Symptoms Of Portal Hypertension Research Articles

Four-dimensional Flow MRI-based Computational Fluid Dynamics Simulation for Noninvasive Portosystemic Pressure Gradient Assessment in Patients with Cirrhosis and Transjugular Intrahepatic Portosystemic Shunt.

Background Transjugular intrahepatic portosystemic shunt (TIPS) dysfunction in patients with liver cirrhosis and recurrent symptoms of portal hypertension is primarily assessed with US and confirmed with invasive catheter venography, which can be used to measure the portosystemic pressure gradient (PSPG) to identify TIPS-refractory portal hypertension. To avoid the risks and costs of invasive catheter venography, noninvasive PSPG evaluation strategies are needed. Purpose To demonstrate the feasibility of the combination of four-dimensional (4D) flow MRI with computational fluid dynamics (CFD) for noninvasive PSPG assessment in participants with cirrhosis and TIPS. Materials and Methods Abdominal 4D flow MRI was performed prospectively in participants with cirrhosis and TIPS between January 2019 and September 2020. Flow rates were measured within the TIPS and inferior vena cava (IVC). The portal vein (PV), TIPS, right hepatic vein, and IVC were segmented on MRI scans to create a CFD mesh. The PV and infrahepatic IVC were defined as inflows for 4D flow MRI-derived flow rates. The suprahepatic IVC was defined as the outflow. CFD simulations were used to noninvasively estimate PSPG as the difference between the simulated pressures in the PV and suprahepatic IVC. Invasive venographic measurements of the PSPG served as the reference standard, and Pearson correlation analysis was conducted to evaluate the relationship between noninvasive estimates and invasive measurements. Results In all 20 participants with cirrhosis (mean age, 58 years ± 9 [SD]; 11 men), 4D flow MRI-based CFD simulations enabled visualization of flow velocities and pressure distributions within the segmented vasculature and TIPS. Noninvasive estimates and invasive measures of PSPG were strongly correlated (r = 0.77; P < .001). The 4D flow MRI-based CFD simulations correctly classified the presence or absence of a post-TIPS PSPG greater than 12 mm Hg in 16 of 20 participants (80%). Conclusion The combination of 4D flow MRI and CFD was feasible for noninvasive PSPG assessment in participants with cirrhosis, portal hypertension, and TIPS. © RSNA, 2024 See also the editorial by Motosugi and Watanabe in this issue.

Medium-to Long-term Outcomes of Rex Shunt in 105 Children With Extrahepatic Portal Vein Obstruction in China

PurposeThe aim of our study was to analyze the medium-to long-term outcomes of Rex shunts in a large series of children with extrahepatic portal vein obstruction (EHPVO). MethodsThe clinical data of 105 children aged between 6 months and 16 years with EHPVO who underwent Rex shunt between October 2014 and June 2021 at our center were retrospectively reviewed after more than 2 years of follow-up. ResultsThe overall patency rate of the Rex shunt was 91.43% (96/105) during a median follow-up of 41 months (range, 24–98 months). Eighty-seven (82.86%) of the 105 patients underwent classical Rex shunt with internal jugular vein (IJV) bypass, and the remaining 18 patients (17.14%) underwent modified Rex shunt with intra-abdominal vein bypass. Patients with a patent shunt experienced portal hypertension resolution, which was characterized by a reduction in portal pressure, disappearance of variceal bleeding, relief of gastroesophageal varices, and relief of splenomegaly or hypersplenism. The rate of Rex shunt thrombosis in our center was 8.57% (9/105), and a repeat Rex shunt was effective for the treatment of graft thrombosis. Anastomotic stenosis occurred in 14.26% (15/105) of the children, 38.46% (5/13) of whom received successful endovascular intervention therapy and experienced remission of portal hypertension symptoms. The patency rate of the classical Rex shunt was higher than that of the modified Rex shunt (97.70% vs. 61.11%), whereas the rate of vascular complications, including anastomotic stenosis and graft thrombosis, of the classical Rex shunt was lower than that of the modified Rex shunt (11.49% vs. 77.78%). Further comparison revealed that the risk of vascular complications was substantially greater in the modified Rex shunt group than in the classical Rex shunt group in the nonadjusted model, minimally adjusted model, and fully adjusted model (RR ranged from 6.77 to 7.07, all p < 0.001). ConclusionsThe Rex shunt provides medium-to long-term benefits for children with EHPVO. The classical Rex shunt with IJV bypass provides the best patency rate and the fewest vascular complications. Levels of EvidenceⅢ Type of StudyRetrospective comparative study.

Intrinsic Causes of Nonfibrotic Portal Hypertension-A Clinicopathologic Review of 56 Patients.

Aim: The differential diagnosis of intrinsic nonfibrotic conditions that may lead to portal hypertension include hepatoportal sclerosis (HPS), nodular regenerative hyperplasia (NRH), and sinusoidal obstruction syndrome (SOS). In this article, we characterize the clinical features and outcome of these lesions when they manifest as portal hypertension. Methods: Data was collected through retrospective patient medical records. Results: Patients (HPS: 28, NRH: 17, SOS: 11) were identified more frequently in recent years. All groups presented with signs and symptoms of portal hypertension. All patients had complex medical histories. An elevated serum alkaline phosphatase occurred in all groups and an elevated bilirubin with SOS. Imaging of the liver with HPS and NRH suggested cirrhosis, which was not seen with SOS. 11%, 12%, and 9% of patients in the HPS, NRH, and SOS respectively, underwent transjugular intrahepatic portosystemic shunt placement to manage the complications of portal hypertension, while 43%, 24%, and 36% of patients respectively, received a liver transplant. Conclusions: Patients with HPS, NRH, and SOS had complex medical histories, likely contributing to the development of these lesions. They are recognized more frequently now. In contrast to HPS and NRH, SOS occurred in liver transplant recipients, was associated with elevated serum bilirubin, and imaging did not suggest the presence of advanced fibrosis/cirrhosis. Liver transplantation appeared to be a viable treatment for complications related to HPS and NRH. Retransplantation for SOS yielded mixed results. HPS, SOS, and NRH should be considered when evaluating liver specimens from patients with unexplained nonfibrotic portal hypertension. Key message: Intrinsic nonfibrotic causes of portal hypertension appear to be increasing in frequency. The differential diagnosis includes NRH, HPS, and SOS. These conditions are associated with complex diseases and possibly due to treatments. Pathologists need to be aware of this differential diagnosis when presented with liver biopsies performed to assess portal hypertension.

Transjugular Intrahepatic Portosystemic Shunt Reduction for Medically Refractory Hepatic Encephalopathy

Abstract Purpose Transjugular intrahepatic portosystemic shunt (TIPS) is an established intervention for symptomatic portal hypertension, with many patients experiencing hepatic encephalopathy (HE) as an undesirable side effect. For medically refractory HE, TIPS reduction can decrease the burden of neurotoxic metabolites. This study aimed to evaluate the efficacy of TIPS reduction for the treatment of medically refractory post-TIPS HE. Methods A retrospective search using an institutional database yielded 45 patients who underwent TIPS reduction between 2011 and 2021. Four patients were excluded due to lack of post-TIPS HE, and 41 patients in total were included. The primary endpoint was improvement of HE after TIPS reduction as measured by the West Haven scores. Secondary endpoints included postreduction recurrence of ascites or gastrointestinal bleeding, procedural complications, and 30-day mortality. Results TIPS reduction was performed in all 41 patients with a 30-day mortality rate of 9.8%. No deaths were attributable to the procedure itself. Twenty-seven patients (65.9%) had improvement in HE and 10 patients (24.4%) proceeded to TIPS occlusion due to refractory HE. The average pre- and postreduction West Haven grades were 2.9 ± 0.5 and 1.9 ± 1.2 (p &lt; 0.001), respectively. One patient (2.4%) had spontaneous TIPS thrombosis after reduction and developed arterial gastrointestinal bleeding, 15 patients (36.6%) experienced recurrent ascites, and there were no cases of variceal hemorrhage. Conclusion In this population, TIPS reduction improved medically refractory HE in 65.9% of patients with a 36.6% risk of recurrent ascites, no cases of variceal hemorrhage, and 9.8% 30-day mortality.

The role of variceal bleeding primary prophylaxis in the management of extrahepatic portal vein obstruction in children

Extrahepatic portal vein obstruction is a major cause of pediatric symptomatic portal hypertension and can lead to profuse variceal hemorrhaging. Purpose - to evaluate the role of primary prophylaxis of the variceal bleeding in children with extrahepatic portal vein obstruction. Materials and methods. A one-center prospective cohort clinical study involved 120 patients with extrahepatic portal vein obstruction who underwent primary or secondary variceal bleeding prophylaxis in 2016-2021. The laboratory, ultrasound, and endoscopy data before and after treatment were collected and evaluated. Results. Variceal bleeding episodes occurred in 5.8% (n=3) of patients who underwent primary prophylaxis and rebleeding occurred in 27.9% (n=19) of those who underwent secondary prophylaxis. Bleeding episodes occurred less frequently in patients from primary prophylaxis group (p=0.013). In patients treated endoscopically (n=53, 44.17%) Variceal bleeding appeared less frequently (n=3, 5.66%) after treatment compared to patients who underwent surgery (n=67 (55.83%), 19 had rebleeding (28.35%)) (p=0.001). In 69.17% patients (n=83) esophageal varices eradication was achieved: in 53.01% (n=44) patients from the primary prophylaxis group and 46.99% (n=39) from the secondary prophylaxis group. Bleeding episodes occurred less frequently after the eradication achievement (p&lt;0.001). The primary prophylaxis led to varices eradication more often than secondary (p=0.003). The varices recurrence episodes rates after the eradication achievement were not different between groups (p=0.51). Conclusions. Primary prophylaxis can reduce the bleeding risk in extrahepatic portal vein obstruction with high risk of variceal bleeding. The prophylaxis by endoscopic variceal banding is an important bridge in the treatment for pediatric patients with portal hypertension that along with portosystemic shunting could potentially improve prophylactic treatment results. The Committee on Clinical Investigation of Bogomolets National Medical University approved this study (Protocol No.141, 27.01.2021). All the studies were conducted according to implemented guidelines in consideration of GCP-ICH and Declaration of Helsinki. The written informed consent of all participants’ parents/guardians was achieved. No conflict of interests was declared by the authors.

Transjugular Intrahepatic Portosystemic Shunt for Advanced Hepatocellular Carcinoma With Main Portal Vein Tumor Thrombus-Related Symptomatic Portal Hypertension

The safety and efficacy of transjugular intrahepatic portosystemic shunt (TIPS) in the treatment of symptomatic portal hypertension (SPH) caused by hepatocellular carcinoma (HCC) with portal vein tumor thrombus (PVTT) of main trunk remains unclear. The purpose of this study was to initially explore the safety and efficacy of TIPS for SPH caused by HCC with PVTT of main trunk. This retrospective study analyzed 16 patients who underwent TIPS for SPH caused by HCC with PVTT of main trunk. The evaluated outcomes were technical success rate, SPH control rate, stent patency rate, overall survival (OS), and complications. From July 2018 to February 2023, sixteen consecutive HCC patients with PVTT of main trunk and SPH were retrospectively identified. Technical success was 93.75%. All patients had complete or partial remission of clinical symptoms, and there were no incidents of acute variceal rebleeding and re-exacerbation of ascites during follow-up. There had no intraoperative TIPS-related complications occurred. One patient developed mild hepatic encephalopathy after TIPS placement during the follow-up period. During follow-up, 13 of 16 patients died of advanced HCC progression, the median OS was 10.0 months, and the cumulative OS of 0.5-, 1-, and 2 years were 66.67%, 45.00%, and 11.25%, respectively. TIPS for SPH caused by HCC with PVTT of main trunk may be safe and effective.

Efficacy, Safety, and Clinical Outcomes of Splenorenal Shunt Surgery as a Therapeutic Intervention for Portal Hypertension Patients

Highlights: 1. This study systematically reviewed the efficacy, clinical outcomes, and safety of the splenorenal shunt procedure with a comprehensive and meticulous approach.2. The splenorenal shunt procedure is an innovative surgical intervention that offers a viable option for the management of portal hypertension. Abstract Portal hypertension is the second most common gastrointestinal bleeding in cirrhosis and non-cirrhosis patients. The splenorenal shunt surgery is a potential intervention that may be considered for portal hypertension patients with clinical symptoms such as upper gastrointestinal bleeding caused by the rupture of gastro-esophageal varices. In this study, the researchers aimed to analyze the efficacy, clinical outcomes, and safety of splenorenal shunt surgery in portal hypertension patients. The sources were obtained from electronic search databases, including PubMed, Google Scholar, and ScienceDirect, using the keywords "Efficacy," "Safety," and "Clinical Outcomes." in relation to splenorenal shunt surgery in portal hypertension patients. The researchers set specific criteria for inclusion and exclusion to select the articles. This systematic review revealed the efficacy of the splenorenal shunt procedure with favorable outcomes. The success rate of splenorenal shunt surgery in reducing the clinical symptoms of portal hypertension varied between 66% and 100%. The prevailing complications observed in this study were shunt thrombosis, rebleeding, and thrombocytopenia. However, notable improvements could be achieved with general treatment. In terms of short- and long-term clinical outcomes, the splenorenal shunt procedure demonstrated favorable results. It can be concluded that splenorenal shunt surgery provides excellent clinical outcomes and should be considered a viable treatment option for patients with both cirrhotic and non-cirrhotic portal hypertension.

A History of Umbilical Vein Catheterization Does Not Preclude Children from a Successful Meso-Rex Bypass.

Umbilical vein catheterization (UVC) can cause portal venous thrombosis, leading to the development of extrahepatic portal venous obstruction (EHPVO) and portal hypertension (PHT). The feasibility of the Meso-Rex bypass (MRB) for the treatment of EHPVO in patients with a history of UVC has been questioned. We compared the feasibility of performing an MRB in patients with or without a history of previous UVC. A retrospective review of patients with EHPVO and known UVC status explored for a possible MRB at our institution was performed (1997-2022). Patients were categorized in two groups: with (UVC(+)) or without (UVC(-)) a history of UVC for comparison. A p-value less than 0.05 was considered significant. One hundred and eighty-seven patients were included (n = 57 in UVC(+); n = 130 in UVC(-)). Patients in the UVC group were significantly younger at surgery and the incidence of prematurity was higher. Other risk factors for the development of EHPVO were similar between the groups, but only history of UVC could predict the ability to receive MRB (odds ratio [OR]: 7.4 [3.5-15.4]; p < 0.001). The success rate of MRB was significantly higher in patients with no history of UVC (28/57 [49.1%] in UVC(+) vs. 114/130 [87.7%] in UVC(-); p < 0.001). However, MRB patency at discharge (25/28 [89.3%] in UVC(+) vs. 106/114 [94.7%] in UVC(-); p = 0.3) was equally high in both groups. Our results indicate that a history of UVC is not a contraindication to MRB. Half of the patients were able to successfully receive an MRB. Patients with symptomatic PHT from EHPVO should not be excluded from consideration for MRB based on UVC history.

TIPS combined with lenvatinib and PD-1 inhibitor in patients with advanced HCC with symptomatic portal hypertension: A retrospective study.

e14628 Background: Symptomatic portal hypertension (SPH) leads to a poor prognosis as a common complication in advanced hepatocellular carcinoma (HCC) patients due to cirrhosis or portal vein tumor thrombus. Although combined therapy including Lenvatinib and PD-1 inhibitors has shown valuable advantages in advanced HCC, the above strategy is limited by liver function impairment due to SPH complications such as variceal bleeding and refractory ascites. Transjugular intrahepatic portosystemic shunt (TIPS) directly relieves portal hypertension which may create a chance for advanced HCC patients with SPH to receive systemic therapy. Thus, TIPS plus following Lenvatinib and PD-1 inhibitor may serve as an effective anticancer modality. This study aimed to evaluate the efficacy and safety of TIPS combined with Lenvatinib and PD-1 inhibitor for advanced HCC patients with SPH. Methods: This retrospective study included thirteen advanced HCC with SPH patients who received TIPS combined with Lenvatinib and PD-1 inhibitor (Tislelizumab/Sintilimab) between January 2020 and January 2023. Main inclusion criteria: a confirmed HCC (BCLC stage C) based on the AASLD with either pathological or imaging findings; no previous systemic anticancer treatment; Child-Pugh score ≤10; ECOG status: 0-1. Portal hypertension with complications (variceal bleeding, refractory ascites). The outcome measures included objective response rate (ORR) (assessed with mRECIST v1.1), disease control rates (DCR), progression-free survival (PFS), overall survival (OS), SPH recurrence rate, and safety. Drug safety was investigated according to CTCAE (version 5.0). Results: With a median follow-up of 6.5 months (range 2.3-19.0), 13 patients with a median age of 57 years (range: 33-70) were evaluable for response and toxicity. 3 (23.1%) patients with Tislelizumab and 10 (76.9%) with Sintilimab. 7 (53.8%) patients were classified as Child-Pugh A class. According to mRECIST v1.1, ORR was 38.5% (95%CI, 11.8%-61.6%) with 1CR and 4 PR, and the DCR was 53.8% (95%CI, 21.3%-73.4%). The median overall survival (OS) was 16.5 months (95%CI,16.1-17.0). The prevalence of rebleeding, ascites and hepatic encephalopathy, was 7.7 %, 15.4%, and 7.7%, respectively. Treatment-related adverse events were confined to grades 1-2 occurring in 3/13 (23.1%) patients as rash (2, 15.4%) and fatigue (1, 7.7%). There were no treatment-related serious adverse events. Conclusions: TIPS combined with Lenvatinib and PD-1 inhibitor demonstrated a valuable anti-cancer strategy for advanced HCC patients with SPH based on the low recurrence rate of SPH and acceptable side effects.

Application of transmesenteric vein extrahepatic portosystemic shunt in treatment of symptomatic portal hypertension with cavernous transformation of portal vein

PurposeTo evaluate the feasibility and efficacy of a transmesenteric vein extrahepatic portosystemic shunt (TmEPS) for the treatment of cavernous transformation of the portal vein (CTPV). Materials and methodsThe clinical data of 20 patients with CTPV who underwent TmEPS between December 2020 and January 2022 ​at Henan Provincial People's Hospital were retrospectively collected. The superior mesenteric vein (SMV) trunk was patent or partially occluded in these patients. An extrahepatic portosystemic shunt between the inferior vena cava and the SMV was established using a stent graft through an infraumbilical median longitudinal mini-laparotomy. The technical success, efficacy, and complication rates were evaluated, and the pre- and postoperative SMV pressures were compared. Patients' clinical outcomes and shunt patency were assessed. ResultsTmEPS was successfully performed in 20 patients. The initial puncture success rate of the balloon-assisted puncture technique is 95%. The mean SMV pressure decreased from 29.1 ​± ​2.9 ​mmHg to 15.6 ​± ​3.3 ​mmHg (p ​< ​0.001). All symptoms of portal hypertension resolved. No fatal procedural complications occurred. During the follow-up period, hepatic encephalopathy occurred in two patients. The remaining patients remained asymptomatic. All shunts were patent. ConclusionsTmEPS is a feasible, safe, and effective treatment option for patients with CTPV.

Abstract No. 47 Efficacy of Post TIPS Ultrasound in Predicting TIPS Dysfunction in the Controlled Expansion Endoprosthesis Era

Evaluate the efficacy of transjugular intrahepatic portosystemic shunt (TIPS) ultrasound (US) in predicting TIPS dysfunction in the controlled expansion endoprosthesis era. A retrospective review of patients who underwent TIPS with a controlled expansion stent (Viatorr CX, WL Gore, Flagstaff, AZ) from January 1, 2018, to March 30, 2021, was performed. Patient demographics, clinical data, US studies and venograms were reviewed. Follow-up US timeline was defined as follows: baseline ≤30 days, early 31-365 days, and late >365 days. US was indicative of ‘TIPS dysfunction’ in the presence of elevated velocity, spatial gradient, temporal gradient, or reversal of flow in PV branches by standard criteria. TIPS occlusion on US was defined as absence of flow on doppler. TIPS venogram with portosystemic gradient (PSG) measurement was performed in all patients with recurrence of portal hypertension symptoms. Patients with TIPS dysfunction on US underwent venogram only if they had recurrence of symptoms or occlusion on US. Mean PSG ≤10 mm Hg was considered abnormal. 121 patients underwent TIPS placement. 83% (n = 100) had follow-up US, approximately 1.3 US per patient. Mean follow-up duration was 425 days. US was done as baseline in 88%, early in 57% and late in 28% of patients. US TIPS occlusion was reported in 5/100 (5%) patients. All those patients were symptomatic and underwent venograms showing occlusion or stenosis with elevated PSG. TIPS dysfunction without occlusion was reported in 56/100 (56%) patients: 28 at baseline, 19 at early and 9 at late US evaluations. Among these, 19/56 underwent venogram due to recurrence of symptoms: 12 had elevated PSG or occlusion and 7 had patent TIPS with normal PSG. 25/99 (25%) patients had venogram due to recurrence of symptoms despite normal US: 9 had elevated PSG or occlusion and 16 had patent TIPS with normal PSG. Mean duration from TIPS placement to stenosis or occlusion on venogram was 84 days. Excluding patients with TIPS occlusion on US, 44/94 (46%) of patients had recurrence of symptoms post TIPS. In this cohort, TIPS dysfunction on US had positive predictive value for stenosis or occlusion of 63%, negative predictive value of 64%, sensitivity of 57% and specificity of 69%. In the era of controlled expansion TIPS stents, current standard sonographic criteria of TIPS dysfunction have limited utility in predicting TIPS stenosis. However, ultrasound remains useful for diagnosing TIPS occlusion.

Significance of a multidisciplinary approach to congenital extrahepatic portosystemic shunt: A changing paradigm for the treatment.

To review the current institutional practice to treat patients with congenital extrahepatic portosystemic shunt (CEPS) and to determine the optimal strategy. We retrospectively reviewed the records of 55 patients diagnosed with CEPS at our center between December 2008 and March 2022. Among these 55 patients, 44 (80.0%) received treatment for CEPS at a median age of 4.7years. The most common indication for treatment was cardiopulmonary complications (45.5%). Therapeutic intervention included shunt closure by endovascular techniques (50.0%) or surgery (40.9%), and liver transplantation (9.1%). A total of 11 were classified as short shunt types, and surgical ligation was performed in all to preserve the major vascular system and prevent complications (p<0.001). Children who received a surgical ligation were more likely to develop complications after shunt closure (p=0.02). Among seven patients with portopulmonary hypertension (POPH), one patient, who received a shunt ligation at <1year-of-age, was only able to completely discontinue medication. Most other CEPS-related complications were completely resolved. Post-treatment complications, including thrombosis and symptoms of portal hypertension, were seen in 16 patients. After shunt closure, one patient was scheduled to undergo liver transplantation for progressive POPH and large residual hepatocellular adenoma. During follow-up, one patient without any treatment for CEPS developed POPH 16years from the diagnosis. Earlier therapeutic interventions should be strongly considered for patients with POPH related to CEPS. However, in view of the invasiveness and treatment complications, special attention should be paid to the management of patients with short shunt types.

Endovascular Alternatives to Transhepatic Shunt Creation.

Portomesenteric decompression is often necessary to treat patients with refractory symptoms of portal hypertension. When transjugular or direct intrahepatic portosystemic shunt creation is not feasible or is inadequate, surgical portosystemic shunt creation is considered, which carries significant morbidity and mortality in these high-risk patients. Surgery is further complicated in patients with portomesenteric thrombosis who require concurrent thrombectomy and long-term anticoagulation. In this article, we outline the technique for performing advanced endovascular alternatives to intrahepatic portosystemic shunt creation including mesocaval and splenorenal shunting. We will also discuss some of the clinical considerations for treating these patients with symptomatic portal hypertension and portomesenteric thrombosis.

Nodular Regenerative Hyperplasia Is Not a Rare Condition After Liver Transplantation: Incidence, Predictive Factors, and Impact on Survival.

The objectives of this study were to evaluate incidence and to identify the risk factors of occurrence and the predictive factors of symptomatic forms of nodular regenerative hyperplasia (NRH) after liver transplantation (LT). To identify risk factors of NRH following LT, we included 1648 patients transplanted from 2004 to 2018 and compared the patients developing NRH after LT to those who did not. To identify predictive factors of symptomatic NRH, we selected 115 biopsies displaying NRH and compared symptomatic to asymptomatic forms. Symptomatic NRH was defined as the presence of ascites, esophageal varices, hepatic encephalopathy, portal thrombosis, retransplantation, or death related to NRH. The incidence of NRH following LT was 5.1%. In multivariate analysis, the independent factor of developing NRH after LT was the donor's age (odds ratio [OR] = 1.02; confidence interval, 1.01-1.03; P = 0.02). Symptomatic forms occurred in 29 (25.2%) patients: 19 (16.5%) patients presented with ascites, 13 (11.3%) with esophageal varices, 4 (3.5%) with hepatic encephalopathy, and 8 (7%) with portal thrombosis. The median period before the onset of symptoms was 8.4 (1.5-11.3) y after LT. The spleen size at diagnosis/before LT ratio (OR = 12.5; 114.17-1.37; P = 0.0252) and thrombectomy during transplantation (OR = 11.17; 1.48-84.11; P = 0.0192) were associated with symptomatic NRH in multivariate analysis. NRH following LT is frequent (5.1%) and leads to symptomatic portal hypertension in 25.2% of patients. Using older grafts increases the risk of developing NRH after LT. Clinicians should screen for signs of portal hypertension, particularly in measuring spleen size.

Study on the comparative analysis of the efficacy of transmesenteric vein extrahepatic portosystemic shunt and transjugular intrahepatic portosystemic shunt in the treatment of cavernous transformation of portal vein

Objective: To compare the safety and efficacy of transmesenteric vein extrahepatic portosystemic shunt (TEPS) and transjugular intrahepatic portosystemic shunt (TIPS) in the treatment of cavernous transformation of the portal vein (CTPV). Methods: The clinical data of CTPV patients with patency or partial patency of the superior mesenteric vein treated with TIPS or TEPS treatment in the Department of Vascular Surgery of Henan Provincial People's Hospital from January 2019 to December 2021 were selected. The differences in baseline data, surgical success rate, complication rate, incidence rate of hepatic encephalopathy, and other related indicators between TIPS and TEPS group were statistically analyzed by independent sample t-test, Mann-Whitney U test, and Chi-square test. Kaplan-Meier survival curve was used to calculate the cumulative patency rate of the shunt and the recurrence rate of postoperative portal hypertension symptoms in both groups. Results: The surgical success rate (100% vs. 65.52%), surgical complication rate (6.67% vs. 36.84%), cumulative shunt patency rate (100% vs. 70.70%), and cumulative symptom recurrence rate (0% vs. 25.71%) of the TEPS group and TIPS group were statistically significantly different (P < 0.05). The time of establishing the shunt [28 (2141) min vs. 82 (51206) min], the number of stents used [1 (12) vs. 2 (15)], and the length of the shunt [10 (912) cm vs. 16 (1220) cm] were statistically significant between the two groups (t = -3.764, -4.059, -1.765, P < 0.05). The incidence of postoperative hepatic encephalopathy in the TEPS group and TIPS group was 6.67% and 15.79% respectively, with no statistically significant difference (Fisher's exact probability method, P = 0.613). The pressure of superior mesenteric vein decreased from (29.33 ± 1.99) mmHg to (14.60 ± 2.80) mmHg in the TEPS group and from (29.68 ± 2.31) mmHg to (15.79 ± 3.01) mmHg in TIPS group after surgery, and the difference was statistically significant (t = 16.625, 15.959, P < 0.01). Conclusion: The best indication of TEPS is in CTPV patients with patency or partial patency of the superior mesenteric vein. TEPS improves the accuracy and success rate of surgery and reduces the incidence of complications.

Tromboembolismo venoso em Idade Pediátrica: Um olhar de 20 anos numa Unidade de Cuidados Intensivos Pediátricos

Objective: Clinical characterization and approach of patients with thromboembolism (VTE). Methods: Retrospective, descriptive study of patients aged &lt;18 years with VTE in a pediatric intensive care unit in a tertiary care hospital over 20 years [2002-2021]. Results: Identified 51 patients: deep vein thrombosis (DVT) (n=30), cerebral vein thrombosis (CVT) (n=21), pulmonary thromboembolism (PTE) (n=4), portal vein thrombosis (PVT) (n=2) and renal vein thrombosis (RVT) (n=1). In 5 patients CVT and DVT coexisted (n=1); PTE and DVT (n=3); and RVT and DVT (n=1). Mean 2.6 cases/year. 52.3% male, median 6 years [1M;17Y]. The presence of a central venous catheter was the main risk factor (72%) in DVT associated with edema in the region; Doppler ultrasound was the exam of choice. All with PTE were adolescents; 75% had chest complaints confirmed by angio-CT; identified =2 risk factors in each patient. The case of RVT was a 2-year-old child with renal neoplasm. The cases of PVT were adolescents with symptoms of portal hypertension. The main symptom in CVT was headache (63.6); 52.4% secondary to upper respiratory infection; thrombophilia study positive in 42.9%. Analytically, all had elevated D-dimers. Most started anticoagulation with enoxaparin (86.3%), which they maintained for 3 to 6 months after discharge. Mean hospital stay was 17.6 days. There was 1 episode of recurrence and 1 death unrelated to VTE.

Hepatoportal Sclerosis-A Clinicopathologic Review of 28 Cases.

The aim of this study was to review a large series of cases with hepatoportal sclerosis (HPS) as a pathologically recognizable entity in liver tissue specimens and describe the associated clinical and radiographic manifestations, along with the outcomes of this entity. Data were collected through a retrospective chart review. Twenty-eight patients were identified that had pathologically defined HPS. All patients had a significant past medical historyand signs and symptoms of portal hypertension. The most consistent laboratory finding was an elevated alkaline phosphatase. Radiographically, 9 patients were mistakenly identified as having advanced fibrosis/cirrhosis. The initial histologic diagnosis was made on biopsy in 20 patients and after transplant in 8 patients. The severity of symptoms was variable andrequired transplantation in 11 patients, 3 were treated with transjugular intrahepatic portosystemic shunt, andtheremaining patients were treated symptomatically. HPS is associated with past medical history that may be causal in nature. Signs and symptoms may be severe enough to require liver transplantation. A significant proportion of patients are radiographically misdiagnosed as cirrhosis. In this small series, overall outcomes for transplanted patients are acceptable.

REX SHUNT IN CHILDREN: COULD DYSFUNCTION BE PROGNOSED? A RETROSPECTIVE ANALYSIS.

The aim: To analyze retrospectively our experience of Rex shunt in children with symptomatic portal hypertension, its effect on hypersplenism regression and varices eradication, assess shunt survival and investigate risk factors, that could lead to shunt dysfunction and thrombosis. Materials and methods: 24 children (16 males, 8 females) ,with portal hypertension included into the study. All surgeries were performed within single center in a period from January 2010 to March 2022. Follow up period was 6.75±1.19 years. Results: Age at diagnosis was 5.39±0.64 years. 5 (20.8%) had umbilical catheter in anamnesis. 16 (66.7%) manifested bleeding episodes as the first sign of portal hypertension. 9 (37.5%) of children manifested severe hypersplenism. Age at Rex shunting was 7.5±0.7 years. In 7 (31.8%) cases Rex shunt thrombosis occurred. 1 successful thrombectomy and 6 splenorenal shunting were performed. Kaplan-Meyer analysis showed Rex shunt survival 0.670 (95%CI 0.420-0.831). Logistic regression model indicated thrombocytes count (p=0.0423) and cytopenia (p=0.0272) as factors that could influence shunt thrombosis. Follow-up group included 18 patients. Spleen volume regression became significant by 1 p/o year p<0,05, thrombocytes significant increasement reached in 1 p/o months (p<0.01), varices involution was achieved by 1 p/o year (p<0,001). Conclusions: Rex shunt effectiveness in study group was 70.9%., shunt survival assessed 0.670 (95%CI 0.420-0.831). Rex shunt was effective in bleeding prophylaxis in all patients of follow up group. Preoperative thrombocytes count (p=0.0423) and cytopenia (p=0.0272) were detected as factors that could influence shunt thrombosis, that is to be considered in RS preoperative period and require following studies.

Hepatic sarcoidosis with symptomatic portal hypertension: A report of 12 cases with review of the literature.

Sarcoidosis is a systemic granulomatosis of unknown etiology, characterized by the presence of immune granulomas. Liver damage is a relatively common extra-pulmonary manifestation, occurring in 3.6-30% of cases. Some patients can develop symptomatic portal hypertension (PH). Few series have evaluated the prognosis of symptomatic PH as well as the efficacy and safety of specific treatment on this complication. This is a multicenter retrospective study of cases of histologically proven hepatic sarcoidosis with symptomatic PH (ascites, digestive hemorrhage) and/or hepatic encephalopathy. Demographic characteristics, comorbidities, clinical manifestations of sarcoidosis, biological data, imaging study of the liver, treatment, and clinical outcomes were collected. Twelve patients were identified, with a mean follow-up of 140 months. The M/F ratio was 1 and Caucasian origin was the most represented (75%). Seven patients presented with hepatic comorbidities: metabolic syndrome, chronic alcoholism or chronic viral hepatitis. Apart from hepatic involvement, mediastino-pulmonary involvement was the most common followed by osteoarticular and skin. Liver damage was inaugural in two thirds of cases. Nine patients developed ascites, six presented esophageal varices complicated by gastrointestinal bleeding. Three patients presented with both ascites and variceal bleeding. One case of hepatic encephalopathy was observed. Five patients presented signs of hepatocellular insufficiency during follow-up, of whom three had hepatic comorbidities. Eight out of 12 patients required second-line treatment after failure of corticosteroids, three patients underwent ligation of esophageal varices but with recurrent digestive bleeding in all cases. Two patients benefited from a transjugular intrahepatic portosystemic shunt (TIPS), also with poor result. At the end of follow-up, five patients were alive and seven patients died. Two patients received a liver transplant, with good result and without recurrence of sarcoidosis on the transplant thereafter. Two patients had quiet sarcoidosis on low dose of corticosteroids and one patient was lost to follow-up. Symptomatic PH related to hepatic sarcoidosis is a severe complication, with high morbidity and mortality, and frequent failure of specific treatments of PH. Early management of these patients, with detection of hepatic comorbidities seems important. In case of therapeutic failure, liver transplantation is an option to consider.

Liver metastases - An unusual cause of portal hypertension: A case report

Portal hypertension is a rare complication of liver metastases. The study highlights that clinician should be aware of conditions mimicking cirrhosis with similar clinical presentation and imaging findings. We present the case of a 29-year-old non-alcoholic lady who presented to our hospital with a history of two months of progressive, painless abdominal distension and progressively increasing yellowish discoloration of the eyes. Physical examination, laboratory investigations, and imaging tests led to a diagnosis of multiple metastases from breast carcinoma to the liver leading to portal hypertension after exclusion of other causes of portal hypertension. However, after three weeks of presentation to the hospital, the patient died before any therapeutic measures were initiated to address breast carcinoma. Liver metastasis from primary breast carcinoma rarely presents with clinical symptoms of portal hypertension. Although portal hypertension secondary to pseudocirrhosis, predominantly linked to ongoing chemotherapy for known cancers, has been previously described in case studies, our case had an unusual presentation leading to diagnostic uncertainty. Our case highlights the rare cause of liver metastasis secondary to breast carcinoma, which presented as portal hypertension.