Portal Vein Thrombosis Xingshun Qi Weifen Xie Editors 123 Portal Vein Thrombosis Xingshun Qi • Weifen Xie Editors Portal Vein Thrombosis Editors Xingshun Qi Weifen Xie Department of Gastroenterology Department of Gastroenterology General Hospital of Northern Changzheng Hospital Theater Command Second Military Medical University Shenyang Shanghai China China ISBN 978-981-33-6537-7 ISBN 978-981-33-6538-4 (eBook) https://doi.org/10.1007/978-981-33-6538-4 © The Editor(s) (if applicable) and The Author(s), under exclusive license to Springer Nature Singapore Pte Ltd. 2021 This work is subject to copyright. All rights are solely and exclusively licensed by the Publisher, whether the whole or part of the material is concerned, specifically the rights of translation, reprinting, reuse of illustrations, recitation, broadcasting, reproduction on microfilms or in any other physical way, and transmission or information storage and retrieval, electronic adaptation, computer software, or by similar or dissimilar methodology now known or hereafter developed. The use of general descriptive names, registered names, trademarks, service marks, etc. in this publication does not imply, even in the absence of a specific statement, that such names are exempt from the relevant protective laws and regulations and therefore free for general use. The publisher, the authors and the editors are safe to assume that the advice and information in this book are believed to be true and accurate at the date of publication. Neither the publisher nor the authors or the editors give a warranty, expressed or implied, with respect to the material contained herein or for any errors or omissions that may have been made. The publisher remains neutral with regard to jurisdictional claims in published maps and institutional affiliations. This Springer imprint is published by the registered company Springer Nature Singapore Pte Ltd. The registered company address is: 152 Beach Road, #21-01/04 Gateway East, Singapore 189721, Singapore Foreword This book is well appreciated because it calls to mind a rare disease which is often overlooked or diagnosed late, the main reason for limited efficacy of treatment and impaired prognosis. In 1856, Rudolf Virchow (1821–1902) first described three cat- egories of factors, known as Virchow’s triad, which are thought to contribute to thrombosis: stasis, vessel wall injury, and hypercoagulability [1]. Stasis is the lead- ing cause of portal vein thrombosis in patients with advanced liver disease. This complication is not uncommon but has probably little impact on outcomes. In con- trast, non-cirrhotic portal vein thrombosis is caused by the categories of vessel wall injury mostly due to local factors (e.g., surgery, inflammation, and trauma) and hypercoagulability. Since Virchow, the knowledge of thrombogenesis has increased dramatically. Unfortunately, treatment of portal vein thrombosis is still unsatisfac- tory, and restitutio ad integrum remains an exception. v vi Foreword This book, written by experts, covers the most important topics of portal vein thrombosis. It is an outstanding achievement which will become an essential con- stituent for clinicians. Congratulations to the editors and the authors. Martin Rössle Department Innere Medizin, Klinik für Innere Medizin II, Gastroenterologie, Hepatologie Endokrinologie und nfektiologie Universitätsklinikum Freiburg Medizinische Fakultät Albert-Ludwigs-Universität Freiburg Freiburg, Germany Reference 1. Virchow, R. (1856). “Thrombose und Embolie. Gefässentzündung und septische Infektion”. Gesammelte Abhandlungen zur wissenschaftlichen Medicin (in German). Frankfurt am Main: Von Meidinger & Sohn. pp. 219–732. Matzdorff AC, Bell WR (1998). Thrombosis and embolie (1846–1856). Canton, Massachusetts: Science History Publications. ISBN 0- 88135- 113- X. Preface Portal vein thrombosis has been a major research interest for me since the initiation of my scientific activities in September 2008. Indeed, my first publication is a correspondence discussing the role of medical treatment and transjugular intrahepatic portosystemic shunt for portal vein thrombosis in liver cirrhosis with variceal bleeding (Hepatology. 2009). Subsequently, based on the medical records provided by Prof. Guohong Han and Prof. Daiming Fan on the Xijing Hospital of Digestive Diseases of the Fourth Military Medical University, my first original paper had been completed, which was a retrospective case series exploring the outcomes of transjugular intrahepatic portosystemic shunt for portal vein thrombosis in liver cirrhosis with variceal bleeding (J Hepatol. 2009), despite being a second co-first author for this paper. At that time, I was very enthusiastic about extensively reading the literature regarding management of portal vein thrombosis, and designed my first randomized controlled trial comparing the medical treatment versus transjugular intrahepatic portosystemic shunt for prevention of variceal bleeding in liver cirrhosis with portal vein thrombosis (BMJ Open. 2010). As known, such a time-consuming and tremendous work has been published (Gut. 2019), although I am a second co-author. Except for transjugular intrahepatic portosystemic shunt for portal vein thrombosis, my researches also involve the risk factors (natural antico- agulants, coagulation factors, fibrinolysis markers, and acquired thrombophilia), outcomes (risk of bleeding and death), and treatment (wait-and-see strategy and preventive and therapeutic anticoagulation) of portal vein thrombosis. Among them, two review papers, which provided more comprehensive insights on the management of portal vein thrombosis, should be particularly emphasized (Nature Rev Gastro Hepatol. 2014, BMC Med. 2018). Certainly, it should be acknowledged that these works could not be optimized without the guidance of many famous experts, includ- ing Prof. Valerio De Stefano, Prof. Dominique Valla, Prof. Eric Yoshida, Prof. Nahum Mendez-Sanchez, Prof. Andrea Mancuso, Prof. Ankur Arora, Prof. Fernando Romeiro, Prof. Frank Tacke, Prof. Aurélie Plessier, Prof. Massimo Primignani, Prof. Carlos Noronha Ferreira, Prof. Cyriac Abby Philips, Prof. Rolf Teschke, and Prof. Saurabh Chawla. On the basis of these accomplishments, Prof. Weifen Xie, who is the head of the Hepatobiliary Disease Study Group of the Chinese Society of Gastroenterology and the co-editor of this book, appointed me to write the Chinese-language consensus for management of portal vein thrombosis in liver cirrhosis in June 2019. I vii viii Preface completed the first draft in September 2019. Since then, this consensus has been further improved through several rounds of face-to-face or online discussions with the members of the Hepatobiliary Disease Study Group until its publication in November 2020. During this period, Prof. Weifen Xie and I initiated the current book project Portal Vein Thrombosis with the support of Springer. Dozens of specialists have been invited to overview the current status and future directions in this field. Shenyang, China Xingshun Qi December 1, 2020 Acknowledgments This book project cannot be completed without the contributions from so many famous experts. As known, all of them have made outstanding contributions to the management of portal vein thrombosis. Some landmark papers written by them should never be neglected, such as a randomized controlled trial regarding anticoagulation for prevention of portal vein thrombosis in liver cirrhosis by Prof. Villa (Gastroenterology. 2012), an excellent review paper regarding portal vein thrombosis, cirrhosis, and liver transplantation by Prof. Durand (J Hepatol. 2012), a large-scale longitudinal study regarding natural history of portal vein thrombosis in liver cirrhosis by Dr. Nery (Hepatology. 2015), a cohort study regarding natural history and management of esophagogastric varices in chronic non-cirrhotic and non-tumoral portal vein thrombosis by Dr. Noronha Ferreira (Hepatology. 2016), and a very comprehensive review paper regarding management of portal vein thrombosis by Dr. Intagliata (Gastroenterology. 2019). Besides, their enthusiastic participation in this book project, especially during the COVID-19 pandemic, should be respected and appreciated. In order to smooth the road to publish this book project, the Springer staff, mainly including Miss Kripa Guruprasad, Miss Xianxian Sun, and Mr. James Bin Hu, gave their assistance in designing the book protocol, communicating with chapter authors, giving comments on the chapters, and formatting and publishing this book. Notably, Kripa, who is also the coordinator of my first Springer book Budd-Chiari Syndrome, is absolutely impressive due to her positive attitude and timely communications. Herein, I would like to pay a heartfelt tribute to her. From my side, such a conscientious staff should be praised and cherished by any publisher. Finally, as always, I must be thankful for the lifelong support of my wife, Jun Liu, and my family. Shenyang, China Xingshun Qi December 1, 2020 ix Contents 1 Anatomy of Portal Vein System . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1 Ruchira Das, James Chambers, and Ankur Arora 2 Epidemiology of Portal Vein Thrombosis . . . . . . . . . . . . . . . . . . . . . . . . 23 Filipe Gaio Nery 3 Risk Factors for Portal Vein Thrombosis . . . . . . . . . . . . . . . . . . . . . . . . 29 Kamran B. Lankarani 4 Imaging of Portal Vein Thrombosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 39 Kumble Seetharama Madhusudhan 5 Classification of Non-malignant Portal Vein Thrombosis . . . . . . . . . . . 65 Matthew J. Stotts and Nicolas M. Intagliata 6 Impact of Non-malignant Portal Vein Thrombosis on Outcomes of Liver Cirrhosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 77 Hajime Takatori, Takehiro Hayashi, Hidetoshi Nakagawa, and Shuichi Kaneko 7 Anticoagulation for Nontumoral Portal Vein Thrombosis . . . . . . . . . . 89 Carlos Noronha Ferreira 8 Thrombolysis for Non-malignant Portal Vein Thrombosis . . . . . . . . . . 103 Tuba Baydas, Necat Irem Abdulhayoglu, Emine Mutlu, Leila Kianifard, and Metin Basaranoglu 9 Transjugular Intrahepatic Portosystemic Shunt for Non-malignant Portal Vein Thrombosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 117 Anshuman Elhence, Shivanand Ramachandra Gamanagatti, and Shalimar 10 Liver Transplantation in the Setting of Non-malignant Portal Vein Thrombosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 131 François Durand, Safi Dokmak, Olivier Roux, and Claire Francoz xi