Ann Thorac Surg 2004;78:333-334
© 2004 The Society of Thoracic Surgeons
Case report
Budd-Chiari syndrome and portal vein thrombosis due to right atrial myxoma
George K. Anagnostopoulos, MDa*,
George Margantinis, MDa,
Panagiotis Kostopoulos, MDa,
Glyceria Papadopoulou, MDb,
Athanassios Roulias, MDb,
George Sakorafas, MD, PhDc,
Nikolaos Liassis, MD, PhDb
a Department of Gastroenterology, 251 Hellenic Air Force and Veterans General Hospital, Athens, Greece
b Department of Radiology, 251 Hellenic Air Force and Veterans General Hospital, Athens, Greece
c Department of Surgery, 251 Hellenic Air Force and Veterans General Hospital, Athens, Greece
Accepted for publication June 3, 2003.
* Address reprint requests to Dr Anagnostopoulos, Department of Gastroenterology, 251 Hellenic Air Force Hospital, 34 Dimokritou St, Agia Paraskevi, 15343 Athens, Greece
e-mail: gkanagnostopoulos{at}yahoo.gr
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Abstract
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Budd-Chiari syndrome is a rare disorder characterized by hepatic venous outflow obstruction. Atrial myxomas account for 35% to 50% of primary cardiac tumors and are usually located in the left atrium. We describe a patient who presented with hepatic inferior vena cava and portal vein thrombosis due to a large myxoma of the right atrium. After the successful removal of the tumor, ascites resolved completely. We conclude that right atrial myxoma is a rare but potentially curable cause of Budd-Chiari syndrome.
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Introduction
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Budd-Chiari syndrome (BCS) is a clinical disorder characterized by the occlusion of the major hepatic veins or the suprahepatic inferior vena cava (IVC). BCS is associated with a heterogeneous group of diseases, including myeloproliferative disorders, thrombophilia, malignancy, and congenital webs [1].
Cardiac tumors have rarely been associated with the development of BCS and constitute a probable curable cause of this syndrome. We describe the case of a patient who presented with BCS and portal vein thrombosis due to a large myxoma of the right atrium.
A 52-year-old man was admitted to our hospital with a 48-hour history of nausea and right upper quadrant pain. Shortness of breath and loss of appetite had developed during the 2 weeks before admission. There was no history of alcohol or drug use.
On physical examination, the liver was tender and palpable 4 cm below the right costal margin. The abdomen was mildly distended, with hypoactive bowel sounds. There was no peripheral edema or jugular venous distention, and heart sounds were normal.
Initial laboratory examinations revealed hematocrit, of 42%, a platelet count of 312,000/mm3, a white blood cell count of 6400/mm3, aspartate aminotransferase at 211 U/L, alanine aminotransferase at 173 U/L, alkaline phosphatase at 134U/L, direct bilirubin at 3.5 mg/dL, albumin at 3.7 mg/dL, and an international normalized ratio of 1.51.
Abdominal ultrasound revealed hepatomegaly and ascites. Doppler ultrasound evaluation showed absent hepatic vein flow, complete thrombosis of the portal vein, and the development of small collaterals. Laboratory results for all probable causes of hypercoagulability were negative. Anticoagulation therapy with 36,000 U/d of unfractionated heparin was instituted.
An abdominal computed tomographic (CT) scan demonstrated hypodense, filling defects in the lumen of IVC on the postcontrast scans consistent with intraluminal thrombi (Fig 1).
Transesophageal echocardiography revealed a right atrial mass measuring 3.17 x 3.11 cm, originating close to the orifice of the IVC (Fig 2). Color Doppler demonstrated that the tumor caused the semiocclusion of the right atrium entrance, impeding blood flow from the IVC and creating enhanced velocities at the pulse wave. The right atrial mass was removed under extracorporeal circulation. After midline sternotomy, bicaval and ascending aortic cannulation were accomplished, and total cardiopulmonary bypass was initiated. Right atriotomy was made, and a tumor was revealed in the right atrium, originating close to the orifice of the IVC. The tumor was resected with adequate margin of atrial wall, and the defect was repaired primarily. Histology was consistent with right atrial myxoma. The postoperative course was uneventful. There was a rapid resolution of ascites by the 12th day. Five months after the operation, the patient had no symptoms, liver function was normal, and repeat transesophageal echocardiography demonstrated no abnormal findings.
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Fig 1. Postcontrast abdominal computed tomographic scan reveals intraluminal filling defects in the inferior vena cava.
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Fig 2. Transesophageal ultrasound demonstrates a large mass (arrow) in the right atrium originating close to the orifice of the inferior vena cava.
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Comment
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Budd-Chiari syndrome is a rare disorder characterized by hepatic venous outflow obstruction, leading to centrilobular hepatic congestion with the subsequent development of fibrosis and cirrhosis a few months after presentation. Most cases of BCS are caused either by hepatic vein thrombosis or mechanical outflow obstruction [1]. The most common risk factors for hepatic vein thrombosis are chronic myeloproliferative diseases and prothrombotic coagulation disorders. Cancer and infections are the most common causes of mechanical outflow obstruction in BCS and are usually associated with the obstruction of the hepatic portion of the IVC [1].
The presentation of BCS is either chronic or acute. Chronic BCS is characterized by a history of vague complaints for about 6 months and the onset of ascites, and it is dominated by features and complications of portal hypertension. Laboratory tests show features of chronic liver disease, such as low albumin, prolonged prothrombin time, and near-normal aminotransferase. The acute form of BCS may present with fulminant hepatic failure, encephalopathy, ascites, jaundice, tender hepatomegaly, and hepatorenal syndrome [1].
Noninvasive imaging modalities have become very useful for the diagnosis of BCS. The abnormalities in BCS detectable by ultrasound are the nonvisualization of the hepatic veins, areas of stenosis, caudate lobe hypertrophy, ostial obstruction, the prominence of collateral veins, and proximal dilatation. Doppler demonstration of altered or absent hepatic vein flow has added significantly to diagnostic accuracy [2]. The finding also of hypodense filling defects in the lumen of the IVC and hepatic veins on postcontrast CT scans is considered highly specific for the diagnosis of thrombosis. Other diagnostic findings include the nonvisualization of the hepatic veins or the IVC and a change of the hepatic attenuation of hepatic parenchyma on postcontrast CT scans [2].
Atrial myxomas account for 35% to 50% of primary cardiac tumors and are usually located in the left atrium, attached to the interatrial septum in the vicinity of the fossa ovalis. Only 20% of myxomas arise from the right atrium [3]. Few case reports in the English literature describe atrial tumors causing BCS [4–8]. In most cases, the tumors were malignant, and the patients died shortly after the diagnosis of the syndrome. The intracardiac extension of hepatocellular [4], renal [5], and testicular [6] carcinoma and leiomomyosarcoma [7] of the IVC have been described as causes of BCS. Our patient had a large atrial myxoma almost attached to the junction of the IVC and the right atrium, impeding blood flow and thus creating favorable conditions for thrombi formation and the subsequent development of BCS. The successful removal of the tumor resulted in the resolution of BCS.
In conclusion, we have described a case of BCS and portal vein thrombosis caused by a right atrial myxoma, which is a potentially curable cause of BCS. Thus, right atrial tumors, including myxomas, should be considered in the differential diagnosis of BCS, especially when other causes are absent.
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References
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- Okuda K. Inferior vena cava thrombosis at its hepatic portion. Semin Liver Dis 2002;22:15-26.
- Even-Sapir E., Iles S.E., Barnes D.C. Liver scan in Budd-Chiari syndrome. Correlation with CT and Doppler. Clin Nucl Med 1993;18:706-708.[Medline]
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- Saisse J., Hardwigsen J., Castellani P., Caus T., Le Treut Y.P. Budd-Chiari syndrome secondary to intracardiac extension of hepatocellular carcinoma. Two cases treated by radical resection. Hepatogastroenterology 2001;48:836-839.[Medline]
- Ciancio G., Soloway M. Renal cell carcinoma invading the hepatic veins. Cancer 2001;92:1836-1842.[Medline]
- Watts R.W.E. Testicular teratoma with extensive intracardiac metastases. Br Heart J 1947;9:175-177.[Free Full Text]
- Kracht M., Becquemin J.P., Anglade M.C., Mathieu D., Hillion M.L., Teboul J.L. Acute Budd-Chiari syndrome secondary to leiomyosarcoma of the inferior vena cava. Ann Vasc Surg 1989;3:268-272.[Medline]
- Cujec B., Ulmer B., McKaigney J.P., Bharadwaj B. Right atrial myxoma presenting as Budd-Chiari syndrome. Ann Thorac Surg 1987;44:658-659.[Abstract/Free Full Text]
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Abstract
Introduction
