DOI:10.2214/AJR.07.3534
AJR 2008; 191:1751-1757
© American Roentgen Ray Society
Causes of TIPS Dysfunction
Marco Cura1,
Alejandro Cura2,
Rajeev Suri1,
Fadi El-Merhi1,
Jorge Lopera1 and
Ghazwan Kroma1
1 Cardiovascular and Special Interventions, Department of Radiology, The
University of Texas Health Science Center, Mail Code 7800, 7703 Floyd Curl
Dr,, San Antonio, TX 78229-3900.
2 Department of Radiology, Hospital de Clinicas, Jose de San Martin, University
of Buenos Aires, Buenos Aires, Argentina.
Received December 11, 2007;
accepted after revision June 22, 2008.
Address correspondence to M. Cura
(curam{at}uthscsa.edu).
CME
This article is available for CME credit. See
www.arrs.org
for more information.
Abstract
OBJECTIVE. Transjugular intrahepatic portosystemic shunt (TIPS)
creation is an effective method to control portal hypertension. TIPS creations
with bare stents have shown limited and unpredictable patency. In nearly all
cases of rebleeding or recurrent ascites after TIPS creation, there is shunt
stenosis or occlusion. The purpose of this article is to review the biologic
and technical factors that predispose to TIPS failure and how the use of an
expandable polytetrafluoroethylene (PTFE)-covered-stent has significantly
improved TIPS patency.
CONCLUSION. Biologic and technical factors may predispose to shunt
failure. The combination of improved technique and expandable PTFE has
significantly improved TIPS patency. The need for follow-up venography and
secondary interventions has been reduced significantly as a result of improved
shunt patency.
Keywords: covered stent • interventional radiology • portal hypertension • transjugular intrahepatic portosystemic shunt
Introduction
The transjugular intrahepatic portosystemic shunt (TIPS) is an effective
method to control the complications of portal hypertension; however, shunt
dysfunction is common. Some shunts may remain patent, whereas others develop
stenoses and thromboses. Recurrent portal hypertension with stenoses greater
than 50% develop in 25–50% of cases 6–12 months after TIPS
creation when bare stents are used
[1–5].
Solving the problems of shunt dysfunction decreases the absolute rates of
rebleeding and recurrent ascites after TIPS and the number of required shunt
revisions. Although many lining materials have been tested as covers for TIPS
stents, only expandable polytetrafluoroethylene (PTFE) grafts appear to
address the biologic causes of shunt dysfunction and provide better patency
rates than TIPS created with bare stents.
Causes of TIPS Dysfunction
Excluding early shunt failure due to technical causes such as stent
shortening or migration, causes of shunt dysfunction in TIPS created with bare
stents include bile-related, non-bile-related, and hepatic vein stenosis
[6]. During TIPS creation, bile
ducts may inadvertently be traversed during needle passes from the hepatic
vein to the portal vein [7]
(Fig. 1). Transected bile ducts
have been associated with TIPS stenosis and occlusion
[8]. Transection of a bile duct
creates communication between the bile ducts and the TIPS lumen, a
biliary–TIPS fistula. The content of bile acids, salts, cholesterol, and
phospholipids makes bile thrombogenic and proinflammatory
[9]. Bile also appears to delay
tract healing by inhibiting smooth muscle proliferation
[10]. Histopathologic analysis
of occluded shunts showed bile pigment in thrombus adherent to a transected
bile duct associated with a granulomatous inflammatory response containing
foreign-body-type giant cells
[11]. Biliary–TIPS
fistulas present with acute thrombosis and recurrent occlusions
[12]
(Fig. 2).
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Fig. 1 —45-year-old man with hepatitis C cirrhosis and variceal
bleeding. Fluoroscopic image shows transjugular puncture of left bile duct
during transjugular intrahepatic portosystemic shunt procedure.
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Fig. 2 —51-year-old man after transjugular intrahepatic portosystemic
shunt (TIPS) who presented with recurrent variceal bleeding. On fluoroscopic
image, contrast injection during revision of thrombosed TIPS shows
opacification of biliary tree.
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Later, nonthrombogenic parenchymal tract stenosis or pseudointimal
hyperplasia is the result of proliferation of myofibroblasts that increase in
number over time [13]. The
pseudointimal hyperplasia responsible for TIPS stenosis has similarities to
the intimal hyperplasia responsible for arterial restenosis after vascular
recanalizations [14]. The
predominant cause of intimal hyperplasia after arterial stent placement is
proliferation and migration of smooth muscle cells from the media. The liver
parenchyma contains no smooth muscle cells; the fibroblasts of the liver
stroma differentiate into myofibroblasts that migrate from the adjacent liver
parenchyma into the TIPS [14,
15]. The fibrotic healing
response to the trauma of shunt creation causes tissue overgrowth through the
stent mesh into the shunt lumen
[8,
16]
(Fig. 3).
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Fig. 3 —49-year-old woman after transjugular intrahepatic
portosystemic shunt (TIPS) who presented with recurrent ascites. TIPS venogram
shows severe intrastent stenosis caused by myofibroblast proliferation.
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Fig. 4 —55-year-old man after transjugular intrahepatic portosystemic
shunt (TIPS) creation who presented with variceal bleeding. TIPS venogram
shows proximal hepatic vein stenosis (arrow) caused by intimal
hyperplasia.
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Leaving the proximal segment of the hepatic vein unstented will predispose
the patient to hepatic vein stenosis as a result of intimal hyperplasia
[17]. The shear stress and
turbulence from increased high-velocity blood flow cause venous intimal
hyperplasia [10,
13]. Hepatic vein stenosis or
intimal hyperplasia of the hepatic vein occurs months to a year after TIPS
creation [16,
18]
(Fig. 4). Late shortening of
self-expanding stents— Wallstents (Boston Scientific)—may occur,
leaving the hepatic vein at risk of subsequent intimal hyperplasia or
recoiling of an unsupported parenchymal tract
[19]. Therefore, extension of
the stent to the junction of the hepatic vein and the inferior vena cava (IVC)
is an important determinant of shunt patency. The optimal location of the
proximal end of the stent is the hepatocaval junction; to determine the exact
location of that junction, a guidewire can be placed in the IVC and a second
guidewire in the TIPS. The sheath is then advanced over the two wires until
its tip reaches the hepatocaval junction. Alternatively, simultaneous contrast
injection may be performed in the TIPS and the IVC to define the hepatic
vein–IVC confluence (Figs.
5A and
5B).
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Fig. 5A —Determining exact location of hepatocaval junction.
Fluoroscopic image in 45-year-old man shows guidewire is placed in inferior
vena cava (IVC) and a second guidewire is placed in transjugular intrahepatic
portosystemic shunt (TIPS). Sheath is advanced over the two wires until its
tip reaches hepatocaval junction (arrow), or simultaneous contrast
injection may be performed in TIPS and IVC to define confluence of hepatic
vein and IVC.
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Fig. 5B —Determining exact location of hepatocaval junction. Left
anterior oblique projection (36°) of venogram in 58-year-old man and
simultaneous contrast injection of shunt and IVC clearly show confluence of
hepatic vein and IVC (arrow).
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Hepatocellular carcinoma (HCC) is the most common primary malignant tumor
of the liver and often occurs in cirrhotic livers with portal hypertension.
HCC is well known to invade venous structures, including the portal and
hepatic veins [20]
(Fig. 6). HCC may grow through
the mesh of the stent or invade the portal and hepatic veins, compromising
portal vein inflow or hepatic vein outflow and resulting in subsequent TIPS
occlusion [21,
22]. If TIPS thrombosis occurs
in a patient with known HCC, bland thrombus should be differentiated from
tumor thrombus before attempting to revise a malfunctioning shunt. CT and MRI
are excellent methods to evaluate tumor extension into blood vessels. Both can
display images in multiple planes and can perform multiphasic enhanced studies
[23,
24]. Portal tumor thrombus
shows early arterial enhancement on dynamic enhanced studies
[25]. Bland thrombus may
respond to mechanical thrombolysis and stenting. On the other hand,
manipulation of tumor thrombus may result in the embolization of tumor
cells.
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Fig. 6 —45-year-old man with hepatitis C cirrhosis and hepatocellular
carcinoma (HCC) who presented with recurrent variceal bleeding. On
nonsubtracted venogram, HCC (T) invasion of portal vein end (PV) of
transjugular intrahepatic portosystemic shunt (TIPS) causes decreased shunt
flow.
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Other recognized causes of shunt dysfunction include iatrogenic dissection
of the portal vein during TIPS creation, resulting in inflow disease and shunt
thrombosis [26].
Hypercoagulability may also predispose to shunt thrombosis and occlusion,
especially in patients with Budd-Chiari syndrome, in whom TIPS dysfunction of
bare stents is fairly common
[21,
27,
28].
Extrahepatic hemodynamic causes of TIPS dysfunction, such as flow stealing
through competent varices or spontaneous mesocaval shunts, may decrease the
flow in the TIPS enough to result in flow stasis and shunt thrombosis.
Mechanical causes of bare stent thrombosis may result from fractures of
balloon-expandable stents deployed in the parenchymal track. The stiffness and
lack of flexibility of balloon-expandable stents may preclude their use in
TIPS (Figs. 7A and
7B).
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Fig. 7A —57-year-old woman after transjugular intrahepatic
portosystemic shunt (TIPS) who presented with abnormal sonographic velocities
and recurrent ascites. TIPS venograms to evaluate recurrent variceal bleeding
show fracture of balloon-expandable stent. After angioplasty, TIPS was revised
with covered stent.
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Fig. 7B —57-year-old woman after transjugular intrahepatic
portosystemic shunt (TIPS) who presented with abnormal sonographic velocities
and recurrent ascites. TIPS venograms to evaluate recurrent variceal bleeding
show fracture of balloon-expandable stent. After angioplasty, TIPS was revised
with covered stent.
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Using the peripheral segment of any hepatic vein to start a TIPS may cause
the shunt to cross the hepatic vein perpendicularly and abut the hepatic vein,
thus reducing the outflow of the shunt. Kinking of the stent may be seen after
peripheral punctures of the portal vein. Intrastent kinking with resultant
reduction in the TIPS diameter may cause hemodynamically significant stenosis
and an increased portosystemic gradient
[29]. Extending the shunt with
additional stents and aligning them smoothly into a shallow arch can reduce
kinks. Evaluation of MRI or CT studies before TIPS creation provides useful
information to determine the existence of variant anatomy or the presence of
accessory veins. Detailed examination of venograms is important to direct the
needle from an appropriate hepatic vein into a corresponding portal vein
branch.
Graft Materials of Covered Stents
Lining stents with graft material to exclude hepatic parenchyma, bile, and
flow dynamic factors from the TIPS lumen ideally addresses, treats, and
prevents the biologic causes of shunt failure and therefore promotes
improvement in shunt patency
[30].
Many graft materials have been tested in TIPS, such as silicone,
polycarbonate urethane, and polyethylene terephthalate. All exhibited equal or
worse shunt patency when compared with bare stents, mainly because of the
development of an inflammatory reaction
[6,
31–33].
On the other hand, expandable PTFE covered stents have shown improved and
durable shunt patency [30].
Histologic and venographic analyses of expandable PTFE TIPS in animals and
humans have shown absence of an inflammatory reaction. Expandable PTFE
prevents myelofibroblasts and extracellular collagen matrix from reaching the
shunt lumen [34]. In addition,
TIPS created with expandable PTFE-covered stents have shown better patency
rates in patients with hypercoagulopathy and Budd-Chiari syndrome
[35].
The Viatorr (Gore) is the commercially available expandable PTFE-covered
stent designed for TIPS. It consists of two portions. A 2-cm-long noncovered
segment in the portal vein allows free blood flow to side branches of the
portal vein through the bare nitinol stent wires. The expandable PTFE portion
of the covered stent covers the inside of the stent and is placed from the
portal vein entry site along the length of the parenchymal tract as far as the
hepatic vein ostium.
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Fig. 8 —Commercially available expandable polytetrafluoroethylene
(PTFE)-covered stent designed for transjugular intrahepatic portosystemic
shunt (Viatorr, Gore) consists of two portions, a 2-cm-long uncovered segment
on portal side (A) allowing free blood flow to side branches of portal vein
through bare nitinol stent wires. Stent-graft portion (B) is covered with
expandable PTFE on inside to cover from portal vein entry to hepatic vein
ostium. Radiopaque gold ring identifies junction between covered and uncovered
segments (arrows), and another radiopaque gold marker identifies
proximal end of device (arrowhead).
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In general, we try to use one endoprosthesis in which the covered segment
will extend from the portal vein entry to the hepatocaval junction. In those
cases in which the deployed stent is short in the hepatic vein end of the
shunt, an additional stent is used to extend the TIPS to the hepatic
vein–IVC confluence.
The expandable PTFE is composed of three layers that are designed to permit
endoluminal enothelization and to minimize fibrous connective tissue cell
penetration. The overlapping three expandable PTFE layers are impermeable to
liquid bile. A radiopaque gold ring marks the transition from the bare to the
covered segment (Fig. 8). The
nitinol skeleton of the stent has considerable radial strength that prevents
recoiling after insertion.
Most TIPS dysfunctions in TIPS created with expandable PTFE covered stents
are related to technical errors or mechanical factors. As in bare-stent TIPS,
a stent short at the hepatic venous end of the TIPS will likely result in
hepatic vein stenosis (Figs.
9A and
9B). Stent kinking at the
portal vein end of the shunt may occur in TIPS as a result of peripheral
portal vein punctures [29],
and in Viatorr-covered stents may be seen at the junction between the covered
and uncovered segments of the stent (Fig.
10). Finally, we have seen mid covered-stent stenosis after TIPS
revision of a Wallstent TIPS with a Viatorr stent, a finding that may be
related to infolding of the graft material (Figs.
11A,
11B,
11C, and
11D).
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Fig. 9A —43-year-old man after transjugular intrahepatic portosystemic
shunt (TIPS) creation with expandable polytetrafluoroethylene covered
(Viatorr, Gore) stent presents with recurrent ascites. Shunt venograms of
Viatorr covered stent TIPS show TIPS ending perpendicular to hepatic vein and
causing functional hepatic vein stenosis (arrow, A). Final
venogram (B) after revision with a Wallstent (Boston Scientific) showed
no residual stenosis.
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Fig. 9B —43-year-old man after transjugular intrahepatic portosystemic
shunt (TIPS) creation with expandable polytetrafluoroethylene covered
(Viatorr, Gore) stent presents with recurrent ascites. Shunt venograms of
Viatorr covered stent TIPS show TIPS ending perpendicular to hepatic vein and
causing functional hepatic vein stenosis (arrow, A). Final
venogram (B) after revision with a Wallstent (Boston Scientific) showed
no residual stenosis.
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Fig. 10 —49-year-old man with transjugular intrahepatic portosystemic
shunt for refractory ascites. Venogram shows peripheral puncture of portal
vein system resulted in kink and angulation of Viatorr (Gore) expandable
polytetrafluoroethylene covered stent at portal vein end of shunt
(arrow). Measured portosystemic gradient across shunt was 13 mm
Hg.
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Fig. 11A —56-year-old man with transjugular intrahepatic portosystemic
shunt (TIPS) for variceal bleeding and refractory ascites. TIPS venograms of
shunt created with a Wallstent (Boston Scientific) show mid-TIPS stenosis
(arrows, A). After angioplasty, Viatorr (Gore) stentgraft was
deployed, covering stenotic segment to junction of hepatic vein and inferior
vena cava (asterisk, B).
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Fig. 11B —56-year-old man with transjugular intrahepatic portosystemic
shunt (TIPS) for variceal bleeding and refractory ascites. TIPS venograms of
shunt created with a Wallstent (Boston Scientific) show mid-TIPS stenosis
(arrows, A). After angioplasty, Viatorr (Gore) stentgraft was
deployed, covering stenotic segment to junction of hepatic vein and inferior
vena cava (asterisk, B).
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Fig. 11C —56-year-old man with transjugular intrahepatic portosystemic
shunt (TIPS) for variceal bleeding and refractory ascites. Shunt venograms of
TIPS created with a Wallstent and revised with Viatorr show mid-stentraft
stenosis (arrows, C) likely related to oversized covered stent
with infolding of graft material that responded well to angioplasty.
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Fig. 11D —56-year-old man with transjugular intrahepatic portosystemic
shunt (TIPS) for variceal bleeding and refractory ascites. Shunt venograms of
TIPS created with a Wallstent and revised with Viatorr show mid-stentraft
stenosis (arrows, C) likely related to oversized covered stent
with infolding of graft material that responded well to angioplasty.
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TIPS Revisions
TIPS patients who present with recurrent signs and symptoms of portal
hypertension as well as abnormal shunt velocities during screening sonography
of the TIPS are referred for TIPS venography and hemodynamic analysis of the
TIPS. Careful patient examination and TIPS venographic and hemodynamic
findings will determine whether the shunt should be revised. After angioplasty
of the stenotic segment or recanalization of the occluded shunt, failed
bare-stent shunts can be revised with Viatorr covered stents. The covered
portion of the covered stent must line the entire tract from the portal vein
entry to the junction between the hepatic vein and the IVC to improve patency
(Figs. 12A,
12B, and
12C). In those cases in which
an occluded TIPS cannot be recanalized, a parallel TIPS can be created to
decompress the portal venous system
[36].
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Fig. 12A —62-year-old woman after transjugular intrahepatic
portosystemic shunt (TIPS) who presented with recurrent variceal bleeding.
Fluoroscopic images and venogram show chronic TIPS occlusion preventing access
to lumen by jugular approach. Therefore, access to TIPS lumen was gained
percutaneously with 21-gauge needle (asterisk, A) and
0.018-inch wire. Retrograde access to inferior vena cava was regained. After
angioplasty, TIPS was revised with covered stent.
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Fig. 12B —62-year-old woman after transjugular intrahepatic
portosystemic shunt (TIPS) who presented with recurrent variceal bleeding.
Fluoroscopic images and venogram show chronic TIPS occlusion preventing access
to lumen by jugular approach. Therefore, access to TIPS lumen was gained
percutaneously with 21-gauge needle (asterisk, A) and
0.018-inch wire. Retrograde access to inferior vena cava was regained. After
angioplasty, TIPS was revised with covered stent.
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Fig. 12C —62-year-old woman after transjugular intrahepatic
portosystemic shunt (TIPS) who presented with recurrent variceal bleeding.
Fluoroscopic images and venogram show chronic TIPS occlusion preventing access
to lumen by jugular approach. Therefore, access to TIPS lumen was gained
percutaneously with 21-gauge needle (asterisk, A) and
0.018-inch wire. Retrograde access to inferior vena cava was regained. After
angioplasty, TIPS was revised with covered stent.
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Sonography After Covered Shunt Placement
Sonography is the screening tool for detecting early shunt dysfunction. It
is performed soon after TIPS creation to obtain baseline velocity values
[37]. However, an acoustic
barrier may prevent examination of the shunt lumen when Doppler sonography is
performed during the first days after covered stent implantation. This
acoustic barrier is believed to be due to the trapping of air bubbles inside
the graft. This usually resolves spontaneously during the first week after
TIPS creation [38]. Then TIPS
hemodynamics can be analyzed as in bare stents (Figs.
13A and
13B). Real-time Doppler
spectral analysis is used to evaluate portal vein and stent (proximal, middle,
and distal segments) blood flow velocities. Doppler results are considered
abnormal on the basis of one or a combination of the following: an absolute
stent velocity < 50 or > 200 cm/s, spatial or temporal stent gradients
> 50 cm/s, a portal vein velocity < 30 cm/s, or the presence of flow
reversal in the portal vein distal to the shunt
[39]. Because of the improved
patency in Viatorr TIPS, frequent routine sonography may not be cost-effective
for long-term surveillance of expandable PTFE-covered stents
[40]. The number and frequency
of sonographic examinations for detection of TIPS malfunction after their
creation with a covered stent are yet to be determined in prospective
trials.
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Fig. 13A —63-year-old woman with portal hypertension after creation of
trans jugular intrahepatic portosystemic shunt (TIPS). Sonogram obtained soon
after TIPS creation with covered stent fails to reveal shunt lumen (S).
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Conclusion
Biologic and technical factors may predispose TIPS to shunt failure. The
combination of improved technique and expandable PTFE has significantly
improved TIPS patency. The need for follow-up venography and secondary
interventions has been reduced significantly as a result of improved shunt
patency,
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Abstract
Introduction
