Va s c u l a r a n d I n t e r ve n t i o n a l R a d i o l o g y • R ev i ew Ferral et al. Budd-Chiari Syndrome

FOCUS ON:

Vascular and Interventional Radiology Review

Hector Ferral1 George Behrens 2 Jorge Lopera3 Ferral H, Behrens G, Lopera J

Budd-Chiari Syndrome OBJECTIVE. Budd-Chiari syndrome (BCS) is an uncommon condition characterized by obstruction of the hepatic venous outflow tract. Presentation may vary from a completely asymptomatic condition to fulminant liver failure. BCS is an example of postsinusoidal portal hypertension. The management can be divided into three main categories: medical, surgical, and endovascular. The purpose of this article is to present an overall perspective of the problem, diagnosis, and management. CONCLUSION. BCS requires accurate, prompt diagnosis and aggressive therapy. Treatment will vary depending on the clinical presentation, cause, and anatomic location of the problem. Patients with BCS are probably best treated in tertiary care centers where liver transplantation is available.

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Keywords: Budd-Chiari syndrome, stents, thrombolysis, transjugular intrahepatic portosystemic shunts DOI:10.2214/AJR.12.9098 Received April 16, 2012; accepted without revision April 23, 2012. 1 Department of Radiology, Section of Interventional Radiology, NorthShore University HealthSystem, 2650 Ridge Ave, Evanston, IL 60201. Address correspondence to H. Ferral ([email protected]). 2 Department of Radiology, Section of Interventional Radiology, Rush University Medical Center, Chicago, IL. 3 Department of Radiology, Section of Interventional Radiology, University of Texas Health Science Center, San Antonio, TX.

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udd-Chiari syndrome (BCS) is an uncommon condition characterized by obstruction of the hepatic venous outflow tract; it has been described to occur in 1 in 100,000 of the population worldwide [1, 2]. The term “BuddChiari” was coined in the late 1800s after the work of George Budd, an internist, who described three cases of hepatic vein thrombosis in 1845 and Hans Chiari, an Austrian pathologist, who reported the first pathologic description of “obliterating endophlebitis of the hepatic veins” in 1899 [1]. The current definition of BCS encompasses a number of conditions that cause obstruction of the hepatic outflow tract from the small hepatic veins to the junction of the inferior vena cava (IVC) and right atrium [3–5]; this condition is a classic example of postsinusoidal portal hypertension [6, 7]. This term does not include cardiac or pericardial causes of hepatic outflow obstruction or the sinusoidal problems related to toxic substance exposure [3, 8]. BCS is classified as primary when the obstruction to hepatic venous outflow is related to a primary venous problem, such as thrombosis, stenosis, or webs [1, 9], and as secondary when it is related to extrinsic compression, such as that caused by abscess, tumor, cyst, or hyperplastic nodules [2, 3, 10–12]. The presentation and cause of BCS may vary depending on the geographic area of presen-

tation [3]. Interestingly, obstruction of the IVC with or without involvement of the hepatic veins is predominant in Asia [3, 13], and pure hepatic vein obstruction predominates in Western countries. Clinical presentation may vary from a completely asymptomatic condition to fulminant liver failure [13]. The management of BCS can be divided into three main categories: medical, surgical, and endovascular [14–17]. The purpose of this article is to present an overview of the most important aspects of this uncommon clinical entity. Cause and Risk Factors Common causes of BCS include inherited and acquired hypercoagulable states [18]. Inherited hypercoagulable states such as factor V Leiden mutation, protein C deficiency, protein S deficiency, the prothrombin G20210A mutation, and antithrombin III deficiency are common causes of hepatic vein thrombosis resulting in BCS. Acquired prothrombotic states such as myeloproliferative disorders (e.g., polycythemia vera, paroxysmal nocturnal hemoglobinuria, essential thrombocytosis, agnogenic myeloid metaplasia, and myelofibrosis) account for more than 50% of BCS cases [3, 4, 18]. Other prothrombotic conditions, such as antiphospholipid syndrome, have also been mentioned. Other conditions have been proposed as risk factors for the development of

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Ferral et al. BCS, including Behçet disease, hypereosinophilic syndrome, and ulcerative colitis [3] and pregnancy, malnutrition, and the use of oral contraceptives [4, 19]. Pathophysiology Once the hepatic veins occlude, the liver venous outflow is compromised, the sinusoidal and portal pressures increase, and the portal flow decreases; if this process continues, it leads to hepatic congestion; formation of ascites; and, in certain cases, portal vein thrombosis [18]. Hepatocytes undergo hypoxic damage that eventually evolves into noninflammatory centrilobular cell necrosis [1, 20]. If this hepatocellular damage is massive, the patient will present with a fulminant form of BCS, which is a potentially fatal condition [21]. Otherwise, the process evolves slowly and allows the patient to develop portal hypertension and ascites; histologically, the liver develops fibrosis and, in later stages, cirrhosis [1]. If BCS is not treated, the natural course is that of a progressive and fatal condition. Previous reports, when no specific therapy was available, showed that 90% of patients with BCS had died by 3 years [3]. The most common causes of death are intractable ascites, gastrointestinal bleeding, and liver failure. Clinical Presentation Clinical presentation of BCS may be fulminant (5%), acute (20%), and subacute or chronic (60%) [13]. BCS may be asymptomatic in approximately 15–20% of cases. A lack of symptoms is associated to the fact that only a single hepatic vein is thrombosed or that venous outflow is carried by large hepatic vein collaterals [3, 22, 23]. These clinical manifestations may be directly related to the speed of the underlying obstructive process [3, 4]. Approximately 75–80% of patients with BCS will have clinical manifestations; the most common include fever, abdominal pain, abdominal distention, ascites, liver failure, lower extremity edema, gastrointestinal bleeding, and encephalopathy [3, 13]. Fulminant Budd-Chiari Syndrome Fulminant BCS develops in a few days; patients present with severe liver failure with elevation of hepatic enzymes, hyperbilirubinemia, encephalopathy, and coagulopathy [24]. The liver is massively enlarged and painful [18, 20, 23]. Ascites and renal failure are constant [20]. Renal failure may be secondary to severe compression of the hepatic vein with compromise of the renal outflow.

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Acute Budd-Chiari Syndrome Acute BCS develops usually within 1 month and is characterized by intractable ascites, abdominal pain, liver enlargement, renal failure, elevation of hepatic enzymes, and coagulopathy [20]. Histologically, hepatic congestion and necrosis are present. Subacute Budd-Chiari Syndrome Subacute BCS is the most common clinical type. This form of BCS has an insidious onset and it may take as long as 3 months to become asymptomatic. Biopsy shows minimal hepatic necrosis and trace or no ascites. The development of the anatomic problem is slow and allows time for decompressive collaterals to develop [18]. Patients with subacute BCS do not have esophageal bleeding. Chronic Budd-Chiari Syndrome The chronic form of BCS is characterized by the development of portal hypertension. Histologically, patients with chronic BCS have congestive cirrhosis [23]. There is progressive abdominal distention due to ascites. The liver function tests may be minimally affected or normal [20]. Renal failure is seen in 50% of patients with chronic BCS [20]. Esophageal bleeding is seen in 5–15% of these patients, and splenomegaly is common in the chronic from [20]. Diagnosis and Morphologic Classification The diagnosis of BCS should be clinically suspected in patients who present with any one of the following findings: fulminant liver failure with abrupt onset of ascites and hepatomegaly; massive ascites with relatively preserved liver function; unexplained chronic liver disease; or liver disease and an associated thrombogenic disorder [1]. BCS may be classified into three types depending on the type of existing venous occlusion [8]. Type I is limited to the IVC. In type II BCS, lesions are within the hepatic veins. If lesions are short-segment occlusions (< 4 cm), type IIa BCS is the diagnosis. BCS type III is the mixed type with involvement of the IVC and hepatic veins. Imaging studies play an important role in confirming the diagnosis of BCS by showing the venous abnormalities. Probably the most useful imaging methods include conventional and Doppler ultrasound, CT, MRI, and catheter venography [8]. Ultrasound An enlarged caudate lobe, hepatomegaly, lack of visualization of the hepatic veins, a

compressed IVC, enlarged intrahepatic collaterals, splenomegaly, and ascites are conventional sonographic findings in patients with BCS [8, 25, 26]. In some instances, an enlarged caudate lobe vein (> 3 mm) can be seen draining directly into the IVC, a spiderweb appearance of hepatic veins or replacement of the hepatic vein by a fibrous, echogenic cord [3, 8, 27–30]. Ultrasound may also show that the stenotic IVC, especially in the intrahepatic segment, is associated with an enlarged caudate lobe [8]. In some chronic cases, large regenerative nodules that may simulate carcinoma may be present [30, 31]. On Doppler evaluation, patients with BCS may present with enlarged hepatic veins with no flow signal or with reversed flow. The identification of collateral vessels with drainage into the subcapsular or intercostal veins is a highly sensitive and specific feature for the diagnosis of BCS [20] CT CT findings in acute BCS may include normal liver morphology, patchy enhancement, an enlarged caudate lobe, a compressed IVC, the absence of hepatic veins, and ascites [25, 27, 32] (Figs. 1–3). The patchy enhancement seen on CT scans of patients with BCS is related to the stasis in the sinusoids and portal vein and is associated with increased enhancement of the central portion of the liver parenchyma [29, 32, 33]. Acute BCS may present with acute thrombosis of the IVC, iliac veins, and femoral veins (Fig. 4); this presentation of acute BCS is rare but may be seen in young patients taking oral contraceptives. Findings in subacute BCS depend on the type of venous involvement and may include the presence of portosystemic and intrahepatic collaterals as well as hepatic artery enlargement. Typically, the IVC is compressed but may be thrombosed; patients may also present with portal vein thrombosis that is secondary to the profound flow disturbance [25, 34]. Chronic BCS is associated with an alteration in the morphology of the liver and regenerative nodule formation. These nodules typically show hyperattenuation in the arterial phase and remain hyperattenuating in the portal phase; patchy enhancement may also be seen in this stage of the disease [25]. MRI MRI is a useful imaging tool in the diagnosis of BCS. Its main advantage is the lack of ionizing radiation. The anatomic findings identified by ultrasound and CT are also depicted on MRI

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Budd-Chiari Syndrome including enlarged liver, inhomogeneous signal intensity of the parenchyma on spin-echo images (Fig. 5), intrahepatic collaterals, caudate lobe enlargement, regenerative nodules, and ascites [25]. BCS is identified on MRI by the absence of blood flow in the occluded veins. Detection of thrombus causing an absence of flow may be difficult on spin-echo images because thrombus may manifest as different signal intensities depending on its age and composition [25]. MR angiography is useful in the diagnosis of BCS because it shows the vascular changes seen in this disease entity. The most useful technique is 3D contrast-enhanced MR angiography because it shows the vascular anatomy in multiple projections, including the presence of intrahepatic collaterals. This technique may also show the presence of thrombus within the hepatic veins, portal vein, and IVC [35]. Catheter Venography Patients with BCS are usually referred to an interventional radiologist to confirm the diagnosis and for therapeutic interventions. Catheter venography is considered the reference standard for the diagnosis of this condition [8]. If properly performed, catheter venography provides anatomic information by precisely depicting the venous problem, hemodynamic information through pressure measurements, and histologic information by obtaining a transjugular liver biopsy and allows the possibility of endovascular management of the condition. Access into the hepatic venous system may be achieved using a transvenous approach via either an internal jugular vein or the common femoral vein (Fig. 6). In cases in which retrograde access into the hepatic veins is difficult or impossible, a transhepatic approach may be used to visualize these veins (Fig. 7). Diagnostic venography in patients with BCS may show severe stenotic areas in the hepatic veins very close to the junction with the IVC (Fig. 6), complete occlusion of the hepatic veins with the formation of large intraparenchymal collaterals (Fig. 7), occlusion of the hepatic veins associated with a spiderweb appearance of the hepatic parenchyma, (Fig. 8) and severe stenosis of the IVC secondary to hypertrophy of the caudate lobe [4] (Fig. 9). Management Medical Management Medical management of BCS is focused on the treatment of the underlying cause: control of portal hypertension, ascites control, sys-

temic or catheter-directed thrombolysis, and anticoagulation [1, 13, 17, 18, 36]. Medical management is usually insufficient and most patients will require either surgical or endovascular intervention [17]. Most patients who undergo endovascular or surgical management will need long-term anticoagulation because most have an underlying prothrombotic disorder [4, 18, 37]. Surgical Management Surgical management includes membrane resection, IVC reconstruction with a pericardial patch, portosystemic shunts, mesoatrial shunts, portoatrial shunts, and liver transplant [15, 38–41]. Surgical portosystemic shunts (portocaval or mesocaval shunt) placed below the diaphragm will fail if the IVC is thrombosed or severely compressed because the system will not be properly decompressed [13]; in these cases, combined endovascular IVC stenting followed by surgical portosystemic shunting is an option for optimal system decompression [42]. Dang and coworkers [39] documented clinical success in 87.7% of patients with BCS who were treated with membrane resection, making it a feasible option for patients with this condition. Inafuku and coworkers [15] evaluated 53 patients with IVC reconstruction with the creation of a pericardial patch. The intraoperative mortality was 3.7% and the 5- and 10-year survival was 89.8% and 70.7%, respectively [15]. Liver transplant is another reported therapeutic approach for BCS. Most investigators agree that patients with fulminant BCS should be immediately listed for a liver transplant and undergo emergency liver transplant as soon as feasible [17]. The first liver transplant for BCS was performed in 1974 [17], and the reported survival rates of patients with BCS 1 and 3 years after liver transplantation are 70% and 45%, respectively [17, 43]. Results from a European registry recorded between 1988 and 1999 reported 1-, 5-, and 10-year survival rates of 248 patients with BCS treated with liver transplant were 75%, 71%, and 68% [17, 44]. Most authors agree that not all patients with BCS should undergo liver transplant and that this therapeutic option should probably be used exclusively in patients with fulminant BCS or in patients with chronic cirrhosis [17, 18]. Endovascular Management Endovascular management of BCS will depend on the type of venous abnormality to

be treated and involves application of various techniques including balloon angioplasty, stent placement, catheter-directed thrombolysis, thrombus maceration, and endovascular portosystemic shunt creation (transjugular intrahepatic portosystemic shunt [TIPS] procedure) [37, 45–50]. Isolated hepatic vein or IVC webs, stenoses, or occlusions may be successfully treated with balloon angioplasty or stent placement only with good technical outcome (Figs. 10 and 11); however, the long-term results of these different therapeutic techniques are not entirely proven because most studies have included a small number of patients or are isolated case reports. If an isolated hepatic vein or IVC intervention fails, the next endovascular step is TIPS creation [18]. The major advantages of the percutaneous approach are that it is minimally invasive and that it creates a portosystemic shunt directly into the suprahepatic IVC, which essentially functions as a portoatrial shunt; if TIPS is properly created, it will effectively decompress the portal vein and IVC with a single procedure. The results of TIPS procedures in patients with BCS have been encouraging, with a more than 90% technical success rate and a more than 75% clinical success rate reported [37, 48, 51]. The most common technical challenge is the recanalization of the occluded hepatic vein. TIPS creation with bare metallic stents has the disadvantage of reduced patency rates [48], whereas a TIPS created with covered stents have shown better patency rates [14, 37, 48, 52]. Gandini and coworkers [48] found much higher patency rates in patients who underwent TIPS with expanded polytetrafluoroethylene covered stents. The mean patency duration was 4.4 months for patients treated with bare stents compared with 22.2 months for patients treated with stent-grafts. The 6- and 12-month patency rates were 100% and 85.7%, respectively, for stent-grafts compared with 16.7% and 0% for bare stents (p < 0.001) [48]. TIPS is minimally invasive and, if performed by experienced operators, it has a high technical and clinical success rate. Longterm shunt patency is one of the most common problems and most patients with BCS require long-term anticoagulation. Conclusion BCS is an uncommon clinical condition that requires accurate, prompt diagnosis and aggressive therapy. Treatment will vary depending on the clinical presentation, cause, and anatomic location of the problem. Medical, surgical, and endovascular therapeutic

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Ferral et al. options are available for the management of this condition. Patients with BCS are probably best treated in tertiary care centers where liver transplant is available. References 1. Aydinli M, Bayraktar Y. Budd-Chiari syndrome: etiology, pathogenesis and diagnosis. World J Gastroenterol 2007; 13:2693–2696 2. Valla DC. Primary Budd-Chiari syndrome. J Hepatol 2009; 50:195–203 3. Plessier A, Valla DC. Budd-Chiari syndrome. Semin Liver Dis 2008; 28:259–269 4. Cura M, Haskal Z, Lopera J. Diagnostic and interventional radiology for Budd-Chiari syndrome. RadioGraphics 2009; 29:669–681 5. Zimmerman MA, Cameron AM, Ghobrial RM. Budd-Chiari syndrome. Clin Liver Dis 2006; 10:259–273 6. Hernández-Guerra M, López E, Bellot P, et al. Systemic hemodynamics, vasoactive systems, and plasma volume in patients with severe Budd-Chiari syndrome. Hepatology 2006; 43:27–33 7. Blendis L. Budd-Chiari syndrome: a clinical model of the hepatorenal reflex? Gastroenterology 2006; 131:671–672 8. Patil P, Deshmukh H, Popat B, Rathod K. Spectrum of imaging in Budd Chiari syndrome. J Med Imaging Radiat Oncol 2012; 56:75–83 9. Gruselle P, Ooghe P, Cauchie P, Van der Cruyssen G. Severe thrombophilic diathesis starting with hepatic vein thrombosis (Budd-Chiari syndrome) in a family with a new protein S gene mutation. Acta Gastroenterol Belg 2006; 69:20–24 10. Larrousse C, Brasseur P, Bissen L, Sukkarieh F, Gouat F. Hepatic amebic abscess complicated by Budd-Chiari syndrome. JBR-BTR 2006; 89:140–141 11. Asrani SK, Larusso NF. Fibrolamellar hepatocellular carcinoma presenting with Budd-Chiari syndrome, right atrial thrombus, and pulmonary emboli. Hepatology 2012; 55:977–978 12. Bedioui H, Nouira K, Ayadi S, et al. Budd-Chiari syndrome secondary to hepatic echinococcosis. Gastroenterol Clin Biol 2007; 31:721–724 13. Senzolo M, Cholongitas EC, Patch D, Burroughs AK. Update on the classification, assessment of prognosis and therapy of Budd-Chiari syndrome. Nat Clin Pract Gastroenterol Hepatol 2005; 2:182–190 14. Darwish Murad S, Plessier A, Hernandez-Guerra M, et al.; EN-Vie (European Network for Vascular Disorders of the Liver). Etiology, management, and outcome of the Budd-Chiari syndrome. Ann Intern Med 2009; 151:167–175 15. Inafuku H, Morishima Y, Nagano T, Arakaki K, Yamashiro S, Kuniyoshi Y. A three-decade experience of radical open endvenectomy with pericardial patch graft for correction of Budd-Chiari syndrome. J Vasc Surg 2009; 50:590–593

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Fig. 1—Axial contrast-enhanced CT scan of abdomen of 30-year-old woman with Budd-Chiari syndrome. Note patchy enhancement of liver (arrow) and absence of hepatic veins. Asterisk = presence of ascites.

J Vasc Interv Radiol 2007; 18:924–927 51. López-Méndez E, Chávez-Tapia NC, Avila-Escobedo L, Cabrera-Aleksandrova T, Uribe M. Early experience of Budd-Chiari syndrome treatment with transjugular intrahepatic portosystemic shunt. Ann Hepatol 2006; 5:157–160 52. Cejna M. Should stent-grafts replace bare stents for primary transjugular intrahepatic portosystemic shunts? Semin Intervent Radiol 2005; 22: 287–299

Fig. 2—Axial contrast-enhanced CT scan of abdomen of 30-year-old woman with Budd-Chiari syndrome. Note massive enlargement of caudate lobe (CL) and patchy enhancement of liver (arrow). Asterisk = presence of ascites, arrowhead = inferior vena cava.

Fig. 3—Contrast-enhanced CT scan of abdomen of 19-year-old woman with subacute Budd-Chiari syndrome. Note absence of hepatic veins, enlargement of liver, compression of inferior vena cava, and presence of ascites (asterisks). Arrowhead = inferior vena cava.

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Fig. 4—15-year-old girl with acute Budd-Chiari syndrome who presented with acute inferior vena cava (IVC) thrombosis. A, Contrast-enhanced CT scan shows enlarged caudate lobe (CL) and lack of opacification of IVC; these findings indicate presence of acute thrombosis. Arrowhead = thrombosed IVC. B, Contrast-enhanced CT scan obtained at lower level than A shows extension of IVC thrombosis down to level of renal veins. Renal veins are also thrombosed (arrowheads). Fig. 5—Axial gadolinium-enhanced MR image of 32-year-old man with Budd-Chiari syndrome. Note hyperintensity of periphery of liver (arrows). Arrowhead = compressed inferior vena cava.

Fig. 6—Intraparenchymal injection of CO2 in 24-year-old woman with Budd-Chiari syndrome. Image was obtained during transjugular intrahepatic portosystemic shunt (TIPS) procedure. Lower accessory hepatic vein is opacified and there is severe stenosis (arrow) of hepatic vein at junction with inferior vena cava. 

Fig. 7—Transhepatic hepatic venogram shows occlusion of middle hepatic vein (arrow) in 24-year-old woman with Budd-Chiari syndrome. Note fugal flow of contrast material into large intrahepatic collaterals and no opacification of inferior vena cava. 

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Fig. 8—Contrast injection into liver parenchyma in 24-year-old man with Budd-Chiari syndrome (BCS). Image was obtained during transjugular liver biopsy procedure. Biopsy needle has been advanced into parenchyma and small amount of contrast material has been injected. Image shows spiderweb appearance typical of BCS. Hepatic vein is not opacified, indicating thrombosis of that vessel. Arrow = spiderweb deformity of veins.

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Fig. 9—Inferior venacavogram of 30-year-old woman with Budd-Chiari syndrome (BCS). Note severe stenosis of intrahepatic inferior vena cava (IVC). This is classic finding in patients with BCS. There is also opacification of hemiazygos system (arrow). RA = right atrium.

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Fig. 10—44-year-old woman with Budd-Chiari syndrome. A and B, Axial contrast-enhanced CT scans of abdomen show enlarged liver (arrow) with heterogeneous enhancement with central hyperattenuation. Note caudate lobe (CL) enlargement, large gastric varices (arrowhead, B), and lack of visualization of hepatic veins and inferior vena cava (IVC). (Fig. 10 continues on next page)

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Fig. 10 (continued)—44-year-old woman with Budd-Chiari syndrome. C, Spot film obtained during catheter venography shows selective catheterization of right hepatic vein (HV). Catheter has been advanced from femoral venous approach. There is complete occlusion of IVC (arrow) just caudad to right atrium. RA = right atrium. D, Spot film obtained during catheter venography corresponds to selective left hepatic venogram. Note small, irregular hepatic vein (HV); opacification of IVC with caudal retrograde flow; and occlusion of IVC (arrow) immediately caudad to right atrium (RA). This case is example of BCS caused by IVC web or occlusion. E, Spot film obtained during balloon angioplasty of IVC web. In this particular case, 14-mm high-pressure balloon was used. IVC occlusion was successfully crossed with wire. Arrow = angioplasty balloon, RA = right atrium. F, IVC venogram obtained after angioplasty shows treatment of IVC occlusion was successful. RA = right atrium.

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Fig. 11—33-year-old woman with Budd-Chiari syndrome managed with transjugular intrahepatic portosystemic shunt (TIPS) procedure. RA = right atrium. A, Spot film obtained during direct portography immediately after gaining transhepatic access into portal vein shows fugal flow into small left gastric, splenic, and inferior mesenteric veins. Also, there is faint opacification of intrahepatic inferior vena cava (IVC), which is severely narrowed. Arrow = transparenchymal tract. B, Spot film obtained during direct portography after TIPS completion shows complete diversion of flow from main portal vein directly into RA. Arrow = transparenchymal tract. C, Spot film obtained during IVC venography shows severe stenosis (arrow) of intrahepatic IVC. Note opacification of hemiazygos vein draining into azygos vein at level of diaphragm. D, Spot film obtained during IVC venography shows IVC immediately after TIPS. Note that intrahepatic IVC has decompressed, lumen of vein is now wide, and flow into hemiazygos system is less prominent. Arrow = less prominent azygos vein.

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