Budd-Chiari syndrome (BCS), or hepatic venous outflow obstruction, is a rare cause of liver disease that should not be missed. Variable clinical presentation among patients with BCS necessitates a high index of suspicion to avoid missing this life-threatening diagnosis. BCS is characterized as primary or secondary, depending on etiology of venous obstruction. Most patients with primary BCS have several contributing risk factors leading to a prothrombotic state. A multidisciplinary stepwise approach is integral in treating BCS. Lifelong anticoagulation is recommended. Long-term monitoring of patients for development of cirrhosis, complications of portal hypertension, hepatocellular carcinoma, and progression of underlying diseases is important.

Budd-Chiari syndrome is a rare disorder caused by hepatic venous outflow obstruction and resulting hepatic dysfunction. Despite a lack of prospective randomized trials, much progress has been made in its management over the last 20 years. The main goals of treatment are to ameliorate hepatic congestion and prevent further thrombosis. The selective use of anticoagulation, vascular stents, transjugular intrahepatic portosystemic stent-shunt and liver transplant has resulted in a significant increase in survival. The diagnosis, initial management and long-term follow-up of patients with Budd-Chiari syndrome is reviewed. The concept of individualization of treatment and a stepwise approach to invasive procedures is also discussed.

Paroxysmal nocturnal hemoglobinuria (PNH) is a rare hematopoietic stem cell disorder characterized by hemolytic anemia, hemoglobinuria, bone marrow failure, and hypercoagulability. Thrombosis is the leading cause of mortality and occurs in one-half of PNH patients, with the hepatic veins being the most common site. Patients with hepatic vein thrombosis (Budd-Chiari syndrome) can present with abdominal pain, hepatomegaly, jaundice, and ascites. Prognosis is poor for these patients; death may occur from liver failure, vessel rupture, intestinal ischemia, infarction, necrosis, or sepsis. The authors report three consecutive cases of successful treatment with catheter-directed thrombolysis and thrombectomy directly in the hepatic veins in patients with PNH who developed acute hepatic vein thrombosis. This treatment represents a potential bridge toward more curative therapies such as allogeneic bone marrow transplant.

Shunting and transplantation are satisfactory methods of treating Budd-Chiari syndrome (BCS). Selection of treatment is based on the degree of hepatic injury (clinical settings), liver biopsy results, potential for parenchymal recovery, and pressure measurements. Shunting is recommended in cases of preserved hepatic function and architecture. In the presence of fulminant forms of BCS, in cases of established cirrhosis or frank fibrosis, or for patients with defined hepatic metabolic defects (e.g., protein C or protein S deficiency), liver transplantation is the treatment of choice. Nonsurgical alternatives, although encouraging, have limited long-term outcome results at the present time. In most cases of BCS, a thrombophilic disorder can be identified. However, it is important to note that postoperative vascular thrombosis has been identified in patients with BCS who do not have a definable hypercoagulable predisposition. It therefore is our practice to recommend early (<24 hours postoperatively) initiation of intravenous heparin therapy in all patients with BCS, who then undergo life-long anticoagulation with coumadin.

Paroxysmal nocturnal hemoglobinuria (PNH) is associated with a high risk of thrombosis, particularly in the peripheral, cerebral, and abdominal veins. We report a patient with an occlusion of the hepatic veins and a slit shape narrowing of the cava inferior consistent with the Budd-Chiari syndrome in whom intravenous fibrinolytic therapy with recombinant tissue plasminogen activator (rt-PA) was applied. Systemic rt-PA was given in a dose of 25 mg rt-PA over 3 h and 25 mg rt-PA as constant intravenous infusion over the next 21 h leading to an incomplete recanalization. The same protocol was applied again 2 days later, resulting in a complete recanalization of the hepatic veins and the vena cava inferior. Our case shows that exclusive systemic application of rt-PA can result in full anatomic and clinical restoration.

Approximately two thirds of cases of hepatic flow obstruction are due to myeloproliferative disorders. Restoration of hepatic blood flow is the essential goal of treatment. Thrombolytic therapy seems to achieve good results at least in selected cases. A 32-year-old woman is presented, with an intermittent increase in platelet count (526-725 x 10(9)/L), two previous spontaneous abortions and acute symptomatic occlusion of hepatic veins, and in whom a diagnosis of essential thrombocythemia was initially carried out in agreement with the polycythemia vera study group criteria. She received recombinant tissue plasminogen activator followed by heparin with restoration of normal hepatic outflow. Asymptomatic re-occlusion of the hepatic veins was observed 1 year later, despite adequate continuous warfarin treatment. Angiography showed marked narrowing of the intrahepatic cava vein due to extrinsic compression by an enlarged liver, not due to a new thrombosis so that no specific intervention could be performed. In the presence of a dearly documented hepatic vein thrombosis, thrombolytic therapy should be considered. The patient was given low-molecular-weight heparin with a dramatic reduction in previously elevated fibrinogen level and a good control of the hepatic function.

To assess the outcomes of current treatment strategies for Budd-Chiari syndrome.

Budd-Chiari syndrome, occlusion or obstruction of hepatic venous outflow, is a disease traditionally managed by portal or mesenteric-systemic shunting. The development of other treatment options, such as catheter-directed thrombolysis, transjugular portosystemic shunting (TIPS), and liver transplantation, has expanded the therapeutic algorithm.

The authors reviewed the medical records of all patients diagnosed with Budd-Chiari syndrome at the Johns Hopkins Hospital during the past 20 years.

A total of 54 patients were identified: 13 (24%) male patients and 41 (76%) female patients, ranging in age from 2 to 76 years (median 33 years). Twenty-one (39%) had polycythemia vera, 3 (5.6%) used estrogens, 11 (20%) had a myeloproliferative or coagulation disorder, and in 7 (13%) the cause remained unknown. Forty-three patients were treated with surgical shunting, 24 mesocaval and 19 mesoatrial. Actuarial survival rates at 1, 3, and 5 years after shunting were 83%, 78%, and 75%, respectively. Of 33 patients surviving more than 4 years, 28 (85%) had relief of clinical symptoms. Five patients required shunt revision and eight had radiologic procedures to maintain shunt patency. Primary and secondary shunt patency rates were 46% and 69% respectively for mesoatrial shunts and 70% and 85% respectively for mesocaval shunts. Clot lysis was successful as primary treatment in seven patients. TIPS was performed in three patients, one after a failed mesocaval shunt. During an average of 4 years of follow-up, these patients required multiple procedures to maintain TIPS patency. Six patients underwent liver transplantation. Of these, three had previous shunt procedures. Five of the transplant recipients are alive with follow-up of 2 to 9 years (median 6).

Both shunting and transplantation can result in a 5-year survival rate of at least 75%, and other treatment modalities may be appropriate for highly selected patients. Optimal management requires that treatment be directed by the predominant clinical symptom (liver failure or portal hypertension) and anatomical considerations and be tempered by careful assessment of surgical risk.

To determine the effects of surgical portal decompression in Budd-Chiari syndrome (BCS) on survival, quality of life, shunt patency, liver function, portal hemodynamics, and hepatic morphology during periods ranging from 3.5 to 27 years.

Experiments in the authors' laboratory showed that surgical portal decompression reversed the deleterious effects of BCS on the liver. This study was aimed at determining whether similar benefit could be obtained in patients with BCS.

From 1972 to 1999, the authors conducted prospective studies of the treatment of 60 patients with BCS who were divided into three groups: the first had occlusion confined to the hepatic veins treated by direct side-to-side portacaval shunt (SSPCS); the second had occlusion involving the inferior vena cava (IVC) treated by a portal decompressive procedure that bypassed the obstructed IVC; and the third group, who had advanced cirrhosis and hepatic decompensation and were referred too late for treatment by portal decompression, required orthotopic liver transplantation.

In the 32 patients with BCS resulting from hepatic vein occlusion alone, SSPCS had a surgical death rate of 3%, and 94% of the patients were alive 3.5 to 27 years after surgery. All 31 survivors remained free of ascites and almost all had normal liver function. No patient with a patent shunt had encephalopathy. The SSPCS remained patent in all but one patient. Liver biopsies showed no evidence of congestion or necrosis, and 48% of the biopsies were diagnosed as normal. Mesoatrial shunt was performed in eight patients with BCS caused by IVC thrombosis. All patients survived surgery, but five subsequently developed thrombosis of the synthetic graft and died. Because of the poor results, mesoatrial shunt was abandoned. Instead, a high-flow combination shunt was introduced, consisting of SSPCS combined with a cavoatrial shunt (CAS) through a Gore-Tex graft. There were no surgical or long-term deaths among 10 patients who underwent combined SSPCS and CAS, and the shunts functioned effectively during 4 to 16 years of follow-up. Ten patients with advanced cirrhosis were referred too late to benefit from surgical portal decompression, and they were approved and listed for orthotopic liver transplantation. Three patients died of liver failure while awaiting a transplant, and four patients died after the transplant. The 1- and 5-year survival rates were 40% and 30%, respectively.

SSPCS in BCS with hepatic vein occlusion alone results in reversal of liver damage, correction of hemodynamic disturbances, prolonged survival, and good quality of life when performed early in the course of BCS. Similarly good results are obtained with combined SSPCS and CAS in patients with BCS resulting from IVC occlusion. In contrast, mesoatrial shunt has been discontinued in the authors' program because of an unacceptable incidence of graft thrombosis and death. In patients with advanced cirrhosis from long-standing, untreated BCS, orthotopic liver transplantation is the only hope of relief and results in the salvage of some patients. The key to long survival in BCS is prompt diagnosis and treatment by portal decompression.

The operations with proven effects on survival in Budd-Chiari syndrome are shunt operations and liver transplantation.

Between 1993 and 1999 (June), 13 cases of Budd-Chiari syndrome have been treated surgically. Four cases had concomitant thrombosis of the inferior vena cava; the others had marked narrowing of the lumen due to the enlarged caudate lobe. Mesoatrial (n = 12) or mesosuperior vena caval (n = 1) shunts were constructed with ringed polytetrafluoroethylene grafts.

The median portal pressure fell from 45 (range 32 to 55) to 20 (range 11 to 27) cm H(2)O (P <0.001). Two patients died in the early postoperative period. One patient who did not comply with anticoagulant treatment had a shunt thrombosis in the second postoperative year. The other 10 patients are alive without problems during a median 42 (range 1 to 76) months of follow-up.

Mesoatrial shunt with a ringed polytetrafluoroethylene graft is effective in Budd-Chiari syndrome cases with thrombosis or significant stenosis in the inferior vena cava.

Many options are available to diagnose and treat patients with the Budd-Chiari syndrome who present with either thrombotic or non-thrombotic occlusion of the major hepatic veins and or vena cava. The goal of therapy is to alleviate venous obstruction and to preserve hepatic function. Low-sodium diets, diuretics, and therapeutic paracentesis are generally ineffective, except for the rare patient who presents with volume overload and incomplete hepatic venous occlusion. Anticoagulants and thrombolytics may be appropriate for selected patients with acute thrombotic venous obstruction. Percutaneous transluminal angioplasty (PTA) of hepatic venous stenoses or caval webs with or without placement of intraluminal stents yield excellent short-term results, but additional studies are warranted to assess long-term efficacy. Transjugular intrahepatic portosystemic shunts (TIPS) may be effective for patients with subacute or chronic disease and ascites refractory to sodium restriction and diuretics. Intrahepatic stents may also serve as a bridge to transplantation for selected patients presenting with fulminant hepatic failure consequent to hepatic venous occlusion. Additional studies will be necessary to assess the role of TIPS in the armamentarium of therapies for patients with the Budd-Chiari syndrome. Decompressive shunts, reconstruction of the vena cava and hepatic venous ostia, transatrial membranotomy, and dorsocranial resection of the liver with hepatoatrial anastomosis are appropriate options for patients with acute or subacute disease who are not candidates for, or fail less invasive therapies. The majority of patients benefit with improvement in liver function tests, ascites, and liver histology; however, hepatic function may deteriorate in patients with marginal reserve. Liver transplantation is reserved for patients with Budd-Chiari syndrome who present with fulminant hepatic failure or end-stage liver disease with portal hypertensive complications. Transplantation is also appropriate for patients who deteriorate after failed attempts at surgical shunting.

To investigate the role of transjugular intrahepatic portosystemic shunt (TIPS) as a bridge to transplantation for patients with Budd-Chiari syndrome (BCS).

Eight patients (five women, three men) with a mean age of 49.8 years (range, 20-61 years) were diagnosed with BCS by means of computed tomography, hepatic venography, and liver biopsy. One patient had acute liver failure, with subacute or chronic failure in seven. TIPS placement was attempted in all eight patients. Clinical follow-up and portograms were obtained in all patients until death or transplantation.

TIPS placement was completed in seven of eight patients (87.5%). During the follow-up period, TIPS occlusion occurred in four patients. TIPS revision in this patient, although successful, was complicated by hemorrhage and multiorgan failure, and the patient died. Assisted patency rate, excluding the technical failure, was 100%. Mean follow-up in the six survivors with TIPS was 342 days (range, 19-660 days). All six survivors had complete resolution of their ascites. Albumin levels improved an average of 0.43 g/dL (range, 0.3-1.4 g/dL). Bilirubin levels improved in five of six patients (83%), decreasing by an average of 5.6 mg/dL (range, 3.0-15.2 mg/dL). Of the six survivors, three underwent elective liver transplantation, one is awaiting transplantation, and one has been removed from the transplantation list because of clinical improvement. One patient was a candidate for transplantation but declined to be put on the list.

Hepatic synthetic dysfunction improves markedly after TIPS placement in patients with BCS. Significant improvement in ascites can also occur. TIPS can be an effective bridge to transplantation for patients with BCS.

To study the efficacy of local infusion of urokinase (UK) in the treatment of symptomatic inferior vena cava (IVC) thrombosis.

Eight patients (five men and three women) who ranged in age from 19 years to 75 years (mean, 56 years) with symptomatic IVC thrombosis underwent local catheter-directed infusion of UK with use of up to three access sites. Infrarenal IVC thrombus and iliac vein thrombus was identified in all patients. Four patients had extension of thrombus proximal to the renal veins. Seven of eight patients had at least one risk factor for IVC thrombosis: hypercoagulable state (n = 3), IVC filter (n = 3), malignancy (n = 2), recent surgery (n = 2), and oral contraceptive use (n = 1). No serious procedure-related complications were encountered, although one patient died 5 days after UK therapy of pulmonary failure due to advanced lung cancer. UK was infused for an average of 79 hours (range, 24-140 hours) and a mean total dose of 7.4 million U of UK (range, 2.9-14.4 million U). Adjunctive balloon angioplasty was performed in three patients. No vascular stents were placed. Clinical and/or radiographic follow-up was obtained in all eight patients.

Thrombolysis was successful in seven of eight (88%) IVCs with no or minimal residual thrombus. The remaining seven patients had no lower extremity swelling 2-24 months (mean, 11 months) after the procedure. Three of seven patients had computed tomographic or venographic follow-up (mean, 9 months; range, 1.5-15 months), demonstrating unchanged or improved IVC patency.

Transcatheter regional infusion of UK for re-establishing venous patency in acute IVC thrombosis appears to be effective with good short-term and mid-term clinical benefit.

Budd-Chiari syndrome (BCS) was initially defined as a symptomatic occlusion of the hepatic veins, but subsequent reports on various obliterative changes that occur in the hepatic portion of the inferior vena cava (IVC) and hepatic vein orifices have resulted in a broadened and ambiguous definition. Membranous obstruction of the inferior vena cava has been regarded by many as a congenital vascular malformation, but its relation to the classical BCS has remained obscure. With modern imaging and recent histological study of new cases, membranous obstruction of the IVC is now considered to be a sequela to thrombosis. How to classify various forms of occlusion and stenosis of the IVC and hepatic vein ostia is a major challenge. In this review, we emphasize that primary hepatic vein thrombosis (classical Budd-Chiari) and an obliterative disease predominantly affecting the hepatic portion of the IVC, both of which account for most patients with venous outflow block, are clinically quite different. In the West, the former is more common than the latter, which constitutes the vast majority of cases of outflow block in developing countries such as Nepal, South Africa, China, and India. The latter is frequently complicated by hepatocellular carcinoma (HCC), and primary hepatic vein thrombosis is not. The major cause of thrombosis is a hypercoagulable state in hepatic vein thrombosis, but more of the latter cases are idiopathic. The clinical presentation of the latter is milder, and onset is frequently inapparent, whereas the former is more severe, sometimes causing acute hepatic failure. Markedly enlarged subcutaneous veins over the body trunk characterize the latter. We propose that these two disorders be clinically distinguished with a suggested term "obliterative hepato-cavopathy" for the latter against classical BCS.

We reviewed our experience of the therapeutic role of radiology in Budd-Chiari syndrome. Patients with stenosis and/or occlusion of the main hepatic veins and/or inferior vena cava (IVC) are suitable for radiological intervention (35% in our series). Eighteen patients (mean age 37.4 years) have undergone radiological intervention over the past 8 years. The site of obstruction was the hepatic veins in 12/18 patients while 6/18 patients had both hepatic vein and IVC obstruction, which in two was due to tumour thrombus. One patient had repeated dilatations of a mesocaval shunt; 49 angiographic venous dilatations were performed (18 during initial intervention, 31 on review) including 10 recanalizations of occlusions. A combined transhepatic-transjugular approach was used for 10/49 procedures. Thrombolysis was performed in 5/18 and stent insertion in 6/18 patients. Three serious complications occurred (IVC stent migration, hepatic artery pseudoaneurysm, myocardial puncture). Follow-up, after initial intervention, has continued for a mean of 24.2 months (range 4 days-92 months). Symptoms related to hepatic venous outflow obstruction were fully relieved in 10/18 (56%) patients and partially relieved in 4/18 (22%) patients. Close monitoring (and re-intervention) during the early post-intervention period is needed because 28% of initial venous dilatations failed to provide adequate venous return in the first instance. Once the patient is stabilized regular review is mandatory as HV restenosis is common after 10 months or more follow-up. The efficacy and safety of radiological intervention make it the preferred first line of treatment in selected patients with Budd-Chiari syndrome.

Budd-Chiari syndrome is the generic term for different forms of hepatic venous outflow obstruction resulting in a clinical picture of portal hypertension and hepatomegaly. Three levels of venous outflow obstruction may be recognized, affecting respectively the small intrahepatic (IVC). Each level of obstruction is related to a different aetiology. Clinical manifestations range from mild symptoms to acute or chronic end-stage liver disease. Treatment is surgical in the great majority of patients. Occlusion of the IVC may be treated by removal of the caval obstruction in selected patients. Hepatic outflow obstruction may be circumvented by different forms of shunting from the portal or upper mesenteric vein to the IVC or right atrium, depending on the level of obstruction and the difference in venous pressure. For the rare patient presenting with acute or chronic end-stage liver failure, hepatic transplantation may be a life-saving procedure.

We report a case of portal vein thrombosis (PVT) in a term neonate following an exchange transfusion through an umbilical vein catheter which was left in situ for 16 h after the transfusion. The baby made a complete recovery after successful thrombolysis with regional streptokinase infusion. To our knowledge, this is the first reported case of a PVT following an indwelling portal venous catheter left in situ with complete resolution following thrombolysis with regional streptokinase infusion.

The patient must be educated to seek medical attention promptly when this malady strikes, and the physician must likewise be taught to institute this therapy as soon as the patient is seen. Less than 6 years ago, it was strongly expressed that there was no rationale for attempting to restore blood flow in the coronary arteries in the setting of myocardial infarction. It was believed that once the diagnosis of myocardial infarction was made, it was too late to relieve myocardial fibers and avert myocyte necrosis. If this thinking and advice of "the damage has already occurred" and "it is too late" prevailed, it would not be known today that treatment of myocardial infarction with thrombolytic therapy within 6 hours of onset of symptoms significantly reduces mortality in comparison with optimal medical treatment (including heparin) without thrombolytic therapy. The concept of thrombolytic therapy is correct. Persistent investigative work in this area will result in better thrombolytic agents and greater dexterity in their use. The thrombolytic agents available today are good, and they can be used safely. To state that agents that have been established to be capable of thrombus resolution should not be used in the treatment of thrombosis is a true example of how not to proceed toward improvement. To be content to remain in the past will not permit entrance into the future.

Budd-Chiari syndrome is characterized by splanchnic congestion due to obstruction of the hepatic venous outflow. A variety of treatment modalities have limited applicability due to their invasive nature, complications or low effectivity. The transjugular intrahepatic portosystemic stent-shunt (TIPS) offers a new treatment by creating an intraparenchymal duct between a main branch of the portal vein and hepatic vein i.e. the intrahepatic part of the inferior vena cava. This paper describes the treatment of two patients with fulminant and subacute Budd-Chiari syndrome treated 2 days and 2 months after the onset of clinical symptoms. It demonstrates that TIPS is a feasible treatment of Budd-Chiari syndrome that restores splanchnic blood flow, reduces collateral circulation and ascites and provides sufficient time to allow for elective liver transplantation, if indicated. Further studies are required to evaluate the effect of TIPS on liver function and survival.

Thrombolytic therapy has been used fairly extensively in the management of acute proximal deep-vein thrombophlebitis of the extremities, acute pulmonary embolism, and acute peripheral arterial thrombosis and embolism in addition to acute thrombotic coronary events. In the presence of acceptable indications and a favorable benefit to risk ratio, this form of therapy, when successful, has served as a useful adjunct in the management of these disorders. In deep-vein thrombophlebitis, lysis of the thrombus before permanent pathological changes (eg, organization, scarring) have occurred can prevent venous valvular dysfunction and postural venous hypertension and its complications, especially the postphlebitic syndrome. In the more severe forms of acute pulmonary embolism, thrombolytic therapy, when applied early after symptom onset, decreases morbidity and is likely to prevent a chronic increase in pulmonary vascular resistance and persistent pulmonary hypertension. In peripheral arterial thrombo-occlusive events, early restoration of flow through thrombolysis has been shown to limit ischemic damage and serve as a useful supplement to angioplasty or surgery. Thrombolytic therapy has been used less extensively in acute strokes. Here the danger of reperfusion causing bleeding into a softened area of brain undergoing infarction has slowed its evaluation for this disorder; its application to stroke remains experimental.

Occlusion or obstruction of hepatic venous outflow results in the Budd-Chiari syndrome. The disorder should be suspected in any patient who suddenly develops massive ascites, and the diagnosis can be confirmed quickly and accurately by hepatic venography. In the absence of surgical intervention, survival is rare. Inferior venacavography and percutaneous liver biopsy can be performed safely in these patients, and both procedures provide useful information for the selection of appropriate surgical therapy. Most cases of the Budd-Chiari syndrome are amenable to mesocaval or mesoatrial shunting. Those patients with documented cirrhosis or fulminant hepatic failure are best managed by orthotopic liver transplantation.

A retrospective study was performed that analyzed 23 patients who had an orthotopic liver transplantation for the Budd-Chiari syndrome with end-stage liver disease. Patient follow-up was as long as 14 years. The technical considerations relevant to the Budd-Chiari syndrome were discussed. There have been no serious complications of postoperative anticoagulation. Three patients, all of whom died, had recurrence of the Budd-Chiari syndrome. No other patient has had evidence of recurrent Budd-Chiari syndrome on postoperative liver biopsies. One-, 3-, and 5-year actuarial survival was 68.8%, 44.7%, and 44.7%, respectively. It was concluded that orthotopic liver transplantation is the most effective treatment for patients with the Budd-Chiari syndrome and end-stage liver disease.

We present a case report of a patient suffering from portal and superior mesenteric vein thrombosis secondary to splenectomy. No surgical procedure could be performed due to the extension of thrombus. Local fibrinolysis treatment with urokinase through a percutaneous transhepatic approach was decided upon, and this procedure had a successful patient outcome.

Of 501 patients with chronic myeloproliferative diseases (c-MPD) 18 developed thrombosis of major abdominal vessels including 6 with hepatic vein thrombosis (Budd-Chiari syndrome). The complication was seen in 14 of 140 (10%) patients with polycythemia vera (PV), 3 of 23 (13%) patients with essential thrombocythemia (ET), 1 of 106 (1%) patients with idiopathic myelofibrosis (IMF), and none of 232 patients with chronic myelogenous leukemia (CML). Leading symptoms and signs were abdominal pain, progressive splenomegaly, widening abdominal girth, ascites, venous collaterals, and nausea and vomiting. The diagnostic modalities with highest specificity were angiography and explorative laparotomy. A causal relationship between the thrombotic event and hematocrit, thrombocyte count, or hemostatic abnormalities at the time of diagnosis could not be established. Detailed laboratory tests of platelet function and coagulation and fibrinolytic parameters of 5 surviving patients did not show any specific defect. Despite medical and surgical intervention, 39% of the patients died within 2 months after diagnosis of the thrombosis. The majority of the survivors developed further complications like liver cirrhosis with portal hypertension and esophageal varices or the short bowel syndrome after extensive bowel resection for mesenterial infarction.

Since 1980 an operation which reestablishes the blood outflow from occluded hepatic veins was performed in 7 patients with Budd-Chiari syndrome by one of us (A. Senning). Using extracorporeal circulation a dorsocranial cylindrical resection of the liver including the confluence of the occluded hepatic veins was performed by transcaval approach. The incised right atrium was sutured around the resected liver area. There was one intraoperative death. In 6 patients with a mean postoperative follow-up of 19.2 months (4-42 months), the patency of hepatoatrial anastomosis was documented by angiography or Doppler-2d-echocardiography. Four patients are free of symptoms and signs of Budd-Chiari syndrome. In one of two patients with associated cirrhosis compression of inferior vena cava reoccurred and in another patient esophageal varices persist. We conclude, that the hepatoatrial anastomosis is an effective treatment of Budd-Chiari syndrome.