Percutaneous recanalization in hepatic vein-type Budd-Chiari syndrome: hepatic or accessory hepatic vein.

PURPOSE: This study was designed to assess the clinical efficiency and long-term outcomes of hepatic vein (HV) and accessory hepatic vein (AHV) recanalization in patients with HV-type Budd-Chiari syndrome (BCS). MATERIAL AND METHODS: A total of 27 patients with HV-type BCS underwent AHV recanalization and 94 patients had HV recanalization at our center from January 2012 to December 2019. The treatment effectiveness and long-term outcomes were compared. RESULTS: Technical success was accomplished in all patients, without any procedure-related complications. The clinical success rates were 96.3% (26/27) and 95.7% (90/94) (p = 1.000). In the AHV and HV groups, re-obstruction was observed in 5 and 36 patients, respectively (p = 0.056). The median primary durations of AHV and HV patency were 64 and 49 months, respectively (p = 0.036), while the median secondary durations of AHV and HV patency were 70 and 64 months, respectively (p = 0.134). The median overall survival after AHV and HV recanalization was 73 and 78 months, respectively (p = 0.263). CONCLUSIONS: Our findings suggest that AHV could be employed as a replacement for HV, as a hepatic drainage vein, in HV-type BCS patients.

Circulating microRNA profile in patients with membranous obstruction of the inferior vena cava.

Membranous obstruction of the inferior vena cava (MOVC) is a common type of Budd-Chiari syndrome. However, the pathogenesis of MOVC has not been fully elucidated. Recent studies demonstrated that microRNAs (miRNAs or miRs) are involved in multiple diseases. To the best of our knowledge, specific changes in the expression of miRNAs in MOVC patients have not been previously assessed. The present study used a microarray analysis, followed by reverse transcription-quantitative polymerase chain reaction (RT-qPCR) validation, with the aim to access the miRNA expression levels in the plasma of 34 MOVC patients, compared with those in healthy controls. The results revealed a total of 16 differentially expressed miRNAs in MOVC patients. Subsequently, RT-qPCR analysis verified the statistically consistent expression of 5 selected miRNAs (miR-125a-5p, miR-133b, miR-423-5p, miR-1228-5p and miR-1266), in line with the results of the microarray analysis. These 5 miRNAs, which were described as crucial regulators in numerous biological processes and vascular diseases, may play an important role in the pathogenesis of MOVC. Bioinformatics analysis of target genes of the differentially expressed miRNAs revealed that these predicted targets were significantly enriched and involved in several key signaling pathways important for MOVC, including the ErbB, Wnt, MAPK and VEGF signaling pathway. In conclusion, miRNAs may involve in multiple signaling pathways contributing to the pathological processes of MOVC. The present study offers an intriguing new perspective on the involvement of miRNAs in MOVC; however, the precise underlying mechanisms require further validation.

Budd-Chiari Syndrome in Young Chinese: Clinical Characteristics, Etiology and Outcome of Recanalization from a Single Center.

PURPOSE: We aimed to characterize the clinical profile, etiology, and outcomes of young Chinese patients with Budd-Chiari syndrome treated with recanalization. METHODS: A total of 35 consecutive young patients (≤25 years of age) with primary Budd-Chiari syndrome treated with recanalization at our center were enrolled in this study between March 2011 and December 2014. Data on baseline information, etiology tests, therapeutic recanalization strategies, and follow-up were collected. RESULTS: The most common clinical feature was ascites, present in 33 cases (94%). Hepatic vein obstruction was present in 60% (21/35) of patients, inferior vena cava obstruction in 3% (1/35), and combined obstruction in 37% (13/35). The most common risk factor for thrombosis was hyperhomocysteinemia (14/35, 40%). Recanalization was technically successful in 32 of 35 patients (91%), and clinically successful in 28 of these 32 patients (88%). The cumulative 1- and 3-year primary patency rates were 75.2 and 54.3%, respectively. The cumulative 1- and 3-year secondary patency rates were 89.3 and 89.3%, respectively. The cumulative 1- and 3-year survival rates were 96.9 and 93.8%, respectively. CONCLUSION: In this study, the most common type of lesion was hepatic vein obstruction, the most common thrombotic risk factor was hyperhomocysteinemia, and recanalization resulted in good mid-term outcomes in young Chinese patients with Budd-Chiari syndrome.

Fibrinolysis status in the Budd-Chiari syndrome in China.

Pathogenesis and clinical characteristics of the Budd-Chiari syndrome (BCS) in Asia are somewhat different from the ones observed in Western countries. Obstruction of the inferior vena cava (IVC) or of the hepatic veins is caused to a greater extent by membranous webs than by thrombosis. Impaired fibrinolysis has been found in European patients with BCS, but its status in Chinese patients with this condition is still unknown. To explore the characteristics of fibrinolysis in BCS patients in this country, we measured the euglobulin lysis time (ELT) for overall fibrinolysis and the plasma levels of five fibrinolytic components in 65 Chinese patients with BCS and 43 healthy controls. In patients, ELTs were slightly shorter than in controls (mean, 293 vs. 357 min, P < 0.02), tissue type plasminogen activator levels were higher than in controls (mean, 239 vs. 185 pg/ml, P < 0.01), and plasminogen activator inhibitor 1 levels were lower than in controls (mean, 1.43 vs. 1.73 ng/ml, P < 0.001). To explore BCS in more detail, we subgrouped the cases according to age, type of venous occlusion, Child-Pugh score, and thrombosis. As a result of this analysis, we found that young patients (age <30 years) had a longer ELT (mean, 440 min) than the older patient groups (30 ≤ age ≤ 44, 45 ≤ age ≤ 54, age>54 years; mean ELT = 242, 198, and 289 min, respectively, all P < 0.05). The independent hepatic vein occlusion subgroup showed a longer ELT (mean, 367 min) than the combined hepatic vein and IVC or the independent IVC occlusion subgroup (mean ELT = 233 and 260 min, both P < 0.05). ELT did not show significant differences between Child-Pugh class A and B subgroups (mean, 267 vs. 333 min, P > 0.05). ELT in the subgroup without thrombosis was shorter than in controls (mean, 288 vs. 358 min, P < 0.05), and in the subgroup with thrombosis, it was also slightly shorter than in controls, without reaching statistical significance (mean, 306 vs. 358 min, P > 0.05). By and large, overall fibrinolytic potential was slightly increased in Chinese patients with BCS in this study, but fibrinolysis differed according to its baseline characteristics. Compared with the one seen in BCS patients from Western countries, BCS in China exhibits certain special changes in fibrinolysis and we were able to explain some of these changes.

Inferior vena cava leiomyosarcoma confirmed by catheter suction biopsy during digital subtraction angiography.

Leiomyosarcoma of vascular origin is a rare disease and most cases arise in the inferior vena cava. Inferior vena cava leiomyosarcoma (IVCLMS) usually presents in females in their sixth decade of life. The clinical symptoms are often non-specific and the diagnosis is often delayed. Current imaging techniques can accurately differentiate inferior vena cava neoplasms from other non-neoplastic lesions. However, definitive diagnosis of IVCLMS needs histologic evidence. We report a case of IVCLMS in a 61-year old Chinese woman. This is the first IVCLMS case confirmed by catheter suction biopsy during digital subtraction angiography.

Comparison osteopontin vs AFP for the diagnosis of HCC: a meta-analysis.

BACKGROUNDS: Osteopontin (OPN) has been reported as one of the most promising markers for hepatocellular carcinoma (HCC). The goal of this meta-analysis was to evaluate the clinical value of the plasma OPN versus alpha-fetoprotein (AFP) in the diagnosis of HCC. METHODS: A comprehensive literature search of PubMed, Foreign Medical Journal Service, Chinese Science Journals Database, and Google Scholar was conducted on articles published before 1st October 2013. The sensitivity, specificity, and other measures of accuracy of OPN in the diagnosis of HCC were pooled using random effects models. The methodological quality of each study was assessed by QUADAS (quality assessment for studies of diagnostic accuracy). Statistical analysis was performed by employing Meta-Disc 1.4 and Stata, version 12. Summary receiver operating characteristic curves were used to summarize overall test performance. RESULTS: Seven studies were included in our meta-analysis. The summary estimates for OPN and AFP in the diagnosis of HCC in these studies as follows: sensitivity, 0.86 (0.79-0.91) vs 0.66 (0.53-0.76), specificity, 0.86 (0.69-0.94) vs 0.95 (0.87-0.98), positive likelihood ratio (PLR), 6.10 (2.43-15.32) vs 13.25 (4.69-37.49), negative likelihood ratio (NLR), 0.16 (0.09-0.28) vs 0.36 (0.26-0.51), diagnostic odds ratio (DOR), 38.52 (8.99-165.08) vs 36.75 (11.04-122.32), and the area under the curve (AUC), 0.92 vs 0.87. CONCLUSION: Our study demonstrates that OPN has a comparable accuracy to AFP for the diagnosis of HCC, while the value of OPN in combination with AFP for HCC detection deserves further investigation.

[Budd-Chiari syndrome in children and adolescents: therapeutic radiological intervention].

OBJECTIVE: Due to its minimal-invasive approach, endovascular procedure had replaced surgery in treating Budd-Chiari syndrome (BCS). The interventional therapy was a safe and effective treatment in adults with BCS and the cure rate was high. However Budd-Chiari syndrome in children and adolescents is rare. Published literature on interventional procedure for Budd-Chiari syndrome in children and adolescents is scarce. The aim of the study was to present results of percutaneous transluminal angioplasty (PTA) and stents placement in children and adolescents with BCS and to evaluate the efficacy and safety in these patients of this approach. METHOD: Twenty-five patients [16 boys and 9 girls; average age of (14.5 ± 3.4) years old; age ranged from 5 to 17 years] with Budd-Chiari syndrome who were hospitalized from December 1990 to August 2012 were presented. All of them were diagnosed by color Doppler ultrasound scan while 12 of them had magnetic resonance venography (MRV) scan. All of the patients had undergone angiographic examination. Four cases with membranous obstruction of the inferior vena cava (IVC) were treated with PTA. One case with segmental block of IVC was treated with PTA and stent placement. Five cases with membranous obstruction of IVC and hepatic vein (/and accessory hepatic vein) were treated with PTA. Among 8 cases with membranous obstruction of hepatic veins, 6 cases were treated with PTA and the others with PTA and stent placement. Among 4 cases with blocks of 3 hepatic veins (HVs), one was treated with PTA, one with PTA plus catheter thrombolysis plus PTA, one with PTA and stent placement and the other one was unsuccessful. Three cases with obstruction of HV and accessory HV (AHV) were treated with PTA. Totally, 24 patients were treated with interventional approach and followed up. RESULT: The procedure was successful in 24 patients. The involved veins (hepatic veins or IVC) were patented after interventional procedure. The pressure of hepatic vein was (42.1 ± 4.2) cm H2O (37-50 cm H2O) (1 cm H2O = 0.098 kPa) before the interventional therapy, while it was (17.3 ± 3.3) cm H2O (14-26 cm H2O) after it. The pressure of IVC was (30.6 ± 2.9) cm H2O (26-36 cm H2O) before the interventional therapy, while it was (18.8 ± 4.2) cm H2O (15-26 cm H2O) after it. The symptoms and signs vanished instantly after interventional procedure. There were no procedure-related complications. The rate of overall initial cure was 96%. The patients were followed up for a mean of 25.8 months (range 6 months to 8 years). Seven cases developed restenosis after first procedure. Five of them were treated with PTA, one with PTA plus catheter thrombolysis plus PTA, one with PTA and stent placement. All of the involved veins were patented again. Clinical symptoms were relieved. There were no procedure-related complications as well. CONCLUSION: The interventional procedure in children and adolescents with BCS is the same as in adults. Radiological therapeutic intervention is efficacious and safe in children and adolescents with BCS.

Combining angioplasty with percutaneous microwave ablation for treating primary Budd-Chiari syndrome associated with hepatocellular carcinoma in two patients: A case report.

Percutaneous transluminal angioplasty using balloon catheters for Budd-Chiari syndrome (BCS) and transcatheter arterial chemoembolization (TACE) for unresectable hepatocellular carcinoma (HCC) have become increasingly accepted as alternative therapeutic modalities. However, few studies have investigated the clinical efficacy of combining percutaneous microwave ablation with angioplasty for patients with BCS complicated by HCC. In the present study, a safe and effective method for treating BCS associated with HCC is presented. Color Doppler ultrasonography, magnetic resonance imaging (MRI), computed tomography (CT), inferior venacavography, hepatic arteriogram and cytological examinations were used for the diagnosis. A KY2000 microwave system with an emission of 915 MHz was also employed for the treatment. Two patients with BCS associated with HCC that were administered different adjuvant drug treatments underwent percutaneous transluminal angioplasty and percutaneous microwave ablation successfully, with no treatment-related complications. Combining angioplasty with percutaneous microwave ablation may represent an alternative method for the treatment of BCS associated with HCC.

[Interventional treatment of idiopathic long-segment occlusion of infrahepatic inferior vena cava complicated with thrombosis].

OBJECTIVE: To evaluate the efficacy of interventional treatment of idiopathic long-segment occlusion of infrahepatic inferior vena cava (IVC) complicated with thrombosis. METHODS: Fourteen patients with idiopathic long-segment occlusion of infrahepatic IVC complicated with thrombosis underwent endovascular recanalization. All procedures were performed under local anesthesia via internal jugular vein in combination with a unilateral femoral approach. First catheter-directed urokinase thrombolysis was performed. Then the occlusion of IVC was treated with balloon angioplasty and/or stent placement. RESULTS: Iliocavogram demonstrated an occlusion of IVC from intrahepatic segment to infrarenal segment in 3 patients and an occlusion of infrahepatic IVC above renal veins in 11 patients. Thrombosis was located in IVC (n = 14) and extended to iliofemoarl veins (n = 12). Technical success was achieved in 12 patients. IVCs were recanalized successfully. Complete or partial thrombus removal was accomplished in 8 and 4 cases, respectively. Recanalization failure occurred in 2 patients. No rethrombosis occurred over a mean follow-up of 12 +/- 6 months (range: 1 - 36). And an asymptomatic restenosis of IVC was diagnosed by duplex scanning. CONCLUSION: Interventional treatment of idiopathic long-segment occlusion of infrahepatic IVC complicated with thrombosis is a safe and effective method.

Interventional endovascular treatment for Budd-Chiari syndrome with long-term follow-up.

PURPOSE: To present results of a 9 year followup of percutaneous transluminal angioplasty (PTA) and stents placement in patients with Budd-Chiari Syndrome (BCS) and to evaluate the clinical value and applicability of this approach. MATERIALS AND METHODS: 44 consecutive patients with BCS (25 male and 19 female; average age, 42.6 years; age range, 19-77 years) were treated with PTA and stents during a 9-year period. The mean duration of symptoms was 46 months. Underlying active malignancy was the cause of occlusion in 5 patients. 3 patients had a history of taking oral contraceptives. The obstructed inferior vena cava (IVC) or hepatic vein (HV) were first dilated by a percutaneous transluminal balloon, and then a self-expanding stent was placed. Clinical patency was defined as absence or improvement of symptoms. Clinical follow-up was supplemented with colour Doppler sonography, CT scan, or both. RESULTS: Technical success was achieved in 97.2% (35/36) IVC and 83% (10/12) HV PTA and stents. Significantly, the IVC pressure decreased from 2.7 kPa (SD = 0.3) to 1.5 kPa (SD = 0.4) and HV pressure dropped from 2.3 kPa (SD = 0.4) to 1.3 kPa (SD = 0.2). The symptoms and signs disappeared or were relieved after operation in most of the patients. A few serious related complications including one stent migration and one pulmonary emboli occurred and were resolved in time. Patients were followed for a mean of 44 months (range 3-102). Short- and long-term results were satisfactory except for 3 patients (9.4%, 1 IVC, 2 HV) who presented with a restenosis or thrombosis and underwent additional therapy. There were 5 deaths owing to underlying malignant disease 3-17 months after the procedures. CONCLUSION: PTA and stent placements proved a safe and effective treatment in BCS and had a good long-term outcome and should be considered in patients who have symptoms or have no adequate alternative therapy.