Ann Thorac Surg 2003;76:278-280
© 2003 The Society of Thoracic Surgeons
Case report
Cavoatrial bypass for occlusion of the inferior caval vein in a patient with budd-chiari syndrome
Markus P. Wilhelm, MDa*,
Gerhard Spillner, MDa,
Martin Rössle, MDb,
Claudia Kurtz, MDc,
Koppany Sarai, MDa,
Friedhelm Beyersdorf, MDa
a Department of Cardiovascular Surgery, University Hospital, Freiburg, Germany
b Department of Gastroenterology, University Hospital, Freiburg, Germany
c Department of Radiology, University Hospital, Freiburg, Germany
Accepted for publication December 6, 2002.
* Address reprint requests to Dr Wilhelm, Department of Cardiovascular Surgery, University Hospital, Hugstetterstr 55, 79106 Freiburg, Germany
e-mail: wilhelm{at}ch11.ukl.uni-freiburg.de
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Abstract
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We report the case of a young man with Budd-Chiari syndrome and occlusion of the inferior caval vein. Peripheral edema was his predominant complaint. Symptoms of portal hypertension were lacking, indicating membranotomy and not portosystemic shunting as the treatment of choice. At operation, membranotomy was not feasible, and a cavoatrial bypass using a 22 mm Gore-Tex graft was placed instead. Shortly after the operation the peripheral edema vanished, diuretic treatment could be withdrawn, and liver function improved. Eighteen months later the bypass remains patent, edema is absent, and liver function is stable. We conclude that cavoatrial bypass is a therapeutic option in patients with occlusion of the inferior vena cava with no clinical symptoms of portal hypertension if transcardiac membranotomy is not feasible.
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Introduction
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Hepatic venous outflow obstruction (Budd-Chiari syndrome) is a relatively uncommon disorder that may result in portal hypertension and liver cirrhosis. Acute and chronic presentation has been reported. The typical clinical signs are ascites, pain in the right upper quadrant of the abdomen as a result of hepatomegaly, and edema of the legs [1]. In the Western world most patients have inherited or acquired thrombophilic disorders. Surgical treatment includes transcardiac membranotomy or cavoatrial bypass [2].
We present a case of a young patient with obliteration of the inferior caval vein as a complication of Budd-Chiari syndrome in whom portal hypertension was mild but symptoms of the caval obstruction severe. Transcardiac membranotomy was not feasible, and therefore, a cavoatrial bypass was implanted.
The 31-year-old male patient was first hospitalized in June 1999 with abdominal pain, edema of the legs, and ascites. The diagnosis of a subacute Budd-Chiari syndrome was confirmed by angiography of the inferior caval vein, demonstrating occlusion of the hepatic veins and significant stenosis of the inferior caval vein. Anticoagulation therapy with warfarin was commenced. During the following year the lower leg edema worsened, and high-dose diuretic treatment was prescribed. The patient was then admitted to our hospital in June 2000.
The clinical examination showed a patient in moderate to good general health with the predominant symptom of edema of the legs. Liver enzymes were slightly elevated (aspartate transaminase, 20 U/L; alanine transaminase, 25 U/L; 
-glutamyltransferase, 129 U/L; alkaline phosphatase, 274 U/L). The bilirubin concentration was 2.0 mg/dL. Despite a slightly elevated concentration of homocysteine (19 µmol/L, normal <10 µmol/L), no evidence of a coagulation disorder was noted.
Duplex sonography demonstrated prograde portal hepatic perfusion with a maximal flow velocity in the extrahepatic portal vein of 17 cm/s. The hepatic veins were occluded and replaced by a network of large collaterals. The inferior vena cava was completely occluded at the level of the original hepatic veins and blood flow below the web was reversed. Computed tomography and nuclear magnetic resonance scans confirmed the duplex sonographic findings, showing a web in the inferior vena cava approximately 1.5 cm below the entry into the right atrium, with collaterals of the right hepatic vein ending below the occlusion, and those of the left hepatic vein above the occlusion (Fig 1).
No ascites could then be detected sonographically.
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Fig 1. Preoperative magnetic resonance imaging scan showing the web in the infrahepatic part of the inferior vena cava. The arrow indicates venous collaterals from the right liver lobe draining into the inferior vena cava.
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Two attempts from the jugular and the femoral vein were undertaken to pass the web for placement of an expandable stent, which proved impossible as the web was extremely rigid.
The surgical strategy was to perform transcardiac membranotomy from the right atrium. The right femoral vein was therefore isolated, and a thick venous catheter was inserted into the inferior vena cava in the infrahepatic part using Seldinger‘s technique. Thereafter, a median sternotomy was done. For extracorporal circulation the ascending aorta and the superior vena cava, through the right atrial appendix, were cannulated. The superior vena cava was encircled with vessel loops. The right atrium was opened, after the extracorporal circulation had been started, with venous drainage through both catheters in the superior and inferior vena cavae (below the liver). Palpation of the upper part of the inferior vena cava showed that it ended blindly, with several large venous collaterals coming from the left liver lobe ending in this part. The venous catheter in the caudal part of the inferior vena cava was palpable from the opened cranial part. It was impossible to reestablish a connection between the two blind-ending parts of the inferior vena cava. For this reason, membranectomy or a patch enlargement was not possible. After opening the peritoneum, the inferior vena cava was isolated caudal to the left liver lobe. An end-to-side anastomosis was performed using a 22-mm Gore-Tex (W.L. Gore & Assoc, Flagstaff, AZ) graft with a thick wall. Then a retroperitoneal tunnel was made dorsal to the left liver lobe with a hole in the diaphragm. The course of the graft is a curve with the concave part being lateral. The graft was anastomosed end-to-side to the middle of the right atrium.
The patient’s postoperative course was uneventful. The leg edema resolved within weeks, and the diuretic medication could be withdrawn. Anticoagulation with warfarin was continued. All biochemical tests normalized, with the exception of -glutamyltransferase (65 U/L) and alkaline phosphatase (254 U/L). At the 18-month follow-up duplex sonography showed a patent bypass with a flow velocity of 40 cm/s. The flow velocity in the portal vein was slightly increased (23 cm/s), suggesting a small decrease in portal hypertension. Duplex sonographic findings were confirmed by computed tomographic scan (Fig 2).
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Fig 2. Three-dimensional reconstruction of a postoperative computed tomographic scan showing the patent Gore-Tex graft from the infrahepatic part of the inferior vena cava to the right atrium.
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Comment
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The Budd-Chiari syndrome is defined as a thrombotic obstruction of hepatic veins leading to acute liver failure or cirrhosis. In most patients the clinical manifestation of the disease is dominated by symptoms of portal hypertension. In addition to hepatic vein thrombosis, the portal vein and inferior vena cava may be involved.
Complete occlusion of the upper inferior vena cava was diagnosed in our patient 1 year after onset of clinical symptoms of the Budd-Chiari syndrome. This was accompanied by increasing leg edema, which was the patient’s main complaint. Portal hypertension was rather mild, as shown by the relatively high flow velocity in the portal vein and the absence of ascites. Hepatic venous outflow was maintained by a well-established network of collaterals. Therefore, portal decompression by a portosystemic or portoatrial shunt in combination with membranectomy or a cavoatrial bypass [3] or transcaval liver resection [4] was not necessary. Balloon dilatation and implantation of a vascular stent as interventional treatment options [5, 6] failed twice. Therefore, surgical transcardiac membranotomy was indicated, as suggested by Ahn and colleagues [2]. As for the interventional approach, surgical membranectomy was again not feasible, and a cavoatrial bypass had to be performed. With prophylactic warfarin therapy, the further course of the disease was uneventful and the bypass remained patent during the 18 months of follow-up.
In summary, a cavoatrial bypass using a synthetic graft seems to be a therapeutic option in patients with intrahepatic obstruction of the inferior vena cava if interventional techniques fail and transcardiac membranotomy is not possible. In patients with no occlusion of the hepatic veins, decompression of the portal system can be expected. However, this could not be achieved in our patient with occluded hepatic veins. In spite of this, hepatic function improved, probably because of a mild but significant increase in hepatic blood flow, which may be explained by facilitating intrahepatic collateral flow.
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References
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- Mitchell M.C., Boitnott J.K., Kaufmann S., Cameron J.L., Maddrey W.C. Budd-Chiari syndrome. Etiology, diagnosis and management. Medicine 1982;61:199-218.[Medline]
- Ahn S.S., Yellin A., Sheng F.C., Colonna J.O., Goldstein L.I., Busuttil R.W. Selective surgical therapy of the Budd-Chiari syndrome provides superior survivor rates than conservative medical management. J Vasc Surg 1987;5:28-37.[Medline]
- Orloff M.J., Daily P.O., Orloff S.L., Girard B., Orloff M.S. A 27-year experience with surgical treatment of Budd-Chiari syndrome. Ann Surg 2000;232:340-352.[Medline]
- Pasic M., Senning A., von Segesser L., Carrel T., Turina M. Transcaval liver resection with hepatoatrial anastomosis for treatment of patients with the Budd-Chiari syndrome: late results. J Thorac Cardiovasc Surg 1993;106:275-282.[Abstract]
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- Pisani-Ceretti A., Intra M., Prestipino F., et al. Surgical and radiologic treatment of primary Budd-Chiari syndrome. World J Surg 1998;22:48-54.[Medline]
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Abstract
Introduction
