Coronary collaterals and left ventricular function early after acute transmural myocardial infarction

The coronary collateral circulation and ventricular function,segmental wall motion and infarct size, were investigated fromhemodynamic and angiographic data in 126 patients with acutetransmural myocardial infarction and complete obstruction ofa major coronary vessel. The patients were divided into twogroups: 74 with obstruction of the right coronary artery and52 with obstruction of the left anterior descending artery.The collateral circulation was rated as absent, poor, fair oradequate: two weeks after infarction, collateral vessels wereobserved in only 27% (poor 15.4%, fair 5.8%, adequate 5.8%)of the patients with an anterior myocardial infarction and inonly 35% (poor 13.5%, fair 16.4%, adequate 4.5%) of the patientswith an inferior myocardial infarction. In both groups of patients, the presence of collateral vesselshad no significant influence on the following parameters: leftventricular performance (left ventricular end-diastolic pressure,left ventricular enddiastolic volume, ejection fraction andmean velocity of fiber shortening), extent of abnormally contractingsegment and segmental wall motion. After anterior myocardialinfarction, there was an insignificant trend to lesser myocardialdamage in patients with coronary collaterals. Thus, coronary collaterals are infrequent in patients with acutetransmural myocardial infarction and total obstruction of acoronary vessel; in these patients we conclude that the collateralshave no effect either on left ventricular function or on thesize of the infarction.     

Transjugular intrahepatic portosystemic shunt for Budd-Chiari syndrome: A comprehensive review

Budd-Chiari syndrome(BCS) is a relatively rare clinical condition with a wide range of symptomatology, caused by the obstruction of the hepatic venous outflow. If left untreated, it has got an high mortality rate. Its management is based on a step-wise approach, depending on the clinical presentation, and includes different treatment from anticoagulation therapy up to Interventional Radiology techniques, such as transjugular intrahepatic portosystemic shunt(TIPS). TIPS is today considered a safe and highly effective treatment and should be recommended for BCS patients, including those awaiting orthotopic liver transplantation. In this review the pathophysiology, diagnosis and treatment options of BCS are presented, with a special focus on published data regarding the techniques and outcomes of TIPS for the treatment of BCS. Moreover, unresolved issues and future research will be discussed.     

Vascular liver disorders (I): diagnosis, treatment and prognosis of Budd-Chiari syndrome

Budd-Chiari syndrome (BCS) is a venous outflow obstruction of the liver that has a dismal outcome if left untreated. Most cases of BCS in the Western world are caused by thrombosis of the hepatic veins, sometimes in combination with thrombosis of the inferior vena cava. Typical presentation consists of abdominal pain, hepatomegaly and ascites, although symptoms may vary significantly. Currently, a prothrombotic risk factor, either inherited or acquired, can be identified in the majority of patients. Moreover, in many patients with BCS a combination of risk factors is present. Myeloproliferative disorders are the most frequent underlying cause, occurring in approximately half of the patients. Recent discovery of the Janus Kinase 2 (JAK2) mutation has significantly contributed to the diagnosis of myeloproliferative disorders. Anticoagulation is indicated for all patients with BCS and additional therapy depends on the severity of symptoms and the extent of venous obstruction. A stepwise therapeutic approach is recommended, with increasing invasiveness and guided by the response to previous treatment. A transjugular intrahepatic portosystemic shunt (TIPS) is proving to be a good therapeutic option in patients with BCS, diminishing the need for surgical shunts. When all other therapy is unsuccessful or in patients with fulminant hepatic failure, a liver transplantation should be considered. Advances in diagnosis and treatment have dramatically improved the prognosis of patients with BCS. Still, many aspects of this complicated disorder remain to be clarified.     

Successful treatment with stent angioplasty for Budd-Chiari syndrome in Behçet’s disease

Budd-Chiari syndrome (BCS) is a very rare vascular complication of Behçets disease (BD) which often leads to death as a result of portal hypertension and liver failure. We report a 45-year-old BD patient who presented with BCS. Diagnosis was confirmed with CT scan and contrast-enhanced MR angiography which showed ascites, short-segment stenosis of the inferior vena cava (IVC), and middle and left hepatic venous thrombosis. Percutaneous transluminal angioplasty (PTA) of the obstructed segment in the IVC was performed and resulted in dramatic reduction of portal venous pressure. Our experience indicates that PTA may be a safe and effective therapeutic modality for BCS in BD which is caused by short segmental obstruction of the IVC.     

Radiological Study of Idiopathic Budd-Chiari Syndrome Complicated by Hepatocellular Carcinoma. A Report of Four Cases

During an 11-yr period (1979-1989), we have experienced five patients with idiopathic Budd-Chiari syndrome (BCS), four (80%) of whom had associated hepatoceilular carcinoma (HCC). In contrast, the incidence of BCS complicated by HCC was 0.7% of a total of 556 patients who underwent surgery for HCC or were autopsied. Hepatitis B virus-related antigen or antibody was positive in one patient each. Four of our five patients were asymptomatic and were initially diagnosed by ultrasonography (n = 3) or computed tomography (n = 1). The hepatic parenchyma histopathological fmdings were cirrhosis and fibrosis in one each. Infection of hepatitis B virus rather than BCS was speculated as a causative factor for HCC in two patients. Membranous obstruction with spotty calcification, intrahepatic bizarre communicating vessels, and the dilated anterior longitudinal veins in spinal canal were recognized in three patients. Three patients had two HCCs which were similar in size and arose from the right and left hepatic lobe, separately, suggesting multicentricity of HCC. Both percutaneous transluminal angioplasty with Gruntzig balloon catheters for the obstruction of the inferior vena cava and hepatic arterial embolization for HCC(s) were performed in three patients. These patients survived 29.3 months on average after the diagnosis of BCS complicated by HCC(s). The opened IVC was confirmed to be patent on an average of 26.3 months after the first angioplasty.     

Budd-Chiari Syndrome Induced by Stage IV Rectal Carcinoid

Budd-Chiari syndrome (BCS) is defined as an obstruction of the hepatic venous outflow anywhere from the small hepatic veins to the suprahepatic inferior vena cava. In this study, a rare case of BCS induced by a metastatic rectal carcinoid is presented. A 57-year-old African American woman with stage IV rectal carcinoid presented with right upper quadrant pain, associated with decreased appetite and weight loss >13 kg over 2 months. Computed tomography scan with contrast enhancement revealed filling defects in the left and middle hepatic veins extending into the suprahepatic inferior vena cava to the junction of the right atrium, suggesting BCS. Thrombophilia workup was negative, and no signs of liver cirrhosis or portal hypertension were found. A hepatitis profile workup yielded negative results. This is the first reported case of BCS that is associated with a metastatic rectal carcinoid. More research is needed to identify the mechanism leading to thrombogenesis in carcinoid tumors.     

Budd-Chiari syndrome： Diagnosis with three-dimensional contrast-enhanced magnetic resonance angiography

AIM: To evaluate the role of three-dimensional contrast-enhanced magnetic resonance angiography (3D CE MRA) in the diagnosis of Budd-Chiari syndrome (BCS). METHODS: Twenty-three patients with BCS underwent 3D CE MRA examination, in which 13 cases were secondary to either hepatocellular carcinoma (11 cases), right adrenalcarcinoma (1 case) or thrombophlebitis (1 case) and 10 suffered from primary BCS. The patency of the inferior vena cava (IVC), hepatic and portal veins as well as the presence of intra- and extrahepatic collaterals, liver parenchymal abnormalities and porto-systemic varices were evaluated. Inferior vena cavography was performed in 10 cases. The diagnosis of IVC obstruction by 3D CE MRA was compared with that demonstrated by inferior vena cavography.RESULTS: The major features of BCS could be clearly displayed on 3D CE MRA. Positive hepatic venous signs included tumor thrombosis (9 cases), tumor compression (2 cases), nonvisualization (4 cases) and focal stenosis (2 cases). Positive IVC findings were noted as severe stenosis or occlusion (10 cases), tumor invasion (2 cases), thrombosis (3 cases), thrombophlebitis (1 case) and septum formation (3 cases). Intrahepatic collaterals were shown in 9 patients,2 of them with “spider web“ sign. The displayed extrahepatic collaterals included dilated azygos and hemi-azygos veins (13 cases) and left renal-inferior phrenic-pericardiophrenic veins (2 cases). The occlusion of the left intrahepatic portal veins was found in 2 cases. Porto-systemic varices were detected in 10 patients. Liver parenchymal abnormalities displayed by 3D CE MRA were enlargement of the caudate lobe (7 cases), heterogenous enhancement (18 cases) and complicated tumors (13 cases). Compared with the inferior vena cavography performed in 10 cases, the accuracy of 3D CE MRA was 100 % in the diagnosis of IVC obstruction.CONCLUSION: 3D CE MRA can display the major features of BCS and provide an accurate diagnosis.     

Acute iatrogenic Budd-Chiari syndrome following hepatectomy for hepatolithiasis: A report of two cases

Budd-Chiari syndrome(BCS)is defined as hepatic venous outflow obstruction at any level from the small hepatic veins to the junction of the inferior vena cava(IVC)and the right atrium,regardless of the cause of obstruction.We present two cases of acute iatrogenic BCS and our clinical management of these cases.The first case was a 43-year-old woman who developed acute BCS following the implantation of an IVC stent for the correction of stenosis in the IVC after hepatectomy for hepatolithiasis.The second case was a61-year-old woman with complete obstruction of the outflow of hepatic veins during bilateral hepatectomy for hepatolithiasis.Acute iatrogenic BCS should be con-sidered a rare complication following hepatectomy for hepatolithiasis.Awareness of potential hepatic outflow obstructions and timely management are critical to avoid poor outcomes when performing hepatectomy for hepatolithiasis.     

布-加综合征、肝血窦阻塞综合征与肝硬化的鉴别

布-加综合征是由肝静脉或其开口以上的下腔静脉阻塞性病变引起的以下腔静脉阻塞、门静脉高压为特点的综合征,诊断依靠影像学检查,治疗的关键是解除梗阻。肝血窦阻塞综合征是指肝窦内皮完整性破坏和肝窦内充血阻塞而产生的肝内窦后性门静脉高压症,临床表现类似布-加综合征,诊断依靠肝组织活检。这两种疾病的治疗和预后均与肝实质病变导致的肝硬化不同,所以临床上需要注意这三种疾病的鉴别。     

Unusual collateral vessel from right subclavian vein to left atrium,a rare complication of superior vena cava obstruction

The most commonly reported collateral systems in the setting of superior vena cava obstruction are azygos venous system, vertebral venous system, external and internal thoracic venous system based on McLntire and Sykes classification. A 49‐year‐old female with renal disease complained dyspnea on exertion. Transesophageal echocardiography showed significant mitral annular calcification, large multi‐lobulated mass at posterior aspect of RA, and complete obstruction of superior vena cava by thrombus formation. Computed tomography angiography showed a collateral vein to the left atrium (LA) roof. This case report is the first one which shows development of collateral vein from right subclavian to LA.     

A caval homograft for Budd-Chiari syndrome due to inferior vena cava obstruction

Transjugular intrahepatic portosystemic shunt (TIPS) is the standard treatment of Budd-Chiari syndrome (BCS) non responsive to medical therapy. However, patients with inferior vena cava (IVC) obstruction proximal to the atrium do not benefit from TIPS and a surgical approach is mandatory. We report the case of BCS due to intrapericardial IVC obstruction. We describe a novel surgical approach using a fresh caval homograft. An attempt to balloon dilatation of the IVC obstruction was complicated by right atrial disruption with tamponade and ventricular fibrillation. Lately, the patient successfully underwent a reconstruction of the cavo-atrial continuity by the interposition of a fresh caval homograft, a novel surgical approach never described before for BCS. Further follow-up revealed progressive reduction and resolution of ascites, and overall clinical improvement. IVC obstruction near to the atrium can be surgically approached with a new technique consisting in inferior vena cava resection and replacement with a caval homograft.     

Redefining Budd-Chiari syndrome:A systematic review

AIM:To re-examine whether hepatic vein thrombosis(HVT)(classical Budd-Chiari syndrome)and hepatic vena cava-Budd Chiari syndrome(HVC-BCS)are the same disorder.METHODS:A systematic review of observational studies conducted in adult subjects with primary BCS,hepatic vein outflow tract obstruction,membranous obstruction of the inferior vena cava(IVC),obliterative hepatocavopathy,or HVT during the period of January2000 until February 2015 was conducted using the following databases:Cochrane Library,CINAHL,MEDLINE,Pub Med and Scopus.RESULTS:Of 1299 articles identified,26 were included in this study.Classical BCS is more common in women with a pure hepatic vein obstruction(49%-74%).HVCBCS is more common in men with the obstruction often located in both the inferior vena cava and hepatic veins(14%-84%).Classical BCS presents with acute abdominal pain,ascites,and hepatomegaly.HVC-BCS presents with chronic abdominal pain and abdominalwall varices.Myeloproliferative neoplasms(MPN)are the most common etiology of classical BCS(16%-62%)with the JAK2V617-F mutation found in 26%-52%.In HVCBCS,MPN are found in 4%-5%,and the JAK2V617-F mutation in 2%-5%.Classical BCS responds well to medical management alone and 1st line management of HVC-BCS involves percutaneous recanalization,with few managed with medical management alone.CONCLUSION:Systematic review of recent data suggests that classical BCS and HVC-BCS may be two clinically different disorders that involve the disruption of hepatic venous outflow.     

Budd-Chiari syndrome: in evolution

Budd-Chiari syndrome (BCS) is a rare but potentially life-threatening disorder caused by hepatic venous obstruction, distinct from cardiac causes of hepatic congestion or sinusoidal obstruction syndrome (formerly known as veno-occlusive disease). BCS may be classified as primary or secondary, depending on the underlying process. Most cases of primary BCS are due to an underlying hypercoagulable disorder. A high index of suspicion is required to make the diagnosis. In most case series, chronic, indolent cases of BCS are more common than acute presentations. Doppler ultrasound, magnetic resonance imaging (MRI), and direct venography are useful in confirming the diagnosis. Systemic anticoagulation should be started expeditiously, as long as there are no contraindications. The use of systemic thrombolysis remains controversial. However, thrombolysis may prove effective when it is administered locally following hepatic venoplasty with or without stenting. Guidelines for the management of more complex cases and of patients who fail medical management are currently in evolution. Budd-Chiari syndrome (BCS) is potentially life-threatening, depending on the extent and rapidity of hepatic venous obstruction. A high index of suspicion is required to clinch the diagnosis, since BCS may be quite indolent or even asymptomatic. Doppler ultrasound or magnetic resonance imaging (MRI) is usually definitive. Systemic anticoagulation should be offered to all patients, unless contraindicated. The role of thrombolysis in BCS remains controversial, and thus it should be reserved for cases undergoing hepatic decompression via percutaneous angioplasty. Guidelines for the management of cases who fail standard medical management are currently in evolution.     

Coronary collateral circulation

The occurrence and influence of coronary collateral circulation and obstruction of the supplying coronary arteries on left ventricular contractility, prevalence of myocardial infarction, and bicycle exercise ergometer test were studied in a random sample of 286 patients with angiographically documented coronary artery disease. Collaterals appeared increasingly in all three main coronary arteries with grade of obstruction. The highest prevalence of collaterals occurred in stenosis of the right coronary artery (60%), followed by the left descending artery (45%); they occurred least in the left circumflex artery (21%) (p less than 0.001). The frequency of intra-arterial collateral circulation was 42%, 11%, and 12%, respectively (p less than 0.001). With total occlusion of the left anterior descending coronary artery, 22% of the patients had normokinetic anterior and apical left ventricular wall when collaterals were present. More often, the inferior wall showed normal contraction with total occlusion of the right coronary artery and collaterals [52%, p less than 0.001 compared with left anterior descending artery (LAD)]. The prevalence of inferior myocardial infarction was 39%, with collateral circulation to the totally occluded right coronary artery. The respective prevalence of anterior infarction and total occlusion in the left coronary artery was 58% (p less than 0.02). The presence or absence of collaterals had no obvious influence on ST-segment response during bicycle ergometer test. In triple-vessel disease, peak work capacity was better when collaterals to LAD were not jeopardized (427 kpm) than when jeopardized (321 kpm) (p less than 0.02).     

Fulminant Budd-Chiari syndrome associated with polycythemia rubra vera and factor V Leiden mutation

Budd-Chiari syndrome (BCS) is a severe disorder characterized by hepatic venous outflow obstruction. Hypercoagulable states are the major etiological factors for the development of BCS and can be identified in about 75% of patients. Multiple etiological factors can be found in the same patient. Hematologic abnormalities, especially myeloproliferative disorders, are the most common causes of BCS. Furthermore, the prevalence of factor V Leiden mutation is three times greater in patients with BCS. Although the clinical course tends to be chronic, BCS may, on rare occasions, cause acute liver failure. Herein, we report a patient who had factor V Leiden mutation and polycythemia rubra vera, presented as fulminant BCS.     

Japanese case of Budd-Chiari syndrome due to hepatic vein thrombosis successfully treated with liver transplantation

A 22‐year‐old Japanese woman was found to have severe esophageal varices and then suffered from hepatic encephalopathy. She was diagnosed with Budd‐Chiari syndrome (BCS) due to hepatic vein (HV) thrombosis accompanied by portal vein thrombosis without inferior vena cava (IVC) obstruction. Latent myeloproliferative neoplasm (MPN) lacking the JAK2‐V617F mutation was considered to be the underlying disease. Liver transplantation was strikingly effective for treating the clinical symptoms attributable to portal hypertension. Although thrombosis of the internal jugular vein occurred due to thrombocythemia, which manifested after transplantation despite anticoagulation therapy with warfarin, the thrombus immediately disappeared with the addition of aspirin. Neither thrombosis nor BCS has recurred in more than 4 years since the amelioration of the last thrombotic event, and post‐transplant immunosuppression with tacrolimus has not accelerated the progression of MPN. In Japan, IVC obstruction, which was a predominant type of BCS, is suggested to have decreased in incidence with recent improvements in hygiene. The precise diagnosis of BCS and causative underlying diseases should be made with attention to the current trend of the disease spectrum, which fluctuates with environmental sanitation levels. Because the stepwise strategy, including liver transplantation, has been proven effective for patients with pure HV obstruction in Western countries, this strategy should also be validated for utilization in Japan and in developing countries where HV obstruction potentially predominates.     

原发性Budd-Chiari综合征的介入治疗

目的分析经皮球囊扩张治疗原发性Budd-Chiari综合征下腔静脉闭塞病变的疗效。方法采用Seldinger穿刺血管方法,行下腔静脉及右心房造影,确定下腔静脉闭塞段,用Brockenbrough穿刺针开通闭塞段,球囊导管扩张病变治疗下腔静脉闭塞病变42例。结果成功率为100％,病变区血管直径扩至12～21mm,平均(18±3．1)mm,下腔静脉压自(17±3)mmHg,降至(7．5±1．8)mmHg,疗效显著(P＜0．01),症状体征明显减轻或消失。结论临床观察表明,Brockenbrough穿刺针开通病变血管是安全的,球囊扩张对绝大部分原发性Budd-Chiari综合征下腔静脉闭塞病变的疗效满意。     

Type II Abernethy Malformation in a Patient with Primary Budd-Chiari Syndrome

Budd-Chiari syndrome (BCS) is a heterogeneous group of disorders characterized by hepatic venous outflow obstruction. Abernethy malformation is a congenital vascular malformation defined by diversion of portal blood away from the liver. Both conditions are rare vascular diseases. We report here the first case of a patient with combined type II Abernethy malformation and BCS from China. The inferior vena cava obstruction was treated with percutaneous balloon angioplasty; close follow-up was elected for the Abernethy malformation.     

Early changes of the portal tract on microcomputed tomography images in a newly‐developed rat model for Budd–Chiari syndrome

Background and Aim: The effect of increased sinusoidal pressure on the portal tract in Budd–Chiari syndrome (BCS) is as yet not elucidated. Our aim was to investigate portal changes in a newly‐developed rat model for BCS. Methods: We created an outflow obstruction in Sprague–Dawley rats (n = 6) by diameter reduction of the inferior vena cava. Left and right liver lobes with portal vein contrast were scanned using microcomputed tomography, and volumes of the portal tree and liver parenchyma were computed by the ANALYZE software program. Results: Portal branching density was significantly lower in BCS than the shams, and decreased over time (P < 0.01). There was a significant drop in volume of both parenchyma and the portal tree in the left but not right lobes. At 6 weeks post‐surgery, the perfusion index (i.e. ratio between both volumes) became equal to (left) or even higher than (right) the shams, suggesting a new equilibrium with preserved portal perfusion. Histological findings were consistent with those observed in humans. Conclusion: As early as day 2, a significant loss of peripheral portal branches was seen, which progressed over time. Inter‐lobar differences in vascular abnormalities suggest compensatory mechanisms. Despite a decrease in both liver and portal vein volume, relative portal perfusion appeared spared.     

Budd–Chiari syndrome: illustrated review of current management

Budd–Chiari syndrome (BCS) is characterized by hepatic venous outflow obstruction at any level from the small hepatic veins to the atriocaval junction. BCS is a complex disease with a wide spectrum of aetiologies and presentations. This article reviews the current literature with respect to presentation, management and prognosis of the disease. Medical, interventional and surgical management of BCS is discussed. Particular attention is paid to interventional and surgical aspects of management. The review is augmented by images, which provide a clinical corollary to the text.