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Martins PL, Silva MJ, Bilhim T, Ribeiro R. Budd-Chiari syndrome in a patient with antiphospholipid syndrome: hiding in plain sight. BMJ Case Rep 2025; 18:e259746. [PMID: 40262925 DOI: 10.1136/bcr-2024-259746] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 04/24/2025] Open
Abstract
We describe the case of a woman in her 40s on lifelong anticoagulation with warfarin due to antiphospholipid syndrome presenting with acute right upper quadrant pain. Liver tests were only slightly elevated, and non-invasive imaging such as Doppler ultrasound and contrast-enhanced CT were non-diagnostic. As suspicion for a thrombotic complication persisted, repeat CT imaging ultimately confirmed the diagnosis of Budd-Chiari syndrome (BCS), and angiography was performed allowing for effective treatment with balloon angioplasty. This case highlights both the need for a high degree of clinical suspicion for BCS in patients with prothrombotic features (even those on anticoagulation) and also the diagnostic and therapeutic potential of interventional radiology in selected cases.
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Affiliation(s)
- Pedro Lages Martins
- Gastroenterology Department, Centro Hospitalar Universitario de Lisboa Central EPE, Lisboa, Portugal
| | - Mário Jorge Silva
- Gastroenterology Department, Centro Hospitalar Universitario de Lisboa Central EPE, Lisboa, Portugal
- NOVA Medical School, Lisboa, Portugal
| | - Tiago Bilhim
- Interventional Radiology Department, Centro Hospitalar Universitario de Lisboa Central EPE, Lisboa, Portugal
| | - Rita Ribeiro
- Internal Medicine Department, Centro Hospitalar Universitario de Lisboa Central EPE, Lisboa, Portugal
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2
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Patel RK, Chandel K, Tripathy T, Behera S, Panigrahi MK, Nayak HK, Pattnaik B, Giri S, Dutta T, Gupta S. Interventions in Budd-Chiari syndrome: an updated review. Abdom Radiol (NY) 2025; 50:1307-1319. [PMID: 39325211 DOI: 10.1007/s00261-024-04558-4] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/03/2024] [Revised: 08/25/2024] [Accepted: 08/29/2024] [Indexed: 09/27/2024]
Abstract
Budd Chiari syndrome is a potentially treatable disease, and imaging is the key to its diagnosis. Clinical presentations may vary, ranging from asymptomatic to fulminant disease. Subacute BCS is the most common type encountered in clinical practice, characterized by ascites, hepatosplenomegaly, dilated abdominal wall veins, and varicosities in the lower limb and scrotum. While hepatic vein thrombosis is the leading cause in the West, membranous and short segmental occlusion are predominant in the Asian populations. These geographical variations have an impact on the treatment algorithm in managing BCS. Anticoagulation alone often fails to prevent disease progression, demanding further interventional therapy. Interventional therapy carries a lower morbidity and mortality than surgery. Anatomical recanalization and portosystemic shunting form the basis of endovascular management. Membranous or short-segment occlusion are best treated by angioplasty, which restores the physiological venous outflow and possibly disease reversal. Suboptimal results with angioplasty require stenting. Transjugular intrahepatic shunt (TIPS) or direct IVC to portal vein shunt (DIPS) decompresses the portal pressure and reduces the sinusoidal congestion, which in turn diminishes hepatocellular damage and hepatic fibrosis. Despite its ability to modify the disease course, TIPS carries several procedure and shunt-related complications, mainly hepatic encephalopathy. Thus, anatomical recanalization precedes TIPS in the traditional step-up approach in managing BCS. However, this concept is challenged by some authors, necessitating future reseach. TIPS is a valid bridge therapy in BCS with acute live failure awaiting liver transplantation. Despite all, interventional therapies fail in a subset of BCS patients, leaving them with only option of liver transplantation.
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Affiliation(s)
- Ranjan Kumar Patel
- Department of Radiodiagnosis, All India Institute of Medical Sciences, Bhubaneswar, 751019, India.
| | - Karamvir Chandel
- Department of Radiodiagnosis, All India Institute of Medical Sciences, Bilaspur, Himachal Pradesh, India
| | - Taraprasad Tripathy
- Department of Radiodiagnosis, All India Institute of Medical Sciences, Bhubaneswar, 751019, India.
| | - Srikant Behera
- Department of General Medicine, All India Institute of Medical Sciences, Bhubaneswar, 751019, India
| | - Manas Kumar Panigrahi
- Department of Gastroenterolgy, All India Institute of Medical Sciences, Bhubaneswar, 751019, India
| | - Hemanta Kumar Nayak
- Department of Gastroenterolgy, All India Institute of Medical Sciences, Bhubaneswar, 751019, India
| | - Bramhadatta Pattnaik
- Department of Surgical Gastroenterolgy, All India Institute of Medical Sciences, Bhubaneswar, 751019, India
| | - Suprabhat Giri
- Department of Gastroenterolgy, Kalinga Institite of Medical Sciences (KIMS), Bhubaneswar, India
| | - Tanmay Dutta
- Department of Surgical Gastroenterolgy, All India Institute of Medical Sciences, Bhubaneswar, 751019, India
| | - Sunita Gupta
- Department of Surgical Gastroenterolgy, All India Institute of Medical Sciences, Bhubaneswar, 751019, India
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Ibrahim MI, Egge AAA, Elmi OA, Abdi MH, Abdilahi MA, Ali AO. A rare pediatric case: Budd-Chiari Syndrome and upper gastrointestinal bleeding in a 5-year-old boy. Int J Surg Case Rep 2025; 127:110992. [PMID: 39889650 PMCID: PMC11834072 DOI: 10.1016/j.ijscr.2025.110992] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/12/2024] [Revised: 01/27/2025] [Accepted: 01/28/2025] [Indexed: 02/03/2025] Open
Abstract
INTRODUCTION AND IMPORTANCE Budd-Chiari Syndrome (BCS) is a rare condition characterized by hepatic venous outflow obstruction, often presenting with ascites, hepatomegaly, and abdominal pain. The diagnosis and management of BCS, especially in resource-limited settings, pose significant challenges that can lead to poor patient outcomes. CASE PRESENTATION This case report describes a previously healthy individual presenting with progressive abdominal distention, hematemesis, and right upper quadrant pain. The patient had a history of trauma and underwent subsequent percutaneous drainage for ascites. Imaging studies revealed non-opacified hepatic veins, splenomegaly, ascites, and a "nutmeg liver" pattern, confirming the diagnosis of BCS. The patient received blood transfusions, diuretics, and nutritional support. A Transjugular Intrahepatic Portosystemic Shunt (TIPS) procedure was planned but ultimately hindered by financial constraints. CLINICAL DISCUSSION This case highlights the challenges of managing BCS in resource-limited settings, particularly regarding access to specialized treatments like TIPS. It emphasizes the need for early diagnosis, multidisciplinary care, and the development of cost-effective treatment strategies to improve patient outcomes. CONCLUSION The management of Budd-Chiari Syndrome is complex, particularly in settings with limited resources. This case underscores the importance of timely intervention and the need for innovative approaches to healthcare delivery that can accommodate financial barriers while ensuring patient safety and care quality.
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Affiliation(s)
- Mohamed Ismail Ibrahim
- College of Health Sciences, School of Medicine and Surgery, Amoud University, Borama, Somalia; Mass CTH, Pediatric department, Somalia
| | - Ahmed Abdi Aw Egge
- College of Health Sciences, School of Medicine and Surgery, Amoud University, Borama, Somalia; School of Postgraduate Studies and Research, Amoud University, Amoud Valley, Borama 25263, Somalia
| | | | | | - Mohamed Ali Abdilahi
- College of Health Sciences, School of Medicine and Surgery, Amoud University, Borama, Somalia
| | - Abdirahman Omer Ali
- College of Health Sciences, School of Medicine and Surgery, Amoud University, Borama, Somalia; School of Postgraduate Studies and Research, Amoud University, Amoud Valley, Borama 25263, Somalia.
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Sharma D, Khera S, Saravagi G, Dhaman PK. Hepatocellular carcinoma as a complication of chronic Budd-Chiari syndrome (BCS) in a child. BMJ Case Rep 2024; 17:e261764. [PMID: 39216892 DOI: 10.1136/bcr-2024-261764] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 09/04/2024] Open
Abstract
Hepatocellular carcinoma (HCC) is an extremely rare long-term complication of Budd-Chiari syndrome (BCS) which may occur due to long-term venous congestion causing fibrosis, cirrhosis and subsequent hepatocellular dysplasia or anaplasia. This complication is even rarer in paediatric BCS and warrants early diagnosis for a favourable prognosis. Benign regenerative nodules seen with BCS are difficult to differentiate from malignant nodular lesion of HCC, thereby making serial imaging less sensitive for early diagnosis of HCC in BCS. Surveillance guidelines like adults do not exist in monitoring chronic paediatric BCS due to rarity of this complication. Six monthly serum alpha-fetoprotein monitoring in addition to radiological surveillance improves the sensitivity of early detection of HCC transformation in BCS and should be the way ahead in paediatric BCS as well. We describe a paediatric patient who presented with advanced HCC after 25-month follow-up for BCS.
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Affiliation(s)
- Divyanshi Sharma
- Department of Pediatrics, Army Hospital Research and Referral, New Delhi, Delhi, India
| | - Sanjeev Khera
- Department of Pediatrics, Army Hospital Research and Referral, New Delhi, Delhi, India
| | - Ganesh Saravagi
- Department of Radiodiagnosis, Army Hospital Research and Referral, New Delhi, Delhi, India
| | - Pawan Kumar Dhaman
- Department of Pathology, Army Hospital Research and Referral, New Delhi, Delhi, India
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Saad Eddin A, Ramzan U, Saad Aldin E. Budd-Chiari syndrome treated with direct intrahepatic portocaval shunt: A case report. Radiol Case Rep 2024; 19:2608-2612. [PMID: 38645960 PMCID: PMC11026531 DOI: 10.1016/j.radcr.2024.03.036] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/14/2023] [Revised: 03/09/2024] [Accepted: 03/13/2024] [Indexed: 04/23/2024] Open
Abstract
Budd-Chiari syndrome is an uncommon disorder characterized by occlusion of hepatic veins. It can lead to portal hypertension. Most common causes of this syndrome are hypercoagulability states. Transjugular intrahepatic portosystemic shunt is often not possible given the portal venous thrombosis. In these cases, direct intrahepatic portocaval shunt, involving the creation of an access between the portal vein and the systemic circulation via the intrahepatic inferior vena cava has proven to be a feasible alternative, and can improve portal hypertension in these patients. Herein, we present a case of a 37-year-old woman diagnosed with Budd-Chiari syndrome that was successfully treated with percutaneous ultrasound (US)-guided direct intrahepatic portocaval shunt.
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Affiliation(s)
- Assim Saad Eddin
- University of Iowa Hospitals and Clinics, Department of Radiology, 200 Hawkins Dr, Iowa City, IA 52242, USA
| | - Umar Ramzan
- Northwestern University Feinberg School of Medicine, 420 E Superior St, Chicago, IL 60611, USA
| | - Ehab Saad Aldin
- University of Nebraska Medical Center, Department of Radiology, 42nd and Emile St, Omaha, NE 68198, USA
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Moreno MOA, Paz CLDSL, Dezan MGF, Cavalcante LN, Lyra AC. TEN-YEAR OUTCOMES OF TIPS FOR BUDD-CHIARI SYNDROME: SYSTEMATIC REVIEW AND META-ANALYSIS. ARQUIVOS DE GASTROENTEROLOGIA 2024; 61:e23171. [PMID: 38896573 DOI: 10.1590/s0004-2803.24612023-171] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Received: 12/12/2023] [Accepted: 02/23/2024] [Indexed: 06/21/2024]
Abstract
BACKGROUND Budd-Chiari syndrome (BCS) results from the obstruction of the hepatic venous flow, usually at the level of the hepatic vein or inferior vena cava. When left untreated, it can progress with several complications, including liver cirrhosis. Transjugular intrahepatic portosystemic shunt (TIPS) appears to be effective in a subgroup of BCS patients. OBJECTIVE To perform a systematic review and meta-analysis of TIPS effectiveness in BCS treatment, considering the survival rate, reduction in portosystemic pressure, need for liver transplantation, technical failure, and shunt dysfunction for up to 10 years of follow-up. METHODS We evaluated 17 studies published in PubMed, Science Direct, Web of Science, and SCOPUS databases, which used TIPS as a treatment for BCS, comprising 618 subjects between 18 and 78 years old. We assessed the bias risk by the NOS, NHI, and JBI scales for cohort stu-dies, before-after studies, and case series, respectively. We conducted the meta-analyses by extracting the number of events and the total patients evaluated to perform the proportion meta-analyses using the R software ("meta" package - version 4.9-6). RESULTS The pooled results (95%CI) showed a 19% (25.9-12.5%) rate of portosystemic pressure reduction, 6% (1-12%) rate for the need for liver transplants despite the use of TIPS, 2% (1-6%) technical failure rate, 30% (18-46%) shunt dysfunction rate, and 88% (81-93%) for the mean frequency of patients alive between 1 and 10 years after the procedure. We stratified survival rate and found an 86% (74-93%) prevalence of living subjects during less than five years, 92% (83-97%) at five years, and a 77% frequency (68-83%) of patients alive ten years after the TIPS placement. CONCLUSION TIPS is an effective treatment for BCS, providing a high 10-year frequency of living patients and a significant decrease in portosystemic pressure. The need for liver transplants after TIPS and the technical failure rate is low.
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Affiliation(s)
| | | | - Maria Gabriela Fernandes Dezan
- Escola Bahiana de Medicina e Saúde Pública; Salvador, BA, Brasil
- Hospital São Rafael, Serviço de Gastro-Hepatologia, Salvador, BA, Brasil
- Universidade Federal da Bahia, PPgMs, Salvador, BA, Brasil
| | - Lourianne Nascimento Cavalcante
- Hospital São Rafael, Serviço de Gastro-Hepatologia, Salvador, BA, Brasil
- Universidade Federal da Bahia, PPgMs, Salvador, BA, Brasil
- Hospital Universitário Professor Edgard Santos, Serviço de Gastro-Hepatologia, Salvador, BA, Brasil
| | - Andre Castro Lyra
- Hospital São Rafael, Serviço de Gastro-Hepatologia, Salvador, BA, Brasil
- Universidade Federal da Bahia, PPgMs, Salvador, BA, Brasil
- Hospital Universitário Professor Edgard Santos, Serviço de Gastro-Hepatologia, Salvador, BA, Brasil
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7
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Riescher-Tuczkiewicz A, Elkrief L, Rautou PE. [Splanchnic vein thrombosis]. Rev Med Interne 2024; 45:17-25. [PMID: 37838484 DOI: 10.1016/j.revmed.2023.07.005] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/15/2023] [Accepted: 07/25/2023] [Indexed: 10/16/2023]
Abstract
Splanchnic vein thrombosis includes Budd-Chiari syndrome and portal vein thrombosis. These diseases share common features: (i) they are rare diseases and (ii) they can lead to portal hypertension and its complications. Budd-Chiari syndrome and portal vein thrombosis in the absence of underlying liver disease share many risk factors, the most common being myeloproliferative neoplasms. A rapid and comprehensive workup for thrombosis risk factors is necessary in these patients. Long-term anticoagulation is indicated in most patients. Portal vein thrombosis can also develop in patients with cirrhosis, and is associated with a worse course of cirrhosis. Indications for anticoagulation in patients with cirrhosis are increasing. Transjugular intrahepatic portosystemic shunt is a second-line procedure in this setting. Because of the rarity of these diseases, high-level evidence studies are rare. However, collaborative studies have provided a better understanding of their natural history and allowed to improve the management of these patients. This review focuses on the causes, diagnosis, and management of patients with Budd-Chiari syndrome, patients with portal vein thrombosis without underlying liver disease, and patients with cirrhosis and portal vein thrombosis.
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Affiliation(s)
- A Riescher-Tuczkiewicz
- Université Paris-Cité, Inserm, centre de recherche sur l'inflammation, UMR 1149, Paris, France.
| | - L Elkrief
- Université de Tours, service d'hépato-gastro-entérologie, CHRU de Tours, Tours, France
| | - P-E Rautou
- Université Paris-Cité, Inserm, centre de recherche sur l'inflammation, UMR 1149, Paris, France; Service d'hépatologie, AP-HP, hôpital Beaujon, DMU DIGEST, centre de référence des maladies vasculaires du foie, FILFOIE, ERN RARE-LIVER, Clichy, France
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8
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Valla DC. Hepatic vein thrombosis and PVT: A personal view on the contemporary development of ideas. Clin Liver Dis (Hoboken) 2024; 23:e0246. [PMID: 38988821 PMCID: PMC11236412 DOI: 10.1097/cld.0000000000000246] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/11/2024] [Accepted: 05/10/2024] [Indexed: 07/12/2024] Open
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Rössle M. Interventional Treatment of Budd-Chiari Syndrome. Diagnostics (Basel) 2023; 13:diagnostics13081458. [PMID: 37189559 DOI: 10.3390/diagnostics13081458] [Citation(s) in RCA: 2] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/06/2023] [Revised: 04/13/2023] [Accepted: 04/15/2023] [Indexed: 05/17/2023] Open
Abstract
Medical treatment is regarded as the primary course of action in patients with Budd-Chiari syndrome (BCS). Its efficacy, however, is limited, and most patients require interventional treatment during follow-up. Short-segment stenosis or the occlusion (the so-called web) of hepatic veins or the inferior vena cava are frequent in Asian countries. An angioplasty with or without stent implantation is the treatment of choice to restore hepatic and splanchnic blood flow. The long-segment thrombotic occlusion of hepatic veins, common in Western countries, is more severe and may require a portocaval shunting procedure to relieve hepatic and splanchnic congestion. Since it was first proposed in a publication in 1993, the transjugular intrahepatic portosystemic shunt (TIPS) has gained more and more attention, and in fact it has been so successful that previously utilized surgical shunts are only used for few patients for whom it does not work. Both interventional treatment options can be performed successfully in about 95% of patients even after the complete obliteration of the hepatic veins. The long-term patency of the TIPS, a considerable problem in its early years, has been improved with PTFE-covered stents. The complication rates of these interventions are low and the survival rate is excellent with five- and ten-year survival rates of 90% and 80%, respectively. Present treatment guidelines recommend a step-up approach indicating interventional treatment after the failure of medical treatment. However, this widely accepted algorithm has several points of contention, and early interventional treatment is proposed instead.
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Affiliation(s)
- Martin Rössle
- Department of Gastroenterology, University Hospital, 79106 Freiburg, Germany
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10
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Elkrief L, Payancé A, Plessier A, d’Alteroche L, Ronot M, Paradis V, Valla D, Rautou PE. Management of splanchnic vein thrombosis. JHEP Rep 2023; 5:100667. [PMID: 36941824 PMCID: PMC10023986 DOI: 10.1016/j.jhepr.2022.100667] [Citation(s) in RCA: 21] [Impact Index Per Article: 10.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/15/2022] [Revised: 12/11/2022] [Accepted: 12/23/2022] [Indexed: 01/04/2023] Open
Abstract
The expression splanchnic vein thrombosis encompasses Budd-Chiari syndrome and portal vein thrombosis. These disorders have common characteristics: they are both rare diseases which can cause portal hypertension and its complications. Budd-Chiari syndrome and portal vein thrombosis in the absence of underlying liver disease share many risk factors, among which myeloproliferative neoplasms represent the most common; a rapid comprehensive work-up for risk factors of thrombosis is needed in these patients. Long-term anticoagulation is indicated in most patients. Portal vein thrombosis can also develop in patients with cirrhosis and in those with porto-sinusoidal vascular liver disease. The presence and nature of underlying liver disease impacts the management of portal vein thrombosis. Indications for anticoagulation in patients with cirrhosis are growing, while transjugular intrahepatic portosystemic shunt is now a second-line option. Due to the rarity of these diseases, studies yielding high-grade evidence are scarce. However, collaborative studies have provided new insight into the management of these patients. This article focuses on the causes, diagnosis, and management of patients with Budd-Chiari syndrome, portal vein thrombosis without underlying liver disease, or cirrhosis with non-malignant portal vein thrombosis.
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Key Words
- BCS, Budd-Chiari syndrome
- CALR, calreticulin
- Cavernoma
- DOACs, direct-acting oral anticoagulants
- Direct oral anticoagulants
- EHPVO, extrahepatic portal vein obstruction
- GFR, glomerular filtration rate
- JAK2, Janus kinase 2
- LMWH, low-molecular-weight heparin
- MPN, myeloproliferative neoplasm
- MTHFR, methylene-tetrahydrofolate reductase
- PNH, paroxysmal nocturnal hemoglobinuria
- PVT, portal vein thrombosis
- Portal biliopathy
- Portal vein recanalisation
- SVT, splanchnic vein thrombosis
- TIPS, transjugular intrahepatic portosystemic shunt
- VKAs, vitamin K antagonists
- Vascular liver diseases
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Affiliation(s)
- Laure Elkrief
- Service d’Hépato-Gastroentérologie CHU de Tours, France
| | - Audrey Payancé
- Université Paris-Cité, Inserm, Centre de recherche sur l'inflammation, UMR 1149, Paris, France
- Service d'Hépatologie, AP-HP, Hôpital Beaujon, DMU DIGEST, Centre de Référence des Maladies Vasculaires du Foie, FILFOIE, ERN RARE-LIVER, Clichy, France
| | - Aurélie Plessier
- Université Paris-Cité, Inserm, Centre de recherche sur l'inflammation, UMR 1149, Paris, France
- Service d'Hépatologie, AP-HP, Hôpital Beaujon, DMU DIGEST, Centre de Référence des Maladies Vasculaires du Foie, FILFOIE, ERN RARE-LIVER, Clichy, France
| | | | - Maxime Ronot
- Université Paris-Cité, Inserm, Centre de recherche sur l'inflammation, UMR 1149, Paris, France
- Service de radiologie, Hôpital Beaujon APHP.Nord, Clichy, France
| | - Valérie Paradis
- Université Paris-Cité, Inserm, Centre de recherche sur l'inflammation, UMR 1149, Paris, France
- Service d’anatomie et cytologie pathologique, Hôpital Beaujon APHP.Nord, Clichy, France
| | - Dominique Valla
- Université Paris-Cité, Inserm, Centre de recherche sur l'inflammation, UMR 1149, Paris, France
- Service d'Hépatologie, AP-HP, Hôpital Beaujon, DMU DIGEST, Centre de Référence des Maladies Vasculaires du Foie, FILFOIE, ERN RARE-LIVER, Clichy, France
| | - Pierre-Emmanuel Rautou
- Université Paris-Cité, Inserm, Centre de recherche sur l'inflammation, UMR 1149, Paris, France
- Service d'Hépatologie, AP-HP, Hôpital Beaujon, DMU DIGEST, Centre de Référence des Maladies Vasculaires du Foie, FILFOIE, ERN RARE-LIVER, Clichy, France
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Semmler G, Lindorfer A, Schäfer B, Bartl S, Hametner-Schreil S, Gensluckner S, Balcar L, Pomej K, Lampichler K, Trauner M, Aigner E, Datz C, Zoller H, Hofer H, Schöfl R, Mandorfer M, Reiberger T, Scheiner B. Outcome of Budd-Chiari Syndrome Patients Treated With Direct Oral Anticoagulants: An Austrian Multicenter Study. Clin Gastroenterol Hepatol 2023; 21:978-987.e2. [PMID: 35533994 DOI: 10.1016/j.cgh.2022.04.024] [Citation(s) in RCA: 8] [Impact Index Per Article: 4.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/08/2021] [Revised: 04/14/2022] [Accepted: 04/15/2022] [Indexed: 02/07/2023]
Abstract
BACKGROUND AND AIMS Direct oral anticoagulants (DOACs) may simplify management of Budd-Chiari syndrome (BCS). Here, we report our experience with off-label use of DOACs for anticoagulation in BCS. METHODS The safety of DOAC vs vitamin K antagonist treatment as well as associated clinical outcomes were retrospectively assessed in 47 BCS patients treated at 6 Austrian centers. RESULTS Mean age at study inclusion was 37.9 ± 14.0 years and mean Model for End-Stage Liver Disease was 13.1 ± 5.1. Overall, 63.8% (n = 30) of patients had decompensated liver disease, and 87.2% (n = 41) showed clinical signs of portal hypertension. During a median follow-up of 82.5 (interquartile range, 43.1-121.8) months, 43 (91.5%) patients received anticoagulation alone or following interventional treatment, including 22 (46.8%) patients treated with DOACs (edoxaban: 10, apixaban: 4, rivaroxaban: 3, dabigatran: 3, more than one DOAC sequentially: 2) for a median of 24.4 (interquartile range, 5.7-35.1) months. While 72.7% (n = 16 of 22) of patients were switched from low-molecular-weight heparin (n = 12) or vitamin K antagonist (n = 4) to DOAC after disease stabilization or improvement, 27.3% (n = 6 of 22) of BCS patients were initially treated with DOAC. Complete response (European Association for the Study of the Liver criteria) was achieved or maintained in 14 (63.6%) of 22 patients, with ongoing response in 2 patients, while disease progressed in 6 patients (including 2 patients with hepatocellular carcinoma). Four major spontaneous bleedings (18.2%; incidence rate 8.8 per 100 patient-years; n = 2 upper gastrointestinal bleeding, n = 1 lower gastrointestinal bleeding, n = 1 hepatocellular carcinoma rupture), 7 minor bleedings, and 1 major procedure-related bleeding (4.5%; 2.2 per 100 patient-years) occurred during DOAC therapy. Overall transplant-free survival was 91.6% at 5 years. CONCLUSIONS DOACs seem to be effective and safe for long-term anticoagulation in patients with BCS, but confirmation by larger prospective studies is needed.
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Affiliation(s)
- Georg Semmler
- Division of Gastroenterology and Hepatology, Department of Internal Medicine III, Medical University of Vienna, Vienna, Austria; Vienna Hepatic Hemodynamic Lab, Division of Gastroenterology and Hepatology, Department of Internal Medicine III, Medical University of Vienna, Vienna, Austria
| | - Alexander Lindorfer
- Department of Internal Medicine IV, Ordensklinikum Linz Barmherzige Schwestern, Linz, Austria
| | - Benedikt Schäfer
- Department of Medicine I, Medical University of Innsbruck, Innsbruck, Austria
| | - Stefan Bartl
- Department of Internal Medicine I, Klinikum Wels-Grieskirchen, Wels, Austria
| | | | - Sophie Gensluckner
- Department of Medicine I, Paracelsus Medical University Salzburg, Salzburg, Austria
| | - Lorenz Balcar
- Division of Gastroenterology and Hepatology, Department of Internal Medicine III, Medical University of Vienna, Vienna, Austria; Vienna Hepatic Hemodynamic Lab, Division of Gastroenterology and Hepatology, Department of Internal Medicine III, Medical University of Vienna, Vienna, Austria
| | - Katharina Pomej
- Division of Gastroenterology and Hepatology, Department of Internal Medicine III, Medical University of Vienna, Vienna, Austria; Vienna Hepatic Hemodynamic Lab, Division of Gastroenterology and Hepatology, Department of Internal Medicine III, Medical University of Vienna, Vienna, Austria
| | - Katharina Lampichler
- Department of Biomedical Imaging and Image-Guided Therapy, Medical University of Vienna, Vienna, Austria
| | - Michael Trauner
- Division of Gastroenterology and Hepatology, Department of Internal Medicine III, Medical University of Vienna, Vienna, Austria
| | - Elmar Aigner
- Department of Medicine I, Paracelsus Medical University Salzburg, Salzburg, Austria
| | - Christian Datz
- Department of Internal Medicine, General Hospital Oberndorf, Teaching Hospital of the Paracelsus Medical University Salzburg, Salzburg, Austria
| | - Heinz Zoller
- Department of Medicine I, Medical University of Innsbruck, Innsbruck, Austria
| | - Harald Hofer
- Department of Internal Medicine I, Klinikum Wels-Grieskirchen, Wels, Austria
| | - Rainer Schöfl
- Department of Internal Medicine IV, Ordensklinikum Linz Barmherzige Schwestern, Linz, Austria
| | - Mattias Mandorfer
- Division of Gastroenterology and Hepatology, Department of Internal Medicine III, Medical University of Vienna, Vienna, Austria; Vienna Hepatic Hemodynamic Lab, Division of Gastroenterology and Hepatology, Department of Internal Medicine III, Medical University of Vienna, Vienna, Austria
| | - Thomas Reiberger
- Division of Gastroenterology and Hepatology, Department of Internal Medicine III, Medical University of Vienna, Vienna, Austria; Vienna Hepatic Hemodynamic Lab, Division of Gastroenterology and Hepatology, Department of Internal Medicine III, Medical University of Vienna, Vienna, Austria; Christian Doppler Laboratory for Portal Hypertension and Liver Fibrosis, Medical University of Vienna, Vienna, Austria.
| | - Bernhard Scheiner
- Division of Gastroenterology and Hepatology, Department of Internal Medicine III, Medical University of Vienna, Vienna, Austria; Vienna Hepatic Hemodynamic Lab, Division of Gastroenterology and Hepatology, Department of Internal Medicine III, Medical University of Vienna, Vienna, Austria
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12
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Găman MA, Cozma MA, Manan MR, Srichawla BS, Dhali A, Ali S, Nahian A, Elton AC, Simhachalam Kutikuppala LV, Suteja RC, Diebel S, Găman AM, Diaconu CC. Budd-Chiari syndrome in myeloproliferative neoplasms: A review of literature. World J Clin Oncol 2023; 14:99-116. [PMID: 37009527 PMCID: PMC10052333 DOI: 10.5306/wjco.v14.i3.99] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/20/2022] [Revised: 02/10/2023] [Accepted: 03/01/2023] [Indexed: 03/19/2023] Open
Abstract
Myeloproliferative neoplasms (MPNs) are defined as clonal disorders of the hematopoietic stem cell in which an exaggerated production of terminally differentiated myeloid cells occurs. Classical, Philadelphia-negative MPNs, i.e., polycythemia vera, essential thrombocythemia and primary myelofibrosis, exhibit a propensity towards the development of thrombotic complications that can occur in unusual sites, e.g., portal, splanchnic or hepatic veins, the placenta or cerebral sinuses. The pathogenesis of thrombotic events in MPNs is complex and requires an intricate mechanism involving endothelial injury, stasis, elevated leukocyte adhesion, integrins, neutrophil extracellular traps, somatic mutations (e.g., the V617F point mutation in the JAK2 gene), microparticles, circulating endothelial cells, and other factors, to name a few. Herein, we review the available data on Budd-Chiari syndrome in Philadelphia-negative MPNs, with a particular focus on its epidemiology, pathogenesis, histopathology, risk factors, classification, clinical presentation, diagnosis, and management.
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Affiliation(s)
- Mihnea-Alexandru Găman
- Faculty of Medicine, “Carol Davila” University of Medicine and Pharmacy, Bucharest 050474, Romania
- Department of Hematology, Center of Hematology and Bone Marrow Transplantation, Fundeni Clinical Institute, Bucharest 022328, Romania
| | - Matei-Alexandru Cozma
- Faculty of Medicine, “Carol Davila” University of Medicine and Pharmacy, Bucharest 050474, Romania
| | | | - Bahadar S Srichawla
- Department of Neurology, University of Massachusetts Chan Medical School, Worcester, Massachusetts 01655, United States
| | - Arkadeep Dhali
- Department of Internal Medicine, Nottingham University Hospitals NHS Trust, Nottingham NG7 2UH, United Kingdom
| | - Sajjad Ali
- Department of Medicine, Ziauddin University, Karachi 75600, Pakistan
| | - Ahmed Nahian
- California Baptist University-Lake Erie College of Osteopathic Medicine, Riverside, CA 92504, United States
| | - Andrew C Elton
- University of Minnesota Medical School, Minneapolis, MN 55455, United States
| | - L V Simhachalam Kutikuppala
- Department of General Surgery, Dr NTR University of Health Sciences, Andhra Pradesh, Vijayawada 520008, India
| | - Richard Christian Suteja
- Department of Undergraduate Medicine, Faculty of Medicine, Udayana University Denpasar, Bali 80232, Indonesia
| | - Sebastian Diebel
- Department of Family Medicine, Northern Ontario School of Medicine Timmins, Ontario 91762, Canada
| | - Amelia Maria Găman
- Department of Pathophysiology, University of Medicine and Pharmacy of Craiova, Romania & Clinic of Hematology, Filantropia City Hospital, Craiova 200143, Romania
| | - Camelia Cristina Diaconu
- Department of Internal Medicine, Faculty of Medicine, “Carol Davila” University of Medicine and Pharmacy, Romania & Internal Medicine Clinic, Clinical Emergency Hospital of Bucharest, Bucharest 105402, Romania
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13
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Mukhiya G, Jiao D, Han X, Zhou X, Pokhrel G. Survival and clinical success of endovascular intervention in patients with Budd-Chiari syndrome: A systematic review. J Clin Imaging Sci 2023; 13:5. [PMID: 36751561 PMCID: PMC9899460 DOI: 10.25259/jcis_130_2022] [Citation(s) in RCA: 4] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/03/2022] [Accepted: 01/14/2023] [Indexed: 01/26/2023] Open
Abstract
Budd-Chiari syndrome is a complex clinical disorder of hepatic venous outflow obstruction, originating from the accessory hepatic vein (HV), large HV, and suprahepatic inferior vena cava (IVC). This disorder includes both HV and IVC obstructions and hepatopathy. This study aimed to conduct a systematic review of the survival rate and clinical success of different types of endovascular treatments for Budd-Chiari syndrome (BCS). All participant studies were retrieved from four databases and selected according to the eligibility criteria for systematic review of patients with BCS. The survival rate, clinical success of endovascular treatments in BCS, and survival rates at 1 and 5 years of publication year were calculated accordingly. A total of 3398 patients underwent an endovascular operation; among them, 93.6% showed clinical improvement after initial endovascular treatment. The median clinical success rates for recanalization, transjugular intrahepatic portosystemic shunt (TIPS), and combined procedures were 51%, 17.50%, and 52.50%, respectively. The median survival rates at 1 and 5 years were 51% and 51% for recanalization, 17.50% and 16% for TIPS, and 52.50% and 49.50% for combined treatment, respectively. Based on the year of publication, the median survival rates at 1 and 5 years were 23.50% and 22.50% before 2000, 41% and 41% in 2000‒2005, 35% and 35% in 2006‒2010, 51% and 48.50% in 2010‒2015, and 56% and 55.50% after 2015, respectively. Our findings indicate that the median survival rate at 1 and 5 years of recanalization treatment is higher than that of TIPS treatment, and recanalization provides better clinical improvement. The publication year findings strongly suggest progressive improvements in interventional endovascular therapy for BCS. Thus, interventional therapy restoring the physiologic hepatic venous outflow of the liver can be considered as the treatment of choice for patients with BCS which is a physiological modification procedure.
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Affiliation(s)
- Gauri Mukhiya
- Department of Interventional Radiology, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, China
| | - Dechao Jiao
- Department of Interventional Radiology, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, China
| | - Xinwei Han
- Department of Interventional Radiology, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, China.,Corresponding author: Xinwei Han, Department of Interventional Radiology, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, China.
| | - Xueliang Zhou
- Department of Interventional Radiology, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, China
| | - Gaurab Pokhrel
- Department of Interventional Radiology, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, China
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14
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Cheng Z, Ju J, Zhu Q, Deng M, Zhang H. Intrahepatic hematoma secondary to transjugular intrahepatic portosystemic stent-shunt procedure: Case report and literature review. Medicine (Baltimore) 2022; 101:e31753. [PMID: 36397433 PMCID: PMC9666193 DOI: 10.1097/md.0000000000031753] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/19/2022] Open
Abstract
RATIONALE Transjugular intrahepatic portosystemic stent-shunt (TIPSS) is a minimally invasive procedure used for the treatment of portal hypertension. Intrahepatic hematoma is the rare complication caused by hepatic arterial injuries from TIPSS procedure. PATIENT CONCERNS This case report illustrated a 77-year-old man with hepatitis B virus-induced cirrhosis who underwent TIPSS. DIAGNOSES The patient suffered from intrahepatic hematoma and hepatic pseudoaneurysm because of the hepatic artery injury after TIPSS. INTERVENTIONS The hepatic artery laceration began at the level of the branch of the left hepatic artery was embolized. OUTCOMES The acute intrahepatic hematoma and hepatic pseudoaneurysm of the patient were cured. CONCLUSION In this report, we describe a cirrhosis patient with a large intrahepatic hematoma secondary to TIPSS, and a literature review is also presented. The intrahepatic hematoma and hepatic pseudoaneurysm should be paid more attention after TIPSS while early-stage prevention should be carried out.
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Affiliation(s)
- Ziyao Cheng
- Department of Gastroenterology, The Affiliated Hospital of Southwest Medical University, Luzhou, Sichuan Province, China
| | - Jiayu Ju
- Department of Gastroenterology, The Affiliated Hospital of Southwest Medical University, Luzhou, Sichuan Province, China
| | - Qingliang Zhu
- Department of Gastroenterology, The Affiliated Hospital of Southwest Medical University, Luzhou, Sichuan Province, China
| | - Mingming Deng
- Department of Gastroenterology, The Affiliated Hospital of Southwest Medical University, Luzhou, Sichuan Province, China
| | - Hailong Zhang
- Department of Gastroenterology, The Affiliated Hospital of Southwest Medical University, Luzhou, Sichuan Province, China
- * Correspondence: Hailong Zhang, The Affiliated Hospital of Southwest Medical University, No. 25, Taiping Street, Luzhou, Sichuan Province 646000, China (e-mail: )
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15
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Mancuso A. Budd-Chiari Syndrome Management: Controversies and Open Issues. Diagnostics (Basel) 2022; 12:2670. [PMID: 36359513 PMCID: PMC9689902 DOI: 10.3390/diagnostics12112670] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/05/2022] [Revised: 10/26/2022] [Accepted: 11/01/2022] [Indexed: 07/03/2024] Open
Abstract
Budd-Chiari Syndrome (BCS) is due to thrombosis of hepatic veins (HVs), inferior vena cava (IVC) or both, leading to impaired hepatic venous outflow [...].
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Affiliation(s)
- Andrea Mancuso
- Centro di Riferimento Regionale Malattie Rare, Sindrome di Budd-Chiari e Teleangectasia Emorragica Ereditaria, Medicina Interna 1, ARNAS Civico-Di Cristina-Benfratelli, Piazzale Leotta 4, 90100 Palermo, Italy
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16
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Elhence A, Shalimar. Treatment Options for Hepatic Venous Outflow Tract Obstruction: Is the Scale Tipping in Favor of TIPS? J Clin Exp Hepatol 2022; 12:1403-1405. [PMID: 36340299 PMCID: PMC9630011 DOI: 10.1016/j.jceh.2022.09.005] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/23/2022] [Accepted: 09/26/2022] [Indexed: 12/12/2022] Open
Affiliation(s)
- Anshuman Elhence
- Department of Gastroenterology, All India Institute of Medical Sciences, Raipur, Chhattisgarh, India
| | - Shalimar
- Department of Gastroenterology and Human Nutrition, All India Institute of Medical Sciences, Delhi, India
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17
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Giri S, Kale A, Shukla A. Efficacy and Safety of Transjugular Intrahepatic Portosystemic Shunt Creation for Budd-Chiari Syndrome: A Systematic Review and Meta-Analysis. J Vasc Interv Radiol 2022; 33:1301-1312.e13. [PMID: 35940361 DOI: 10.1016/j.jvir.2022.07.022] [Citation(s) in RCA: 15] [Impact Index Per Article: 5.0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/24/2022] [Revised: 07/04/2022] [Accepted: 07/22/2022] [Indexed: 12/15/2022] Open
Abstract
PURPOSE To assess the critical role of transjugular intrahepatic portosystemic shunt (TIPS) in the management of Budd-Chiari syndrome (BCS), as the data with respect to the safety and outcome of TIPS in patients with BCS are scarce because of the rarity of the disease. MATERIALS AND METHODS A comprehensive search of literature of various databases from 2000 to October 2021 was conducted for studies evaluating the outcome of TIPS in patients with BCS. The primary outcomes of the analysis were technical and clinical success, adverse events and mortality associated with TIPS, dysfunction of TIPS, need for TIPS revision, need for liver transplantation (LT), and 1-year survival. RESULTS A total of 33 studies (1,395 patients) were included in this meta-analysis. The pooled rates and 95% confidence intervals of various outcomes were 98.6% (97.6-99.7) for technical success, 90.3% (86.0-94.6) for clinical success, 10.0% (6.5-13.6) for major adverse events, 0.5% (0.2-1.0) for TIPS-related mortality, 11.6% (7.8-15.4) for post-TIPS hepatic encephalopathy (HE), 40.1% (32.5-47.7) for TIPS dysfunction, 8.6% (4.9-12.4) for the need for TIPS revision, 4.5% (2.8-6.2) for the need for LT, and 94.6% (93.1-96.1) for 1-year survival. Publication bias was seen with all outcomes except for post-TIPS HE, TIPS dysfunction, and the need for LT. CONCLUSIONS The existing literature supports the feasibility, safety, and efficacy of TIPS in the treatment of BCS. Deciding the optimal timing of TIPS in BCS needs further studies.
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Affiliation(s)
- Suprabhat Giri
- Department of Gastroenterology, Nizam's Institute of Medical Sciences, Hyderabad, Telangana, India.
| | - Aditya Kale
- Department of Gastroenterology, Seth GS Medical College and KEM Hospital, Mumbai, Maharashtra, India
| | - Akash Shukla
- Department of Gastroenterology, Seth GS Medical College and KEM Hospital, Mumbai, Maharashtra, India
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18
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Evaluation of outcome from endovascular therapy for Budd-Chiari syndrome: a systematic review and meta-analysis. Sci Rep 2022; 12:16166. [PMID: 36171454 PMCID: PMC9519873 DOI: 10.1038/s41598-022-20399-x] [Citation(s) in RCA: 4] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/17/2021] [Accepted: 09/13/2022] [Indexed: 11/19/2022] Open
Abstract
This study was performed to evaluate the outcome of endovascular intervention therapy for Budd-Chiari syndrome (BCS) and compare recanalization, transjugular intrahepatic portosystemic shunt (TIPS)/direct intrahepatic portosystemic shunt (DIPS), and combined procedure treatment. For the meta-analysis, 71 studies were identified by searching four databases. The individual studies’ samples were used to calculate a confidence interval (CI 95%), and data were pooled using a fixed-effect model and random effect model. The pooled measure and an equal-weighted average rate were calculated in all participant studies. Heterogeneity between the studies was assessed with I2, and T2 tests, and publication bias was estimated using Egger’s regression test. A total of 4,407 BCS patients had undergone an endovascular intervention procedure. The pooled results were 98.9% (95% CI 97.8‒98.9%) for a technical success operation, and 96.9% (95% CI 94.9‒98.9%) for a clinical success operation. The re-intervention rate after the initial intervention procedure was 18.9% (95% CI 14.7‒22.9%), and the survival rates at 1 and 5 years after the initial intervention procedure were 98.9% (95% CI 96.8‒98.9%) and 94.9% (95% CI 92.9‒96.9%), respectively. Patients receiving recanalization treatment (98%) had a better prognosis than those with a combined procedure (95.6%) and TIPS/DIPS treatment (94.5%). The systematic review and meta-analysis further solidify the role of endovascular intervention treatment in BCS as safe and effective. It maintains high technical and clinical success and long-term survival rates. The recanalization treatment had a better prognosis and outcome than the combined procedures and TIPS/DIPS treatment.
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19
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Elkilany A, Alwarraky M, Denecke T, Geisel D. Percutaneous transluminal angioplasty for symptomatic hepatic vein-type Budd-Chiari syndrome: feasibility and long-term outcomes. Sci Rep 2022; 12:14095. [PMID: 35982064 PMCID: PMC9388522 DOI: 10.1038/s41598-022-16818-8] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/26/2022] [Accepted: 07/15/2022] [Indexed: 11/09/2022] Open
Abstract
For management of Budd-Chiari syndrome (BCS), a step-wise therapeutic approach starting with medical treatment, followed by endovascular recanalization, transjugular intrahepatic portosystemic shunt, and finally liver transplantation has been adopted. We retrospectively analyzed 51 patients with symptomatic short segment (≤ 30 mm) hepatic vein (HV)-type BCS who underwent percutaneous transluminal balloon angioplasty (PTBA) with/without stenting to determine the feasibility, clinical effectiveness, and long-term outcomes. The intervention was technically successful in 94.1% of cases (48/51)—32 patients underwent PTBA and 16 patients underwent HV stenting. Procedure-related complications occurred in 14 patients (29.1%). The clinical success rate at 4 weeks was 91.7% (44/48). Nine patients underwent reintervention, six patients due to restenosis/occlusion and three patients with clinical failure. The mean primary patency duration was 64.6 ± 19.9 months (CI, 58.5–70.8; range, 1.2–81.7 months). The cumulative 1-, 2-, and 5-year primary patency rates were 85.4, 74.5, and 58.3%, respectively. The cumulative 1-, 2-, and 5-year secondary patency rates were 93.8, 87.2, and 75%, respectively. The cumulative 1-, 2-, and 5-year survival rates were 97.9, 91.5, and 50%, respectively. Percutaneous transluminal angioplasty with and without stenting is effective and achieves excellent long-term patency and survival rates in patients with symptomatic HV-type BCS. With its lower incidence of re-occlusion and higher clinical success rate, HV angioplasty combined with stenting should be the preferred option especially in patients with segmental HV-type BCS.
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Affiliation(s)
- Aboelyazid Elkilany
- Department of Diagnostic and Interventional Radiology, Charité-Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin, Humboldt-Universität Zu Berlin, Berlin Institute of Health, Augustenburger Platz 1, 13353, Berlin, Germany. .,Department of Diagnostic Medical Imaging and Interventional Radiology, National Liver Institute, Menoufia University, Menoufia, Egypt.
| | - Mohamed Alwarraky
- Department of Diagnostic Medical Imaging and Interventional Radiology, National Liver Institute, Menoufia University, Menoufia, Egypt
| | - Timm Denecke
- Department of Diagnostic and Interventional Radiology, Leipzig University Hospital, Leipzig, Germany
| | - Dominik Geisel
- Department of Diagnostic and Interventional Radiology, Charité-Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin, Humboldt-Universität Zu Berlin, Berlin Institute of Health, Augustenburger Platz 1, 13353, Berlin, Germany
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20
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Jindal A, Sarin SK. Epidemiology of liver failure in Asia-Pacific region. Liver Int 2022; 42:2093-2109. [PMID: 35635298 DOI: 10.1111/liv.15328] [Citation(s) in RCA: 12] [Impact Index Per Article: 4.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/05/2022] [Revised: 05/26/2022] [Accepted: 05/27/2022] [Indexed: 02/13/2023]
Abstract
The global burden of deaths caused by liver failure is substantial. The Asia-Pacific region is home to more than half of the global population and accounted for 62.6% of global deaths because of liver diseases in 2015. The aetiology of liver failure varies in different countries at different times. Viruses (Hepatitis A, B and E), drugs (herbs and anti-tuberculous drugs), toxins (alcohol use) and autoimmune flares are mainly responsible of majority of liver failure in individuals with normal liver (acute liver failure; ALF); else these may precipitate liver failure in those with chronic liver disease (acute-on-chronic liver failure; ACLF). Concomitant increases in alcohol misuse and metabolic syndrome in recent years are concerning. Ongoing efforts to address liver failure-related morbidity and mortality require accurate contemporary estimates of epidemiology and outcomes. In light of the ever-changing nature of liver disease epidemiology, accurate estimates for the burden of liver failure across the countries are vital for setting clinical, research and policy priorities. In this review, we aimed to describe the current as well as changing epidemiological trends of common liver failure syndromes, ALF and ACLF in the Asia-Pacific region.
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Affiliation(s)
- Ankur Jindal
- Department of Hepatology, Institute of Liver and Biliary Sciences, New Delhi, India
| | - Shiv K Sarin
- Department of Hepatology, Institute of Liver and Biliary Sciences, New Delhi, India
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21
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Boike JR, Thornburg BG, Asrani SK, Fallon MB, Fortune BE, Izzy MJ, Verna EC, Abraldes JG, Allegretti AS, Bajaj JS, Biggins SW, Darcy MD, Farr MA, Farsad K, Garcia-Tsao G, Hall SA, Jadlowiec CC, Krowka MJ, Laberge J, Lee EW, Mulligan DC, Nadim MK, Northup PG, Salem R, Shatzel JJ, Shaw CJ, Simonetto DA, Susman J, Kolli KP, VanWagner LB. North American Practice-Based Recommendations for Transjugular Intrahepatic Portosystemic Shunts in Portal Hypertension. Clin Gastroenterol Hepatol 2022; 20:1636-1662.e36. [PMID: 34274511 PMCID: PMC8760361 DOI: 10.1016/j.cgh.2021.07.018] [Citation(s) in RCA: 134] [Impact Index Per Article: 44.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/30/2021] [Revised: 07/01/2021] [Accepted: 07/13/2021] [Indexed: 02/07/2023]
Abstract
Complications of portal hypertension, including ascites, gastrointestinal bleeding, hepatic hydrothorax, and hepatic encephalopathy, are associated with significant morbidity and mortality. Despite few high-quality randomized controlled trials to guide therapeutic decisions, transjugular intrahepatic portosystemic shunt (TIPS) creation has emerged as a crucial therapeutic option to treat complications of portal hypertension. In North America, the decision to perform TIPS involves gastroenterologists, hepatologists, and interventional radiologists, but TIPS creation is performed by interventional radiologists. This is in contrast to other parts of the world where TIPS creation is performed primarily by hepatologists. Thus, the successful use of TIPS in North America is dependent on a multidisciplinary approach and technical expertise, so as to optimize outcomes. Recently, new procedural techniques, TIPS stent technology, and indications for TIPS have emerged. As a result, practices and outcomes vary greatly across institutions and significant knowledge gaps exist. In this consensus statement, the Advancing Liver Therapeutic Approaches group critically reviews the application of TIPS in the management of portal hypertension. Advancing Liver Therapeutic Approaches convened a multidisciplinary group of North American experts from hepatology, interventional radiology, transplant surgery, nephrology, cardiology, pulmonology, and hematology to critically review existing literature and develop practice-based recommendations for the use of TIPS in patients with any cause of portal hypertension in terms of candidate selection, procedural best practices and, post-TIPS management; and to develop areas of consensus for TIPS indications and the prevention of complications. Finally, future research directions are identified related to TIPS for the management of portal hypertension.
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Affiliation(s)
- Justin R. Boike
- Department of Medicine, Division of Gastroenterology & Hepatology, Northwestern University Feinberg School of Medicine, Chicago, IL, USA
| | - Bartley G. Thornburg
- Department of Radiology, Division of Vascular and Interventional Radiology, Northwestern University Feinberg School of Medicine, Chicago, IL, USA
| | | | - Michael B. Fallon
- Department of Medicine, Division of Gastroenterology and Hepatology, Banner - University Medical Center Phoenix, Phoenix, AZ, USA
| | - Brett E. Fortune
- Department of Medicine, Division of Gastroenterology and Hepatology, Weill Cornell Medical College, New York, NY, USA
| | - Manhal J. Izzy
- Department of Medicine, Division of Gastroenterology, Hepatology, and Nutrition, Vanderbilt University Medical Center, Nashville, TN, USA
| | - Elizabeth C. Verna
- Department of Medicine, Division of Digestive and Liver Diseases, Columbia University College of Physicians & Surgeons, New York, NY, USA
| | - Juan G. Abraldes
- Division of Gastroenterology (Liver Unit), University of Alberta, Edmonton, AB, Canada
| | - Andrew S. Allegretti
- Department of Medicine, Division of Nephrology, Massachusetts General Hospital, Boston, MA, USA
| | - Jasmohan S. Bajaj
- Department of Internal Medicine, Division of Gastroenterology, Hepatology, and Nutrition, Virginia Commonwealth University and Central Virginia Veterans Healthcare System, Richmond, VA, USA
| | - Scott W. Biggins
- Department of Medicine, Division of Gastroenterology & Hepatology, University of Washington Medical Center, Seattle, WA, USA
| | - Michael D. Darcy
- Department of Radiology, Division of Interventional Radiology, Washington University School of Medicine, St. Louis, MO, USA
| | - Maryjane A. Farr
- Department of Medicine, Division of Cardiology, Columbia University College of Physicians & Surgeons, New York, NY, USA
| | - Khashayar Farsad
- Dotter Department of Interventional Radiology, Oregon Health and Science University, Portland, OR, USA
| | - Guadalupe Garcia-Tsao
- Department of Digestive Diseases, Yale University, Yale University School of Medicine, and VA-CT Healthcare System, CT, USA
| | - Shelley A. Hall
- Department of Internal Medicine, Division of Cardiology, Baylor University Medical Center, Dallas, TX, USA
| | - Caroline C. Jadlowiec
- Department of Surgery, Division of Transplant Surgery, Mayo Clinic, Phoenix, AZ, USA
| | - Michael J. Krowka
- Department of Pulmonary and Critical Care Medicine, Division of Gastroenterology and Hepatology, Mayo Clinic, Rochester, MN, USA
| | - Jeanne Laberge
- Department of Radiology and Biomedical Imaging, Division of Interventional Radiology, University of California San Francisco, San Francisco, CA, USA
| | - Edward W. Lee
- Department of Radiology, Division of Interventional Radiology, University of California-Los Angeles David Geffen School of Medicine, Los Angeles, CA, USA
| | - David C. Mulligan
- Department of Surgery, Division of Transplantation, Yale University School of Medicine, New Haven, CT, USA
| | - Mitra K. Nadim
- Department of Medicine, Division of Nephrology and Hypertension, University of Southern California, Los Angeles, California, USA
| | - Patrick G. Northup
- Department of Medicine, Division of Gastroenterology and Hepatology, University of Virginia, Charlottesville, VA, USA
| | - Riad Salem
- Department of Radiology, Division of Vascular and Interventional Radiology, Northwestern University Feinberg School of Medicine, Chicago, IL, USA
| | - Joseph J. Shatzel
- Division of Hematology and Medical Oncology, Oregon Health and Science University, Portland, OR, USA
| | - Cathryn J. Shaw
- Department of Radiology, Division of Interventional Radiology, Baylor University Medical Center, Dallas, TX, USA
| | - Douglas A. Simonetto
- Department of Physiology, Division of Gastroenterology and Hepatology, Mayo Clinic, Rochester, MN, USA
| | - Jonathan Susman
- Department of Radiology, Division of Interventional Radiology, Columbia University Irving Medical Center, New York, NY, USA
| | - K. Pallav Kolli
- Department of Radiology and Biomedical Imaging, Division of Interventional Radiology, University of California San Francisco, San Francisco, CA, USA
| | - Lisa B. VanWagner
- Department of Medicine, Division of Gastroenterology & Hepatology, Northwestern University Feinberg School of Medicine, Chicago, IL, USA,Department of Preventive Medicine, Northwestern University Feinberg School of Medicine, Chicago, IL, USA,Address for correspondence: Lisa B. VanWagner MD MSc FAST FAHA, Assistant Professor of Medicine and Preventive Medicine, Divisions of Gastroenterology & Hepatology and Epidemiology, Northwestern University Feinberg School of Medicine, 676 N. St Clair St - Suite 1400, Chicago, Illinois 60611 USA, Phone: 312 695 1632, Fax: 312 695 0036,
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22
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Kageyama K, Yamamoto A, Jogo A, Sohgawa E, Hagihara A, Fujii H, Uchida-Kobayashi S, Kawada N, Miki Y. The Albumin-bilirubin Score Detects Changes in the Liver Function during Treatment for Budd-Chiari Syndrome: A Retrospective Observational Study. Intern Med 2022; 61:959-967. [PMID: 34544949 PMCID: PMC9038474 DOI: 10.2169/internalmedicine.8020-21] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/30/2022] Open
Abstract
Objective Mapping the long-term prognosis of Budd-Chiari syndrome (BCS) is difficult, as the prognosis is associated with changes in the liver function. The present study evaluated the time course changes in the liver function in a treatment group with percutaneous old balloon angioplasty (POBA) and a non-treatment group using the albumin-bilirubin score (ALBI) and Child-Pugh score during long-term follow-up. Methods In this retrospective study, 13 consecutive patients diagnosed with BCS at our hospital between 2007 and 2020 were categorized into a treatment group (n=8), which received POBA, and a non-treatment group (n=5). Differences in the liver function in the ALBI and Child-Pugh scores between the initial visit and one- and three-year follow-up were calculated and statistically evaluated. We investigated the changes in the liver function during the long-term follow-up, including events such as re-stenosis and re-treatment. Results While the Child-Pugh scores in the treatment group did not differ significantly between the initial visit and 1- or 3-year follow-up, the ALBI scores in this group improved significantly between the initial visit and the 1- or 3-year follow-up visit (p=0.0078 and 0.0156, respectively). The liver function according to the ALBI score in the treatment group showed gradual improvement from the initial value but gradual worsening in the non-treatment group. The ALBI scores also revealed that the liver function varies according to re-stenosis and re-POBA in BCS patients. Conclusion Unlike the Child-Pugh score, the ALBI score was able to capture changes in the liver function of BCS patients during the long-term course of BCS.
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Affiliation(s)
- Ken Kageyama
- Department of Diagnostic and Interventional Radiology, Graduate School of Medicine, Osaka City University, Japan
| | - Akira Yamamoto
- Department of Diagnostic and Interventional Radiology, Graduate School of Medicine, Osaka City University, Japan
| | - Atsushi Jogo
- Department of Diagnostic and Interventional Radiology, Graduate School of Medicine, Osaka City University, Japan
| | - Etsuji Sohgawa
- Department of Diagnostic and Interventional Radiology, Graduate School of Medicine, Osaka City University, Japan
| | - Atsushi Hagihara
- Department of Hepatology, Graduate School of Medicine, Osaka City University, Japan
| | - Hideki Fujii
- Department of Hepatology, Graduate School of Medicine, Osaka City University, Japan
| | | | - Norifumi Kawada
- Department of Hepatology, Graduate School of Medicine, Osaka City University, Japan
| | - Yukio Miki
- Department of Diagnostic and Interventional Radiology, Graduate School of Medicine, Osaka City University, Japan
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Comparison of long-term outcomes of balloon angioplasty with or without stent placement for hepatic vein type Budd-Chiari syndrome. Eur J Gastroenterol Hepatol 2021; 33:e843-e850. [PMID: 34402468 DOI: 10.1097/meg.0000000000002277] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/10/2022]
Abstract
PURPOSE To compare percutaneous transluminal balloon angioplasty (PTBA) alone with PTBA plus stent placement for Budd-Chiari syndrome (BCS) patients with short-segmental obstruction of hepatic vein. METHODS Between May 2001 and December 2010, 91 hepatic vein type BCS patients (mean age, 32.8 ± 10.8; M:F ratio 50:41) were included and divided into two groups (PTBA alone, as group A, n = 48; PTBA plus stent, as group B, n = 43). The incidence of restenosis, long-term patency, and survival were evaluated. RESULTS All 91 patients were successfully treated with hepatic vein recanalization. During follow-up period (median 161 months, range: 84-234), group B had a significantly higher proportion of hepatic vein restenosis (56%, 24/43) than group A (33%, 16/48; P < 0.05). Cumulative primary patency rates at 1, 5, 10, 15 years were 96%, 81%, 69%, 65% and 91%, 60%, 47%, 47% in group A and group B, respectively (log-rank P < 0.05). Secondary technical success rates of target hepatic vein were 93% and 57% in group A and group B, respectively (P < 0.05). Clinical complete response rates were 94% and 86% in group A and group B, respectively (P > 0.05). The 15-year cumulative survival rates were 98% and 95% in group A and group B, respectively (P > 0.05). Major procedure-related complications occurred in 3 (3%) patients (1 in group A, 2 in group B). CONCLUSIONS Hepatic vein recanalization is safe and efficient for hepatic vein type BCS patients with hepatic vein short segmental obstruction. Restenosis after hepatic vein stenting is more common and difficult to manage than that after hepatic vein balloon angioplasty alone.
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Shukla A, Shreshtha A, Mukund A, Bihari C, Eapen CE, Han G, Deshmukh H, Cua IHY, Lesmana CRA, Al Meshtab M, Kage M, Chaiteeraki R, Treeprasertsuk S, Giri S, Punamiya S, Paradis V, Qi X, Sugawara Y, Abbas Z, Sarin SK. Budd-Chiari syndrome: consensus guidance of the Asian Pacific Association for the study of the liver (APASL). Hepatol Int 2021; 15:531-567. [PMID: 34240318 DOI: 10.1007/s12072-021-10189-4] [Citation(s) in RCA: 46] [Impact Index Per Article: 11.5] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/28/2020] [Accepted: 04/11/2021] [Indexed: 02/07/2023]
Abstract
Budd Chiari syndrome (BCS) is a diverse disease with regard to the site of obstruction, the predisposing thrombophilic disorders and clinical presentation across the Asia-Pacific region. The hepatic vein ostial stenosis and short segment thrombosis are common in some parts of Asia-Pacific region, while membranous obstruction of the vena cava is common in some and complete thrombosis of hepatic veins in others. Prevalence of myeloproliferative neoplasms and other thrombophilic disorders in BCS varies from region to region and with different sites of obstruction. This heterogeneity also raises several issues and dilemmas in evaluation and approach to management of a patient with BCS. The opportunity to recanalize hepatic vein in patients with hepatic vein ostial stenosis or inferior vena cava stenting or pasty among those membranous obstruction of the vena cava is a unique opportunity in the Asia-Pacific region to restore hepatic outflow closely mimicking physiology. In order to address these issues arising out of the diversity as well as the unique features in the region, the Asia Pacific Association for Study of Liver has formulated these guidelines for clinicians.
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Affiliation(s)
- Akash Shukla
- Department of Gastroenterology, King Edward Memorial Hospital and Seth Gordhandas Sunderdas Medical College, Mumbai, India.
| | | | - Amar Mukund
- Institute of Liver and Biliary Sciences, New Delhi, India
| | - Chhagan Bihari
- Institute of Liver and Biliary Sciences, New Delhi, India
| | - C E Eapen
- Christian Medical College, Vellore, India
| | - Guohong Han
- Xijing Hospital of Digestive Diseases, Fourth Military Medical University, Xian, China
| | - Hemant Deshmukh
- Dean and Head of Radiology, King Edward Memorial Hospital and Seth Gordhandas Sunderdas Medical College, Mumbai, India
| | - Ian Homer Y Cua
- Institute of Digestive and Liver Diseases, St Lukes Medical Center, Global City, Philippines
| | - Cosmas Rinaldi Adithya Lesmana
- Dr. Cipto Mangunkusumo National General Hospital, Universitas Indonesia, Jakarta, Indonesia
- Digestive Disease & GI Oncology Center, Medistra Hospital, Jakarta, Indonesia
| | - Mamun Al Meshtab
- Bangabandhu Sheikh Mujib Medical University, Dhaka, Bangladesh
- Center for Innovative Cancer Therapy, Kurume University Research, 67 Asahi-machi, Kurume, 830-0011, Japan
| | - Masayoshi Kage
- Department of Gastroenterology, King Edward Memorial Hospital and Seth Gordhandas Sunderdas Medical College, Mumbai, India
| | - Roongruedee Chaiteeraki
- Division of Gastroenterology, Department of Medicine, Faculty of Medicine, Chulalongkorn University, Bangkok, Thailand
| | - Sombat Treeprasertsuk
- Division of Gastroenterology, Department of Medicine, Faculty of Medicine, Chulalongkorn University, Bangkok, Thailand
| | - Suprabhat Giri
- Department of Gastroenterology, King Edward Memorial Hospital and Seth Gordhandas Sunderdas Medical College, Mumbai, India
| | - Sundeep Punamiya
- Vascular and Interventional Radiology, Tan Tock Seng Hospital, Singapore, Singapore
| | - Valerie Paradis
- Dpt dAnatomie Pathologique, Hôpital Beaujon, 100 bd du Gal Leclerc, Clichy, 92110, France
| | - Xingshun Qi
- General Hospital of Northern Theater Command (formerly General Hospital of Shenyang Military Area), No. 83 Wenhua Road, Shenyang, China
| | - Yasuhiko Sugawara
- Department of Transplantation and Pediatric Surgery, Kumamoto University, Kumamoto, Japan
| | - Zaigham Abbas
- Department of Hepatogastroenterology, Dr. Ziauddin University Hospital Clifton, Karachi, Pakistan
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Sharma A, Keshava SN, Eapen A, Elias E, Eapen CE. An Update on the Management of Budd-Chiari Syndrome. Dig Dis Sci 2021; 66:1780-1790. [PMID: 32691382 DOI: 10.1007/s10620-020-06485-y] [Citation(s) in RCA: 25] [Impact Index Per Article: 6.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/05/2020] [Accepted: 07/04/2020] [Indexed: 12/17/2022]
Abstract
Budd-Chiari syndrome (BCS) is an uncommon condition, caused by obstruction to hepatic venous outflow. It is largely underdiagnosed, and a high index of suspicion is required for any patient with unexplained portal hypertension. The understanding of its etiology and pathology is improving with advances in diagnostic techniques. Recent studies reported an identifiable etiology in > 80% of cases. Myeloproliferative neoplasm (MPN) is the most common etiology, and genetic studies help in diagnosing latent MPN. Better cross-sectional imaging helps delineate the site of obstruction accurately. The majority of BCS patients are now treated by endovascular intervention and anticoagulation which have improved survival in this disease. Angioplasty of hepatic veins/inferior vena cava remains under-utilized at present. While surgical porto-systemic shunts are no longer done for BCS, liver transplantation is reserved for select indications. Some of the unresolved issues in the current management of BCS are also discussed in this review.
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Affiliation(s)
- A Sharma
- Hepatology Department, Christian Medical College, Vellore, Tamil Nadu, India
| | - S N Keshava
- Department of Interventional Radiology, Christian Medical College, Vellore, India
| | - A Eapen
- Department of Radiodiagnosis, Christian Medical College, Vellore, India
| | - E Elias
- Hepatology Department, Christian Medical College, Vellore, Tamil Nadu, India.,Liver Unit, University Hospitals Birmingham, Birmingham, UK
| | - C E Eapen
- Hepatology Department, Christian Medical College, Vellore, Tamil Nadu, India.
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26
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Patidar Y, Pal CK, Mukund A, Kumar G, Sarin SK. Retrospective comparative study of efficacy, safety and outcome of percutaneous intervention for Budd-Chiari syndrome patients with bilirubin less than 3 and 3-6 mg/dl. Br J Radiol 2021; 94:20201157. [PMID: 33595338 DOI: 10.1259/bjr.20201157] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/28/2022] Open
Abstract
OBJECTIVE Comparing the efficacy, safety and outcome of percutaneous intrervention for Budd-Chiari Syndrome (BCS) patients with bilirubin less than 3 and 3-6 mg dl-1. METHODS AND MATERIALS 188 BCS patients having serum bilirubin ≤6 mg dl-1 and underwent percutaneous interventions were divided into two groups based on bilirubin level: 151 patients having bilirubin <3 mg dl-1 were included in Group 1; and 37 patients having bilirubin 3-6 mg dl-1 were included in Group 2. Both group were compare for technical success (successful recanalization of hepatic venous stenosis or creation of portocaval shunt with post-procedure gradient ≤5 mm of Hg), Safety (procedure-related mortality/morbidity or patient required transplantation) and outcome (resolution of clinical symptoms and survival). RESULTS Technical success was 94.7% in Group 1-89.1% in Group 2 with overall success rate was 93.6%. No significant differences observed between the two groups in regards to procedure related complication. Overall transplant-free survival at 1 and 5 years after intervention in both groups was 96.3 and 91.2% respectively. 1-year and 5-year survivals in Group 1 was 96.7%, and 93.1%, whereas Group 2 was 94.6 and 90.1% with no statically significantly difference between the two groups (p = 0.59). Percutaneous intervention results are good in patients having bilirubin up to 6 mg dl-1, i.e. mild to moderate liver dysfunctions. CONCLUSION Technical success, survival and outcome of percutaneous intervention in BCS patients having serum bilirubin 3-6 mg dl-1 was comparable to patients having bilirubin level <3 mg dl-1. ADVANCES IN KNOWLEDGE Percutaneous intervention treatment is suitable for treatment for symptomatic BCS patients having bilirubin up to 6 mg dl-1.
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Affiliation(s)
- Yashwant Patidar
- Department of Interventional Radiology, Institute of Liver & Biliary Sciences, New Delhi, India
| | - Chandan Kumar Pal
- Department of Interventional Radiology, Institute of Liver & Biliary Sciences, New Delhi, India
| | - Amar Mukund
- Department of Interventional Radiology, Institute of Liver & Biliary Sciences, New Delhi, India
| | - Guresh Kumar
- Department of Clinical Research, Institute of Liver and Biliary Sciences, New Delhi, India
| | - Shiv Kumar Sarin
- Department of Hepatology, Institute of Liver and Biliary Sciences, New Delhi, India
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27
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Mukund A, Biradar B. IR Management of Budd–Chiari Syndrome. BASICS OF HEPATOBILIARY INTERVENTIONS 2021:107-118. [DOI: 10.1007/978-981-15-6856-5_8] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 01/04/2025]
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Long-Term Outcomes of Endovascular Interventions in More than 500 patients with Budd-Chiari Syndrome. J Vasc Interv Radiol 2020; 32:61-69.e1. [PMID: 33218919 DOI: 10.1016/j.jvir.2020.08.035] [Citation(s) in RCA: 9] [Impact Index Per Article: 1.8] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/06/2020] [Revised: 08/29/2020] [Accepted: 08/31/2020] [Indexed: 01/10/2023] Open
Abstract
PURPOSE To assess and compare the long-term outcomes of various endovascular interventions in patients with Budd-Chiari syndrome (BCS). MATERIALS AND METHODS In this single-center retrospective study, 510 consecutive patients with BCS who had undergone a total of 618 endovascular procedures from January 2001 to December 2019 were included. Details of the type of endovascular intervention, technical success, clinical success, patency rate, complications, and survival outcomes were analyzed. RESULTS The overall technical success rate was 96% (593 of 618 procedures; 500 in treatment-naïve patients and 93 repeat interventions for recurrent disease). Endovascular procedures included recanalization procedures (angioplasty and stent placement) in 355 patients (71%) and transjugular intrahepatic portosystemic shunt (TIPS) creation in 145 (29%). Major postprocedure complications occurred in 14 patients (2.8%). Vascular/stent restenosis occurred in 95 patients (19%), and successful repeat intervention was performed in 82 of those 95 (86.3%). An additional 11 of these 82 (13.4%) underwent a third intervention for restenosis. In the recanalization and TIPS groups, the 1- and 5-y cumulative patency rates were 87% and 74% and 95% and 68%, respectively. The 1- and 5-y survival rates were 96% and 89% and 90% and 76%, respectively. CONCLUSIONS Endovascular interventions for BCS are feasible and safe in the majority of patients, with excellent short- and long-term patency and survival rates.
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29
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Venous thrombosis of the liver: current and emerging concepts in management. Transl Res 2020; 225:54-69. [PMID: 32407789 DOI: 10.1016/j.trsl.2020.04.012] [Citation(s) in RCA: 6] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/26/2020] [Revised: 04/11/2020] [Accepted: 04/16/2020] [Indexed: 02/08/2023]
Abstract
Venous thrombosis within the hepatic vasculature is associated with a distinct array of risk factors, characteristics, and potential complication. As such, it entails unique management considerations and strategies relative to the more common categories of venous thromboembolic disease. Although broadly divided into thrombosis of the afferent vasculature (the portal venous system) and efferent vasculature (the hepatic venous system), presentations and management strategies within these groupings are heterogeneous. Management decisions are influenced by a variety of factors including the chronicity, extent, and etiology of thrombosis. In this review we examine both portal vein thrombosis and hepatic vein thrombosis (and the associated Budd-Chiari Syndrome). We consider those factors which most impact presentation and most influence treatment. In so doing, we see how the particulars of specific cases introduce nuance into clinical decisions. At the same time we attempt to organize our understanding of such cases to help facilitate a more systematic approach. Critically, we must recognize that although increasing evidence is emerging to help guide our management strategies, the available data remain limited and largely retrospective. Indeed, current paradigms are based largely on observational experiences and expert consensus. As new and more rigorous studies emerge, treatment strategies are likely to be continually refined, and paradigm shifts are sure to occur.
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30
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Sharma A, Goel A, Moses V, Keshava SN, Zachariah UG, Elias E, Eapen CE. Anticoagulating Budd-Chiari syndrome patients presenting with variceal bleed: A retrospective study. J Gastroenterol Hepatol 2020; 35:1397-1403. [PMID: 31900982 DOI: 10.1111/jgh.14971] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/15/2019] [Revised: 12/21/2019] [Accepted: 01/02/2020] [Indexed: 12/09/2022]
Abstract
BACKGROUND AND AIM This aims to study incidence of re-bleeding on anticoagulation and survival of Budd-Chiari syndrome (BCS) patients presenting with variceal bleeding. METHODS Budd-Chiari syndrome patients presenting with variceal bleed between 01/01/2007 and 01/05/2019 were retrospectively studied. Patients underwent endoscopic treatment ± endovascular therapy, followed by anticoagulation. Variceal re-bleed (on anticoagulation) and survival were studied. RESULTS Of 376 BCS patients diagnosed during the study period, 40 (10.7%) patients, presenting with variceal bleed (age 33 [25-40] years; male patients 70%; Rotterdam score 1.13 [0.63-1.22]), Group 1 were compared with 40 randomly selected age-matched BCS patients presenting with ascites, no bleeds (40 [23-42] years; male patients 42.5%; Rotterdam score 1.11 [1.09-1.16]), Group 2. The commonest site of obstruction was hepatic vein (65%) in Group 1 and combined hepatic veins and inferior vena cava (57.5%) in Group 2 (P < 0.01). Thirty-six Group 1 patients underwent endoscopic intervention (variceal ligation, 33; sclerotherapy, 2; glue injection, 1). Endovascular intervention was performed in 30 Group 1 patients (angioplasty ± stent, 22; endovascular shunt, 8) and in 34 Group 2 patients (angioplasty ± stent, 26; endovascular shunt, 8). All 80 patients were started on anticoagulation. Variceal bleed on anticoagulation occurred in five patients in Group 1 and three patients in Group 2. One-year and 5-year survival were 94.2% and 87.5%, respectively, in Group 1 and 100% and 80%, respectively, in Group 2. CONCLUSIONS About one-tenth of BCS patients present with variceal bleed. On management with endoscopic ± endovascular therapy, followed by anticoagulation, variceal re-bleed in these patients were comparable with those in BCS patients presenting with ascites and survival was excellent at 1 and 5 years.
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Affiliation(s)
- Anand Sharma
- Department of Hepatology, Christian Medical College, Vellore, India
| | - Ashish Goel
- Department of Hepatology, Christian Medical College, Vellore, India
| | - Vinu Moses
- Department of Radiology, Christian Medical College, Vellore, India
| | | | | | - Elwyn Elias
- Department of Hepatology, Christian Medical College, Vellore, India.,Liver Unit, University Hospital Birmingham, Birmingham, UK
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Haque LYK, Lim JK. Budd-Chiari Syndrome: An Uncommon Cause of Chronic Liver Disease that Cannot Be Missed. Clin Liver Dis 2020; 24:453-481. [PMID: 32620283 DOI: 10.1016/j.cld.2020.04.012] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/31/2023]
Abstract
Budd-Chiari syndrome (BCS), or hepatic venous outflow obstruction, is a rare cause of liver disease that should not be missed. Variable clinical presentation among patients with BCS necessitates a high index of suspicion to avoid missing this life-threatening diagnosis. BCS is characterized as primary or secondary, depending on etiology of venous obstruction. Most patients with primary BCS have several contributing risk factors leading to a prothrombotic state. A multidisciplinary stepwise approach is integral in treating BCS. Lifelong anticoagulation is recommended. Long-term monitoring of patients for development of cirrhosis, complications of portal hypertension, hepatocellular carcinoma, and progression of underlying diseases is important.
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Affiliation(s)
- Lamia Y K Haque
- Section of Digestive Diseases, Yale University School of Medicine, 333 Cedar Street, LMP 1080, New Haven, CT 06520, USA
| | - Joseph K Lim
- Section of Digestive Diseases, Yale Liver Center, Yale University School of Medicine, 333 Cedar Street, LMP 1080, New Haven, CT 06520, USA.
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Anand AC, Nandi B, Acharya SK, Arora A, Babu S, Batra Y, Chawla YK, Chowdhury A, Chaoudhuri A, Eapen EC, Devarbhavi H, Dhiman R, Datta Gupta S, Duseja A, Jothimani D, Kapoor D, Kar P, Khuroo MS, Kumar A, Madan K, Mallick B, Maiwall R, Mohan N, Nagral A, Nath P, Panigrahi SC, Pawar A, Philips CA, Prahraj D, Puri P, Rastogi A, Saraswat VA, Saigal S, Shalimar, Shukla A, Singh SP, Verghese T, Wadhawan M, The INASL Task-Force on Acute Liver Failure. Indian National Association for the Study of the Liver Consensus Statement on Acute Liver Failure (Part 1): Epidemiology, Pathogenesis, Presentation and Prognosis. J Clin Exp Hepatol 2020; 10:339-376. [PMID: 32655238 PMCID: PMC7335721 DOI: 10.1016/j.jceh.2020.04.012] [Citation(s) in RCA: 42] [Impact Index Per Article: 8.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/22/2020] [Accepted: 04/12/2020] [Indexed: 12/12/2022] Open
Abstract
Acute liver failure (ALF) is an infrequent, unpredictable, potentially fatal complication of acute liver injury (ALI) consequent to varied etiologies. Etiologies of ALF as reported in the literature have regional differences, which affects the clinical presentation and natural course. In this part of the consensus article designed to reflect the clinical practices in India, disease burden, epidemiology, clinical presentation, monitoring, and prognostication have been discussed. In India, viral hepatitis is the most frequent cause of ALF, with drug-induced hepatitis due to antituberculosis drugs being the second most frequent cause. The clinical presentation of ALF is characterized by jaundice, coagulopathy, and encephalopathy. It is important to differentiate ALF from other causes of liver failure, including acute on chronic liver failure, subacute liver failure, as well as certain tropical infections which can mimic this presentation. The disease often has a fulminant clinical course with high short-term mortality. Death is usually attributable to cerebral complications, infections, and resultant multiorgan failure. Timely liver transplantation (LT) can change the outcome, and hence, it is vital to provide intensive care to patients until LT can be arranged. It is equally important to assess prognosis to select patients who are suitable for LT. Several prognostic scores have been proposed, and their comparisons show that indigenously developed dynamic scores have an edge over scores described from the Western world. Management of ALF will be described in part 2 of this document.
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Key Words
- ACLF, acute on chronic liver failure
- AFLP, acute fatty liver of pregnancy
- AKI, Acute kidney injury
- ALF, Acute liver failure
- ALFED, Acute Liver Failure Early Dynamic
- ALT, alanine transaminase
- ANA, antinuclear antibody
- AP, Alkaline phosphatase
- APTT, activated partial thromboplastin time
- ASM, alternative system of medicine
- ASMA, antismooth muscle antibody
- AST, aspartate transaminase
- ATN, Acute tubular necrosis
- ATP, adenosine triphosphate
- ATT, anti-TB therapy
- AUROC, Area under the receiver operating characteristics curve
- BCS, Budd-Chiari syndrome
- BMI, body mass index
- CBF, cerebral blood flow
- CBFV, cerebral blood flow volume
- CE, cerebral edema
- CHBV, chronic HBV
- CLD, chronic liver disease
- CNS, central nervous system
- CPI, clinical prognostic indicator
- CSF, cerebrospinal fluid
- DAMPs, Damage-associated molecular patterns
- DILI, drug-induced liver injury
- EBV, Epstein-Barr virus
- ETCO2, End tidal CO2
- GRADE, Grading of Recommendations Assessment Development and Evaluation
- HAV, hepatitis A virus
- HBV, Hepatitis B virus
- HELLP, hemolysis
- HEV, hepatitis E virus
- HLH, Hemophagocytic lymphohistiocytosis
- HSV, herpes simplex virus
- HV, hepatic vein
- HVOTO, hepatic venous outflow tract obstruction
- IAHG, International Autoimmune Hepatitis Group
- ICH, intracerebral hypertension
- ICP, intracerebral pressure
- ICU, intensive care unit
- IFN, interferon
- IL, interleukin
- IND-ALF, ALF of indeterminate etiology
- INDILI, Indian Network for DILI
- KCC, King's College Criteria
- LC, liver cirrhosis
- LDLT, living donor liver transplantation
- LT, liver transplantation
- MAP, mean arterial pressure
- MHN, massive hepatic necrosis
- MPT, mitochondrial permeability transition
- MUAC, mid-upper arm circumference
- NAPQI, n-acetyl-p-benzo-quinone-imine
- NPV, negative predictive value
- NWI, New Wilson's Index
- ONSD, optic nerve sheath diameter
- PAMPs, pathogen-associated molecular patterns
- PCR, polymerase chain reaction
- PELD, Pediatric End-Stage Liver Disease
- PPV, positive predictive value
- PT, prothrombin time
- RAAS, renin–angiotensin–aldosterone system
- SHF, subacute hepatic failure
- SIRS, systemic inflammatory response syndrome
- SNS, sympathetic nervous system
- TB, tuberculosis
- TCD, transcranial Doppler
- TGF, tumor growth factor
- TJLB, transjugular liver biopsy
- TLR, toll-like receptor
- TNF, tumor necrosis factor
- TSFT, triceps skin fold thickness
- US, ultrasound
- USALF, US Acute Liver Failure
- VZV, varicella-zoster virus
- WD, Wilson disease
- Wilson disease (WD)
- YP, yellow phosphorus
- acute liver failure
- autoimmune hepatitis (AIH)
- drug-induced liver injury
- elevated liver enzymes, low platelets
- sALI, severe acute liver injury
- viral hepatitis
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Affiliation(s)
- Anil C. Anand
- Department of Gastroenterology, Kaliga Institute of Medical Sciences, Bhubaneswar, 751024, India
| | - Bhaskar Nandi
- Department of Gastroenterology, Sarvodaya Hospital and Research Centre, Faridababd, Haryana, India
| | - Subrat K. Acharya
- Department of Gastroenterology and Hepatology, KIIT University, Patia, Bhubaneswar, Odisha, 751 024, India
| | - Anil Arora
- Institute of Liver Gastroenterology &Pancreatico Biliary Sciences, Sir Ganga Ram Hospital, Rajinder Nagar, New Delhi, 110 060, India
| | - Sethu Babu
- Department of Gastroenterology, Krishna Institute of Medical Sciences, Hyderabad 500003, India
| | - Yogesh Batra
- Department of Gastroenterology, Indraprastha Apollo Hospital, SaritaVihar, New Delhi, 110 076, India
| | - Yogesh K. Chawla
- Department of Gastroenterology, Kalinga Institute of Medical Sciences (KIMS), Kushabhadra Campus (KIIT Campus-5), Patia, Bhubaneswar, Odisha, 751 024, India
| | - Abhijit Chowdhury
- Department of Hepatology, School of Digestive and Liver Diseases, Institute of Post Graduate Medical Education & Research, Kolkata, 700020, India
| | - Ashok Chaoudhuri
- Hepatology and Liver Transplant, Institute of Liver & Biliary Sciences, D-1 Vasant Kunj, New Delhi, India
| | - Eapen C. Eapen
- Department of Hepatology, Christian Medical College, Vellore, India
| | - Harshad Devarbhavi
- Department of Gastroenterology and Hepatology, St. John's Medical College Hospital, Bangalore, 560034, India
| | - RadhaKrishan Dhiman
- Department of Hepatology, Post graduate Institute of Medical Education and Research, Chandigarh, 160 012, India
| | - Siddhartha Datta Gupta
- Department of Pathology, All India Institute of Medical Sciences, Ansari Nagar, New Delhi, 110 029, India
| | - Ajay Duseja
- Department of Hepatology, Post graduate Institute of Medical Education and Research, Chandigarh, 160 012, India
| | - Dinesh Jothimani
- Institute of Liver Disease and Transplantation, Dr Rela Institute and Medical Centre, Chrompet, Chennai, 600044, India
| | | | - Premashish Kar
- Department of Gastroenterology and Hepatology, Max Super Speciality Hospital, Vaishali, Ghaziabad, Uttar Pradesh, 201 012, India
| | - Mohamad S. Khuroo
- Department of Gastroenterology, Dr Khuroo’ S Medical Clinic, Srinagar, Kashmir, India
| | - Ashish Kumar
- Institute of Liver Gastroenterology &Pancreatico Biliary Sciences, Sir Ganga Ram Hospital, Rajinder Nagar, New Delhi, 110 060, India
| | - Kaushal Madan
- Gastroenterology and Hepatology, Max Smart Super Specialty Hospital, Saket, New Delhi, India
| | - Bipadabhanjan Mallick
- Department of Gastroenterology, Kalinga Institute of Medical Sciences, Bhubaneswar, 751024, India
| | - Rakhi Maiwall
- Hepatology Incharge Liver Intensive Care, Institute of Liver & Biliary Sciences, D-1 Vasant Kunj, New Delhi, India
| | - Neelam Mohan
- Department of Pediatric Gastroenterology, Hepatology & Liver Transplantation, Medanta – the Medicity Hospital, Sector – 38, Gurgaon, Haryana, India
| | - Aabha Nagral
- Department of Gastroenterology, Apollo and Jaslok Hospital & Research Centre, 15, Dr Deshmukh Marg, Pedder Road, Mumbai, Maharashtra, 400 026, India
| | - Preetam Nath
- Department of Gastroenterology, Kaliga Institute of Medical Sciences, Bhubaneswar, 751024, India
| | - Sarat C. Panigrahi
- Department of Gastroenterology, Kaliga Institute of Medical Sciences, Bhubaneswar, 751024, India
| | - Ankush Pawar
- Liver & Digestive Diseases Institute, Fortis Escorts Hospital, Okhla Road, New Delhi, 110 025, India
| | - Cyriac A. Philips
- The Liver Unit and Monarch Liver Lab, Cochin Gastroenterology Group, Ernakulam Medical Centre, Kochi, 682028, Kerala, India
| | - Dibyalochan Prahraj
- Department of Gastroenterology, Kaliga Institute of Medical Sciences, Bhubaneswar, 751024, India
| | - Pankaj Puri
- Department of Hepatology and Gastroenterology, Fortis Escorts Liver & Digestive Diseases Institute (FELDI), Fortis Escorts Hospital, Delhi, India
| | - Amit Rastogi
- Department of Liver Transplantation, Medanta – the MedicityHospital, Sector – 38, Gurgaon, Haryana, India
| | - Vivek A. Saraswat
- Department of Gastroenterology, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Raibareli Road, Lucknow, Uttar Pradesh, 226 014, India
| | - Sanjiv Saigal
- Department of Hepatology, Department of Liver Transplantation, India
| | - Shalimar
- Department of Gastroenterology and Human Nutrition Unit, All India Institute of Medical Sciences, New Delhi, 29, India
| | - Akash Shukla
- Department of Gastroenterology, LTM Medical College & Sion Hospital, India
| | - Shivaram P. Singh
- Department of Gastroenterology, SCB Medical College, Cuttack, Dock Road, Manglabag, Cuttack, Odisha, 753 007, India
| | - Thomas Verghese
- Department of Gastroenterology, Government Medical College, Kozikhode, India
| | - Manav Wadhawan
- Institute of Liver & Digestive Diseases and Head of Hepatology & Liver Transplant (Medicine), BLK Super Speciality Hospital, Delhi, India
| | - The INASL Task-Force on Acute Liver Failure
- Department of Gastroenterology, Kaliga Institute of Medical Sciences, Bhubaneswar, 751024, India
- Department of Gastroenterology, Sarvodaya Hospital and Research Centre, Faridababd, Haryana, India
- Department of Gastroenterology and Hepatology, KIIT University, Patia, Bhubaneswar, Odisha, 751 024, India
- Institute of Liver Gastroenterology &Pancreatico Biliary Sciences, Sir Ganga Ram Hospital, Rajinder Nagar, New Delhi, 110 060, India
- Department of Gastroenterology, Krishna Institute of Medical Sciences, Hyderabad 500003, India
- Department of Gastroenterology, Indraprastha Apollo Hospital, SaritaVihar, New Delhi, 110 076, India
- Department of Gastroenterology, Kalinga Institute of Medical Sciences (KIMS), Kushabhadra Campus (KIIT Campus-5), Patia, Bhubaneswar, Odisha, 751 024, India
- Department of Hepatology, School of Digestive and Liver Diseases, Institute of Post Graduate Medical Education & Research, Kolkata, 700020, India
- Hepatology and Liver Transplant, Institute of Liver & Biliary Sciences, D-1 Vasant Kunj, New Delhi, India
- Department of Hepatology, Christian Medical College, Vellore, India
- Department of Gastroenterology and Hepatology, St. John's Medical College Hospital, Bangalore, 560034, India
- Department of Hepatology, Post graduate Institute of Medical Education and Research, Chandigarh, 160 012, India
- Department of Pathology, All India Institute of Medical Sciences, Ansari Nagar, New Delhi, 110 029, India
- Institute of Liver Disease and Transplantation, Dr Rela Institute and Medical Centre, Chrompet, Chennai, 600044, India
- Gleneagles Global Hospitals, Hyderabad, Telangana, India
- Department of Gastroenterology and Hepatology, Max Super Speciality Hospital, Vaishali, Ghaziabad, Uttar Pradesh, 201 012, India
- Department of Gastroenterology, Dr Khuroo’ S Medical Clinic, Srinagar, Kashmir, India
- Gastroenterology and Hepatology, Max Smart Super Specialty Hospital, Saket, New Delhi, India
- Department of Gastroenterology, Kalinga Institute of Medical Sciences, Bhubaneswar, 751024, India
- Hepatology Incharge Liver Intensive Care, Institute of Liver & Biliary Sciences, D-1 Vasant Kunj, New Delhi, India
- Department of Pediatric Gastroenterology, Hepatology & Liver Transplantation, Medanta – the Medicity Hospital, Sector – 38, Gurgaon, Haryana, India
- Department of Gastroenterology, Apollo and Jaslok Hospital & Research Centre, 15, Dr Deshmukh Marg, Pedder Road, Mumbai, Maharashtra, 400 026, India
- Liver & Digestive Diseases Institute, Fortis Escorts Hospital, Okhla Road, New Delhi, 110 025, India
- The Liver Unit and Monarch Liver Lab, Cochin Gastroenterology Group, Ernakulam Medical Centre, Kochi, 682028, Kerala, India
- Department of Hepatology and Gastroenterology, Fortis Escorts Liver & Digestive Diseases Institute (FELDI), Fortis Escorts Hospital, Delhi, India
- Department of Liver Transplantation, Medanta – the MedicityHospital, Sector – 38, Gurgaon, Haryana, India
- Department of Gastroenterology, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Raibareli Road, Lucknow, Uttar Pradesh, 226 014, India
- Department of Hepatology, Department of Liver Transplantation, India
- Department of Gastroenterology and Human Nutrition Unit, All India Institute of Medical Sciences, New Delhi, 29, India
- Department of Gastroenterology, LTM Medical College & Sion Hospital, India
- Department of Gastroenterology, SCB Medical College, Cuttack, Dock Road, Manglabag, Cuttack, Odisha, 753 007, India
- Department of Gastroenterology, Government Medical College, Kozikhode, India
- Institute of Liver & Digestive Diseases and Head of Hepatology & Liver Transplant (Medicine), BLK Super Speciality Hospital, Delhi, India
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Li DM, Yin X, Yang F, Zhang LG, Liu TG, Fu YF. Accessory hepatic vein recanalization for hepatic vein-type Budd-Chiari syndrome. MINIM INVASIV THER 2020; 30:239-244. [PMID: 32022611 DOI: 10.1080/13645706.2020.1723110] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/30/2022]
Abstract
PURPOSE To explore the clinical efficacy and long-term outcomes of accessory hepatic vein (AHV) recanalization as a means of treating hepatic vein (HV)-type Budd-Chiari syndrome (BCS). METHODS Between January 2011 and December 2018, a total of 46 symptomatic HV-type BCS patients were treated by AHV recanalization in our hospital. The technical and clinical success of this treatment, as well as associated long-term patient prognosis was assessed herein. RESULTS The AHV recanalization approach was technically successful in 100% of patients, without any instances of complications associated with the operation. This procedure was 95.7% (44/46) clinically successful and resultant. AHV re-obstruction occurred in 12 patients. The cumulative primary one-, two-, and five-year patency rates were 77.3%, 71.7%, and 71.7%, respectively. The secondary cumulative one-, two-, and five-year patency rates were 97.7, 87.1, and 87.1%, respectively. The five-year patency rates did not differ significantly between patients treated with balloons and stents (p = .674). Based on Cox-regression analysis, younger age was an independent predictor of re-obstruction (p = .005). The cumulative one-, two-, and five-year survival rates were 97.7, 92.2, and 92.2%, respectively. CONCLUSIONS AHV recanalization is a safe and effective treatment for HV-type BCS.
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Affiliation(s)
- Dong-Mei Li
- Department of Infectious and Liver Disease, Affiliated Hospital of Binzhou Medical College, Binzhou, China
| | - Xue Yin
- Department of Infectious and Liver Disease, Affiliated Hospital of Binzhou Medical College, Binzhou, China
| | - Fang Yang
- Department of Infectious and Liver Disease, Affiliated Hospital of Binzhou Medical College, Binzhou, China
| | - Li-Guo Zhang
- Department of Infectious and Liver Disease, Affiliated Hospital of Binzhou Medical College, Binzhou, China
| | - Tong-Gang Liu
- Department of Infectious and Liver Disease, Affiliated Hospital of Binzhou Medical College, Binzhou, China
| | - Yu-Fei Fu
- Department of Radiology, Xuzhou Central Hospital, Xuzhou, China
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Mukund A, Taunk A. Percutaneous Recanalization for Budd–Chiari Syndrome. BUDD-CHIARI SYNDROME 2020:147-156. [DOI: 10.1007/978-981-32-9232-1_11] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 01/04/2025]
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Sugawara Y, Hibi T. Liver Transplantation for Budd–Chiari Syndrome. BUDD-CHIARI SYNDROME 2020:181-187. [DOI: 10.1007/978-981-32-9232-1_14] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 01/03/2025]
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Abstract
Disorders of the mesenteric, portal, and hepatic veins and mesenteric and hepatic arteries have important clinical consequences and may lead to acute liver failure, chronic liver disease, noncirrhotic portal hypertension, cirrhosis, and hepatocellular carcinoma. Although literature in the field of vascular liver disorders is scant, these disorders are common in clinical practice, and general practitioners, gastroenterologists, and hepatologists may benefit from expert guidance and recommendations for management of these conditions. These guidelines represent the official practice recommendations of the American College of Gastroenterology. Key concept statements based on author expert opinion and review of literature and specific recommendations based on PICO/GRADE analysis have been developed to aid in the management of vascular liver disorders. These recommendations and guidelines should be tailored to individual patients and circumstances in routine clinical practice.
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Payancé A, Plessier A. Anticoagulation for Budd–Chiari Syndrome. BUDD-CHIARI SYNDROME 2020:131-145. [DOI: 10.1007/978-981-32-9232-1_10] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 01/04/2025]
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Mancuso A. Controversies in the Management of Budd–Chiari Syndrome. BUDD-CHIARI SYNDROME 2020:245-252. [DOI: 10.1007/978-981-32-9232-1_19] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 01/04/2025]
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Seetharam A. Intensive Care Management of Acute Liver Failure: Considerations While Awaiting Liver Transplantation. J Clin Transl Hepatol 2019; 7:384-391. [PMID: 31915608 PMCID: PMC6943205 DOI: 10.14218/jcth.2019.00032] [Citation(s) in RCA: 8] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/31/2019] [Revised: 10/06/2019] [Accepted: 10/27/2019] [Indexed: 12/15/2022] Open
Abstract
Acute liver failure is a unique clinical phenomenon characterized by abrupt deterioration in liver function and altered mentation. The development of high-grade encephalopathy and multisystem organ dysfunction herald poor prognosis. Etiologic-specific treatments and supportive measures are routinely employed; however, liver transplantation remains the only chance for cure in those who do not spontaneously recover. The utility of artificial and bioartificial assist therapies as supportive care-to allow time for hepatic recovery or as a bridge to liver transplantation-has been examined but studies have been small, with mixed results. Given the severity of derangements, intensive critical care is needed to successfully bridge patients to transplant, and evaluation of candidates occurs rapidly in parallel with serial reassessments of operative fitness. Psychosocial assessment is often suboptimal and relative contraindications to transplant, such as ventilator-dependence may be overlooked. While often employed to guide evaluation, no single prognostic model discriminates those who will spontaneously recover and those who will require transplant. The purpose of this review will be to summarize approaches in critical care, prognostic modeling, and medical evaluation of the acute liver failure transplant candidate.
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Affiliation(s)
- Anil Seetharam
- Correspondence to: Anil Seetharam, Banner Transplant and Advanced Liver Disease, University of Arizona College of Medicine, 441 N. 12th Street, 2nd Floor, Phoenix, AZ 85006, USA. Tel: +1-602-521-5800; Fax: +1-602-521-5337, E-mail:
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Kageyama K, Yamamoto A, Jogo A, Nota T, Murai K, Ogawa S, Nakano MM, Sohgawa E, Hamamoto S, Hamuro M, Kaminou T, Nishida N, Takahashi K, Yamamoto K, Miki Y. Usefulness of Venous Pressure Measurement in Endovascular Treatment of Budd-Chiari Syndrome: A Retrospective Cohort Study. Intern Med 2019; 58:2923-2929. [PMID: 31243212 PMCID: PMC6859396 DOI: 10.2169/internalmedicine.2704-19] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 01/17/2023] Open
Abstract
Objective Therapeutic predictors derived from the venous pressure before therapy have not been identified for Budd-Chiari syndrome (BCS). The aim of this study was to determine whether or not measuring the distal pressure or pressure gradient was useful for predicting treatment efficacy in BCS. Methods We retrospectively analyzed seven consecutive patients diagnosed with symptomatic BCS at our hospital between 2008 and 2017. Distal and proximal venous pressures at occlusion sites of BCS were measured before treatment in all cases. The pressure gradient was defined as the difference between distal and proximal venous pressures. A receiver operating characteristics (ROC) analysis was performed for venous pressures. Results Percutaneous old balloon angioplasty (POBA) was performed in seven cases, with technical success achieved in all cases (100%). No complications were encountered. The median primary patency was 574 (interquartile range, 439.5-1,056.5) days. The 1-year primary patency rate was 71.73%. Six cases (85.7%) showed resolution of symptoms, representing clinical success. The ROC analysis revealed a high distal pressure (area under the ROC curve = 0.83, cut-off=12 mmHg) as a predictor of treatment efficacy of POBA for symptomatic BCS. In addition, the pressure gradient was considered significant from a clinical perspective, because the 6 successful cases with resolution of symptoms showed a large pressure gradient (range, 8-21 mmHg) before treatment, whereas the failed case showed a relatively small pressure gradient (7 mmHg). Conclusion High distal pressure and a large pressure gradient might predict the treatment efficacy of balloon angioplasty for symptomatic BCS.
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Affiliation(s)
- Ken Kageyama
- Department of Diagnostic and Interventional Radiology, Osaka City University Graduate School of Medicine, Japan
| | - Akira Yamamoto
- Department of Diagnostic and Interventional Radiology, Osaka City University Graduate School of Medicine, Japan
| | - Atsushi Jogo
- Department of Diagnostic and Interventional Radiology, Osaka City University Graduate School of Medicine, Japan
| | - Takehito Nota
- Department of Diagnostic and Interventional Radiology, Osaka City University Graduate School of Medicine, Japan
| | - Kazuki Murai
- Department of Diagnostic and Interventional Radiology, Osaka City University Graduate School of Medicine, Japan
| | - Satoyuki Ogawa
- Department of Diagnostic and Interventional Radiology, Osaka City University Graduate School of Medicine, Japan
| | - Mariko M Nakano
- Department of Diagnostic and Interventional Radiology, Osaka City University Graduate School of Medicine, Japan
| | - Etsuji Sohgawa
- Department of Diagnostic and Interventional Radiology, Osaka City University Graduate School of Medicine, Japan
| | - Shinichi Hamamoto
- Department of Diagnostic and Interventional Radiology, Osaka City University Graduate School of Medicine, Japan
| | - Masao Hamuro
- Department of Radiology, Izumiotsu Municipal Hospital, Japan
| | - Toshio Kaminou
- Department of Radiology, National Hospital Organization Osaka Minami Medical Center, Japan
| | - Norifumi Nishida
- Department of Radiology, Osaka Saiseikai Nakatsu Hospital, Japan
| | - Kanae Takahashi
- Department of Medical Statistics, Osaka City University Graduate School of Medicine, Japan
| | - Kouji Yamamoto
- Department of Medical Statistics, Osaka City University Graduate School of Medicine, Japan
| | - Yukio Miki
- Department of Diagnostic and Interventional Radiology, Osaka City University Graduate School of Medicine, Japan
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Wang Q, Li K, He C, Yuan X, Luo B, Qi X, Guo W, Bai W, Yu T, Fan J, Wang Z, Yuan J, Li X, Zhu Y, Han N, Niu J, Lv Y, Liu L, Li J, Tang S, Guo S, Wang E, Xia D, Wang Z, Cai H, Wang J, Yin Z, Xia J, Fan D, Han G. Angioplasty with versus without routine stent placement for Budd-Chiari syndrome: a randomised controlled trial. Lancet Gastroenterol Hepatol 2019; 4:686-697. [PMID: 31279647 DOI: 10.1016/s2468-1253(19)30177-3] [Citation(s) in RCA: 28] [Impact Index Per Article: 4.7] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/01/2019] [Revised: 04/18/2019] [Accepted: 04/23/2019] [Indexed: 02/07/2023]
Abstract
BACKGROUND Angioplasty recanalisation is recommended as the first-line interventional procedure for Budd-Chiari syndrome, but subsequent restenosis is common. We aimed to test whether use of routine, non-selective stenting in angioplasty could improve patency and treatment efficacy with adequate safety in Budd-Chiari syndrome. METHODS We did a randomised controlled trial, for which patients aged 18-75 years with Budd-Chiari syndrome with membranous obstruction or short-length stenosis (≤4 cm), and a Child-Pugh score of less than 13 were considered eligible. Patients were excluded if they had obstruction not amenable to angioplasty, were recommended to be treated with transjugular intrahepatic portosystemic shunt or liver transplantation, or had contraindications for angioplasty. Eligible patients were randomly assigned (1:1) to an angioplasty-only group or an angioplasty plus routine stenting group, with use of a web-based allocation system (Pocock and Simon's minimisation method, stratified by obstruction features and Child-Pugh score). Recanalisation procedures were done within 24 h of randomisation. The statistician and investigators responsible for data collection data and endpoint assessment were masked to group allocation. The primary outcome was the proportion of patients free of restenosis, analysed in the intention-to-treat population. The study is registered on ClinicalTrials.gov (NCT02201485) and is completed. FINDINGS Between July 28, 2014, and Sept 29, 2017, 88 (59%) of 150 screened patients were enrolled and assigned either the angioplasty-only group (n=45) or the angioplasty plus routine stenting group (n=43). During a median follow-up period of 27 months (IQR 19-41), the angioplasty plus routine stenting group had significantly higher proportion of patients free of restenosis (42 [98%] of 43 patients) than did the angioplasty-only group (27 [60%] of 45 patients; p<0·0001). In the survival analysis, 3-year restenosis-free survival was 96·0% (95% CI 88·6-100·0) in the routine stenting group versus 60·4% (46·4-78·7) in the angioplasty-only group (log-rank p<0·0001). The hazard ratio for restenosis was 0·04 (95% CI 0·01-0·31) in favour of routine stenting, with an absolute risk reduction of 35·6% (95% CI 24·2-55·0). Two (5%) patients in the angioplasty plus routine stenting group and one (2%) patient in the angioplasty-only group died during follow-up. One (2%) patient from the angioplasty plus routine stenting group had puncture site haematoma, which was not related to stenting. No stent fracture or migration occurred. Anticoagulation-related adverse events occurred in five (11%) patients from angioplasty alone group and five (12%) patients from angioplasty plus routine stenting group. INTERPRETATION Routine stenting with angioplasty is superior to angioplasty alone for preventing restenosis in patients with Budd-Chiari syndrome with short-length stenosis and is safe to use as part of first-line invasive treatment. Further validation is needed in similar settings and other regions in which different characteristics of Budd-Chiari syndrome are more prevalent. FUNDING National Natural Science Foundation of China, National Key Technology R&D Programme, Optimised Overall Project of Shaanxi Province, Boost Programme of Xijing Hospital.
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Affiliation(s)
- Qiuhe Wang
- Department of Liver Diseases and Digestive Interventional Radiology, National Clinical Research Centre for Digestive Diseases and Xijing Hospital of Digestive Diseases, Fourth Military Medical University, Xi'an, Shaanxi, China
| | - Kai Li
- Department of Liver Diseases and Digestive Interventional Radiology, National Clinical Research Centre for Digestive Diseases and Xijing Hospital of Digestive Diseases, Fourth Military Medical University, Xi'an, Shaanxi, China
| | - Chuangye He
- Department of Liver Diseases and Digestive Interventional Radiology, National Clinical Research Centre for Digestive Diseases and Xijing Hospital of Digestive Diseases, Fourth Military Medical University, Xi'an, Shaanxi, China
| | - Xulong Yuan
- Department of Liver Diseases and Digestive Interventional Radiology, National Clinical Research Centre for Digestive Diseases and Xijing Hospital of Digestive Diseases, Fourth Military Medical University, Xi'an, Shaanxi, China
| | - Bohan Luo
- Department of Liver Diseases and Digestive Interventional Radiology, National Clinical Research Centre for Digestive Diseases and Xijing Hospital of Digestive Diseases, Fourth Military Medical University, Xi'an, Shaanxi, China
| | - Xingshun Qi
- Department of Liver Diseases and Digestive Interventional Radiology, National Clinical Research Centre for Digestive Diseases and Xijing Hospital of Digestive Diseases, Fourth Military Medical University, Xi'an, Shaanxi, China
| | - Wengang Guo
- Department of Liver Diseases and Digestive Interventional Radiology, National Clinical Research Centre for Digestive Diseases and Xijing Hospital of Digestive Diseases, Fourth Military Medical University, Xi'an, Shaanxi, China
| | - Wei Bai
- Department of Liver Diseases and Digestive Interventional Radiology, National Clinical Research Centre for Digestive Diseases and Xijing Hospital of Digestive Diseases, Fourth Military Medical University, Xi'an, Shaanxi, China
| | - Tianlei Yu
- Department of Liver Diseases and Digestive Interventional Radiology, National Clinical Research Centre for Digestive Diseases and Xijing Hospital of Digestive Diseases, Fourth Military Medical University, Xi'an, Shaanxi, China
| | - Jiahao Fan
- Department of Liver Diseases and Digestive Interventional Radiology, National Clinical Research Centre for Digestive Diseases and Xijing Hospital of Digestive Diseases, Fourth Military Medical University, Xi'an, Shaanxi, China
| | - Zhengyu Wang
- Department of Liver Diseases and Digestive Interventional Radiology, National Clinical Research Centre for Digestive Diseases and Xijing Hospital of Digestive Diseases, Fourth Military Medical University, Xi'an, Shaanxi, China
| | - Jie Yuan
- Department of Liver Diseases and Digestive Interventional Radiology, National Clinical Research Centre for Digestive Diseases and Xijing Hospital of Digestive Diseases, Fourth Military Medical University, Xi'an, Shaanxi, China
| | - Xiaomei Li
- Department of Liver Diseases and Digestive Interventional Radiology, National Clinical Research Centre for Digestive Diseases and Xijing Hospital of Digestive Diseases, Fourth Military Medical University, Xi'an, Shaanxi, China
| | - Ying Zhu
- Department of Liver Diseases and Digestive Interventional Radiology, National Clinical Research Centre for Digestive Diseases and Xijing Hospital of Digestive Diseases, Fourth Military Medical University, Xi'an, Shaanxi, China
| | - Na Han
- Department of Liver Diseases and Digestive Interventional Radiology, National Clinical Research Centre for Digestive Diseases and Xijing Hospital of Digestive Diseases, Fourth Military Medical University, Xi'an, Shaanxi, China
| | - Jing Niu
- Department of Liver Diseases and Digestive Interventional Radiology, National Clinical Research Centre for Digestive Diseases and Xijing Hospital of Digestive Diseases, Fourth Military Medical University, Xi'an, Shaanxi, China
| | - Yong Lv
- Department of Liver Diseases and Digestive Interventional Radiology, National Clinical Research Centre for Digestive Diseases and Xijing Hospital of Digestive Diseases, Fourth Military Medical University, Xi'an, Shaanxi, China
| | - Lei Liu
- Department of Liver Diseases and Digestive Interventional Radiology, National Clinical Research Centre for Digestive Diseases and Xijing Hospital of Digestive Diseases, Fourth Military Medical University, Xi'an, Shaanxi, China
| | - Jing Li
- Department of Liver Diseases and Digestive Interventional Radiology, National Clinical Research Centre for Digestive Diseases and Xijing Hospital of Digestive Diseases, Fourth Military Medical University, Xi'an, Shaanxi, China
| | - Shihao Tang
- Department of Liver Diseases and Digestive Interventional Radiology, National Clinical Research Centre for Digestive Diseases and Xijing Hospital of Digestive Diseases, Fourth Military Medical University, Xi'an, Shaanxi, China
| | - Shuai Guo
- Department of Liver Diseases and Digestive Interventional Radiology, National Clinical Research Centre for Digestive Diseases and Xijing Hospital of Digestive Diseases, Fourth Military Medical University, Xi'an, Shaanxi, China
| | - Enxing Wang
- Department of Liver Diseases and Digestive Interventional Radiology, National Clinical Research Centre for Digestive Diseases and Xijing Hospital of Digestive Diseases, Fourth Military Medical University, Xi'an, Shaanxi, China
| | - Dongdong Xia
- Department of Liver Diseases and Digestive Interventional Radiology, National Clinical Research Centre for Digestive Diseases and Xijing Hospital of Digestive Diseases, Fourth Military Medical University, Xi'an, Shaanxi, China
| | - Zhexuan Wang
- Department of Liver Diseases and Digestive Interventional Radiology, National Clinical Research Centre for Digestive Diseases and Xijing Hospital of Digestive Diseases, Fourth Military Medical University, Xi'an, Shaanxi, China
| | - Hongwei Cai
- Department of Medical Statistics, School of Preventive Medicine, Fourth Military Medical University, Xi'an, Shaanxi, China; Information Technology Department, First Affiliated Hospital of Xi'an Jiaotong University, Xi'an, Shaanxi, China
| | - Jianhong Wang
- Department of Ultrasound, Xijing Hospital of Digestive Diseases, Fourth Military Medical University, Xi'an, Shaanxi, China
| | - Zhanxin Yin
- Department of Liver Diseases and Digestive Interventional Radiology, National Clinical Research Centre for Digestive Diseases and Xijing Hospital of Digestive Diseases, Fourth Military Medical University, Xi'an, Shaanxi, China
| | - Jielai Xia
- Department of Medical Statistics, School of Preventive Medicine, Fourth Military Medical University, Xi'an, Shaanxi, China
| | - Daiming Fan
- State Key Laboratory of Cancer Biology, National Clinical Research Centre for Digestive Diseases and Xijing Hospital of Digestive Diseases, Fourth Military Medical University, Xi'an, Shaanxi, China
| | - Guohong Han
- Department of Liver Diseases and Digestive Interventional Radiology, National Clinical Research Centre for Digestive Diseases and Xijing Hospital of Digestive Diseases, Fourth Military Medical University, Xi'an, Shaanxi, China.
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Sonavane AD, Amarapurkar DN, Rathod KR, Punamiya SJ. Long Term Survival of Patients Undergoing TIPS in Budd-Chiari Syndrome. J Clin Exp Hepatol 2019; 9:56-61. [PMID: 30765940 PMCID: PMC6363956 DOI: 10.1016/j.jceh.2018.02.008] [Citation(s) in RCA: 11] [Impact Index Per Article: 1.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/08/2017] [Accepted: 02/20/2018] [Indexed: 02/07/2023] Open
Abstract
BACKGROUND There has been significant improvement in understanding the etiology and management of Budd-Chiari Syndrome (BCS). Patients with chronic or acute-on-chronic BCS need radiological interventions in the form of angioplasty, hepatic vein/inferior vena cava stenting or Transjugular Intrahepatic Portosystemic Shunt (TIPS). Data regarding the long term follow up of patients undergoing TIPS is limited. We thus prospectively followed-up BCS patients who underwent TIPS at our center. METHODS This study included 42 patients with BCS who underwent TIPS with a covered stent between 2004 and 2014. We analyzed the etiology, symptoms, severity, laboratory parameters and imaging pre and post TIPS. All patients underwent surveillance for hepatocellular carcinoma. RESULTS Patients demographics included 26 males and 16 females with a mean age of 40.5 years (19-68 years). The mean Model for End-Stage Liver Disease score of the entire cohort was 15.38 (range: 9-25). Thirty-four patients were grouped into Rotterdam Class 2 and remaining into Class 3. There was significant improvement in ascites, gastrointestinal bleed, renal function and transaminase levels post TIPS. There were 11 deaths over the follow-up period - 4 within one month, 2 within six months and the rest after 3 years following TIPS. Median duration from clinical presentation to TIPS was 2.1 weeks and median survival till follow-up was 45.5 months (0-130 months). 33/42 patients underwent TIPS prior to 2013, and their median survival till follow-up was 55 months. Six out of eleven deaths that occurred within six months post-TIPS were before 2006; when the technique of TIPS creation was evolving. The cumulative 1 year, 5 years and 10 years OLT-free survival was 86%, 81% and 76%, respectively. Two patients underwent a liver transplant at 4 and 7 years after TIPS. CONCLUSION Our results validate the role of TIPS in the management of patients with BCS. With the accessibility of TIPS, the requirement for liver transplantation has become rare.
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Key Words
- AT III, Antithrombin III
- BCS, Budd-Chiari Syndrome
- Budd Chiari syndrome
- CT, Computerized Tomography
- CTP, Child-Turcotte-Pugh
- HV, Hepatic Vein
- INR, International Normalized Ratio
- IVC, Inferior Vena Cava
- JAK-2, Janus Kinase 2
- MELD, Model for End-Stage Liver Disease
- MRI, Magnetic Resonance Imaging
- MTHFR, Methylene Tetrahydrofolate Reductase
- OLT, Orthotopic Liver Transplantation
- SD, Standard Deviation
- TIPS, Transjugular Intrahepatic Portosystemic Shunt
- TIPS-BCS PI score, BCS-TIPS Prognostic Index score
- long term survival
- orthotopic liver transplantation free survival
- transjugular intrahepatic portosystemic shunt
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Affiliation(s)
- Amey D. Sonavane
- Post-graduate Resident, Department of Gastroenterology, Bombay Hospital and Institute of Medical Sciences, Mumbai, India,Address for correspondence: Amey D. Sonavane, C-1804, Azziano, Rustomjee Urbania, Majiwada, Thane (West), Maharashtra 400601, India.
| | - Deepak N. Amarapurkar
- Consultant Gastroenterologist, Department of Gastroenterology, Bombay Hospital and Institute of Medical Sciences, Mumbai, India
| | - Krantikumar R. Rathod
- Consultant, Department of Vascular and Interventional Radiology, Bombay Hospital and Institute of Medical Sciences, Mumbai, India
| | - Sundeep J. Punamiya
- Consultant, Department of Vascular and Interventional Radiology, Tan Tock Seng Hospital, Singapore
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Bi Y, Chen H, Ding P, Zhou P, Han X, Ren J. Excellent long-term outcomes of endovascular treatment in budd-chiari syndrome with hepatic veins involvement: A STROBE-compliant article. Medicine (Baltimore) 2018; 97:e12944. [PMID: 30412110 PMCID: PMC6221688 DOI: 10.1097/md.0000000000012944] [Citation(s) in RCA: 9] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/24/2018] [Accepted: 10/01/2018] [Indexed: 12/23/2022] Open
Abstract
This study aimed to evaluate the long-term efficacy and safety of percutaneous transhepatic balloon angioplasty (PTBA) and transjugular intrahepatic portosystemic stent-shunt (TIPSS) in the treatment of Budd-Chiari syndrome (BCS) with hepatic veins involvement. Between June 2008 and August 2016, a total of 60 BCS patients with hepatic vein involvement in our department were enrolled in this study. Thirty-three cases underwent hepatic vein balloon angioplasty in PTBA Group and 27 cases underwent TIPSS. Data were retrospectively collected, and follow-up observations were performed. TIPSS Group showed significantly higher thrombotic/segmental obstruction and peripheral stenosis/obstruction compared with PTBA Group. The success rates were 93.9% and 100.0% in PTBA Group and TIPSS Group, respectively. The mean portal vein pressure decreased significantly after stenting. Except for 1 patient died from repeated hemorrhage, other sever complications had not been observed in both group. Twenty-six patients and 21 patients were clinically cured in PTBA Group and TIPSS Group, respectively. The primary patency rates were 89.7%, 79.3%, and 79.3% for short-term, mid-term and longterm in PTBA Group, which were significantly higher than TIPSS Group for long-term follow up. The second patency rates were 100.0%, 96.6% and 96.6% for short-term, mid-term and long-term in PTBA Group, which were similar to TIPSS Group (P = 1.0000). In conclusion, PTBA and TIPSS are safe and effective in the treatment of BCS with hepatic veins involvement, with an excellent long-term patency rate of hepatic vein and TIPSS shunt. TIPSS can be used to treat patients with all 3 hepatic veins lesion and failure PTBA.
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Affiliation(s)
- Yonghua Bi
- Department of Interventional Radiology, The First Affiliated Hospital of Zhengzhou University
| | - Hongmei Chen
- Department of Ultrasound, Zhengzhou Central Hospital Affiliated to Zhengzhou University, Zhengzhou, China
| | - Pengxu Ding
- Department of Interventional Radiology, The First Affiliated Hospital of Zhengzhou University
| | - Pengli Zhou
- Department of Interventional Radiology, The First Affiliated Hospital of Zhengzhou University
| | - Xinwei Han
- Department of Interventional Radiology, The First Affiliated Hospital of Zhengzhou University
| | - Jianzhuang Ren
- Department of Interventional Radiology, The First Affiliated Hospital of Zhengzhou University
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Direct Intrahepatic Portosystemic Shunt in Budd-Chiari Syndrome: A Case Report and Review of the Literature. Case Rep Radiol 2018; 2018:9261268. [PMID: 30210895 PMCID: PMC6126074 DOI: 10.1155/2018/9261268] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/07/2018] [Accepted: 06/27/2018] [Indexed: 11/17/2022] Open
Abstract
Transjugular intrahepatic portosystemic shunt (TIPS) is an alternative interventional procedure used to manage refractory Budd-Chiari syndrome (BCS) when conservative medical therapy has failed. However, TIPS is not always technically successful because of hepatic vein thrombosis and inability to catheterize the hepatic veins. In these situations, direct intrahepatic portosystemic shunt (DIPS) with access to the portal vein from the IVC has been shown to be a viable alternative that may ameliorate portal hypertension in these patients. Typically, DIPS involves the use of transabdominal ultrasound to target the portal vein. Herein a case in which a 39-year-old female underwent DIPS without the use of ultrasound guidance is presented. Instead, a hepatic venogram generated using collateral circulation was used to opacify and guide access to the portal vein.
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Cheng DL, Zhu N, Xu H, Li CL, Lv WF, Fang WW, Li CT. Outcomes of endovascular interventional therapy for primary Budd-Chiari syndrome caused by hepatic venous obstruction. Exp Ther Med 2018; 16:4141-4149. [PMID: 30402156 PMCID: PMC6201046 DOI: 10.3892/etm.2018.6708] [Citation(s) in RCA: 7] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/11/2018] [Accepted: 08/09/2018] [Indexed: 12/13/2022] Open
Abstract
To date, interventional therapy for patients with Budd-Chiari syndrome (BCS) due to hepatic vein obstruction (HVO) has not been standardized in China. In Western countries, BCS primarily occurs due to thrombosis and the majority of patients receive thrombolysis. In China, BCS is mostly caused by the membranous occlusion of the HV or IVC. The present retrospective study evaluated the efficacy of recanalization techniques in patients with primary BCS due to HVO. The data of 69 patients with BCS due to HVO, who underwent endovascular therapy at 2 centers in China between December 2010 and December 2012, were analyzed. All of the patients underwent balloon angioplasty. In addition, 14, 6 and 5 patients received thrombolysis, endovascular stent and thrombolysis + endovascular stent, respectively. The overall technical success rate was 95.7% (66/69), and was comparable among the treatments. The HV pressure after the treatments was significantly lower compared with that prior to the procedures (23.3±6.9 vs. 46.5±8.6 cmH2O; P<0.001). The mean follow-up duration was 75 months (range, 60–84 months). During the 5-year follow-up, 10 patients (15.2%) had developed a recurrence of BCS-associated symptoms, of which 7 were successfully treated. The cumulative survival rates at 12, 36 and 60 months after endovascular interventional therapy (balloon angioplasty or combined treatment) were 98.5, 98.5 and 93.9%, respectively. After treatment by endovascular therapy, the patients with BCS caused by HVO had high survival rates and low recurrence rates in the short- and mid-term.
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Affiliation(s)
- De-Lei Cheng
- Shandong Medical Imaging Research Institute, Shandong University, Jinan, Shandong 250021, P.R. China.,Department of Interventional Radiology, Anhui Provincial Hospital, Hefei, Anhui 230000, P.R. China
| | - Nan Zhu
- Department of Radiology, People's Hospital of Wuhe County, Bengbu, Anhui 233300, P.R. China
| | - Hao Xu
- Department of Interventional Radiology, Affiliated Hospital of Xuzhou Medical University, Xuzhou, Jiangsu 221100, P.R. China
| | - Cheng-Li Li
- Shandong Medical Imaging Research Institute, Shandong University, Jinan, Shandong 250021, P.R. China
| | - Wei-Fu Lv
- Department of Interventional Radiology, Anhui Provincial Hospital, Hefei, Anhui 230000, P.R. China
| | - Wei-Wei Fang
- Department of Interventional Radiology, Anhui Provincial Hospital, Hefei, Anhui 230000, P.R. China
| | - Chuan-Ting Li
- Shandong Medical Imaging Research Institute, Shandong University, Jinan, Shandong 250021, P.R. China
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Image-guided treatment of Budd-Chiari syndrome: a giant leap from the past, a small step towards the future. Abdom Radiol (NY) 2018; 43:1908-1919. [PMID: 28988356 DOI: 10.1007/s00261-017-1341-y] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/06/2023]
Abstract
Budd-Chiari syndrome (BCS) is a relatively rare vascular disease characterized by hepatic outflow tract obstruction, and image-guided endovascular treatment, namely percutaneous angioplasty, stenting, and transjugular intrahepatic portosystemic shunt (TIPS), has proven to be effective treatment modalities to alleviate symptoms and markedly improve the prognosis of the disease. Specifically, a step-wise approach is recommended, i.e., angioplasty and stenting are the prioritized choice for patients with membranous obstruction and short-length stenosis, whereas TIPS is the option for patients who fail this treatment. Currently, 5-year survival with the step-wise approach is about 75%, and the most promising way to further improve this value is to identify candidates who are at high risk of failing angioplasty, and perform pre-emptive TIPS in these patients.
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Bi Y, Yu Z, Ding P, Ren J, Zhou P, Han X. Long-Term Outcomes of Endoluminal Sharp Recanalization of Occluded Inferior Vena Cava in Budd-Chiari Syndrome. J Laparoendosc Adv Surg Tech A 2018; 29:309-315. [PMID: 30036126 DOI: 10.1089/lap.2018.0385] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/18/2022] Open
Abstract
PURPOSE To report the long-term results of endoluminal sharp recanalization of occluded inferior vena cava (IVC) in patients with the Budd-Chiari syndrome (BCS). PATIENTS AND METHODS Seventy-two consecutive patients with BCS and IVC occlusion underwent endoluminal recanalization of the IVC occlusion during a 6-year period. BCS with occlusive IVC was detected by reviewing patient history and color Doppler ultrasonography. Data on technical success, morbidity, mortality, complications, and color Doppler sonographic outcome were collected and analyzed. RESULTS Seventy-seven recanalizations were performed in 72 patients. Technical success (patent IVC with good blood flow) was achieved in 70 (97.4%) patients. No perioperative death was observed. Six complications were found during recanalization, and 10 complications were found for total interventional procedures. The complication rates were 7.8% and 7.3% for recanalization and total procedure, respectively. Bleeding of access veins was the most common complication, and 2 patients showed slight rupture of IVC. The postoperative IVC diameter and blood speed of IVC increased significantly. Fifty-seven patients (79.2%) were clinical cured, and 2 patients (2.8%) showed no improvement. The 1-year, 3-year, and 5-year primary patency rates were 92.5%, 86.8%, and 77.3%, respectively. The second patency rates were 100.0%, 97.8%, and 91.8% for 1, 3, and 5 years, respectively. There were 2 deaths during follow-up. CONCLUSION Endoluminal sharp recanalization of occluded IVC in patients with BCS is safe and effective with good long-term outcomes.
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Affiliation(s)
- Yonghua Bi
- Department of Interventional Radiology, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, China
| | - Zepeng Yu
- Department of Interventional Radiology, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, China
| | - Pengxu Ding
- Department of Interventional Radiology, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, China
| | - Jianzhuang Ren
- Department of Interventional Radiology, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, China
| | - Pengli Zhou
- Department of Interventional Radiology, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, China
| | - Xinwei Han
- Department of Interventional Radiology, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, China
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Bi Y, Chen H, Ding P, Zhou P, Ren J, Han X. Percutaneous Transhepatic Hepatic Vein Angioplasty in Budd-Chiari Syndrome After Transvenous Failure. J Laparoendosc Adv Surg Tech A 2018; 28:1346-1351. [PMID: 29851361 DOI: 10.1089/lap.2018.0156] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/12/2022] Open
Abstract
PURPOSE To evaluate the safety and efficacy of percutaneous transhepatic route creation for hepatic vein (HV) angioplasty in Budd-Chiari syndrome (BCS). PATIENTS AND METHODS Between April 2012 and August 2016, a total of 19 BCS patients underwent percutaneous transhepatic route creation for HV angioplasty after transvenous catheterization failure in this study. Color Doppler ultrasonography was required in all patients after admission and during follow-up. Data were retrospectively collected, and follow-up observations were performed. RESULTS Technical and clinical success was achieved in 18 patients. Except for 1 failure of route creation, 19 routes were successfully created in 18 patients, with a technology success rate of 95.0%. Twenty-two balloon angioplasties were performed in 18 patients, with a mean balloon diameter of 13.6 ± 0.5 mm. Blood pressure and length of occlusive HV decreased significantly, and blood flow velocity and diameter of HV increased significantly after procedure. Abdominal distension/pain and ascites decreased significantly after procedure. One procedure-related death occurred, who died of gastrointestinal bleeding 6 days later. Except for the failure case, the rest of 18 patients were successfully followed up. The remaining 18 patients survived during follow-up, with a 5-year survival rate of 94.9%. One patient had a restenosis of HV after 47 days, and had undergone successful dilation. The 5-year primary and second patency rates were 94.1% and 100%, respectively. CONCLUSION Percutaneous transhepatic route creation is safe and effective for HV angioplasty, and can be used to treat BCS patients after transvenous catheterization failure.
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Affiliation(s)
- Yonghua Bi
- 1 Department of Interventional Radiology, The First Affiliated Hospital of Zhengzhou University , Zhengzhou, China
| | - Hongmei Chen
- 2 Department of Ultrasound, Zhengzhou Central Hospital Affiliated to Zhengzhou University , Zhengzhou, China
| | - Pengxu Ding
- 1 Department of Interventional Radiology, The First Affiliated Hospital of Zhengzhou University , Zhengzhou, China
| | - Pengli Zhou
- 1 Department of Interventional Radiology, The First Affiliated Hospital of Zhengzhou University , Zhengzhou, China
| | - Jianzhuang Ren
- 1 Department of Interventional Radiology, The First Affiliated Hospital of Zhengzhou University , Zhengzhou, China
| | - Xinwei Han
- 1 Department of Interventional Radiology, The First Affiliated Hospital of Zhengzhou University , Zhengzhou, China
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Angioplasty for Budd-Chiari Syndrome in a Child with 26-Year Follow-up. Ann Vasc Surg 2018; 51:328.e7-328.e12. [PMID: 29777844 DOI: 10.1016/j.avsg.2018.03.004] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/28/2017] [Revised: 02/10/2018] [Accepted: 03/05/2018] [Indexed: 11/23/2022]
Abstract
Budd-Chiari syndrome (BCS) is a rare but life-threatening disease. If not treated promptly, it is almost always lethal with progressive liver failure, severe nutritional depletion, and renal failure at the late stage. We report the successful treatment of a 5-year-old boy with BCS due to a stenosis of the inferior vena cava (IVC) with a 26-year follow-up. We performed a percutaneous transluminal angioplasty, which resulted in a great improvement of BCS and the disappearance of ascites. Restenosis occurred at 25 years after the initial angioplasty, for which balloon angioplasty was repeatedly performed. The IVC was patent at 1-year follow-up after the second angioplasty.
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Abstract
Acute liver failure (ALF) is a rare but highly fatal condition. The most common causes include drug-induced and viral hepatitis, but other less common etiologies, especially autoimmune hepatitis, Budd-Chiari syndrome, and Wilson disease, need to be considered. Because diagnosis is frequently tied to potential for reversibility of ALF and prognosis, early identification in a timely manner is crucial. Other causes of ALF are more easily recognizable based on specific circumstances, such as ALF in pregnancy or ischemic hepatitis. Ultimately, maintaining a wide differential diagnosis in patients with ALF is essential to identifying the proper treatment and prognosis.
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Affiliation(s)
- Russell Rosenblatt
- Division of Gastroenterology and Hepatology, Weill Cornell Medical College, 1305 York Avenue, 4th Floor, New York, NY 10021, USA
| | - Robert S Brown
- Division of Gastroenterology and Hepatology, Weill Cornell Medical College, 1305 York Avenue, 4th Floor, New York, NY 10021, USA.
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