months. TIPS dysfunction was defined
as an increase in portosystemic gradient above 10 to 12
mmHg and clinical signs of portal hypertension.
In our liver transplantation program the modified
piggyback technique by Belghiti  has been used as a
routine surgical procedure since 2001. Before 2001, the
so-called piggyback technique had been applied [34,35].
Descriptive statistics were provided as mean standard
deviation (SD) and as range. The cumulative survival
probability was estimated by the method described by
Kaplan and Meier. All analyses were carried out in
Microsoft Excel and SPSS.
The study protocol conformed to the ethical
guidelines of the Helsinki Declaration, and was approved by
the ethics committee of the University of Heidelberg.
20 patients, 4 male and 16 female, were included in our
study. Patient age ranged from 14 to 60 with a mean age
of 34 12 years. Patients characteristics are given in table
1. Myeloproliferative disorders (especially PV) were the
cause of BCS in 6 patients, 7 patients had plasmatic
coagulation abnormalities, 1 patient took oestrogen medication
as a possible underlying hypercoagulable condition and in
6 patients the aetiology of BCS remained unclear. As not
all of our patients were screened for the JAK2V617F
mutation latent MPD may have been missed in several patients.
12 patients presented with acute BCS, 8 with subacute or
chronic disease. 8 patients presented with abdominal pain,
3 with new onset of ascites, 7 with abdominal pain plus
new onset of ascites, 1 with gastrointestinal bleeding plus
ascites and 1 with acute liver failure.
Table 1 Baseline Characteristics of all Patients
Patient Underlying diagnosis
1 Polycythemia vera
2 Protein C and S deficiency
Factor V (Leiden) mutation
Treatment and outcome
13 patients underwent TIPS (10 women, 3 men) and 4
underwent OLT (3 women, 1 man) as initial therapy.
One of the remaining 3 patients died of fulminant liver
failure rapidly after the initial diagnosis, the other
patients have not had an intervention, yet as their status
was stable under a symptomatic anticoagulation therapy.
In the TIPS group mean age was 36 13 years (range:
20-60 years) and in the transplant group 27 9 years
(range: 14-34 years). 8 of 13 patients in the TIPS group
underwent TIPS within one week after the diagnosis of
BCS was confirmed, 3 patients underwent TIPS within 1
month, 1 patient within 2 months and in 1 patient time
period until TIPS placement was 48 months.
The porto-systemic pressure gradient was lowered by
a mean of 21 10 mmHg (range: 6-40 mmHg) in the
13 patients initially treated with TIPS. After a median
Time between primary
Table 2 Angiographic findings and interventions in the TIPS group
*HV: hepatic veins; PV: portal vein; SMV: superior mesenteric vein; IVC: inferior vena cava
**Numbers indicate original portosystemic pressure gradient followed by post-TIPS gradient in mmHg.
follow-up of 4 years (range: 6 months to 12 years) 11 of
these 13 patients (85%) developed TIPS dysfunction
requiring reintervention. In most cases TIPS dysfunction
was due to thrombosis or pseudointimal hyperplasia and
could be managed by dilation. On average, 2.5 2.2
revisions per patient were necessary (range: 0-8) (see
table 2). 12 out of 13 patients survived within the follow
up period (92%). One patient died in the course of the
underlying hematologic disease. After TIPS placement
no patient developed clinical relevant hepatic
encephalopathy. None of the patients in the TIPS group had to
undergo OLT subsequently.
After a mean of 30 13 months after diagnosis
(range: 12-48 months) patients in the transplant group
underwent OLT. Each patient who underwent OLT as
initial therapy, had to undergo re-OLT. The first
patient had retransplantation within a few days
because of portal vein thrombosis and graft failure.
Today, 20 years after re-OLT she is still alive and has
an excellent graft function. The second patient had to
undergo 2 retransplantations. She had ischemic
cholangiopathy in all explants and in the second explant
ischemic cholangiopathy in relation with arterial
thrombosis was found, histologically. The patient died
due to septic complications 3 months after the third
OLT. The third patient also had retransplantation
within a few days because of vascular obliteration of
the hepatic artery and a necrosis of the common bile
duct. This patient is still alive without further
complications. The fourth patient had to undergo
retransplantation 9 months after initial OLT because of
ischemic graft failure. She is still alive 15 years after
OLT without limitations. Although no recurrence of
BCS in the transplants has been detected, each patient
in the transplant group suffered from various
thrombotic complications. Within the observation
period 3 out of 4 patients survived (75%).
The overall probability of survival on an intention to
treat basis for the whole cohort of 20 patients with BCS
was 72.6% after a median follow-up of 5 years (range: 0
to 20 years), (see figure 1).
In this study, we assessed the clinical course and long
term outcome of patients with BCS referred to our
university hospital analysing the data of all patients
admitted with a primary diagnosis of BCS between 1988
Sequential use of increasingly invasive procedures
based upon the clinical response has been recommended
for the treatment of BCS [8,25,27]. In one study in 14
patients, treated with diuretics and anticoagulation
alone, a mortality rate of 86% during a period of 6
months was reported , however acceptable long
term survival rates with only medical therapy has been
reported in other trials . Surprisingly, in the recently
published first prospective study  nearly 50% of the
patients were managed conservatively, too. But there
may be limitations of this study; on the one hand the
study was not restricted to patients with severe disease
and on the other hand the median follow-up was only
17 months. In our present trial only 2 patients have not
had an invasive therapeutic procedure and are still
without clinical symptoms under anticoagulation therapy.
Both patients have incomplete hepatic venous outflow
obstruction and presented with chronic or subacute
BCS. In addition, in one of these two patients the
follow-up period of half a year is relatively short. On the
basis of this very limited experience, we may confirm
the value of medical treatment alone in patients with
Figure 1 Probability of survival given for all BCS patients. On the x-axis the time of follow-up in years is given. On the y-axis the probability
of survival is shown.
limited disease. In contrast, in the patients with severe
disease or symptomatic cirrhosis, medical treatment was
not effective and interventional treatment (TIPS) or
transplantation had to be applied.
Different from the last Baveno consensus 
treatment was only partly applied in a stepwise manner. On
the basis of radiological imaging and the severity of
clinical presentation a decision concerning adequate
treatment was reached. In our collective 13 patients
underwent TIPS as initial therapy. In the follow-up, one
patient died in the course of the underlying hematologic
disease so that an overall survival of 92% was achieved.
In all patients transplantation could be avoided. Similar
5 year transplant free survival rates between 77 and
100% after TIPS were observed in other studies
[11-13,22-25]. These results seem to justify primary
TIPS treatment in patients with severe disease without
awaiting failure of a previous treatment step. Both the
high survival and the prevention of OLT in the patients
initially treated with TIPS demonstrate an advantage
over a stepwise strategy recommended by Baveno and
applied in the study by Plessier et al. . Although, the
latter found high survival rates using therapeutic
procedures by order of increasing invasiveness, the need for
transplantation after TIPS was high (38%) compared to
our study. This may be due to the fact that TIPS is
more efficient when inserted early. Therefore, our
results suggest that TIPS should no longer be
considered as a bridge to OLT but as a definitive treatment
option in BCS, especially when prompt intervention and
a high grade of interventional experience is available.
With regard to the current situation of liver donor
shortage these results are important.
Although, our protocol involved resumption of
anticoagulation immediately after TIPS placement, 85% of
our patients had at least one reintervention within a
mean follow-up of 6 years. In all these patients TIPS
revision was technically successful and effective.
However, TIPS dysfunction seems to be a common problem
[38,39]. Since, covered stents have a considerable
advantage over bare stents with a lower dysfunction rate for
the treatment of BCS patients , covered stents
should be preferred.
Four patients underwent OLT as initial therapy. Two
were transplanted before TIPS had been introduced as a
treatment option of BCS and two had cirrhosis with
signs of chronic liver failure. Survival following OLT
depends upon the underlying cause of BCS and the
patients condition at the time of transplantation . A
large series with 510 patients  found a 3 year patient
survival of 85% after introduction of the Model for
EndStage Liver Disease (MELD) score versus a 3 year
patient survival of 73% in the pre-MELD era. In two
other studies [41,43] 10 year survival rates were reported
between 69 and 68%, respectively. It has been suggested,
that the outcome of OLT in BCS patients does not
differ from that of other etiologies of liver failure when
adequate longterm anticoagulation is administered .
Even in patients with myeloproliferative diseases survival
rates are similar to those in patients with other
underlying etiologies . Although, the number of patients
transplanted in the present trial does not allow any
conclusions on survival, the overall survival rate of 75%
during a mean follow-up period of 11 years is comparable
to the reported results.
Each patient who underwent OLT as initial therapy,
had to undergo re-OLT, which is one of the most
important results of our study. All patients had graft
failure due to thrombotic or vascular complications.
These complications have been described in other series
and severe thrombotic complications occurred despite
routine early posttransplantation anticoagulation [8,41].
In accordance to other reports, these findings support
the concept that aggressive anticoagulation as early as
possible may reduce the risk of thrombembolic
complications as well as the late recurrence of BCS after OLT
However, it seems likely, that in some cases
thrombembolic complications will not be preventable, despite
of aggressive anticoagulation as observed in one patient
in our series.
In conclusion, therapy for BCS usually requires TIPS or
OLT. The treatment modality is dependent on duration
of illness, extent of thrombosis and degree of liver
dysfunction. TIPS is successful as initial therapy as it
promotes clinical improvement in the long run even
though shunt revisions are generally needed. In the
present study the necessity of subsequent OLT was
remarkably low and thus TIPS could be regarded as definitive
treatment option in BCS. OLT in BCS is associated with
an increased risk of thrombembolic complications and
early graft failure in spite of consequent anticoagulation
AZ contributed substantially to the design of the study, performed data
collection, data analysis and wrote the paper. DG contributed to the data
collection and analysis. KHW contributed to the data collection and analysis.
GR performed the radiological interventions, contributed to the data
collection and analysis. JS contributed to the data collection and analysis
concerning the transplant patients. WS contributed substantially to the
design of the study and to the interpretation of data. PS developed the
original idea of the study, was involved in data analysis and reviewed the
manuscript finally. All authors read and approved the final manuscript.
The authors declare that they have no competing interests.
The pre-publication history for this paper can be accessed here:http://www.
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