Diagnostic Methods for Cirrhosis and Portal Hypertension

337 Pages·2018·9.93 MB·English

by  Annalisa Berzigotti, Jaime Bosch

Diagnostic Methods
for Cirrhosis and
Portal Hypertension
Annalisa Berzigotti
Jaime Bosch
Editors
123
Diagnostic Methods for Cirrhosis
and Portal Hypertension
Annalisa Berzigotti • Jaime Bosch
Editors
Diagnostic Methods
for Cirrhosis and Portal
Hypertension
Editors
Annalisa Berzigotti Jaime Bosch
Inselspital, University of Bern Hospital Clinic
UVCM, DMLL, Hepatology Inselspital, Hepatic Hemodynamic Laboratory Hospital
University of Bern Clinic
Berne Barcelona
Switzerland Spain
ISBN 978-3-319-72627-4 ISBN 978-3-319-72628-1 (eBook)
https://doi.org/10.1007/978-3-319-72628-1
Library of Congress Control Number: 2018935245
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Preface
The past years have witnessed an enormous advancement in all areas of hepatology,
from molecular pathophysiology to diagnostic techniques and therapy, to the point
that we have now effective therapies for most liver diseases and noninvasive diag-
nostic tests are creating new gold standards for diagnosis that before required diffi-
cult and demanding invasive techniques.
These changes are paramount most especially in the diagnosis of cirrhosis and
portal hypertension. We come from an era when cirrhosis was diagnosed based on
liver biopsy to one when pathologists prefer to use the term “advanced chronic liver
disease” instead of “cirrhosis,” thus underlining the dynamic nature of disease pro-
cess. In this scenario, different disease stages are better defined on the basis of clini-
cal, imaging, and hemodynamic characteristics than by the biopsy findings that
correlate poorly with patient outcome. This is well illustrated by the fact that with
similar liver biopsy findings, patient prognosis can be very good (as exemplified by
the compensated patient without portal hypertension) or extremely poor (as in the
case of the decompensated patient with multiple complications).
The above considerations emphasize one of the major requirements in modern
medicine, that is, diagnostic tests should be able to inform on prognosis, therefore
providing the basis for both risk stratification at the time of diagnosis and personal-
izing treatment.
This approach has been used to devise this book that reviews the more recent
advances in the diagnostic methods for cirrhosis, the main complication of portal
hypertension, and non-cirrhotic causes of portal hypertension. We are making spe-
cial emphasis on new noninvasive methods and on the use of these tests in the dif-
ferent stages of cirrhosis and different complications of portal hypertension. After
being part of the standard of care for the adult population, noninvasive diagnostic
methods are increasingly used in the pediatric population with cirrhosis and/or por-
tal hypertension, and this aspect as well as the specificities of diagnostics in Western
and Eastern countries is taken into account.
Our aim is to offer to the general hepatologists and hepatologists in training the
current state of the art regarding the many different techniques available and under
development for clinical decision making. We hope the reader will find in the differ-
ent chapters of this book—all written by well-known opinion leaders in their
fields—a concise but comprehensive and updated, clinically focused guide to
answer difficult questions, such as when to think about rare causes of non-cirrhotic
v
vi Preface
portal hypertension (e.g., long-lasting porto-sinusoidal disease), when to start endo-
scopic surveillance in a given patient, or when to shift from a completely noninva-
sive assessment to an invasive measurement of HVPG in different clinical scenarios
(e.g., sustained virological response after treatment with direct-acting antivirals).
We would like to acknowledge the commitment and efforts of all the authors
from different disciplines (hepatology, endoscopy, radiology, pathology) and from
the different areas of the world that have contributed to this book. They provide an
outstanding example of what interdisciplinary collaboration can bring into the com-
plex field of hepatology.
We hope that this book will be helpful for hepatologists and physicians interested
in liver diseases in order to select the most appropriate diagnostic methods for their
patients with cirrhosis and/or portal hypertension.
Berne, Switzerland Annalisa Berzigotti
Barcelona, Spain Jaime Bosch
Contents
1 Cirrhosis and Portal Hypertension: Staging and Prognosis . . . . . . . . . . . 1
Guadalupe Garcia-Tsao
Part I Gold-Standard Invasive Diagnostic Methods
2 Liver Biopsy Diagnosis of Cirrhosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 17
Zachary D. Goodman
3 Hepatic Venous Pressure Measurement and Other
Diagnostic Hepatic Hemodynamic Techniques . . . . . . . . . . . . . . . . . . . . 33
Annalisa Berzigotti and Jaime Bosch
4 Endoscopy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 49
Alessandra Dell’Era and Roberto de Franchis
Part II Non-Invasive Diagnostic Methods
5 Non-invasive Serum Markers of Fibrosis . . . . . . . . . . . . . . . . . . . . . . . . . 63
Thomas Pembroke and Giada Sebastiani
6 Ultrasound Elastography: General and Technical Overview . . . . . . . . . 83
Veronica Salvatore and Fabio Piscaglia
7 Liver Stiffness by Ultrasound Elastography . . . . . . . . . . . . . . . . . . . . . . 95
Laurent Castera
8 Spleen Stiffness by Ultrasound Elastography . . . . . . . . . . . . . . . . . . . . 113
Antonio Colecchia, Federico Ravaioli, Giovanni Marasco,
and Davide Festi
9 Diagnostic Methods for Cirrhosis and Portal Hypertension:
Imaging: Ultrasound and Doppler Ultrasonography . . . . . . . . . . . . . . 139
Soon Koo Baik and Moon Young Kim
10 Contrast-Enhanced Ultrasonography for the Diagnosis
of Portal Hypertension . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 149
Hitoshi Maruyama and Naoya Kato
vii
viii Contents
11 Subharmonic Aided Pressure Estimation (SHAPE) . . . . . . . . . . . . . . . 159
Ipshita Gupta, John R. Eisenbrey, and Flemming Forsberg
12 Endoscopic Ultrasound and Portal Hypertension . . . . . . . . . . . . . . . . . 169
Oriol Sendino and Angels Ginès
13 Computed Tomography . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 183
Maxime Ronot, Romain Pommier, Paul Calame, Yvonne Purcell,
and Valérie Vilgrain
14 Magnetic Resonance Imaging Methods for Assessing Cirrhosis
and Portal Hypertension . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 211
Naaventhan Palaniyappan, Indra Neil Guha,
and Guruprasad Padur Aithal
15 Magnetic Resonance Elastography of the Liver . . . . . . . . . . . . . . . . . . . 225
Sumeet K. Asrani and Jayant A. Talwalkar
Part III Invasive and Non-Invasive Diagnostic Methods
in Special Conditions
16 Budd-Chiari Syndrome: The Western Perspective . . . . . . . . . . . . . . . . 241
Aurélie Plessier, Audrey Payancé, and Dominique Valla
17 Budd-Chiari Syndrome and Inferior Vena Cava Obstruction:
The Asian Perspective . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 257
Qiuhe Wang and Guohong Han
18 Extrahepatic Portal Vein Obstruction: Asian
and Global Perspective . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 271
Rakhi Maiwall and Shiv Kumar Sarin
19 Idiopathic Portal Hypertension (Portosinusoidal Disease) . . . . . . . . . . 301
Virginia Hernández-Gea, Ernest Belmonte, Angeles García- Criado,
and Juan Carlos García-Pagán
20 Hereditary Hemorrhagic Telangiectasia
(Osler-Weber-Rendu Syndrome) and Liver
Vascular Malformations . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 309
Elisabetta Buscarini and Guido Manfredi
21 Diagnostic Methods of Cirrhosis and Portal Hypertension:
Specifics of the Pediatric Population . . . . . . . . . . . . . . . . . . . . . . . . . . . . 325
Daniel H. Leung, Milton J. Finegold, and Benjamin L. Shneider
Cirrhosis and Portal Hypertension: 1
Staging and Prognosis
Guadalupe Garcia-Tsao
Cirrhosis is considered the end-stage of chronic liver disease of any etiology with a
broad spectrum of clinical manifestations, from an entirely asymptomatic stage to a
stage characterized by multiorgan failure. The clinical manifestations of cirrhosis
are due mostly to portal hypertension and its hemodynamic consequences and/or to
liver insufficiency.
Numerous prognostic studies over the years have indicated that the natural his-
tory of cirrhosis does not represent, as in most disease states, a continuum of a sin-
gle entity but that cirrhosis is an entity that progresses across different stages, each
with different prognosis, predictors of death and pathophysiological mechanisms.
A systematic review of 116 prognostic studies in cirrhosis had already demon-
strated that cirrhosis is a heterogeneous disease with median survival times that
ranged widely between 1–186 months [1]. This review also showed that, when
patients are classified into two stages depending on the presence or absence of clini-
cally evident liver-related events (specifically ascites, variceal hemorrhage, hepatic
encephalopathy [HE] and/or jaundice), 1-year survival in patients without these
events (compensated patients) is 95% (interquartile range 91–98%) while in those
with any of these events (decompensated patients), it is 61% (interquartile range
56–70%) [1]. This systematic review also revealed that predictors of death are dif-
ferent in patients with compensated compared with those with decompensated
cirrhosis [1].
From another perspective, analysis of individual patient data from two prospec-
tive Italian cohort studies including over 1600 patients demonstrated a median
survival of greater than 12 years in patients with compensated cirrhosis, while
patients with decompensated cirrhosis have a median survival of 1.8 years [1].
G. Garcia-Tsao, M.D.
Section of Digestive Diseases, Yale University School of Medicine, New Haven, CT, USA
Digestive Diseases Section, VA-CT Healthcare System, West Haven, CT, USA
e-mail: [email protected]
© Springer International Publishing AG, part of Springer Nature 2018 1
A. Berzigotti, J. Bosch (eds.), Diagnostic Methods for Cirrhosis and Portal
Hypertension, https://doi.org/10.1007/978-3-319-72628-1_1
2 G. Garcia-Tsao
These results have been confirmed in a recent prospective study that analyzed a
concurrent cohort of patients with cirrhosis (both compensated and decompensated)
and showed that decompensation was, by far, the strongest predictor of death in cir-
rhosis. Furthermore, both stages have different predictors of death (age for compen-
sated; Model for End-Stage Liver Disease [MELD] score for decompensated), and
predictors that were common to both stages (albumin, platelet count) have different
strengths of association [2].
Therefore, compensated cirrhosis and decompensated cirrhosis should be con-
sidered as two different disease entities, with different probabilities of death and
different predictors of death and should be described, studied and managed
separately.
Within these two main stages of cirrhosis, recent advances have provided further
granularity that has allowed sub-staging of compensated and decompensated cir-
rhosis not only regarding prognosis but also regarding the predominant pathogenic
mechanisms (Fig. 1.1). Before detailing this sub-staging, it is important to briefly
summarize the pathophysiology of the complications of cirrhosis.
Stages of cirrhosis and pathogenic mechanisms
Histological F1-F3 F4 (Cirrhosis)
Clinical Non-cirrhotic Compensated Compensated Decomp Further
Dec Æ AoCLF
None (no None (varices Ascites, Rec VH, RA,
Symptoms None
varices) present) VH, HE HRS Æ MOF
Portal hyper- >6 >10 >12 >20
tension
(HVPG) Inflammation
Hyperdynamic circulation
Liver insufficiency
Biological Fibrogenesis Scar and Thick (acellular)
Insoluble scar Insoluble scar
and neovasc- X-linking scar and nodules
ularization
Fig. 1.1 Stages of cirrhosis and pathogenic mechanisms. The figure shows the different stages of
chronic liver disease from a non-cirrhotic stage to a stage of further (late) decompensation with the
severity of portal hypertension and the different pathogenic mechanisms at each stage, as well as
the biology of fibrogenesis at each stage. VH variceal hemorrhage, HE hepatic encephalopathy,
Rec recurrent, RA refractory ascites, HRS hepatorenal syndrome, MOF multiorgan failure, HVPG
hepatic venous pressure gradient, an indirect measure of portal hypertension