Clin Liver Dis 10 (2006) 259–273

Budd-Chiari Syndrome
Michael A. Zimmerman, MD,
Andrew M. Cameron, MD, PhD,
R. Mark Ghobrial, MD, PhD*
Division of Liver and Pancreas Transplantation, Department of Surgery,
The Pfleger Liver Institute, The Dumont-UCLA Transplant Center,
The David Geffen School of Medicine at the University of California Los Angeles,
10833 Le Conte Avenue, Los Angeles, CA 90095, USA

Budd-Chiari syndrome (BCS) represents a spectrum of disease states re-

sulting in hepatic venous outflow occlusion. Obstruction can occur at any
level from the hepatic venules to the right atrium of the heart. Anatomic ab-
normalities including vascular webs or strictures may be present and possi-
bly predispose to venous thrombosis. Hypercoagulable states often are
present, and the incidence of concomitant disorders is increasing (Box 1)
[1]. Untreated, BCS has a mortality rate close to 80% [2]. The most common
causes of BCS in the Western world are myeloproliferative disorders, includ-
ing polycythemia vera and essential thrombocytosis [3]. Pregnancy, use of
oral contraceptives, and cancer are also documented causes. An evolving
pathologic concept advocates the presence of a genetic mutation in one or
more genes leading to a hypercoagulable predisposition. Coupled with an
acquired thrombogenic stimulus (ie, cancer), the result is hepatic venous
thrombosis and outflow occlusion. Spontaneous resolution has been re-
ported [4], and up to 25% of patients remain asymptomatic [5]. Most pa-
tients, however, require an aggressive multidisciplinary approach,
including invasive radiologic procedures and surgery, to control symptoms
and prevent disease progression. Liver transplantation recently has emerged
as the preferred therapy for patients who have fulminant liver failure or cir-
rhosis [6]. Although the cadaveric organ pool is limited, portosystemic
shunts remain a therapeutic alternative in patients who have preserved liver
function. This article outlines the approach to clinical diagnosis and sup-
portive medical therapy in patients who have BCS and reviews the clinical

* Corresponding author. The Dumont-UCLA Transplant Center, 77-120 CHS, Box

957054, 10833 Le Conte Avenue, Los Angeles, CA 90095.
E-mail address: rghobria@mednet.ucla.edu (R.M. Ghobrial).

1089-3261/06/$ - see front matter Ó 2006 Elsevier Inc. All rights reserved.
doi:10.1016/j.cld.2006.05.005 liver.theclinics.com
260 ZIMMERMAN et al

Box 1. Causes and associated disorders of Budd-Chiari

syndrome
Prothrombotic conditions
Factor V Leiden mutation
Antiphospholipid syndrome
Antithrombin III deficiency
Protein C, S deficiency
Hyperhomocysteinemia
Myeloproliferative disorders
Polycythemia vera
Essential thrombocytosis
Paroxysmal nocturnal hemoglobinuria
Oral contraceptive use
Pregnancy
Cancer
Anatomic webs (inferior vena cava, hepatic veins)
Behçet’s syndrome
Hepatic abscess
Hepatitis
Trauma
Inflammatory bowel disease
Polycystic liver disease
Idiopathic

data supporting surgical shunting and liver transplantation as viable treat-

ment options in this patient population.

Diagnosis
Clinical manifestations of BCS classically include hepatomegaly, right
upper quadrant pain, and abdominal ascites. These findings are present in
the majority of patients [7]. The degree of symptomatology corresponds di-
rectly with the rapidity with which hepatic outflow occlusion occurs. Fulmi-
nant and acute forms of BCS are characterized by rapidly worsening
encephalopathy and accumulation of abdominal ascites. Menon and col-
leagues [8] note that subacute BCS is the most common form and presents
with minimal ascites and hepatic necrosis because of sinusoidal decompres-
sion through the collateral venous circulation. Patients who have the
chronic form of BCS present with the usual manifestations of end-stage liver
disease and portal hypertension.
The initial study of choice for documenting hepatic outflow obstruction is
Doppler ultrasound. With an overall accuracy of approximately 70% [9],
 BUDD-CHIARI SYNDROME 261

ultrasound is relatively inexpensive and is readily available at most institu-

tions. A second line of investigation includes either CT scan or MRI. Each
imaging modality has specific advantages. CT scanning allows the evalua-
tion of hepatic vein patency, parenchymal abnormalities, and the degree
of abdominal ascites. The presence of caudate lobe hypertrophy also may
be assessed (Fig. 1) [10]. A well-documented compensatory mechanism fol-
lowing hepatic venous outflow obstruction, significant caudate lobe enlarge-
ment, has serious implications for surgical intervention. The role of MRI
continues to evolve as technology is enhanced. Noone and colleagues [11]
suggest that MRI may be valuable in differentiating acute from chronic dis-
ease. More importantly, it may allow further definition of the vascular anat-
omy, including patency of the supra- and infrahepatic vena cava, as well
distinguishing benign from malignant disease [12]. Although not absolutely
essential, hepatic venography still is considered the criterion standard imag-
ing technique. Precise definition of vascular abnormalities may directly in-
fluence therapeutic interventions. Further, infra- and suprahepatic caval

Fig. 1. Portosystemic shunt with caudate lobe hypertrophy. (A) The caudate lobe is enlarged on
both sides of the inferior vena cava (IVC) and portal vein. (B) A side-to-side portacaval shunt is
in place and patent.
262 ZIMMERMAN et al

pressures can be measured, and a liver biopsy can be obtained to assess the
degree of inflammation or fibrosis [13].

Pathology
Hepatic venous outflow occlusion provides an excellent clinicopathologic
model of hepatocellular injury and regeneration. The continuum of histo-
logic alteration ranging from severe sinusoidal congestion and inflammation
to fibrosis and ultimately cirrhosis is well documented in this patient popu-
lation [14]. Fortunately, there is no clinical correlation between the histo-
logic presence of congestion or fibrosis and overall patient survival [15].
Hepatic explants following liver transplantation in patients who have BCS
contain regenerative hyperplastic nodules [16]. These neoplastic foci repre-
sent a spectrum of cellular changes from hyperplasia to adenomas. Al-
though the relationship once was considered controversial, hepatocellular
carcinoma also is associated with BCS by two mechanisms. Hepatocellular
carcinoma can cause hepatic vein or inferior vena cava (IVC) obstruction by
direct vascular invasion of the tumor, resulting in secondary outflow occlu-
sion. Alternatively, hepatocellular carcinoma can develop de novo in pa-
tients who have BCS and cirrhosis [17].
An important study by Tanaka and colleagues [18] describes the ‘‘two-
hit’’ vascular hypothesis for the development of regenerative nodules char-
acteristic of BCS. Examining 15 explants following liver transplantation,
they define several different forms of cirrhosis in patients who have chronic
disease. This study identified a definitive link between the presence or ab-
sence of portal vein thrombosis and the specific pattern of cirrhosis. Patients
who had severe portal vein obliteration developed veno-portal cirrhosis,
a form of fibrosis in which the septa bridge the portal tracts and hepatic
veins. Specimens without portal vein occlusion develop fibrosis that includes
only the hepatic vein. Further, the authors propose a vascular mechanism
for the formation of regenerative nodules initiated by ischemia and inflam-
mation subsequent to outflow obstruction. A concomitant decrease in portal
perfusion leads to portal vein thrombosis with a compensatory increase in
arterial perfusion. Nodular regeneration is histologically related to these
areas of parenchymal hyperemia. Other groups have confirmed these obser-
vations [19–22]. Whether these regenerative lesions undergo malignant
transformation is unknown.

Medical management
Medical management of BCS centers on diagnosis and aggressive treat-
ment of the underlying cause as well as controlling abdominal pain and as-
cites. All patients should undergo a hypercoagulable work-up to identify
any predisposition to venous thrombosis. Anticoagulation therapy with
 BUDD-CHIARI SYNDROME 263

heparin in the acute setting, followed by Warfarin for long-term therapy, is

recommended [8]. A general strategy to control abdominal ascites includes
reduction of daily sodium intake to approximately 60 to 90 mEq/d, which
is roughly equal to 1500 to 2000 mg of salt [23]. In patients who have severe
hyponatremia and whole-body fluid overload, fluid intake should be re-
stricted to 1000 cm3/d. Patients who have moderate-volume ascites should
also be managed with oral diuretic therapy in the form of furosemide (20–
40 mg/d) and spironolactone (50–200 mg/d). Finally, patients who have
large-volume or tense ascites can undergo abdominal paracentesis at regular
intervals [24]. Control of abdominal ascites will reduce abdominal pain and
discomfort.
Although medical therapy alone can achieve reasonable long-term results
in select patients [25,26], BCS is largely a surgical disease. Medical therapy
as a sole intervention is recommended only for asymptomatic patients who
have no ongoing necrosis, normal liver function, and easily controllable as-
cites [8]. In addition, high-risk patients who have serious comorbid condi-
tions also may be candidates for a more conservative approach.
Unfortunately, medical management alone has been associated with a mor-
tality rate higher than 80%, with liver failure the primary cause of death
[27]. A retrospective analysis of patients who had BCS at the University
of California, Los Angeles (UCLA) revealed a 2-year survival of 9% for pa-
tients treated with medical therapy alone [28]. Patients treated with a surgical
shunt had a 54% survival in the same time interval. These data must be in-
terpreted with caution, however, because liver transplantation was not con-
sidered as a surgical treatment option, and the shunt operations were not
standardized.

Thrombolytic therapy and interventional techniques

Since the first report of thrombolytic therapy in the early 1970s [29], nu-
merous case reports have documented short-term success in patients who
had acute and subacute BCS [30–32]. As experience with this therapeutic op-
tion evolves, several questions remain to be answered. Currently, there are
no level I data comparing the efficacy of various drug regimes (ie, streptoki-
nase versus tissue plasminogen activator). Further, optimal drug delivery
(systemic versus local) and the appropriate timing of thrombolytic adminis-
tration are unknown. Although success with systemic therapy has been
documented in select patients [33], local infusion may prove more effective
[34]. The risks of thrombolytic therapy include bleeding and pulmonary em-
bolism [35]. Contraindications to thrombolysis include history of a bleeding
disorder, previous cerebrovascular accident, and hypertension. A recent
study by Sharma and colleagues [36] chronicles a 12-year experience with
thrombolytic therapy in 10 patients who had BCS. The authors conclude
that several specific conditions must exist for local lytic therapy to be
264 ZIMMERMAN et al

successful. Primarily, the thrombus must be recent and incompletely occlu-

sive. Total occlusion of the vessel portends a worse prognosis. To achieve
high concentrations of thrombolytic agent, the drug must be delivered either
immediately proximal or within the thrombus. They suggest that tissue plas-
minogen activator is the preferred agent because it has a very short half-life
(5 minutes), and that systemic therapy is of little value.
Balloon angioplasty and stent placement in the IVC and hepatic veins are
useful adjuncts to medical therapy. Stent placement is recommended be-
cause reocclusion occurs frequently after angioplasty [37]. A recent series
of 115 patients who had BCS reported successful stent placement in the
IVC and hepatic veins of 94% and 87%, respectively [38]. Stent patency
with a mean follow-up of more than 45 months was greater than 90%. Clin-
ically, almost all patients who had patent stents improved symptomatically.
Seventeen patients had combined IVC and hepatic vein occlusion. Angio-
plasty and stent placement were undertaken first in the IVC, with the hepatic
vein stent placed 1 week later. During long-term follow-up only one patient
developed hepatic vein occlusion (possibly reocclusion), and all IVC stents
were open. A caveat of this report is that all patients who had hepatic
vein occlusion had obstruction of all three veins. Only one vein was stented
in each case, with a universally successful result. Thus, the entire liver can be
decompressed by intrahepatic venous collaterals.
Although thrombolytic therapy, angioplasty, and stent placement can be
effective in the acute setting, most patients present weeks to months after he-
patic vein occlusion. Thus, formation of an intrahepatic shunt between the
hepatic veins and main branch of the portal vein, transjugular intrahepatic
portosystemic shunt (TIPS), is an extremely effective method of splanchnic
decompression [39]. Indications for the TIPS procedure have been extended
to select patients who have BCS in both elective and emergent situations
[40,41]. Several authors have documented rapid improvement in symptoms
and liver function after successful shunt placement [42,43]. Similar to angio-
plasty and stenting, TIPS does not require surgical exploration and can be
applied in high-risk patients. TIPS also can be employed to manage patients
who have concomitant hepatic outflow obstruction and portal vein throm-
bosis [44].
Few large series exist documenting TIPS placement in patients who have
BCS. Attwell and colleagues [45] at the University of Colorado recently re-
ported their experience in 17 patients who had BCS and undergoing TIPS
placement. With 100% success with stent placement, 82% of patients expe-
rienced improved symptoms or liver function in the short term. Unfortu-
nately, at 3-years follow-up only 47% of patients were clinically well.
Twenty-nine percent of the patients in this series ultimately required liver
transplantation. Because three of these patients presented with fulminant
hepatic failure and were acutely stabilized using TIPS, the authors conclude
that TIPS may serve as a bridge to definitive therapy. Almost one in four
patients experienced a complication, however. Overall, seven patients
 BUDD-CHIARI SYNDROME 265

required shunt revisions, and five patients required shunt replacement.

Long-term success was limited. Recently, Molmenti and colleagues [46] re-
ported a reduction in portal pressures by nearly 44% after TIPS placement
[46]. Long-term follow-up notes that 7 of 11 patients required one or more
shunt revisions. Other groups have reported similar problems [47,48].

Surgical management
Operative intervention for BCS has been in evolution during the last 3 de-
cades. Because the cadaveric organ pool is limited, liver transplantation is
not available to all patients. As a result, the role of portosystemic shunt pro-
cedures has increased. As the complex application of liver transplantation
and shunt surgery for BCS has developed, several basic principles have
emerged. Liver disease is a well-recognized clinical and pathologic contin-
uum. Therefore, it not surprising that the decision to place a shunt between
the portal circulation and the IVC is based on the severity and character of
disease (see Fig. 1). Secondly, a portosystemic shunt does not preclude pa-
tients from liver transplantation in the future. Shunting can be used as
bridge to liver transplantation, because previous shunt surgery has no influ-
ence on outcome following transplantation [49,50]. Finally, these operations
should be undertaken at centers that have a large volume of experience and
a carefully considered multidisciplinary approach.

Portosystemic shunting
Acute hepatic venous outflow occlusion leads to sinusoidal congestion,
hepatocellular necrosis, and ultimately cirrhosis. Fibrosis has been observed
as early as 60 days after the onset of symptoms [7,51]. Portal hypertension,
characterized in the short term by significant abdominal ascites, is a central
feature in this disease. The first report of a portacaval shunt in BCS was
documented by Blakemore [52] in 1948. Thirty years passed before portosys-
temic shunts were shown to be more effective than medical therapy alone
[53]. Five-year survival rates after surgery reportedly have been as high as
90% [5]. As the clinical experience with shunt surgery for BCS accumulates,
it is difficult to interpret because of the heterogeneous nature of the proce-
dures performed. Further, the controls against which shunt patients are
compared ranges from medical therapy or radiologic procedures to liver
transplantation [4,54]. Few studies include more than 40 patients (Table 1).
Nonetheless, important information regarding surgical strategy and overall
outcome in BCS has accumulated over the last decade. Several studies do-
cument the success of mesenteric vein–systemic (mesocaval) venous shunts
(Fig. 2) [28,55]. Five-year survival of up to 75% has been reported with
primary and secondary patency rates of 70% and 85%, respectively [56].
In 1978 Cameron and Maddrey [57] first described the mesoatrial shunt in
a single patient who had IVC thrombosis. With a Dacron graft, a shunt was
266 ZIMMERMAN et al

Table 1
Surgical therapy for Budd-Chiari syndrome: portosystemic shunt versus orthotopic liver
transplantation
Author Liver
[Reference] # in Study Shunt (#) Transplant (#) Comments
Ringe [59] 50 12 43 10-year survival after transplant was
69%. Success rate in shunt group
was only 29%.
Mahmoud [4] 44 16 10 37% of shunt patients died within
30 days of surgery. 90% of patients
died after OLT.
Zeitoun [26] 82 82 0 Overall 10-year survival 57%. Shunt
surgery had no impact on survival
versus medical therapy alone.
Orloff [60] 60 50 7 Overall survival rate of 94% after
porto-caval shunt at a mean follow-
up of 13.6 years. 5-year survival rate
after OLT was 30%.
Slakey [56] 43 43 6 5-year survival rate at least 75% for
both shunt and OLT when
treatment was directed at portal
hypertension versus liver failure.
Xu [58] 1360 1360 0 89% success rate at a mean follow-up
of 6.8 years when type of shunt
procedure was tailored to specific
anatomic defect. Recurrence rate
almost 7%.
Abbreviation: OLT, orthotopic liver transplantation.

constructed between the superior mesenteric vein and the right atrium. Portal
pressures were reduced by 20 cm H2O. A recent surgical experience in more
than 1300 patients, including both mesocaval and mesoatrial shunts, reports
an overall complication rate of 15% and a mortality rate of only 3% [58].
Orloff and colleagues [60] recently reported a prospective series of 60 pa-
tients who had BCS over a 27-year period, treated with a portosystemic
shunt or liver transplantation. Overall, 50 patients underwent a shunt oper-
ation, and 10 were referred for liver transplantation. The authors make sev-
eral important observations. This is the single largest reported series of
patients who had BCS with isolated hepatic vein occlusion treated by
a side-to-side portacaval shunt (n ¼ 32). With a mean follow-up of almost
14 years, 30-day and overall survival rates are 97% and 94%, respectively.
Only 3% of patients in this group have developed ascites or hepatic enceph-
alopathy or have required diuretics in this time interval. Additionally, only
10% of these patients have abnormal liver function tests. Ninety-seven per-
cent had no evidence of parenchymal congestion or hepatocellular necrosis
on biopsy.
A second group of patients had combined hepatic vein obstruction and
IVC occlusion/stenosis (Fig. 3). Over the course of this study the treatment
 BUDD-CHIARI SYNDROME 267

Fig. 2. Mesocaval shunt. Synthetic Gore-Tex H-graft shunt between the superior mesenteric
vein and the vena cava.

strategy for IVC occlusion changed dramatically. Eight patients were

treated with a mesoatrial shunt (Fig. 4). Unfortunately, five of these patients
developed shunt thrombosis and died. With a 5-year survival of 38%, mes-
oatrial shunting was abandoned in favor of a side-to-side portacaval shunt
combined with a cavoatrial Gore-Tex stent graft. Ten patients treated with
the combined approach have 30-day and overall survival rates of 100% with
mean follow-up of 9 years. Finally, the side-to-side portacaval shunt had an
overall thrombosis rate of 0.2%. The authors conclude that, once the diag-
nosis of BCS is made, there is no reason to delay portal decompression in
the absence of end-stage liver disease. When performed early, portacaval
shunting can stop or reverse ongoing parenchymal injury.

Orthotopic liver transplantation

Liver transplantation is the preferred treatment for patients who have
BCS and acute fulminant failure or cirrhosis and decompensated disease.
Since the first successful transplantation in a patient who had BCS was
268 ZIMMERMAN et al

Fig. 3. Magnetic resonance venogram with IVC stenosis. (A) Inferior vena cava obstruction
(arrow) is shown at the level of the caudate lobe. (B) Caudate lobe hypertrophy (arrow) is noted
inferior to segment 5.

reported in 1976 [61], several small studies have documented acceptable

long-term survival rates [6,56,62]. In a series of 43 patients who had BCS,
Ringe and colleagues [59] reported 10-year survival of nearly 70% following
transplantation. The authors’ experience at UCLA reveals an actuarial sur-
vival rate of 76% at 3 years following liver transplantation [54]. Long-term
laboratory data suggest a slow progression of hepatic disease in shunted pa-
tients. Conversely, patients receiving a transplant maintain higher serum al-
bumin levels and improved synthetic function. Although a cadaveric organ
shortage exists in most countries around the world, many groups are refin-
ing a treatment algorithm that begins with conservative management. Med-
ical therapy and interventional techniques are used early in the disease
process before the patient develops end-stage decompensation. A recent se-
ries notes successful conservative therapy for up to 8 years before transplan-
tation [63].
Unfortunately, lifelong anticoagulation therapy is recommended after
transplantation because BCS does recur [64]. Re-emergence of outflow ob-
struction has been observed from 4 months to 7 years after transplantation
[6]. A series of 23 patients from the University of Pittsburgh receiving trans-
plants for BCS notes three recurrences [65]. All three patients died. The re-
currence rate of outflow occlusion has been reported to be as high as 10%
and may require retransplantation [13]. Technical concerns at the time of
transplantation center around prior shunt placement and caudate lobe hy-
pertrophy. Pre-existing portacaval shunts can make the hilar dissection par-
ticularly challenging. At UCLA, all patients who have significant portal
hypertension and prior shunt surgery are placed on venovenous bypass
(both peripheral and portal). This bypass allows portal decompression
 BUDD-CHIARI SYNDROME 269

Fig. 4. Mesoatrial shunt. Gore-Tex graft from the superior mesenteric vein to the right atrium
of the heart.

during the dissection, thus reducing blood loss and preserving hemodynamic
stability. Caudate lobe enlargement may make mobilization of the liver ex-
tremely difficult. Optimal exposure is mandatory.

Comments
BCS is a spectrum of disease processes characterized by hepatic venous
outflow occlusion, acute hepatic parenchymal congestion and inflammation,
and ultimately liver dysfunction and cirrhosis. Untreated, BCS is an ex-
tremely morbid and lethal condition. Early experience indicates that medical
therapy alone is unsuccessful in most patients. Liver transplantation is the
preferred treatment in the setting of decompensated disease. With a limited
cadaveric organ pool, however, alternate interventions including TIPS and
portosystemic shunts have been employed increasingly. These procedures
may serve as a bridge to transplantation when they are instituted in the acute
270 ZIMMERMAN et al

setting, in patients who have preserved hepatocellular function. Patients

should be listed for transplantation when cirrhosis and decompensation are
documented. Currently, living-donor liver transplantation is becoming
a viable treatment option for relatively healthy patients who have end-stage
liver disease. Unfortunately, anatomic abnormalities including caval stenosis
may require complete resection of the IVC at the time of transplantation.
Vascular reconstruction of the hepatic veins and IVC may render living-
donor liver transplantation a practical alternative to cadaveric transplanta-
tion for BCS in the future.

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