Complications Of Portal Hypertension Research Articles

Myeloproliferative neoplasms and splanchnic vein thrombosis: Contemporary diagnostic and therapeutic strategies.

Myeloproliferative neoplasms (MPNs) are the most common etiologies of primary splanchnic vein thrombosis, present in almost forty percent of patients with Budd-Chiari syndrome or portal vein thrombosis. Diagnosis of MPNs can be difficult in these patients because key characteristics, such as elevated blood cell counts and splenomegaly, are confounded by portal hypertension or bleeding complications. In recent years, diagnostic tools have improved to provide more accurate diagnosis and classification of MPNs. Although bone marrow biopsy findings remain a major diagnostic criterion, molecular markers are playing an increasing role not only in diagnosis but also in better estimating prognosis. Therefore, though screening for JAK2V617F mutation should be the starting point of the diagnostic workup performed in all patients with splanchnic vein thrombosis, a multidisciplinary approach is needed to accurately diagnose the subtype of myeloproliferative neoplasm, recommend the useful additional tests (bone marrow biopsy, search for an additional mutation using targeted next-generation sequencing), and suggest the best treatment strategy. Indeed, providing a specific expert care pathway for patients with splanchnic vein thrombosis and underlying myeloproliferative neoplasm is crucial to determine the optimal management to reduce the risk of both hematological and hepatic complications.

A100 CAVERNOUS TRANSFORMATION OF THE PORTAL VEIN IN A PEDIATRIC PATIENT WITH GOLDENHAR SYNDROME: A CASE REPORT

Abstract Background Goldenhar syndrome is a rare congenital condition thought to arise from the first and second branchial arches and typically asymmetrically affects the eyes, ears, and spine. Portal vein thrombosis/cavernous transformation is one of the main causes of portal hypertension in children. It is often associated with risk factors such as catheterization of the umbilical vein in the neonatal period, omphalitis, intra-abdominal infections in the neonatal period and prothrombotic conditions. This is a challenging clinical scenario as it leads to portal hypertension and variceal bleed with no chronic liver disease stigmata and liver function is essentially normal. While previous European literature from the early-2000s reported associated portal vein anomalies in Goldenhar syndrome, we are not aware of any recent and/or Canadian pediatric cases. Purpose We report a Canadian case of cavernous transformation of the portal vein with resulting gastrointestinal bleeding in a child with Goldenhar syndrome. Method Case report Result(s) A pediatric patient with a postnatal suspicion of Goldenhar syndrome, with confirmation by 6 months of age, presented with an acute 3-day history of melena, on the context of a recent viral illness and ibuprofen use. Laboratory testing showed a normocytic anemia (Hb 68) with a normal INR (1.1). The rest of the workup was unremarkable. There was no history of catheterization of the umbilical vein on the neonatal period nor other hematological complications. To assess for potential duplication cyst as differential diagnosis, a computerized tomography of the chest, abdomen and pelvis was ordered and revealed suspected cavernous transformation of the portal vein with suspected varices near the gastroesophageal junction and within the abdomen, and hepatomegaly with spleen size at the upper limit of normal. Octreotide was started after the CT results. A gastroscopy was done 24h after presentation and found portal hypertensive gastropathy and esophageal varices (2 Grade II and 1 Grade I). Sclerotherapy was performed. Post-gastroscopy ultrasound supported earlier radiographic findings of cavernous portal vein transformation and hepatosplenomegaly. Moreover, the ultrasound noted normal flow direction in the portal and hepatic veins. The patient has not re-presented for further episodes of gastrointestinal bleeding. Conclusion(s) This case report supports the previously reported association between Goldenhar syndrome and portal venous anomalies. Early consideration may lead to prompt diagnosis and management of the potentially life-threatening complications of portal hypertension in this population. Please acknowledge all funding agencies by checking the applicable boxes below None Disclosure of Interest None Declared

Fontan-associated liver disease: Diagnosis, surveillance, and management.

The Fontan operation is a lifesaving procedure for patients with functional single-ventricle congenital heart disease, where hypoplastic left heart syndrome is the most frequent anomaly. Hemodynamic changes following Fontan circulation creation are now increasingly recognized to cause multiorgan affection, where the development of a chronic liver disease, Fontan-associated liver disease (FALD), is one of the most important morbidities. Virtually, all patients with a Fontan circulation develop liver congestion, resulting in fibrosis and cirrhosis, and most patients experience childhood onset. FALD is a distinctive type of congestive hepatopathy, and its pathogenesis is thought to be a multifactorial process driven by increased nonpulsatile central venous pressure and decreased cardiac output, both of which are inherent in the Fontan circulation. In the advanced stage of liver injury, complications of portal hypertension often occur, and there is a risk of developing secondary liver cancer, reported at young age. However, FALD develops with few clinical symptoms, a surprisingly variable degree of severity in liver disease, and with little relation to poor cardiac function. The disease mechanisms and modifying factors of its development are still not fully understood. As one of the more important noncardiac complications of the Fontan circulation, FALD needs to be diagnosed in a timely manner with a structured monitoring scheme of disease development, early detection of malignancy, and determination of the optimal time point for transplantation. There is also a clear need for consensus on the best surveillance strategy for FALD. In this regard, imaging plays an important role together with clinical scoring systems, biochemical workups, and histology. Patients operated on with a Fontan circulation are generally followed up in cardiology units. Ultimately, the resulting multiorgan affection requires a multidisciplinary team of healthcare personnel to address the different organ complications. This article discusses the current concepts, diagnosis, and management of FALD, with special emphasis on the role of different imaging techniques in the diagnosis and monitoring of disease progression, as well as current recommendations for liver disease surveillance.

Utilizing ultra-small volume graft in auxiliary liver transplantation for portal hypertension

Objective: To examine the clinical effect of auxiliary liver transplantation with ultra-small volume graft in the treatment of portal hypertension. Methods: Twelve cases of portal hypertension treated by auxiliary liver transplantation with small volume graft at Liver Transplantation Center,Beijing Friendship Hospital, Capital Medical University between December 2014 and March 2022 were studied retrospectively. There were 8 males and 4 females,aged 14 to 66 years. Model for end-stage liver disease scores were 1 to 15 points and Child scores were 6 to 11 points. The grafts was derived from living donors in 9 cases,from split cadaveric donors in 2 cases,from whole cadaveric liver of child in 1 case. The graft recipient body weight ratios of 3 cadaveric donor livers were 0.79% to 0.90%, and of 9 living donor livers were 0.31% to 0.55%.In these cases, ultra-small volume grafts were implanted. The survivals of patient and graft, complications, portal vein blood flow of residual liver and graft, abdominal drainage and biochemical indexes of liver function were observed. Results: All the grafts and patients survived. Complications included outflow tract torsion in 2 cases, acute rejection in 1 case, bile leakage in 1 case, and thyroid cancer at the later stage of follow-up in 1 case, all of which were cured. The torsion of outflow tract was attributed to the change of anastomotic angle after the growth of donor liver. After the improvement of anastomotic method, the complication did not recur in the later stage. There was no complication of portal hypertension. The measurement of ultrasonic portal vein blood flow velocity showed that the blood flow of residual liver decreased significantly in the early stage after operation, and maintained a very low blood flow velocity or occlusion in the long term after operation, and the blood flow of transplanted liver was stable. Conclusions: Auxiliary liver transplantation can implant ultra-small donor liver through compensation of residual liver. This method may promote the development of living donor left lobe donation and split liver transplantation. However, the auxiliary liver transplantation is complex, and it is difficult to control the complications. Therefore, this method is currently limited to centers that are skilled in living related liver transplantation and that have complete ability to monitor and deal with complications.

Editorial: Appraising liver fibrosis with eagle eyes

Liver fibrosis is the pivotal component of essentially all chronic liver diseases.1 Tremendous efforts have been contributed by many basic and clinical researchers over the last few decades. By understanding the pathological mechanisms of liver fibrosis, exciting developments keep emerging from the basic, diagnostic, prognostic and therapeutic themes. In the current issue of the Journal of Gastroenterology and Hepatology, Zhao et al. lead us through the fascinating development of liver fibrosis research via the bibliometric and visualized analytic insights over the last two decades—nearly 9000 articles with over 45 000 authors from 97 countries had published in more than 1300 journals.2 This important piece of information highlighted the remarkable achievements in this field. This paper brings us an amazing fast-forward view of all these incredible and thrilling advances in understanding liver fibrosis. I found the publications about artificial intelligence (AI) and noninvasive assessments for liver fibrosis, the heated topics in recent years, particularly appealing.3 Noninvasive assessments are also positioned as accurate prognostic for the prediction of various important clinical outcomes, namely, portal hypertension and related complications, hepatocellular carcinoma (HCC), and deaths.4 The importance of the prognostic role of liver fibrosis is further supported by the fact that the most cited original research paper in this field is about liver fibrosis and long-term outcomes in patients with nonalcoholic fatty liver disease.5 AI facilitates the utilization of data from histopathology, clinical parameters, and/or imaging data to predict liver fibrosis with high accuracy. Laboratory-based AI models generally have high applicability as some common laboratory parameters are part of the routine liver panel. On the other hand, AI-assisted histologic scores enable investigators to improve the quality of clinical trials and facilitate comparison across studies. AI models may function as built-in models in the electronic clinical management systems or via some smartphone applications.6 Currently, many AI models are under rigorous evaluations for their ability to assess and monitor disease severity and predict future outcomes. By then, clinicians will have the necessary tools to improve patient care. Moving one step further, the recent fiery debate over ChatGPT-assisted diagnosis in medicine may bring us the future much closer,7 with a great deal of ethical issues to be reconciled.8 With the wide applicability of the important research findings in the field of liver fibrosis, the management of two billion patients with chronic liver diseases worldwide is amidst a revolutionary process.

Editorial introductions.

Current Opinion in Gastroenterology was launched in 1985. It is one of a successful series of review journals whose unique format is designed to provide a systematic and critical assessment of the literature as presented in the many primary journals. The field of gastroenterology is divided into 12 sections that are reviewed once a year. Each section is assigned a Section Editor, a leading authority in the area, who identifies the most important topics at that time. Here we are pleased to introduce the Section Editors for this issue. SECTION EDITORS Chantal HoussetChantal HoussetChantal Housset is Professor of Cell Biology at Sorbone Université, Paris, France, since 2000. She is Course Director of molecular and cellular biology at Sorbonne Université Medical School & Life Science Department. She is Hepatologist in the Reference Center for Inflammatory Biliary Diseases and Auto-immune Hepatitis, in Saint-Antoine Hospital, and of the Rare Liver Disease National Network (FILFOIE). She is the Head of an INSERM-Sorbonne Université Laboratory of translational research on metabolic and biliary diseases of the liver in Centre de Recherche Saint-Antoine (CRSA), Paris. Dr Housset is member of the University National Council (CNU) in Cell Biology. She published 193 articles. Her main scientific contributions relate to the pathophysiology of bile secretion and biliary diseases, the progression of metabolic and biliary diseases towards fibrosis, the origins and functions of myofibroblasts in the liver. Don C. RockeyDon C. RockeyDr Don Rockey completed internship, residency, chief residency, and fellowship at the University of California, San Francisco, USA. He is currently Director of the Medical University of South Carolina Digestive Disease Center. He has active, basic and clinical research interests in liver disease. His basic research focuses on understanding the molecular basis of fibrosis and portal hypertension. His laboratory initially demonstrated that a key player, the hepatic stellate cell, exhibits a contractile phenotype and transforms to a myofibroblast. Further, following liver injury, stellate cells not only produce increases quantities of extracellular matrix, but also exhibit exaggerated contractility, constrict liver sinusoids, and contribute to increased intrahepatic vascular resistance typical of portal hypertension. Dr Rockey also has an active clinical research program, focused on clinical management problems in gastrointestinal bleeding, drug induced liver injury, and chronic liver diseases including complications of portal hypertension. Dr Rockey and colleagues have published some 300 peer reviewed original scientific papers and over 100 editorials, reviews, and book chapters. He has served as a mentor for over 125 trainees, including students, residents, fellows, and junior faculty members - the vast majority of whom have pursued or who are pursuing careers in academic medicine. He has had a number of editorial responsibilities, including as Associate Editor for Hepatology, Liver International, and the Journal of Investigative Medicine, and has served on multiple NIH and other study sections, and editorial boards.

Abstract No. 213 Does Portosystemic Shunt Creation for Complication of Portal Hypertension after Liver Transplant Modify Outcomes?

Abstract No. 213 Does Portosystemic Shunt Creation for Complication of Portal Hypertension after Liver Transplant Modify Outcomes?

Portal Vein Interventions for Portal Hypertension: Complications and Management

AbstractPercutaneous management of complications of portal hypertension entails reducing portal pressures, controlling varices, and redirecting collateral flow using embolization, obliterative, restorative, recanalization, and shunt creation techniques. Management is based on the clinical status and symptoms and the physiologic and anatomic abnormalities causing portal hypertension. This article briefly describes percutaneous management strategies, their results, and related iatrogenic and physiologic complications.

Transjugular Intrahepatic Portosystemic Shunt Creation in Children Weighing

Transjugular intrahepatic portosystemic shunt (TIPS) creation is a standard therapeutic procedure for the management of complications of portal hypertension. However, in small children, this procedure is infrequently performed because of technical difficulties owing to their small size and scarce information on outcomes. This article presents the results of treatment of variceal bleeding with TIPS creation in 8 children weighing <10 kg, along with the description of the unconventional techniques and materials required. Technical and clinical success rates were 100%. Although this was a small cohort of patients, the results obtained suggest that this is a safe and technically and clinically effective treatment option for complications of portal hypertension in this age group.

The Role of Non-Selective Beta-Blockers (NSBBs) in Liver Cirrhosis

Non-selective beta-blockers (NSBBs) are one of the recommended treatments for portal hypertension in liver cirrhosis (LC). NSBB plays the role of primary as well as secondary prophylactic upper gastrointestinal bleeding in LC. NSBB therapy has been shown to effectively reduce the risk of variceal bleeding, reduction of portal pressure, and treat other complications of portal hypertension. The safety of NSBB therapy in cirrhosis requires a good therapeutic guide and considers the side effects. The indication of NSBB administration is adjusted according to the cirrhosis stage and the specific pathophysiology that occurs in cirrhosis. Conventional NSBBs such as propranolol and nadolol which are antagonists of β1 and β2 adrenergic receptors would induce decreased cardiac output and splanchnic vasoconstriction. Carvedilol is an NSBB with the addition of α1-adrenergic activity in reducing portal pressure stronger than conventional NSBB, so carvedilol can cause greater systemic arterial pressure reduction when compared to conventional NSBB. The appropriate treatment strategies can be applied in the use of NSBB to prevent more severe complications and reduce morbidity and mortality.

Natural history and prognosis of pediatric PSC with updates on management.

Natural history and prognosis of pediatric PSC with updates on management.

Smaller Diameter and Adjustable Diameter Transjugular Intrahepatic Portosystemic Shunts.

Complications of overshunting, including hepatic encephalopathy and hepatic insufficiency, remain prevalent following transjugular intrahepatic portosystemic shunt (TIPS) creation. Smaller diameter TIPS may reduce the risk of overshunting, but the use of smaller stents must be weighed against the risk of undershunting and persistent or recurrent hemorrhage, ascites, and other complications of portal hypertension. This article explores the question of optimal shunt diameter by examining outcomes for smaller diameter TIPS stent-grafts (<10 mm), underdilated stent-grafts, and variable diameter stent-grafts.

Gamma-glutamyl transferase and disease course in pediatric-onset primary sclerosing cholangitis: A single-center cohort study.

Patients with pediatric-onset primary sclerosing cholangitis (PSC) are at risk of developing hepatic complications with liver transplantation as only curative treatment. Complications usually occur over many years, underlining the need for reliable surrogate markers to predict the clinical course. Recently, gamma-glutamyl transferase (GGT) has been suggested to allow prediction of the clinical course. In a single-center cohort study, we tested the potency of GGT in this respect. We used longitudinal data of patients from our academic center, diagnosed with pediatric-onset PSC between 2000 and 2020. Patients with a GGT decrease from baseline >25% (n = 36) were compared with those who did not have this decrease (n = 7). We performed Kaplan-Meier analysis and log-rank testing to assess the occurrence of portal hypertensive or biliary complications, hepatobiliary malignancies, liver transplantation, or death. The median age diagnosis was 15.2 years and 12.1 years in the group with ≤25% decrease of GGT and the group with >25% decrease, respectively (p = 0.078). The probability of developing ≥1 complications in the first 5 years after diagnosis was 50% in the group with ≤25% decrease of GGT and 20% in the group with >25% decrease of GGT (p = 0.031). The use of medication was not associated with the development of complications. In a retrospective cohort study, we report that a GGT decrease of >25% within 1 year of diagnosis of pediatric-onset PSC is associated with a lower occurrence of complications within 5 years. Our results provide further support for the recently hypothesized predictive value of first-year GGT change in predicting the disease course in pediatric-onset PSC.

Transjugular Intrahepatic Portosystemic Shunt Reduction Techniques.

Transjugular intrahepatic portosystemic shunt (TIPS) creation treats complications of portal hypertension in appropriately selected patients by lowering the portal venous pressure. While this can be a lifesaving intervention, portal venous flow diversion is not without potential consequences. Overshunting can lead to hepatic decompensation and encephalopathy. TIPS reduction and TIPS occlusion are therapeutic options used to mitigate overshunting, with reduction being the initial alternative due to retained shunt patency and lower potential for venous thrombosis. Patient selection, techniques for TIPS reduction, and patient outcomes are reviewed in this article.

Transjugular Intrahepatic Portosystemic Shunt Reductions.

Transjugular intrahepatic portosystemic shunts (TIPS) are effective in reducing the portosystemic gradient and relieving complications of portal hypertension. Despite optimal patient selection, TIPS placement can be limited due to worsening hepatic encephalopathy and liver failure. In these cases, TIPS reduction may be necessary. A brief history of TIPS reduction and techniques for reduction are reviewed.

P210 Long-term outcome of very early onset inflammatory bowel disease associated with primary sclerosing cholangitis: a multicenter study from the Pediatric IBD Porto Group of ESPGHAN

Abstract Background Primary sclerosing cholangitis related to inflammatory bowel disease (PSC-IBD) diagnosed under the age of 6 years (i.e., VEO-IBD) may have unique characteristics and disease course. We aimed to analyze the characteristics and natural history of children with VEO PSC-IBD and compare them to children diagnosed with PSC-IBD at an older age. Methods This was a multicenter, retrospective, study evaluating patients diagnosed with both IBD and PSC before (VEO-PSC-IBD) or after the age of 6 years (PSC-IBD), followed at 14 centers affiliated with the Porto IBD Interest group of ESPGHAN. Demographic, clinical, laboratory, endoscopic, and imaging data were collected at baseline and every six months thereafter for a minimum follow-up of 12 months. IBD-related (clinical remission, need for systemic steroids, therapy escalation and surgery) and PSC-related outcomes (biliary and portal hypertensive complications, need for treatment escalation and liver transplantation, cholangiocarcinoma, and death) were analyzed at 1, 3, and 5 years. Results A total of 69 children with both IBD and PSC were included: 28 with VEO PSC-IBD [median age 5.2 (3.7-5.9) years, 21 UC (75%), 4 IBDU (14%) and 3 (15%) CD] and 41 with PSC-IBD [median age 15.7 (13.3-16.9) years, 34 UC (83%), 6 IBDU (1.5%) and 1 (0.2%) CD]. IBD was diagnosed prior to PSC in 28 of the 69 patients (40%), simultaneously in 30 (43%), and after PSC in 11 (16%), with no significant differences between VEO PSC-IBD and PSC-IBD. Most patients with UC presented with extensive disease at diagnosis (89% in VEO PSC-UC vs. 89% in PSC-UC, p = 0.72). Both groups presented most often with mild intestinal disease at diagnosis (mean PUCAI of VEO IBD-PSC 34±16, vs 31±19 of IBD-PSC, p=0.11; mean PCDAI 31±33 vs. 21±27, respectively, p=0.14). A higher number of VEO-IBD-PSC patients were diagnosed with autoimmune sclerosing cholangitis than older children [24 (86%) vs. 27 (66%) PSC-IBD, p=0.04], whereas no other differences were found for PSC-related variables. During follow-up, no significant differences were found in major IBD outcomes. The risk of developing biliary strictures and escalating therapy to vancomycin from ursodeoxycholic acid was lower in the VEO-PSC-IBD group (Figure 1 and 2, Log-rank p=0.02 and p=0.02), while no difference was found for portal hypertension and liver transplantation at 5-year follow-up. No cases of cholangiocarcinoma or death were reported. Conclusion IBD-PSC has similar baseline characteristics whether diagnosed as VEO-IBD or thereafter. However, a milder disease course in terms of biliary complications and the need for PSC-related therapy escalation, with an overall mild intestinal disease at presentation in all patients, characterize this specific subcohort of patients.

Expert consensus on emergency procedures for portal hypertension bleeding (2022)

Expert consensus proposes an emergency treatment protocol for portal hypertension bleeding. Herein, the emergency treatment procedures, which include first aid, medical, interventional, and surgical treatments, are described. In addition, the indications, contraindications, operating norms, precautions, and prevention of complications of portal hypertension are described to optimize the first aid process.

THE FACTOR ANALYSIS OF THE RESULTS OF MODERN TREATMENT OF PATIENTS WITH LIVER CIRRHOSIS WITH PORTAL HYPERTENSION

The purpose of this study is to study and evaluate the results of conventional methods of endovascular and endoscopic treatment of patients with complicated portal hypertension. Methods. In our study until 2021, 56 patients were included for analysis. In the course of the research, in 12 cases the endovascular method of percutaneous hepatic embolization of esophagus and gastric varicose vein in combination with splenic artery embolization, endoscopic sclerotherapy method in 23 cases, and endoscopic ligation according to the traditional method in 21 cases were used. The result of research work has showed that isolated use of endovascular methods (left gastric vein embolization, splenic artery embolization) in patients with liver cirrhosis is characterized by a low 2-year survival rate - 14.2%, with frequent anatomical and technical complications of this procedure - up to 64.7%, unstable primary hemostasis – 40.4%, esophagus and gastric varicose vein are explained by a high rate of recurrence of bleeding from varicose veins – 67.4%, risk of death – 21.8%. Conclusions. The clinical advantages of EL compared to different methods of ES have been achieved in terms of the rate of ineffective primary hemostasis, the risk of recurrence of bleeding from EGVV, short-term and long-term results, as well as improved 2-year survival rates. However, in general, their isolated use is associated with a relatively low chance of complete prevention of exacerbation of hemorrhagic syndrome relapses, which makes it possible to choose a combined approach to the treatment of patients with LC with PH and risk of bleeding from EGVV requires the development of tactical algorithms. In endovascular transhepatic embolization of esophageal and gastric varices, good results of treatment in the follow-up period of up to 2 years were very low, while unsatisfactory results of ES prevailed over good results, in turn, the best results were observed in the EL group. The results of endovascular transhepatic embolization in EGVV remain unknown in 1/3 of the studies.

RECURRENT STOMAL VARICEAL BLEEDING IN CIRRHOSIS: MANAGEMENT WITH TRANSJUGULAR INTRAHEPATIC PORTOSYSTEMIC SHUNT AND EMBOLIZATION

Gastrointestinal (GI) bleeding is a common complication of cirrhosis and portal hypertension. Most cases are secondary to gastro-esophageal variceal rupture, managed endoscopically1. Stomal bleeding is rare and there’s no standard therapy2. We present a case of a cirrhotic patient with recurrent stomal hemorrhage successfully treated with transjugular intrahepatic portosystemic shunt (TIPS) and stomal varices embolization.

RECURRENT STOMAL VARICEAL BLEEDING IN CIRRHOSIS: MANAGEMENT WITH TRANSJUGULAR INTRAHEPATIC PORTOSYSTEMIC SHUNT AND EMBOLIZATION

Gastrointestinal (GI) bleeding is a common complication of cirrhosis and portal hypertension. Most cases are secondary to gastro-esophageal variceal rupture, managed endoscopically1. Stomal bleeding is rare and there’s no standard therapy2. We present a case of a cirrhotic patient with recurrent stomal hemorrhage successfully treated with transjugular intrahepatic portosystemic shunt (TIPS) and stomal varices embolization.