Catheter-Directed Thrombolysis for Massive Pulmonary Embolism Resulting from Thrombosis in a Duplicated Inferior Vena Cava: A Case Report.

Pulmonary embolism (PE) associated with duplicated inferior vena cava (IVC) is rare, and there are no reports of this condition treated with catheter-directed thrombolysis. We present the case of a 54-year-old man who developed massive PE caused by thrombi in a duplicated IVC that detached during transcatheter angiography. After implantation of a retrievable filter in the common IVC, the patient underwent catheter-directed thrombolysis. He was asymptomatic at discharge, with complete resolution of thrombosis and successful retrieval of the filter. The patient had an uneventful 9-month follow-up with no further complications.

Comparative study of MRI manifestations of acute and chronic Budd-Chiari syndrome.

BACKGROUND: Magnetic resonance imaging (MRI) is able to diagnose Budd-Chiari syndrome (BCS) by distinguishing differential imaging features of acute and chronic forms of the disease. However, the characteristic imaging differences are still not clear as previous data were mostly obtained from scattered small samples instead of large comparative study. AIM: To investigate MRI manifestations of acute and chronic (BCS), and to evaluate the value of MRI for diagnosis of acute and chronic BCS. METHODS AND RESULTS: We retrospectively compared MRI results of 24 patients with acute and 82 patients with chronic BCS using Mann-Whitney U test for ascites volume, and Fisher's exact test for intrahepatic venous collaterals and extra-hepatic venous collaterals. In the acute group, MRI findings suggested thrombosis in hepatic vein (HV) in all acute patients and additional inferior vena cava (IVC) thrombosis in 5 patients. In the chronic BCS group, 6 and 15 patients showed solitary obstruction either in the IVC or HV, respectively, while 61 patients showed combined IVC and HV obstruction. More patients with acute BCS presented with ascites accompanied with high signals on T2WI from intravenous obstructive lesions. Further, the average maximal spleen diameter in patients with acute BCS, and the ratio of patients with acute BCS developing intrahepatic venous collaterals and extra-hepatic venous collaterals were also lower compared with chronic BCS. All these differences were statistically significant. CONCLUSION: MRI indicates direct and indirect features of BCS, and therefore enables accurate diagnosis of acute and chronic BCS.

Combined thrombus aspiration and recanalization in treating Budd-Chiari syndrome with inferior vena cava thrombosis.

PURPOSE: To investigate the feasibility, safety, and effectiveness of combined thrombus aspiration and inferior vena cava (IVC) recanalization for Budd-Chiari syndrome (BCS) patients with IVC thrombosis. MATERIALS AND METHODS: From March 2011 to October 2014, 17 consecutive BCS patients with IVC thrombosis [male 13, female 4 (mean age 52.6 ± 8.4 years, range 43-72 years)] treated by combined thrombus aspiration and IVC recanalization were enrolled in this retrospective study. An 8F guiding catheter was used as the aspiration catheter. During the treatment, the aspiration catheter was placed from the right femoral vein to the IVC thrombi, and a 20-ml syringe was connected with the aspiration catheter for thrombus aspiration. IVC recanalization was performed after thrombus aspiration. Data on technical success, clinical success, and follow-up were analyzed. RESULTS: Technical success was achieved in all patients. After thrombus aspiration, 12 patients had no visible thrombi on IVC venography, while 5 patients were shown to have the IVC mural thrombi. Afterwards, 13 patients were treated by IVC balloon dilation, and 4 patients were treated by IVC stent insertion. No patient experienced dyspnea after treatment. The average IVC pressure decreased from 29.8 ± 3.4 cmH2O to 8.6 ± 2.1 cmH2O (P < 0.001). Clinical success was achieved in all patients. The average follow-up period was 15.3 ± 11.6 months (range 2-44 months). Long-term IVC patency was achieved in 15 of 17 patients. CONCLUSION: Combined thrombus aspiration and IVC recanalization can be a safe and effective method for BCS patients with IVC thrombosis.

[Application of suprarenal inferior vena cava filter placement in patients with venous thromboembolism].

OBJECTIVE: To evaluate the safety and efficacy of suprarenal inferior vena cava (IVC) filter implantation in patients with venous thromboembolism (VTE). METHODS: Between May 2006 and December 2014, 28 patients with VTE underwent suprarenal IVC filter implantation, anticoagulant treatment and/or catheter-directed thrombolysis at the affiliated hospital of Xuzhou medical college. Follow up examination with color Doppler ultrasound was taken after treatment to eassess the patency of IVC. RESULTS: One filter was successfully implanted in suprarenal IVC in each patient intraoperatively. The filter was retrieved in 26 patients after indwelling of 5 to 17 (mean 11 ± 3) days. The filter was permanently indwelled in 2 patients. There were no complications of filter tilt and migration in all cases. Twenty eight patients were followed up for 2 to 104 (mean 34 ± 34) months. None of the 2 patients whose filters were permanently indwelled presented complications of recurrent pulmonary embolism and IVC occlusion due to the filter. Among 26 patients whose filters were retrieved, the IVC was patent. CONCLUSION: Suprarenal IVC filter placement is a safe and effective method in the treatment of VTE.

[MRV comparison of the angle between the right hepatic vein and the inferior vena cava for patients with membranous obstruction of the inferior vena cava].

OBJECTIVE: To determine whether there are differences in both the right hepatic vein (RHV) morphology and the size of the angle between the inferior vena cava and the RHV in patients with membranous obstruction of the inferior vena cava (MOVC),in healthy individuals and in patients with cinhosis (HLC), in order to help guide development of an effective interventional treatment program. METHODS: Consecutive patients (n=248) were divided into the following three groups: group A (control; n=94), group B (MOVC patients; n=68), group C (HLC patients; n=86). The angle between the hepatic vein and inferior vena cava was measured and defined as the T value. The morphology of the RHV was classified as N, U, or I. The difference of the constituent ratio was compared among the three groups for the T value and the angle type.Measurement data was calculated as x ± s,and groups were compared using one-way ANOVA; count data was calculated as relative number, and groups were compared using the chi-square test. RESULTS: The average T value of group B was significantly higher than that of group A (56.1 ± 13.7 vs. 49.3 ± 7.8, P=0.010) and of group C (vs. 51.5 ± 10.0, P < 0.001); the difference was statistically significant (F=8.750, P < 0.001), but there was no significant difference between the groups A and C.N-type proportion of B group was 48.5% (33/68), greater than that of group A(16.0%,15/94) and C (16.3%, 14/86), x² = 20.1, x² =18.6.U-type proportion of B group was 11.8% (8/68), smaller than that of groups A (28.7%,27/94) and C (37.2%, 32/86), 2 2 = 6.70, x² =12.8, and the differences were statistically significant (P < 0.01). For groups A and C, the N and U types were not significantly different. CONCLUSION: The angle between the RHV and the inferior vena cava in MOVC patients is morphologically different from that in healthy humans, with the angle value in MOVC patients being slightly larger. However, this difference is irrelevant to cirrhosis.

Clinical outcomes of warfarin anticoagulation after balloon dilation alone for the treatment of Budd-Chiari syndrome complicated by old inferior vena cava thrombosis.

BACKGROUND: To evaluate the safety and clinical efficacy of warfarin anticoagulation after balloon dilation alone for the treatment of Budd-Chiari syndrome (BCS) complicated by old inferior vena cava (IVC) thrombosis. METHODS: From January 2008 to November 2013, 19 BCS patients complicated with old IVC thrombosis were treated with balloon dilation followed by oral administration of anticoagulant warfarin. Follow-up was performed at 1 week, then 1, 2, 3, 6, and 12 months after balloon dilation, and then annually thereafter. IVC patency and morphologic changes of the old thrombus were examined by ultrasound, and clinical symptoms and signs were determined by clinical examinations during follow-up. RESULTS: Successful IVC balloon dilation was achieved in the 19 patients (100%). Inferior vena cavography demonstrated the patency of IVC lumen, and the size of the old thrombus was not altered. The mean pressure gradient between IVC and the right atrium was reduced from 27.5 ± 3.0 cm H2O (range, 22-35) before treatment to 5.4 ± 1.3 cm H2O (range: 2-7) after treatment (t = 41.6, P < 0.05; 1 cm H2O = 0.098 kPa). Patients were followed up as outpatients for an average of 15.9 ± 14.4 months (range, 3-66). Anticoagulation with warfarin was well tolerated in all patients after balloon dilation alone. Of the 19 patients, complete resolution of the old thrombus was achieved in 12 patients and partial resolution was achieved in 7 patients. Color Doppler ultrasound showed that 17 patients had IVC lumen patency, and 2 patients had IVC reocclusion. None of the patients had recurrence of thrombosis, symptomatic pulmonary embolism, and bleeding complications throughout the follow-up period. CONCLUSIONS: Our results indicate that warfarin anticoagulation after balloon dilation alone is a safe and effective therapy for BCS patients with old IVC thrombosis.

Liver Injury and Its Impact on Prognosis in Patients with HBV-Related Hepatocellular Carcinoma Undergoing Transarterial Chemoembolization Combined with Tyrosine Kinase Inhibitors Plus Immune Checkpoint Inhibitors.

Purpose: Recently, the triple therapy of transarterial chemoembolization (TACE) combined with tyrosine kinase inhibitors (TKIs) plus immune checkpoint inhibitors (ICIs) has become a new treatment option for advanced or unresectable hepatocellular carcinoma (HCC) patients. We aimed to explore the liver injury and its effect on overall survival (OS) in patients treated with this combination therapy. Patients and Methods: Patients with HBV-related HCC who were treated with TACE-TKIs-ICIs from January 2020 to December 2021 were enrolled. Liver injury and survival time were the main endpoints of the study. Logistic regression analysis was used to analyze the factors associated with liver injury. Cox regression and Kaplan-Meier analysis were used to determine prognostic factors for OS. Results: As of March 2022, 52 of the 119 enrolled patients developed any grade hepatotoxicity: 15 cases with grade 1, 19 cases with grade 2, 16 cases with grade 3 and 2 cases with grade 4. Our analysis indicated that lack of antiviral prevention was a risk factor for liver injury (OR = 0.149; 95% CI: 0.050-0.442; P = 0.001). The findings suggested that liver injury events (HR = 1.912; 95% CI: 1.031-3.546; P = 0.040) was associated with patient death. The median OS of patients without liver injury, grade 1-2 and grade 3-4 liver injury were undefined, 13.7 months and 11.1 months, respectively (log-rank P = 0.034). Conclusion: Liver injury adverse events are common in HBV-related HCC patients treated with TACE-TKIs-ICIs. Patients who developed liver injury had a poor prognosis. For HBV-related HCC patients, effective prophylactic antiviral therapy and regular liver function testing are required before and during this triple therapy.

Mid- to long-term outcomes of initial transjugular intrahepatic portosystemic shunt versus anticoagulation for pyrrolizidine alkaloid-induced hepatic sinusoidal obstruction syndrome.

BACKGROUND: Anticoagulation therapy (AT) is often used as the initial treatment for pyrrolizidine alkaloid (PA)-induced hepatic sinusoidal obstruction syndrome (HSOS). However, transjugular intrahepatic portosystemic shunt (TIPS) is an alternative treatment. This study aimed to determine the mid- to long-term outcomes of TIPS versus AT as the initial treatment for PA-induced HSOS. METHODS: We retrospectively analyzed the clinical data of 61 patients with PA-induced HSOS that were collected between November 2015 and July 2021. The patients were allocated to the TIPS group ( n = 20) or the AT group ( n = 41). These two groups were divided into subgroups according to the severity grading. The clinical data of the patients in both groups were analyzed. Cumulative survival rates were calculated and compared between the two groups and among the subgroups. RESULTS: The clinical symptoms and signs improved or stabilized in 100% of the patients following TIPS and in 85% of the patients following AT at discharge ( P = 0.166). The mortality rate was 0.0% in the TIPS group and 34.1% in the AT group ( P = 0.005). The patients were followed up for 2-69 months (mean, 26.3 ± 20.5 months). In the mild- and moderate-grade subgroups, there was no difference in the cumulative survival rate between the TIPS and AT groups ( P = 0.589 and P = 0.364, respectively). In the severe and very severe-grade subgroups, the cumulative survival rate was higher in the TIPS group than in the AT group ( P = 0.018 and P = 0.025, respectively). CONCLUSION: AT is a suitable initial treatment for mild or moderate PA-induced HSOS, whereas TIPS should be considered the appropriate initial treatment for severe or very severe PA-induced HSOS.

Efficacy, feasibility and safety of TIPS in the treatment of recurrent portal hypertension with variceal bleeding after open splenectomy and esophagogastric devascularization.

PURPOSE: To investigate the efficacy, feasibility, and safety of transjugular intrahepatic portosystemic shunt (TIPS) as a treatment for patients with recurrent portal hypertension with variceal bleeding (RPHVB) who have previously undergone open splenectomy and esophagogastric devascularization (OSED). METHODS: The data were retrospectively retrieved from 39 cirrhotic RPHVB patients who had undergone OSED from August 2015 to December 2020. All patients were treated with TIPS using the Viabahn stent. RESULTS: Out of the 39 patients included in the study, TIPS was successfully performed in 38 patients with a success rate of 97.44%. One patient had a failed attempt due to cavernous transformation of the portal vein (CTPV). Among the 38 patients who underwent TIPS, 33 patients also underwent varicose vein embolization, while the remaining 5 patients only underwent TIPS procedure. A total of 39 Viabahn stents were implanted, with 5 patients receiving stents expanded to their nominal diameter of 8 mm and the remaining 33 patients having their shunt maintained at a diameter of 6 mm. The postoperative hemostasis rate was 97.37% (37/38). The portal vein pressure (PVP) and portal pressure gradient (PPG) decreased significantly from (31.28 ± 6.24) and (20.61 ± 5.14) mmHg to (19.58 ± 4.69) and (9.24 ± 3.07) mmHg, respectively (P < 0.001). During the follow-up period, the rebleeding rate was 6.09% (2/29), while the incidence of hepatic encephalopathy (HE) and shunt dysfunction was 13.79% (4/29) for each. CONCLUSION: Transjugular intrahepatic portosystemic shunt is an effective, feasible and safe treatment for RPHVB patients who have previously undergone OSED. A satisfactory clinical outcome could be achieved with a 6 mm-diameter shunt in most patients.

Efficacy and safety of camrelizumab plus transarterial chemoembolization in intermediate to advanced hepatocellular carcinoma patients: A prospective, multi-center, real-world study.

Objective: Camrelizumab is a newly developed program-death receptor one inhibitor; the real-world evidence about its application in hepatocellular carcinoma (HCC) treatment is lacking. Therefore, this prospective, multi-center, real-world study evaluated the efficacy and safety of camrelizumab plus transarterial chemoembolization (TACE) in treating intermediate-to-advanced HCC patients. Methods: This study consecutively enrolled 101 intermediate to advanced HCC patients. All patients received camrelizumab-based treatment within 30 days of the perioperative period of the TACE operation. The primary outcome was progression-free survival (PFS), and the secondary effects were overall survival (OS), objective response rate (ORR), disease control rate (DCR), and AEs. Results: Specifically, the median PFS was 9.7 (95% confidence interval: 7.4-12.0) months, with a 1-year PFS rate of 30.6%. Meanwhile, the median OS was not reached (NR) yet, with a 1-year OS rate of 61.9%. Besides, the CR, PR, SD, and PD rates were 12.8%, 44.9%, 29.5%, and 12.8%, respectively. The ORR and DCR were 57.7% and 87.2%, respectively. More cycles of camrelizumab were independently correlated with prolonged PFS (hazard ratio (HR): 0.415, P = 0.002), whereas longer intervals between camrelizumab administration and TACE were independently associated with unfavorable PFS (HR: 1.873, P = 0.032). The incidence of total AEs was 90.1%; most AEs were grade 1 (20.8%), grade 2 (28.7%) and grade 3 (37.6%), while only 3 (3.0%) patients had grade 4 AEs. Conclusion: The camrelizumab plus TACE regimen is effective and safe, indicating its potential to serve as a promising treatment choice for intermediate to advanced HCC patients.

[Study on between magnetic resonance venography and digital subtraction angiography on the inferior vena cava obstructive interface morphology of Budd-Chiari syndrome].

OBJECTIVE: To evaluate magnetic resonance venography (MRV) in diagnosing obstructive interface morphology of Budd-Chiari syndrome(BCS). METHODS: MRV examination was performed on 44 cases of BCS, and the images of obstructive interface morphology of the inferior vena cava were reviewed by two radiologists. RESULTS: In all 44 cases, there were 37 cases with complete obstruction and 7 with incomplete obstruction. MRV showed 4 cases with membrane with hole of incomplete obstruction. The morphologies MRV demonstrated that the proximal part of the 37 cases with complete obstruction were mainly divided into the cone type (36 cases) and the planum type (1 case). Besides, the type of distal end of obstruction were the cone type (30 cases), the planum type (4 cases) and the irregular type (3 cases). The overall sensitivity, specificity, positive and negative predictive values for the diagnosis of MRV were respectively 100%%, 57.1%, 92.5% and 100% as compared to the DSA. CONCLUSION: The examination of MRV is capable of revealing the obstructive interface morphology of the inferior vena cava, especially for the distal end of obstruction. MRV can provide guidelines in interventional treatment of Budd-Chiari syndrome.

Long-term outcomes of interventional treatment for Budd-Chiari syndrome with chronic accessory hepatic vein obstruction.

OBJECTIVE: Interventional treatment is the main therapy for Budd-Chiari syndrome (BCS) with hepatic vein obstruction. The aim of this study was to investigate the long-term outcomes of endovascular management for BCS with chronic accessory hepatic vein (AHV) obstruction. METHODS: In total, 68 patients with primary BCS who underwent AHV dilation with or without stenting from January 2008 to December 2018 were included in this retrospective study. The technical success rate and complications were recorded. Cumulative patency rates were estimated by the Kaplan-Meier curves and compared using the log-rank test. RESULTS: Technical success was achieved in all 68 patients, and AHV patency was confirmed by postoperative angiography. Fifty-two patients underwent balloon dilation alone and 16 underwent adjunctive stent implantation. During a mean follow-up period of 60.2 ± 30.5 months, AHV reocclusion occurred in 14 patients in the balloon group and four patients in the stent group (χ2 = 0.034, P = 0.855). The overall cumulative 1-, 3-, 5-, and 7-year primary and secondary AHV patency rates were 90.6, 76.6, 72.0, and 68.2% and 98.4, 91.4, 89.2, and 89.2%, respectively. The cumulative 1-, 3-, 5-, and 7-year primary patency rates of the AHV were 89.8, 73.8, 71.2, and 71.2% in the balloon group and 93.3, 86.2, 75.4, and 60.3% in the stent group, respectively. There was no statistically significant difference between the two groups (P = 0.934). CONCLUSION: Interventional treatment of BCS with chronic AHV obstruction has good long-term outcomes. Both balloon dilation alone and adjunctive stent implantation can be used for AHV recanalization.

[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.

Review of Budd-Chiari Syndrome.

This study aims to report the Budd-Chiari syndrome clinical research status and progress that has occurred in over nearly 30 years in China, and emphasize the value of imaging in facilitating the diagnosis of Budd-Chiari syndrome based on more than 2500 cases. Findings on ultrasonography, computed tomography, magnetic resonance imaging, and digital subtraction angiography images are used to propose new Budd-Chiari syndrome types and subtypes. The new subtype classification presented here has important value for guiding interventional treatment. This study also proposes a new concept of anatomical and functional obstruction of hepatic vein that stresses the compensatory value of accessory hepatic vein and azygos vein and describes the risk of manipulation of the communication branch of inferior vena cava obstruction in interventional therapy.

Hepatocellular carcinoma arising from left accessory liver lobe supplied by the branch of left hepatic artery: A case report.

INTRODUCTION: Accessory liver lobe (ALL) is a rare congenital anomaly. ALL combined with hepatocellular carcinoma (HCC) is even rarer. Most ALLs with HCC are often located in the right liver, and are not supplied by the left hepatic artery. PATIENT CONCERNS: A 77-year-old man was referred to our hospital because of the level of serum alpha-fetoprotein (AFP) continually increased for 1 month. He had no history of chronic hepatitis, alcohol abuse, or cirrhosis of the liver. DIAGNOSES: Preoperative computed tomography (CT) scan revealed a 3.0 × 3.0 × 1.0 cm ovaloid-shaped solid mass in the left subphrenic area with isodensity. Magnetic resonance imaging (MRI) showed a mass with a heterogeneous signal on T1- and T2-weighted images. On contrast-enhanced CT and MRI, the mass showed a pattern of early enhancement and washout. Digital subtraction angiography (DSA) confirmed the mass was fed by the branch of left liver artery. INTERVENTIONS: The mass was treated by transatheter arterial embolization (TAE) followed by surgical resection. Histopathologically showed HCC, consistent with a moderately differentiated. OUTCOMES: Follow-up of 3 months after surgery, the level of AFP returned to normal gradually. CONCLUSIONS: In this report, we describe a rare case of ALL with HCC, located in the left subphrenic area, especially which was supplied by the branch of left hepatic artery has rarely been described. The clinical presentation, radiological features are described in the literature.

The application and efficacy of stent place for Budd-Chiari syndrome.

Objective To evaluate the application value and efficacy on stent place for Budd-Chiari syndrome (BCS). Methods From January 1990 to May 2017, 2228 patients with BCS were admitted to our institution. The mean age was 43.3 years. Stents were placed in inferior vena cava (IVC), hepatic vein (HV), or both after balloon dilation. During follow-up period, the patency of stent was evaluated by ultrasound regularly and the clinic sign was surveyed by letter, telephone or clinic visit. The restenosis of stent were treated with balloon dilatation and thrombolysis to restore the its function. Results IVC type was diagnosed in 1492 cases, HV type in 510 cases, and mixed type in 226 cases. Eighteen patients aborted treatment because of economic reasons, advanced liver cancer, severe scoliosis, or both bilateral iliac veins and total IVC occlusion. Among the other 2210 cases who underwent endovascular therapy, stents were implanted into IVC in 339 cases, HV in 97 cases, mixed type in 64 cases. The rate of restenosis in IVC stent was 11.50% (39/339). After repeat angioplasty, the long-term patency rate reached to 98.12%. The incidence of HV occlusion caused by IVC stent was 12.09% (n = 41). Restenosis occurred in 47 cases (48.45%) after HV stent placement. However, the 5-year patency rate was 91.75% (89/97) after repeat dilatation and stent re-implantation. The incidence of IVC obstruction caused by HV was 3.33% (3 cases). Conclusion IVC stent placement appears to be an effective treatment for the cases of IVC segmental occlusion, and at the same time, the stent has the dual role of compression and fixation of thrombus and support of lumen. The HV and accessory hepatic vein obstruction could happen when the IVC stent crossed these veins ostium. The incidence of the stent restenosis in the HV was higher than that in the IVC.

Number and function of circulating endothelial progenitor cells in patients with primary Budd-Chiari syndrome.

BACKGROUND AND AIM: Primary Budd-Chiari syndrome (BCS) is associated with vascular endothelial injury. Circulating endothelial progenitor cells (EPCs) provide an endogenous mechanism to repair endothelial injury. This study investigated the levels and functionality of EPCs in patients with primary BCS. METHODS: EPCs (CD34+/CD133+/KDR+) were quantified in 82 patients with primary BCS (inferior vena cava type: n=19; hepatic vein type: n=22; and mixed type: n=41), 10 cirrhosis controls (CC group) and 10 age-matched healthy controls (HC group), using flow cytometry. EPCs proliferation was detected by MTT assay, adhesion by adhesion activity assay, and migration capacity by Transwell assay. RESULTS: EPCs levels were significantly lower in the BCS group (0.020±0.005%) than in the CC and HC groups (0.260±0.201%, 0.038±0.007%; P<0.001 for each). EPCs cultured in vitro from BCS and CC groups had, respectively, lower proliferation activity (0.20±0.04, 0.23±0.06 vs 0.58±0.07, each P<0.001), adhesion activity (15.8±1.7, 18.2±4.3 vs 35.0±2.5 cells/random microscopic field (RMF), each P<0.001) and migration activity (16.1±1.5, 16.7±3.0 vs 23.9±2.0 cells/RMF, each P<0.001) than in the HC group. EPCs functionality did not significantly differ between the BCS and CC groups. The numbers and functions of EPCs did not significantly differ among patients with inferior vena cava type, hepatic vein type and mixed type of BCS. CONCLUSION: Patients with primary BCS had lower EPCs levels, with less proliferation, adhesion and migration activities. These findings suggest that lower levels of less functional EPCs may be associated with venous occlusion in primary BCS patients.

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.

Comparison of Long-Term Outcomes of Endovascular Management for Membranous and Segmental Inferior Vena Cava Obstruction in Patients With Primary Budd-Chiari Syndrome.

BACKGROUND: Endovascular management is important for the treatment of primary Budd-Chiari syndrome, which is caused by inferior vena cava (IVC) obstruction. The aims of this study were to compare long-term outcomes of endovascular management for primary Budd-Chiari syndrome patients with membranous obstruction of IVC (MOVC) and segmental obstruction of IVC (SOVC) and explore the optimal endovascular strategy for these conditions. METHODS AND RESULTS: Clinical data of 265 patients with Budd-Chiari syndrome who received endovascular management (MOVC group, n=136; SOVC group, n = 129) were retrospectively reviewed. Cumulative IVC patency rates were generated by the Kaplan-Meier method and compared by log-rank test. In total, 245 patients were followed up from 3 to 72 months after treatment. The difference of long-term outcomes of balloon dilation alone versus stent placement was not significant in each group. The overall cumulative 1-, 3-, and 5-year primary IVC patency rates were 98.3%, 90.7%, and 83.8% in the MOVC group and 88.3%, 79.1%, and 67.9% in the SOVC group (P=0.007), respectively. The long-term IVC patency rates were lower in the SOVC group than in the MOVC group for patients who underwent balloon dilation alone (P=0.001) and did not significantly differ for patients who underwent stent placement between both the groups (P=0.687). CONCLUSIONS: The long-term treatment outcome of endovascular management was better for primary Budd-Chiari syndrome patients with MOVC than for those with SOVC. Balloon dilation alone could be the optimal treatment for patients with MOVC. However, stent placement should be more strongly recommended for patients with SOVC.

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 January 2000 until February 2015 was conducted using the following databases: Cochrane Library, CINAHL, MEDLINE, PubMed 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%). HVC-BCS 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 abdominal wall varices. Myeloproliferative neoplasms (MPN) are the most common etiology of classical BCS (16%-62%) with the JAK2V617-F mutation found in 26%-52%. In HVC-BCS, MPN are found in 4%-5%, and the JAK2V617-F mutation in 2%-5%. Classical BCS responds well to medical management alone and 1(st) 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.