DOI:10.2214/AJR.07.3496
AJR 2008; 191:560-564
© American Roentgen Ray Society
Percutaneous Sonographic Guidance for TIPS in Budd-Chiari Syndrome: Direct Simultaneous Puncture of the Portal Vein and Inferior Vena Cava
Fatih Boyvat1,
Ali Harman1,
Umut Ozyer1,
Cuneyt Aytekin1 and
Zubeyde Arat2
1 Radiology Department, Baskent University, Fevzi Cakmak Cad. 10. Sok. No. 45
06490, Ankara, Turkey.
2 Nephrology Department, Baskent University, Ankara, Turkey.
Received December 4, 2007;
accepted after revision February 23, 2008.
Address correspondence to F. Boyvat
(boyvatf{at}yahoo.com).
Abstract
OBJECTIVE. Budd-Chiari syndrome (BCS) is a clinical condition
characterized by hepatic venous outflow obstruction. A transjugular
intrahepatic portosystemic shunt (TIPS) is an effective means of decompressing
the portal system in patients unresponsive to traditional medical therapy.
TIPS may be difficult in patients with BCS owing to the presence of hepatic
venous occlusive disease. We present our experience using direct percutaneous
simultaneous puncture of the portal vein and the inferior vena cava to place a
TIPS in patients with BCS.
MATERIALS AND METHODS. Between September 2003 and October 2006,
percutaneous sonographically guided TIPS was performed on 11 patients (five
women and a girl, four men and a boy; age range, 6–43 years).
Indications for the TIPS procedure were intractable ascites in nine patients
and intractable ascites and variceal bleeding in two patients.
RESULTS. Technical success was achieved in all patients. The mean
portosystemic pressure gradient was reduced from 23.5 to 9.8 mm Hg. The
cumulative rate of primary patency was 60% at 1 year. Nine revisions were
performed in five patients. In nine of the 11 patients, ascites resolved
completely, and in two patients, it was relieved.
CONCLUSION. Excellent technical and clinical success can be achieved
with percutaneous sonographically guided direct simultaneous puncture of the
portal vein and inferior vena cava in patients with BCS.
Keywords: ascites • Budd-Chiari syndrome • hepatic vein • stent • transjugular intrahepatic portosystemic shunt
Introduction
Budd-Chiari syndrome (BCS) is a heterogeneous group of disorders
characterized by obstruction of hepatic venous outflow at any level from the
small hepatic veins to the junction of the inferior vena cava (IVC) and the
right atrium. Venous stasis and congestion lead to centrilobular fibrosis,
nodular regenerative hyperplasia, and ultimately liver cell loss and cirrhosis
[1,
2].
The clinical presentation of BCS varies depending on the extent and
rapidity of obstruction of the hepatic vein and on the presence of collateral
veins that decompress the liver sinusoids. According to the clinical
presentation, BCS can be classified into four categories: fulminant, acute,
subacute, and chronic [3].
Patients with the fulminant presentation have hepatic encephalopathy within a
few weeks after the development of jaundice; this form is less common than the
others. The acute form is characterized by intractable ascites and hepatic
necrosis. There is no time for the venous collateral vessels to develop. The
subacute form, which is the most common, is characterized by the presence of
collateral veins, minimal areas of hepatic necrosis, and ascites. The chronic
presentation is indolent development of ascites, portal hypertensive bleeding,
or both.
The primary goal of treatment is to eliminate the morbidity and mortality
associated with hepatic congestion. Medical management is aimed at controlling
further development of ascites, preventing further extension of venous
thrombosis, and managing the underlying cause
[4]. Decompression of the
hepatic venous system remains an option. The options for patients unresponsive
to medical treatment are transjugular intrahepatic portosystemic shunt (TIPS),
membranotomy, radical resection of membrane and thrombus, surgical shunt, and
liver transplantation. TIPS is an effective technique in the treatment of
patients with BCS because it decompresses the congested liver. Caudate lobe
enlargement does not compromise TIPS efficacy. TIPS also can serve as a bridge
to liver transplantation
[5].
The most critical and difficult part of TIPS placement is gaining access to
the portal vein. Because patients with BCS have occluded hepatic veins,
puncture from the IVC through the liver to the portal vein is necessary.
Although a TIPS can be placed in most patients with BCS, much time and effort
can be spent attempting to gain access to the portal vein. In addition,
complications during the procedure are associated with puncture and access to
the portal vein. Boyvat et al.
[6] described a method of TIPS
insertion designed to overcome the problems associated with portal venous
puncture performed by the transjugular route alone. The technique involves
percutaneous insertion of a needle under sonographic guidance through a portal
venous branch and then into the IVC to facilitate insertion of a guidewire,
which is snared through a jugular venous puncture. We present our experience
with this technique of percutaneous sonographically guided direct simultaneous
puncture of the portal vein and the IVC for performance of a TIPS procedure in
patients with BCS.
Materials and Methods
Eleven patients (five women and a girl, four men and a boy; mean age, 26
years; range, 6–43 years) were referred to our interventional radiology
service for a TIPS procedure because of intractable ascites in nine cases and
intractable ascites with variceal bleeding in two cases. The diagnosis of BCS
was based on clinical presentation, radiologic findings, and results of liver
biopsy. The clinical and demographic characteristics are shown in
Table 1.
Screening Doppler sonography was performed to delineate the anatomic
features and predict the possible route of percutaneous simultaneous puncture
of the portal vein and IVC. Patients were given local anesthesia, and the
procedures were performed with the patient under conscious sedation. The right
side of the patient's neck (at the entrance of the right internal jugular
vein) and the abdomen were prepared for percutaneous puncture. Percutaneous
punctures were performed with an 18-gauge, 20-cm Chiba needle under real-time
sonographic guidance.
Punctures were made into the right portal vein to the IVC in three cases or
the left portal vein to the IVC in eight cases by a single operator using a
freehand technique. In the three patients in whom right portal venous puncture
was performed, an intercostal approach was necessary so that ascitic fluid
could be drained before the procedure to facilitate percutaneous puncture. We
preferred left portal venous puncture because it was technically easy. Left
portal venous puncture was performed on the main left portal venous trunk
where it passes anteriorly before dividing into the branches of liver segments
II and III. Right portal venous puncture to the right anterior portal vein was
performed close to the right portal venous trunk. When portal venous puncture
was accomplished, contrast medium was injected for visualization of the
location of the needle tip, and the needle was further advanced without a
change in the insertion angle to puncture the IVC (Figs.
1A,
1B, and
1C).
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Fig. 1A —43-year-old woman with Budd-Chiari syndrome in Child-Pugh
class B and intractable ascites. Subcostal sonographic image shows left portal
vein (thin arrow) and inferior vena cava (thick arrow) in
same plane.
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The position of the needle was again confirmed with injection of contrast
medium. A stiff 0.035-inch guidewire was advanced through the Chiba needle to
the IVC. Guidewire manipulation was kept to a minimum to reduce the risk of
shearing the coating of glidewires introduced through a bevelled needle. At
this stage, an 8-French sheath was placed into the IVC through the right
internal jugular vein. An Amplatz gooseneck snare was introduced through the
right internal jugular vein to capture the glidewire to achieve
through-and-through access.
The Chiba needle was pulled back 2 cm to dilate the intrahepatic track with
an 8-mm ultrathin balloon advanced through the 8-French sheath. When the
balloon catheter was advanced to the intrahepatic track, sonography was
performed to determine that the balloon was not advanced beyond the entrance
to the portal vein. An 8-French sheath was advanced to the entrance of the
portal vein. A 5-French cobra or Davis catheter and a 0.035-inch glidewire
were introduced through the sheath, and both catheter and glidewire were
manipulated into the main portal vein and the superior mesenteric vein.
Heparin (5,000 IU) was administered IV. The through-and-through glidewire was
kept in place for safety. After portography and pressure measurement, a
0.035-inch Amplatz guidewire was advanced to the portal system. The hepatic
track was again dilated with an 8-mm balloon. The through-and-through
glidewire was removed, the Chiba needle was withdrawn near the liver capsule,
and the track was embolized with a mixture of n-butyl cyanoacrylate
(Histoacryl, Braun) and iodized oil (Lipiodol, Guerbet) at a1:1 ratio.
The intrahepatic track was stented, and 15 stents (Wallstent, Boston
Scientific) were used in 11 patients (four of the patients had two stents).
The stents used during the procedure measured 10 x 42 mm in three cases,
10 x 68 mm in six cases, and 10 x 94 mm in six cases. All stents
were bare stents, and they were extended into the upper retrohepatic vena cava
above the compressed IVC so that they were directed upward. The stent in
patient 5 was dilated to 8 mm, and the others were dilated to 10 mm.
Portography was performed, and portal vein and IVC pressures were measured
again.
The patients received heparin IV for anticoagulation, and administration of
warfarin was started the day after the procedure. Control Doppler sonography
was performed on days 1 and 7 after the procedure, then monthly for the first
3 months and every 3 months thereafter.
Results
TIPS placement was technically successful in all patients. The mean
portosystemic pressure gradient was reduced from 23.45 ± 7.3 to 9.82
± 4.6 mm Hg. Percutaneous punctures were performed either from the
right portal vein to the IVC in three cases or from the left portal vein to
the IVC in eight cases. The IVC was compressed in two patients and occluded in
one patient, so in those cases, sonographic visualization was not possible for
percutaneous puncture. For this reason, IVC recanalization was performed
before the TIPS procedure, and a balloon was placed to the suprahepatic and
intrahepatic IVC. This balloon was used as a target during the percutaneous
puncture (Figs. 2A,
2B, and
2C).
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Fig. 2A —6-year-old girl who underwent percutaneous shunt placement
because of variceal bleeding and ascites. Sonographic image shows needle tip
was in left portal vein (black arrow). Balloon catheter was placed in
inferior vena cava to facilitate percutaneous puncture (white
arrows).
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Fig. 2C —6-year-old girl who underwent percutaneous shunt placement
because of variceal bleeding and ascites. Portal venogram after transjugular
portosystemic shunt placement shows excellent shunt.
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Because of the age (4 years) of patient 5, the stent was dilated to a
diameter of 8 mm, reducing the portacaval pressure gradient from 14 to 4 mm
Hg. Ascites did not resolve completely, so a stent was placed into the IVC 10
days after the TIPS procedure. In patient 4, placement of the TIPS was
followed by an immediate increase in preload, and transient right heart
failure developed. The symptoms responded quickly to treatment, and the
patient was discharged from the hospital 3 days after the TIPS procedure.
Although we started IV heparin during the procedure and warfarin therapy the
next day to keep the international normalized ratio between 2 and 3, two early
stent occlusions (first and third days) were detected and were managed with
thrombolysis and percutaneous transluminal angioplasty. These early stent
occlusions might have been caused by the hypercoagulable state of these
patients. Patient 5 had factor V Leiden mutation, but no gene mutation was
found in patient 4.
The cumulative rate of primary stent patency was 60% 1 year after the TIPS
procedure; the secondary stent patency rate was 100%.
Table 2 shows the pressure
gradients before and after TIPS placement, the number of revisions, and the
duration of patency. TIPS dysfunction occurred in five (45%) of 11 patients
during the follow-up period. Three of these patients underwent one revision,
and two patients had three revisions. The other six patients did not need
shunt revision. Among the patients with TIPS dysfunction, six patients had
shunt occlusion, and three had shunt stenosis. Five cases of TIPS dysfunction
responded to angioplasty alone; in four cases an additional bare stent was
needed. The transjugular approach was performed in seven cases of shunt
dysfunction. In the other two cases, occlusion was due to stent retraction,
and jugular access was not possible, so sonography- and fluoroscopy-guided
percutaneous puncture was performed to gain access to the stents. The
guidewire was snared through the right internal jugular vein, and endovascular
intervention was performed through this access.
In nine of 11 patients, ascites resolved completely, and in the other two
it was relieved. Patient 6 underwent orthotopic liver transplantation because
progressive deterioration of liver function was detected during 8 months of
follow-up.
Discussion
The therapeutic approach to BCS includes medical management and relief of
hepatic venous outflow tract obstruction. When medical therapy alone is
insufficient, a TIPS or surgical portosystemic shunt may be indicated.
Surgical shunts can be very successful, but hemodynamic and anatomic factors
can limit this option [7,
8]. A side-to-side portacaval
shunt in cases of hepatic venous occlusion corrects the hemodynamic
abnormalities, but caudate lobe hypertrophy presents a technical
contraindication to this shunt. When BCS involves thrombosis or occlusion of
the IVC, cavoatrial shunting in combination with a side-to-side portacaval
shunt has been used, but mesoatrial shunts are associated with increased risk
of thrombosis [9,
10]. TIPS placement has been
found effective for relieving portal hypertension and hepatic congestion. If
the caudate lobe compresses the IVC, a TIPS is more technically feasible than
a surgical shunt. Even though portal venous decompression may be successful
after a TIPS procedure, hepatic function may deteriorate, and liver
transplantation may be necessary.
The most critical and difficult part of TIPS placement is gaining access to
the portal vein. Sonographic guidance can be used to locate the portal vein
and facilitate the procedure
[11,
12]. Intravascular sonographic
guidance for placement of a direct portacaval shunt has been described by
Petersen and Binkert [13] but
requires special equipment that adds cost to the procedure. Even though there
are complementary imaging techniques, these techniques may not always increase
the success of the procedure. In patients with BCS, the standard TIPS
procedure is performed through the ostial remnant of the hepatic vein or by
direct puncture of the intrahepatic portion of the IVC. The first TIPS
placement in a patient with BCS was performed in 1993 by Peltzer and
associates [14]. One problem
with conventional TIPS techniques in BCS is difficulty puncturing the portal
vein, and this difficulty can lead to long fluoroscopy times.
Percutaneous sonographically guided direct simultaneous puncture of the
portal vein and vena cava has been performed with good outcome in one patient
[6]. The patient had no shunt
dysfunction during 21 months of follow-up, but the authors did report stent
shortening. In our series, patients were treated exclusively with this
technique. We encountered six cases of shunt occlusion and three of shunt
stenosis; two of the shunt occlusions were caused by stent shortening. Despite
maintenance of anticoagulation treatment, the probability of TIPS occlusion or
stenosis necessitating shunt revision was similar to that in other series
[2,
3]. This technique facilitates
easy, straightforward, and accurate puncture of the portal vein and IVC
simultaneously to prevent complications related to portal venous puncture.
This technique decreases fluoroscopy time, and in cases in which portal venous
puncture is not possible, operator difficulty is reduced.
TIPS is effective in decreasing portal venous pressure, improving liver
function, and controlling ascites. An important factor limiting the success of
TIPS is stenosis of the shunt
[15]. Molmenti et al.
[16] and Ryu et al.
[17] found TIPS particularly
useful in the treatment of BCS patients but not as durable as liver
transplantation. Because TIPS dysfunction is common, repeated intervention is
frequently necessary to keep the shunt open. Polytetrafluoroethylene
(PTFE)-covered stentgrafts for TIPS in patients with BCS have been described
by Hernandez-Guerra [2] and
Gandini [3] and their
associates. Those authors concluded that PTFE-covered stentgrafts increased
primary patency significantly more than did bare stents, and PTFE-covered
stent-grafts had a lower dysfunction rate and necessitated fewer repeated
interventions. PTFE-covered stent-grafts would probably increase the incidence
of IVC occlusion if the covered part were to extend into the IVC. We did not
use PTFE-covered stent-grafts because our patients' insurance systems did not
pay for the use of PTFE-covered stents.
Our early experience suggests that percutaneous sonographically guided
direct simultaneous puncture of the portal vein and IVC is an effective
technique for performing a TIPS procedure.
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Abstract
Introduction
