Open Journal of Gastroenterology, 2013, 3, 1-4 OJGas Published Online February 2013 (
Successful transjugular intrahepatic portal-systemic shunt
in an ineligible liver transplant patient with primary
biliary cirrhosis with refractory ascites and aplastic anemia
Christopher M. Moore1, George Behrens2, Hector Ferral2, David H. Van Thiel1
1Section of Hepatology, Department of Medicine, Rush University Medical Center, Chicago, USA
2Section of Interventional Radiology, Department of Radiology, Rush University Medical Center, Chicago, USA
Received 24 October 2012; revised 24 November 2012; accepted 1 December 2012
A transjugular intrahepatic portal-systemic shunt
(TIPS) is a standard way to decompress the portal
system in cirrhotic patients as a bridge to orthotopic
liver transplantation (OLT). Traditionally, TIPS has
been indicated for certain portal hypertensive seque-
lae such as refractory ascites, varices treatment and
even hepato-hydrothorax. Herein is a case report on
the efficacy of TIPS in an OLT ineligible patient with
primary biliary cirrhosis and aplastic anemia who
had developed refractive ascites requiring serial
paracentesis and esophageal varices. He survived 2.5
years post-TIPS placement and died from complica-
tions related to severe leucopenia and the develop-
ment of sepsis.
Keywords: Aplastic Anemia; Ascites; Liver
Transplantation; Paracentesis; TIPS
The Transjugular intrahepatic portal-systemic shunt ( TIP S)
is a standard interventional radiological technique that is
utilized in cirrhotic patients to treat major complications
of portal hypertension such as refractory ascites and
hepato-hydrothorax, secondary prevention of esophageal
varices, and primary prevention of gastric varices [1-4].
TIPS is not a cure for decompensated liver disease, but
rather conceived of as a bridge to orthotopic liver trans-
plantation (OLT) [3-5].
OLT is a complex stand ard-of-care surgery for eligible
chronic decompensated liver disease and fulminant liver
failure patients [4]. The success of this technology has
rested upon improvements in immunosuppressive thera-
pies, surgical technique and appropriate patient selection.
The MELD score [1-3], albeit imperfect, has been in-
strumental in risk stratifying patients by overall 90 day
mortality. The score is the product of a mathematical
equation comprising serum bilirubin, creatinine and INR
that is updated periodically on transplant eligible pa-
In brief, the TIPS procedure bypasses the hepatic pa-
renchyma by means of a metallic stent directly linking a
branch of the portal vein to a hepatic vein with resultant
decompres sion of the portal system and high blood flow
into the cardiopulmonary system [3,4]. Given the tech-
nical challenges of TIPS, and the expected physiologic
changes induced, eligible patients must fulfill a number
of criteria including absence of sepsis, absence of severe
cardiopulmonary disease, and few prior episodes of he-
patic encephalopathy (HE). More comprehensively, it
has been determined that the best candidates for TIPS are
usually those patients with a Ch ilds-Turcotte-Pugh (CTP)
class of A, or a model for end-stage liver disease (MELD)
score of <14 [1-3]. TIPS has known side-effects includ-
ing bleeding, infection, stent malposition/thrombosis, HE,
abdominal pain and hepatic injury/necrosis.
Herein is a report of a case for the successful utiliza-
tion of a TIPS procedure in a patient with decompensated
primary biliary cirrhosis (PBC), deemed ineligible for
OLT given the high morbidity and mortality of his aplas-
tic anemia.
A 62-year-old white ma le with underlyin g aplastic ane mia
and PBC developed portal hypertensive sequelae includ-
ing diuretic-resistant refractory ascites and esophageal
varices. His diagnosis of aplastic anemia was made in
2007. He had been treated previously for the aplastic
anemia with sequential therapies consisting of steroids,
rituximab, and cyclosporine. He was receiving intrave-
nous immunoglobulin therapy at the time of his referral.
His diagnosis of PBC was based upon a combination of
data, including: an elevated serum alkaline phosphatase,
anti-mitochondrial antibody positivity, increased total
serum cholesterol and IgM levels, and a computed to-
C. M. Moore et al. / Open Journal of Gastroenterology 3 (2013) 1-4
mography (CT) scan documented hepatosplenomegaly
with increased hepatic heterogenicity.
Approximately 1 year prior to our initial evaluation, he
developed portal hypertensive ascites which was initially
responsive to sodium diet restriction and diuretic therapy,
consisting of a combination of furosemide and spiro-
nolactone at 120 mg and 300 mg per day, respectively.
However, his blood urea nitrogen (BUN) and serum
creatinine levels increased over time, reflecting worsen-
ing kidney injury, such that he became diuretic-resistant.
He consequently required serial large-volume paracente-
sis (LVP) every two weeks for a three-month duration in
order to manage the rapidly re-accumulating ascitic fluid
in the setting of inadequate diuresis. Additionally, he
underwent standard esophagogastric-duodenoscopy (EGD )
for varices surveillance, and was found to have large
esophageal varices which were band ligated. Subsequent ly,
he was maintained on beta-blocker therapy for variceal
prophylaxis. He has never had a variceal bleeding epi-
sode. He has never had an episode of HE. Given his
known diagnosis of aplastic anemia, the referring heap-
tologists were concerned about the risks of future eso-
phageal variceal bleeding and consequence of continued
LVP upon his physiologic reserve capacity and state of
nutrition. Thus, he was referred for more definitive
treatment in the form of OLT. Additionally, it was noted
that he was quite malnourished with severe generalized
muscle wasting and a markedly protuberant abdomen
with large-volume ascites. His overall medical history
was reviewed thoroughly by the multi-disciplinary liver
transplant team, which declined him for OLT because of
his aplastic anemia and a related life expectancy of less
than 5 years even with an OLT.
Following this decision, he was evaluated for a TIPS
procedure recognizing that it would not be as a bridge
therapy to OLT, but rather, that it would simplify his
continued medical management and reduce the catabolic
state in the setting of portal hypertension and aplastic
anemia. He underwent a diagnostic and therapeutic LVP,
with removal of 2.1 L of culture negative ascitic fluid,
containing 10 white blood cells (WBC) per mm3, and an
ascitic albumin level of 2.0 g/d, which when subtracted
from his serum albumin level yielded a serum ascites-
albumin gradient (SAAG) of 1.2, consistent with his di-
agnosis of portal hypertension. A triple-phase CT scan
re-demonstrated findings consistent with advanced cir-
rhosis with arterial-venous shunts, a few hepatic cysts,
no focal lesions, and patent hepatic, portal and splenic
veins, as well as large esophageal varices.
Based upon these findings and further evaluation yi eld-
ing no significant cardiopulmonary disease, a TIPS pro-
cedure was offered to the patient, who at the time had the
following laboratory values, listed in Ta bl e 1 . The TIPS
procedure was performed in standard fashion with the
implementation of 28 cm × 10 mm Viatorr© polytetra-
fluoroethylene (PTFE)-covered stent without any com-
plications. Pre- and post-TIPS hepatic pressures as well
as the hepatic wedge pressure gradient are documented
in Table 2 and demonstrate adequate portal venous de-
compression. The patient did well post-TIPS procedure
and was discharged home the following day.
Table 1. Clinical laboratory findings in the patient.
Pre-TIPS Day of TIPS Day after TIPS Normal value
WBC count 0.81 1.27 1.45
4 - 10 × 103/µl
Hemoglobin 7.6 9.7 10.7
13.5 - 17.5 g/dl
MCV 94.3 90.8 90.1 82 - 103 fl
Platelet count 42 35 26
150 - 399 × 103/µl
BUN 34 30 29
8 - 21 mg/dl
Creatinine 1.1 1.1 1.1 0.6 - 1.3 mg/dl
Blood glucose 94 135 93 60 - 109 mg/dl
Total protein 7.8 5.6 5.5
6.0 - 8.2 g/dl
Albumin 3.4 2.5 2.5
3.5 - 5.0 g/dl
Total bilirubin 1.3 1.3 1.4 0.2 - 1.3 mg/d
AST 20 35 36 3 - 44 U/l
ALT 13 20 24 0 - 40 U/l
Prothrombin time 13.7 X 15.7 9.5 - 14.0 s
INR 1.17 X
1.39 0.83 - 1.23 (no unit)
aPTT 34 X X 23 - 33 s
WBC = white b l o od cells; MC V = mean corpuscular volume; BUN = blood urea nitrogen; AST = aspar t ate aminotransferase; ALT = alanine ami-
notransferase; INR= i n t ernational normalized r atio; aPTT = activated partial thromboplastin time; X = not available. Abnormal data in bold font.
Copyright © 2013 SciRes. OPEN ACCESS
C. M. Moore et al. / Open Journal of Gastroenterology 3 (2013) 1-4 3
Table 2. Calculated portal vein pressures (mmHg).
Pre-TIPS Post-TIPS Normal value
Hepatic vein 19 20 <5
Wedged portal vein 32 27 5 - 10
Gradient 13 7 0 - 5
At a follow-up outpatient visit two weeks later, an ab-
dominal ultrasound (US) with a complete duplex Dop-
pler study was performed and documented peak systolic
velocities at the portal vein end of the TIPS, mid TIPS,
and hepatic vein end of the TIPS of 57, 163 and 119
cm/sec with no high velocity jets, respectively. In the
following weeks, the patient’s ascites resolved, and his
diuretic requirements were reduced to 20 mg furosemide
and 50 mg spironolactone per day in the setting of stable
kidney disease and an adequate diuresis. In the post-TIPS
state, he no longer required paracentesis, his esophageal
varices were decompressed, his nutritional state and
overall clinical appearance improved. Most importantly,
he did not develop any complications of his TIPS proce-
dure and survived an additional 2.5 years at which time
he died as a result of severe leucopenic sepsis.
Cirrhosis is manifested by an extensive fibronodular re-
placement of the liver parenchyma with resultant portal
hypertension and progressive synthetic dysfunction [4].
The development of portal hypertension leads to a num-
ber of complications including HE, ascites and variceal
bleeding, which are each associated with an increased
morbidity and mortality rate [2-5]. Ascites is treated with
a combination of dietary sodium restriction and diuretics,
primarily consisting of furosemide and spironolactone. In
approximately 10% of ascites patients, a diagnosis of
refractory ascites develops wherein they no longer re-
spond to or cannot tolerate these diuretic therapies. In
such cases, LVP is instituted, and while efficacious, is
associated with complications consisting of peritoneal
bleeding, infection, and increased catabolic rate. Fur-
thermore, there are ongoing concerns of long-term cost
and quality-of-life issues [2,3,6].
It is within this setting that the theory and implementa-
tion of TIPS arose. TIPS provides a non-surgical method
of delivering blood from the portal to hepatic venous
system through the utilization of a transhepatic bypass
stent [3]. It is generally accepted that a TIPS procedure is
utilized best in patients having been classified as CTP
class A patient or with a MELD score < 14 who is await-
ing OLT, and who has experienced a number of portal
hypertensive complications consisting of refractory as-
cites or variceal bleeding. Other indications for a TIPS
include the development of a hepato-hydrothorax, al-
though experience with this problem is less-well docu-
mented [3-5]. Furthermore, given the expected physiol-
ogic effect of TIPS a number of contra-indications to its
implementation have been identified such as congestive
heart failure and severe pulmonary hypertension. While
TIPS has been successful in ameliorating the complica-
tions of portal hypertension, its long-term effectiveness,
effect on quality-of-life, and health-care cost have also
been matters of debate when compared to serial LVP [2,3,
6]. In part, some of these issues revolv e around the stud-
ies of uncovered TIPS stent compared to LVP, whereas in
the current period covered TIPS stents, which are less
likely to become occluded, are the standard-of-care [7].
In this case report, the patient had several common
portal hypertensive sequelae of his PBC, including eso-
phageal varices and refractory ascites. Furthermore, the
ascites required serial LVP, which despite being a fairly
common procedure, is nevertheless associated with com-
plications and costs that are substantial over time. Worse
still is the patient’s underlying aplastic anemia, a d isease
process itself manifested by pancytopenia including
symptomatic anemia, hemorrhage, and a risk of infection.
In addition, there is also an increased risk for the devel-
opment of hematologic cancer [8]. It was this underlying
disease process and its associated morbidity and mortal-
ity that both excluded the patient from OLT considera-
tion and made continued LVP a prohibitive option given
the well-recognized probability of complications over
Given these facts, the present case report is interesting
for several reasons. Firstly, it is as best as we can deter-
mine the only case in which a TIPS procedure has been
performed in a cirrhotic individual with underlying apla s-
tic anemia. Importantly, the TIPS procedure was suc-
cessful in reducing his ascites and the need for repetitive
LVP as well as decompressing his esophageal varices.
Importantly, no complications were experienced in the
procedure or experienced in his post-procedural follow-
up. Moreover, he was able to reduce his diuretic re-
quirements and reduce his catabolic state. The benefits to
his esophageal varices were important, as any future
bleeding episodes would be complicated not only by his
baseline anemia and thrombocytopenia, but also in the
difficulty of finding an appropriate blood match given
the number of anti-platelet antibodies he had developed
over the preceding years of transfusion.
As he no longer required LVP, his nutrition status, as
assessed by physical appearance improved. LVP results
in significant ascitic fluid protein losses when compared
to diuretic therapy [9]. Additionally, in the post-TIPS
setting in the absence of ascites there is an associated
increased mobility, energy and protein intake compared
to continued LVP [10]. Post-TIPS, his quality-of-life im-
proved, a general finding well-documented in the litera-
Copyright © 2013 SciRes. OPEN ACCESS
C. M. Moore et al. / Open Journal of Gastroenterology 3 (2013) 1-4
ture [6], although this metric was not quantitatively stu-
died in the patient reported. The underlying aplastic
anemia did not precipitate any perceived complications
regarding the TIPS procedure per se or his clinical status
afterwards until his death as a result of leucopenic sepsis
2.5 years later. These outcomes support the pragmatism
of the therapeutic go als: that he was appropriately denied
an OLT given his underlying high-mortality state, but
that he could benefit clinically from a non-standard indi-
cation TIPS procedure given the severity of his portal
hypertension and aplastic anemia. Organ allocation is
especially stringent for OLT given the relative paucity of
available donors to recipients.
In summary, this case report details the non-standard
application of a TIPS procedure in a PBC OLT ineligible
patient with aplastic anemia and refractory ascites re-
quiring serial LVP. The TIPS procedure was effective in
ameliorating sequelae of his portal hypertension and was
accomplished without any complications related to his
underlying aplastic anemia. Careful application of such a
procedure, although not traditionally indicated for such a
patient as in this case report, can have reasonable bene-
fits upon quality of life, and possibly even health-care
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