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FIFTH EDITION
EDITORS
CATHERINE M. OTTO, MD
Professor of Medicine
J. Ward Kennedy-Hamilton Endowed Chair in Cardiology
Department of Medicine
Division of Cardiology
University of Washington School of Medicine
Seattle, Washington
No part of this publication may be reproduced or transmitted in any form or by any means, electronic or
mechanical, including photocopying, recording, or any information storage and retrieval system, without
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Publisher’s permissions policies and our arrangements with organizations such as the Copyright Clearance
Center and the Copyright Licensing Agency, can be found at our website: www.elsevier.com/permissions.
This book and the individual contributions contained in it are protected under copyright by the Publisher
(other than as may be noted herein).
Notice
Practitioners and researchers must always rely on their own experience and knowledge in evaluating
and using any information, methods, compounds or experiments described herein. Because of rapid
advances in the medical sciences, in particular, independent verification of diagnoses and drug dosages
should be made. To the fullest extent of the law, no responsibility is assumed by Elsevier, authors, editors
or contributors for any injury and/or damage to persons or property as a matter of products liability,
negligence or otherwise, or from any use or operation of any methods, products, instructions, or ideas
contained in the material herein.
Previous editions copyrighted 2014, 2009, 2004, 1999 by Saunders, an imprint of Elsevier Inc.
Printed in Canada.
v
vi CONTRIBUTORS
Valvular heart disease remains an important clinical problem. An esti- for open-heart surgery in the care of these patients. Increasingly, the “Heart
mated 17 million persons worldwide are affected by this condition, Team,” consisting of a cardiologist, a physician experienced in multi-
with about 20,000 deaths and 100,000 hospitalizations each year in the modality imaging, an interventional cardiologist, and a cardiac surgeon,
United States alone. Recent years have witnessed numerous important should be available to manage patients with valvular heart disease.
developments in the epidemiology, assessment, and management of Dr. Otto and Dr. Bonow, the editors of Valvular Heart Disease, are
valvular heart disease. Despite the virtual disappearance of rheumatic world leaders in this field. In this book, they cover systematically and
fever in high-income nations, the prevalence of valvular heart disease in depth the pathogenesis, pathophysiology, clinical findings, imaging,
in these regions is rising steadily because of the increase in valvular natural history, and therapeutic options. They also describe challenges
diseases that accompany the progressive aging of the population. involved in the care of patients who have undergone successful valve
The frequency of valvular heart disease in low-income countries is also replacement.
rising because the incidence of new cases of rheumatic heart disease This fifth edition of Valvular Heart Disease builds on the previous
has not (yet) fallen, while the number of older people and the accom- editions and has become the leading textbook in the field. All chapters
panying age-related valve diseases increase simultaneously. have been thoroughly revised, and four new chapters have been added.
In the assessment of patients with valvular heart disease, the clinical There are eighteen new authors, each an authority in the area that they
history remains of paramount importance and must not be neglected discuss. We congratulate and thank the editors and authors for their
because the assessment of disability and estimate of the clinical trajectory important contributions and welcome this outstanding new edition to
has critical importance in the decision to undertake invasive interven- our growing family of companions to Braunwald’s Heart Disease.
tions. Noninvasive imaging techniques, including three-dimensional
echocardiography, cardiac magnetic resonance imaging, and computed Eugene Braunwald, MD
tomography, and the constant improvements in these technologies Peter Libby, MD
provide rich anatomic and functional information in these patients. Douglas L. Mann, MD
Catheter-based therapies represent the most important advances in Gordon F. Tomaselli, MD
treatment of valvular conditions. Indeed, a new subspecialty, structural Deepak Bhatt, MD, MPH
heart disease, has emerged, largely as a consequence of catheter-based Scott Solomon, MD
valvular therapy. Despite these advances, there remains an important role
vii
P R E FAC E
The scientific underpinnings, clinical evaluation, and treatment of val- This edition of Valvular Heart Disease includes more than 750 new
vular heart disease continue to advance at a startling rate. In the context and updated illustrations of anatomy and physiology, methodology, flow
of this rapidly expanding knowledge base, we are pleased to present the charts, and clinical examples, with additional figures and video content
fifth edition of Valvular Heart Disease: A Companion to Braunwald’s available in the online version. The chapters also include the current
Heart Disease, which we believe will be a valuable, authoritative re- guideline recommendations of the American College of Cardiology/
source for cardiology and surgery practitioners, physicians-in-training, American Heart Association and the European Society of Cardiology/
and students of all levels. European Association of Cardio-Thoracic Surgery, with recommenda-
In keeping with the previous editions of Valvular Heart Disease, the tions summarized in convenient tables in each chapter.
fifth edition covers the breadth of the field, providing the basics of We are indebted to all of our authors for their commitment of
diagnosis and treatment while highlighting the new exciting advances considerable time and effort to ensure the high quality and authori-
and their potential to transform the outcome of patients with heart tative nature of this edition of Valvular Heart Disease. We are also
valve disorders. With the help of our internationally recognized au- delighted that this book remains a member of the growing family of
thors from the United States, Canada, and Europe, we have thoroughly companion texts to Braunwald’s Heart Disease: A Textbook of Cardio-
revised this edition to keep the content vibrant, stimulating, and up to vascular Medicine. As a member of the Braunwald’s companion
date. We have added new authors for about 50% of the 28 chapters, all series, the book is available online on the companion Expert Consult
highly accomplished and recognized in their respective disciplines. website, and the electronic version of this work includes a greater
Chapters have been reorganized to closely link imaging and interven- numbers of figures and tables than the print version can accommo-
tional approaches for aortic and mitral valve disease. date. Figures and tables can be downloaded directly from the website
The book starts with the foundation for understanding valvular for electronic slide presentations. In addition, there is a large portfo-
heart disease, including epidemiology, 3D anatomy, molecular mecha- lio of video content that supplements the print content of many of
nisms of disease, genetic and clinical risk factors, basic principles of our chapters.
medical therapy, and the approach to estimating procedural risk in an Despite the advances in diagnosis and treatments (both surgical
individual patient. There are now several chapters on aortic valve disease and interventional), valvular heart disease remains a major cause of
with separate discussions of aortic stenosis, aortic regurgitation, and morbidity and mortality throughout the world. Rheumatic heart dis-
bicuspid valve disease, plus additional chapters detailing patient selec- ease remains a scourge in developing countries of the world, and
tion and procedural aspects for transcatheter and surgical aortic valve congenital forms of aortic and mitral valve disease create a steady
replacement. The next eight chapters cover different aspects of mitral stream of young and middle-aged adults with aortic stenosis, aortic
valve disease, including rheumatic mitral stenosis, primary mitral regur- regurgitation, and mitral regurgitation in both developed and develop-
gitation, secondary mitral regurgitation, transcatheter interventions, ing countries. The aging of the population around the world results in
and mitral valve surgery. Focused chapters discuss the role of imaging an increasing number of elderly patients who have degenerative forms
during transcatheter interventions and in patients undergoing surgical of aortic stenosis and mitral regurgitation and often present with age-
procedures. The final section of the book discusses diverse topics, related medical comorbidities that confound medical decision making.
including tricuspid valve disease, pulmonic valve disease, endocarditis, Unlike most other forms of cardiovascular disease, in which manage-
prosthetic valves, transcatheter intervention for prosthetic valve steno- ment decisions can be guided by the evidence base created by multiple
sis, and management of valvular heart disease during pregnancy. large-scale randomized controlled clinical trials, the evidence base in
Hot topics and key procedures covered in this book include the valve disease is limited by a dearth of clinical trials. In this field, more
timing and approach to transcatheter aortic valve replacement (TAVR), than any other, expert clinical judgment and experience are the corner-
transcatheter balloon mitral valvuloplasty, transcatheter repair for stones of rational decision making and optimal patient management.
primary and secondary mitral regurgitation, surgical mitral valve re- We believe that the collective knowledge, experience, and expert clinical
pair and replacement, interventions for tricuspid valve regurgitation, judgment of the accomplished authors of Valvular Heart Disease
transcatheter pulmonic valve implantation and valve-in-valve trans- will serve as an invaluable resource for all of us who are called upon to
catheter aortic valve replacement, management of patients with a provide care for our patients with these diseases.
prosthetic valve, advanced imaging of the aortic and mitral valves,
management of valve disease in pregnant women, and advances in Robert O. Bonow, MD, MS
diagnosis and treatment of endocarditis. Catherine M. Otto, MD
viii
AC K N OW L E D G M E N T S
Sincere thanks are due to the many individuals who helped make this book a reality. In particular, we would
like to express our deep appreciation to the distinguished chapter authors for their time and effort in pro-
viding excellent chapters. We also thank the publishing staff at Elsevier for their guidance and close working
relationship. Finally, most importantly, we would like to thank our families for their constant understand-
ing, encouragement, and support.
ix
CONTENTS
1 Epidemiology of Valvular Heart Disease, 1 14 Surgical Approach to Diseases of the Aortic Valve
John B. Chambers and Aortic Root, 267
S. Chris Malaisrie, Patrick M. McCarthy
2 Three-Dimensional Anatomy of the Aortic and Mitral
Valves, 22 15 Diagnostic Evaluation of Mitral Regurgitation, 289
Wendy Tsang, Benjamin H. Freed, Roberto M. Lang Akhil Narang, Jyothy Puthumana, James D. Thomas
3 Molecular Mechanisms of Calcific Aortic Valve 16 Rheumatic and Calcific Mitral Stenosis and Mitral
Disease, 43 Commissurotomy, 311
Russell J. Everett, David E. Newby, Marc R. Dweck Bernard Iung, Alec Vahanian
4 Clinical and Genetic Risk Factors for Calcific Valve 17 Mitral Valve Prolapse, 337
Disease, 66 David Messika-Zeitoun, Maurice Enriquez-Sarano
George Thanassoulis
18 Secondary (Functional) Mitral Regurgitation in Ischemic
5 Left Ventricular and Vascular Changes in Valvular Heart and Dilated Cardiomyopathy, 354
Disease, 79 Paul A. Grayburn
Brian R. Lindman
19 Surgical Mitral Valve Repair and Replacement, 370
6 Principles of Medical Therapy for Patients With Valvular Javier G. Castillo, David H. Adams
Heart Disease, 94 20 Transcatheter Mitral Valve Repair and Replacement, 390
John P. Erwin III, Catherine M. Otto
Howard C. Herrmann
7 Surgical and Procedural Risk Assessment of Patients 21 Imaging Guidance of Transcatheter Mitral Valve
With Valvular Heart Disease, 114 Procedures, 404
Mohanad Hamandi, Michael J. Mack
Ernesto E. Salcedo, Robert A. Quaife, John D. Carroll
8 Imaging the Aortic Valve, 124 22 Intraoperative Echocardiography for Mitral Valve
Rebecca T. Hahn, João L. Cavalcante
Surgery, 431
9 Aortic Stenosis: Clinical Presentation, Disease Stages, Donald C. Oxorn
and Timing of Intervention, 156 23 Diseases of the Tricuspid Valve, 451
Jason P. Linefsky, Catherine M. Otto
Grace Lin
10 Aortic Regurgitation: Clinical Presentation, Disease 24 Pulmonic Valve Disease in Adults, 471
Stages, and Management, 179 Yuli Y. Kim
Arturo Evangelista, Pilar Tornos, Robert O. Bonow
25 Infective Endocarditis, 496
11 The Bicuspid Aortic Valve and Associated Aortic Andrew Wang, Thomas Michael Bashore
Disease, 197
Alan C. Braverman, Andrew Cheng 26 Prosthetic Heart Valves, 521
Philippe Pibarot, Patrick T. O’Gara
12 Transcatheter Aortic Valve Replacement: Indications,
Procedure, and Outcomes, 223 27 Management of Bioprosthetic Valve Degeneration, 547
Amisha Patel, Susheel Kodali Danny Dvir
13 Imaging Assessment for Transcatheter Aortic Valve 28 Valvular Heart Disease in Pregnancy, 566
Replacement, 241 Karen K. Stout, Eric V. Krieger
James Lee, Paul Schoenhagen, Milind Desai
x
B R A U N WA L D ’ S H E A R T D I S E A S E
FA M I LY O F B O O K S
xi
xii BRAUNWALD’S HEART DISEASE FAMILY OF BOOKS
VALVULAR
HEART
DISEASE
A COMPANION TO BRAUNWALD’S HEART DISEASE
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1
Epidemiology of Valvular Heart Disease
John B. Chambers
CHAPTER OUTLINE
Incidence and Prevalence of Valve Disease, 3 Congenital Conditions, 12
Valve Disease in Industrially Underdeveloped Areas, 3 Systemic Inflammatory Conditions, 13
Incidence of Rheumatic Fever and Chronic Rheumatic Disease, 3 Carcinoid, Drugs, and Radiation, 13
Prevalence of Rheumatic Disease, 4 Carcinoid, 13
Valve Disease in Industrially Developed Areas, 7 Drugs, 13
Causes of Valve Disease, 7 Radiation, 14
Rheumatic Fever, 7 Disease by Valve Type, 14
Endomyocardial Fibrosis, 9 Aortic Stenosis and Regurgitation, 14
Calcific Aortic Stenosis, 9 Mitral Stenosis, 15
Mitral Prolapse, 10 Mitral Regurgitation, 16
Secondary Mitral Regurgitation, 11 Right-Sided Valve Disease, 16
Secondary Aortic Regurgitation Resulting from Aortic Dilation, 11 Tricuspid Valve Disease, 16
Secondary Tricuspid Regurgitation, 11 Pulmonary Valve Disease, 16
Infective Endocarditis, 11 Multiple Valve Disease, 17
Replacement Heart Valves, 12 Conclusions, 17
KEY POINTS
• Valve disease is globally common, affecting approximately 2.5% of • Drug-induced valve disease is increasing as a result of 5-HT2B
the population. receptor agonists.
• In industrially underdeveloped countries, rheumatic disease • Infective endocarditis is increasingly related to medical devices and
(RhD) is the most common cause. Endomyocardial fibrosis is intravenous drug use.
an underresearched disease common in equatorial Africa. • Failure of biological replacement valves is a major burden in all
• In industrially developed regions, diseases of old age predomi- regions of the world.
nate, particularly calcific aortic stenosis and secondary mitral • Substantial variation in access to health care exists in all countries,
regurgitation. including those that are industrially developed.
• In the United States, valve disease is most common among • The main global challenge is to prevent chronic RhD, which
the elderly, with a prevalence of 13% among those older than will require collaborations among social, political, and medical
75 years. programs.
Heart valve disease is the term for any deviation from normal valve Mozambique, New Caledonia, New Zealand, Nicaragua, Pakistan,
anatomy or function. This includes a wide spectrum of severity, from Samoa, South Africa, Tonga, Yemen),3 but most surveys of RhD are
mild mitral prolapse to a flail mitral leaflet or aortic sclerosis to critical of children and young adults and are restricted geographically and
aortic stenosis. The term does not describe a unitary diagnosis but by time. Countrywide estimates are usually extrapolated from these
instead a set of conditions affecting one or more of the four heart small studies to people of all ages and socioeconomic classes and
valves caused by genetic, environmental, or acquired pathologic pro- therefore remain uncertain. In the industrially developed world,
cesses that vary with geography and demography. there have been population surveys of all types of valve disease in
Our knowledge of the epidemiology of valve disease is incomplete. the United States4 and Norway5 and studies of the elderly in the
The global disease survey1,2 used 56,356 unique sources of data from United Kingdom.6 Otherwise, work has focused on aortic stenosis,7,8
195 countries to estimate incidence, prevalence, mortality, and disabil- mitral prolapse,9 or bicuspid aortic valve.10,11
ity rates arising from 328 diseases and injuries. Rheumatic disease There are other limitations to our knowledge. Estimates of the
(RhD) and infective endocarditis (IE) were the only valve diseases prevalence of valve disease vary with the method of diagnosis, which
recorded. for RhD was originally clinical but more recently includes echocar-
National or large-scale screening programs for RhD exist in diography, which is approximately 10 times more sensitive than
some countries (e.g., Australia, Cambodia, Fiji, India, Laos, Mali, auscultation.12 The prevalence and types of valve disease differ whether
1
2 CHAPTER 1 Epidemiology of Valvular Heart Disease
TABLE 1.1 Principal Causes of Valve Disease with Prevalence or Incidence When Known.
Prevalence in Economically Prevalence in Economically
Poorer Countries Richer Countries References
Causes
Chronic rheumatic heart disease 0.9%–4.0% 0.3% 17–22
Endomyocardial fibrosis 20%a 23
Calcific aortic stenosis 0.4%b 3
Bicuspid aortic valve 0.5%–0.8% b
9,10
Mitral regurgitation 1.7% 3
Mitral prolapse 2%–3% 8,23
Prolapse and mitral regurgitation 0.2%–0.3% 20,21
Secondary mitral regurgitation 1.4%c
Failing biological replacement valves
Aortic dilation
Inflammatory conditions (e.g., SLE, rheuma-
toid arthritis)
Drugs, carcinoid, radiation
assessment occurs by community screening,4,6,8 open access echocar- aneurysms are the third most common cause of mitral regurgitation
diography,13,14 or hospital services.15 For example, the frequency of after RhD and mitral prolapse in sub-Saharan Africa.
bicuspid aortic valves is 0.5% to 0.8% in community surveys,10,11 2% The second pattern occurs in economically richer areas, where the
in a postmortem study,16 and approximately 67% among patients in epidemiology of valve disease changed (Box 1.1)27 in the second half of
their 40s who have aortic valve replacement.17 the 20th century, with a decrease in the incidence of rheumatic fever as
The limitations make it difficult to talk about the epidemiology a result of better housing to reduce overcrowding, better nutrition,21
of valve disease in general, but there are two broad patterns and better health care. There also was a change in streptococcal sero-
(Table 1.1).3,8–10,17–26 The first occurs in economically disadvantaged types. Rheumatogenic serotypes were found in 50% of children with
areas of the world characterized by poor housing, sanitation, and nu- pharyngitis in Chicago between 1961 and 1968, compared with 11%
trition and with inadequate access to medical care. These areas include between 2000 and 2004.22 Calcific degeneration, secondary mitral re-
parts of Africa, the Indian subcontinent, the Middle East, and South gurgitation, and aortic dilation leading to aortic regurgitation pre-
America. dominantly affect older people; mitral prolapse and bicuspid aortic
Valve disease is predominantly caused by rheumatic fever (RhF) valve are found in younger people.
and chronic RhD. Patients are typically young, and the morbidity and New types of valve disease are increasing in frequency as a result of
mortality rates are high. Acute RhF occurs mainly between the ages of drugs or radiation exposure. IE has a different pattern from that in the
5 and 15 years, and heart failure, usually as a result of acute rheumatic developing world. With older populations, more Staphylococcus aureus
mitral regurgitation, occurs most commonly within a year of the acute and more cases as a result of health care, mainly hemodialysis and
episode.18,19 In Ethiopia, 20% with RhD die before the age of 5 years implanted electrical devices.28 Tricuspid regurgitation as a result of
and 80% before 25 years.20 Endocarditis, stroke, and heart failure are endocardial pacing systems and defibrillators is also increasingly
common sequelae. Mitral prolapse occurs within the differential diag- seen.23
nosis of chronic RhD diagnosed by echocardiography. There are also a These two patterns are usually taken to apply broadly to the indus-
number of geographically localized diseases. Endomyocardial fibrosis trially underdeveloped and developed regions of the world. However,
(EMF) is second in incidence to RhD in Equatorial Africa. Subvalvar this is an oversimplification because pockets of poverty exist in the
CHAPTER 1 Epidemiology of Valvular Heart Disease 3
Prevalence (%)
prophylaxis)
Fall in rheumatogenic serotypesa 50
• Increasing age expectancy, leading to more degenerative disease
• New types of valve disease requiring treatment (e.g., radiation, drugs) 40
• More health care–related endocarditis
• Biological replacement valves used in younger patients resulting in more 30
reoperations, including valve-in-valve transcatheter procedures
• More mild disease as a result of increased use of echocardiography 20
a
There were rheumatogenic serotypes in 50% of children with pharyn-
10
gitis in Chicago between 1961 and 1968, compared with 11% between
2000 and 2004.27.
0
65–69 70–74 75–79 80–84 85+
industrially developed world (e.g., indigenous populations of Australia Age (years)
and New Zealand). Conversely, richer people in the disadvantaged ar-
Fig. 1.1 Newly Detected and Previously Known Valvular Heart Dis-
eas of the world are more likely to reflect patterns of valve disease ease (VHD) by Age From the OxVALVE Study. Subjects 65 years of
usually seen in the industrially developed areas. Many countries, such age or older without known valve disease and who were registered with
as India and China, are in the process of rapid development and have one of five primary care medical centers were invited to participate. For
a falling incidence of RhF while retaining a higher prevalence of the first 2500 enrolled participants, the mean age (SD) was 73 (6) years,
chronic rheumatic heart disease among the more elderly population 51.5% were female, and 99% were white. The plot illustrates that a
than seen in the United States or Western Europe.18 substantial burden of valve disease was not previously detected. (From
d’Arcy JL, Coffey S, Loudon MA, et al. Large-scale community echocar-
diographic screening reveals a major burden of undiagnosed valvular
INCIDENCE AND PREVALENCE OF VALVE DISEASE heart disease in older people: the OxVALVE Population Cohort Study. Eur
Heart J 2016;37:3515-3522.)
Valve disease remains underdetected6,12 (Fig. 1.1) in developed and
developing countries. In the developing world, the detection rate is
approximately 10% using clinical examination alone without system-
atic echocardiographic screening.12,24 In the United States,4 the preva- physicians. The elderly are particularly disadvantaged, and about one
lence of moderate or severe valve disease is 1.8% when estimated from third with aortic stenosis are denied surgery inappropriately.34 Devel-
echocardiograms performed as clinically indicated compared with an oping a transcatheter program leads to an increase in conventional
age-corrected population prevalence of 2.5%. In the U.K. Oxford Valve surgical rates35 because clinically inappropriate perceptual barriers to
(OxVALVE) study,6 the prevalence of previously undiagnosed moder- referral are lifted.
ate or severe valve disease was 6.4% for people aged 65 years of age or
older and was already identified in another 4.9% (see Fig. 1.1). Valve Disease in Industrially Underdeveloped Areas
A survey of open access studies found that significant valve disease The global burden of RhD was assessed in a World Health Organiza-
was suspected from a murmur in 127 patients but was unsuspected in tion (WHO) report in 2005,36 two reports in 2011, 18,37 and the global
177 cases.14 Postmortem examinations show that only about 50% of burden of disease study most recently updated in 2017.1,2 All but the
aortic stenosis cases may be diagnosed before death,25 and undetected first of these used echocardiographic diagnosis, but the criteria for
or underinvestigated aortic stenosis is an important cause of periop- echocardiographic diagnosis have changed.
erative and maternal deaths.26 Detection rates may increase in the fu-
ture with improved access to point-of-care echocardiography,29,30 Incidence of Rheumatic Fever and Chronic Rheumatic Disease
perhaps aided by improved analysis of murmurs using artificial intel- The annual incidence of all first or subsequent attacks of acute RhF
ligence linked with mobile phone technology. worldwide is estimated at 471,000 cases38 based on a meta-analysis of
Access to valve surgery is restricted. In India and Africa, valve op- regional reports. The median annual incidence ranges from 10 to 374
erations are performed at a rate of 1.8 per 100,000 people each year.31 cases per 100,000 people.38,39 The incidence is decreasing in all WHO
This compares with 28 operations per 100,000 people in the Nether- regions for which data exist, except in the Americas, where the inci-
lands32 and 122 operations per 100,000 people in the United States. In dence is increasing slightly (Fig, 1.2).18,19,39 It is higher in surveys with
Brazil, only 11,000 operations are performed each year, and 80% of active surveillance (8 to 51 cases per 100,000) than in surveys using
those needing surgery remain on a waiting list.27 There is also variation passive surveillance (5 to 35 per 100,000).40 Estimates of the incidence
in access to surgery in industrially developed countries. A comparison of first attack are based on few data but annual incidences are 23 per
of rates of aortic valve replacement in the United Kingdom against 100,000 people per annum (p.a.) in Kuwait, 35 per 100,000 in Iran,
estimated need found a variance between observed and expected rang- 51 per 100,000 in India, 80 per 100,000 in a Maori population in
ing between 2356 and 1230.33 The causes of this variance have New Zealand,40 and 194 per 100,000 in an indigenous population in
not been explored but may partly reflect the activity of community the Northern Territories of Australia.41
4 CHAPTER 1 Epidemiology of Valvular Heart Disease
1970
1972
1974
1976
1978
1980
1982
1984
1986
1988
1990
1992
1994
1996
1998
2000
2002
2004
2006
2008
A Year B Year
Incidence per 100,000
150 80
60
100
40
50 20
0 0
1970
1972
1974
1976
1978
1980
1982
1984
1986
1988
1990
1992
1994
1996
1998
2000
2002
2004
2006
2008
1970
1972
1974
1976
1978
1980
1982
1984
1986
1988
1990
1992
1994
1996
1998
2000
2002
2004
2006
2008
C Year D Year
250 250
200 200
150 150
100 100
50 50
0 0
1970
1972
1974
1976
1978
1980
1982
1984
1986
1988
1990
1992
1994
1996
1998
2000
2002
2004
2006
2008
1970
1972
1974
1976
1978
1980
1982
1984
1986
1988
1990
1992
1994
1996
1998
2000
2002
2004
2006
2008
E Year F Year
Fig. 1.2 Patterns of Change in Incidence of Acute Rheumatic Fever in World Health Organization
(WHO) Regions. Incidence was approximately stable in every WHO region other than the Americas, where
there is a slight increase. (A) The Americas. (B) Europe. (C) Africa. (D) Eastern Mediterranean. (E) Western
Pacific. (F) Southeast Asia. Acute rheumatic fever continues to occur sporadically in industrially developed
areas, such as in Utah,39 and the annual incidence is 30 cases per 100,000 people37 for all attacks but less
than 10 cases per 100,000 people p.a. for first attacks.19 (From Seckeler MD, Hoke TR. The worldwide epide-
miology of acute rheumatic fever and rheumatic heart disease. Clin Epidemiol 2011;3:67-84.)
Males and females were equally affected by first attacks in one Europe (Fig. 1.3).18,22,40–43 RhD is estimated to cause 4.3 million cases
study,40 and females more frequently affected in another.41 The of heart failure, which is 6.9% of the total number of cases, and to
incidence peaks at age 5 to 14 at 150 per 100,000 men, falling to produce an age-standardized mortality rate of 4.7 deaths per 100,000
approximately 55 per 100,000 at 15 to 24 years of age and to 25 per people p.a.2
100,000 at 25 to 34 years of age.41 The recurrence rate is 4.5% during In surveys concentrating on RhD, prevalence estimates have
the first year and 12.5% at 5 years.41 There are no data on the incidence changed in recent years with the use of echocardiography and with the
of first attack from Africa. evolution of echocardiographic criteria for diagnosis. A seminal study
The incidence of new RhD is 27% to 35% at 1 year, 44% to 51% by in 2007 in Cambodia and Mozambique12 found a prevalence of
5 years, and 52% to 61% at 10 years.42,43 Allowing that 60% of people 230 cases per 100,000 children as estimated by clinical examination
with acute RhF develop chronic RhD, the annual incidence of new cases but 2810 cases per 100,000 using echocardiography. The WHO 2006
of RhD globally has been estimated at 282,000.38 However, there are criteria42 used a combination of clinical examination and echocardiog-
major geographic differences, with a mean of 160 cases per 100,000 raphy to give a prevalence of 2110 cases per 100,000 (95% CI: 1410–
people (95% CI: 80–230) based on a meta-analysis of 37 population 3140),43 compared with only 270 cases per 100,000 (95% CI: 160–440)
surveys.43 The estimated incidence is 23.5 cases per 100,000 people in based on clinical diagnosis.43
Soweto,44 but with a J-shaped age-dependence curve and with 30 cases There were concerns about the limitations of clinical examination
per 100,000 for those 15 to 19 years of age, 15 cases per 100,000 for those and the specificity and variability of echocardiographic criteria. A
19 years of age, and 53 cases per 100,000 for those older than 60 years. retrospective re-evaluation of results gave a range in prevalence for the
same population of 510 to 3040 cases per 100,000, depending on the
Prevalence of Rheumatic Disease echocardiographic criteria used.45 These concerns led to a World Heart
The global disease survey1 estimated a worldwide prevalence of chronic Federation consensus46 statement in 2012 that categorized RhD as
RhD of at just under 30 million in 2016, an increase of 3.4% compared definite or borderline using a combination of regurgitation, gradient,
with 2006. The prevalence is increasing in all WHO regions other than and morphology (Fig. 1.4 and Box 1.2),45 Using these criteria, the
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pronounce nothing but divine truth. For the time being the poor
helpless clod is made the shrine of the sacred presence, and
converted into an oracle a thousand times more infallible than the
asphyxiated Pythoness of Delphi; and, unlike her mantic frenzy,
which was exhibited before the multitude, this holy sleep is
witnessed only within the sacred precinct by those few of the adepts
who are worthy to stand in the presence of the Adonai.
The description which Isaiah gives of the purification necessary for
a prophet to undergo before he is worthy to be the mouthpiece of
heaven, applies to the case in point. In customary metaphor he says:
“Then flew one of the seraphim unto me having a live coal in his
hand, which he had taken with the tongs from off the altar ... and he
laid it upon my mouth and said, Lo! this hath touched thy lips and
thine iniquity is taken away.”
The invocation of his own Augoeides, by the purified adept, is
described in words of unparalleled beauty by Bulwer-Lytton in
Zanoni, and there he gives us to understand that the slightest touch
of mortal passion unfits the hierophant to hold communion with his
spotless soul. Not only are there few who can successfully perform
the ceremony, but even these rarely resort to it except for the
instruction of some neophytes, and to obtain knowledge of the most
solemn importance.
And yet how little is the knowledge treasured up by these
hierophants understood or appreciated by the general public! “There
is another collection of writings and traditions bearing the title of
Kabala, attributed to Oriental scholars,” says the author of Art-Magic;
“but as this remarkable work is of little or no value without a key,
which can only be furnished by Oriental fraternities, its transcript
would be of no value to the general reader.”[577] And how they are
ridiculed by every Houndsditch commercial traveller who wanders
through India in pursuit of “orders” and writes to the Times, and
misrepresented by every nimble-fingered trickster who pretends to
show by legerdemain, to the gaping crowd, the feats of true Oriental
magicians!
But, notwithstanding his unfairness in the Algerian affair, Robert
Houdin, an authority on the art of prestidigitation, and Moreau-Cinti,
another, gave honest testimony in behalf of the French mediums.
They both testified, when cross-examined by the Academicians, that
none but the “mediums” could possibly produce the phenomena of
table-rapping and levitation without a suitable preparation and
furniture adapted for the purpose. They also showed that the so-
called “levitations without contact” were feats utterly beyond the
power of the professional juggler; that for them, such levitations,
unless produced in a room supplied with secret machinery and
concave mirrors, was impossible. They added moreover, that the
simple apparition of a diaphanous hand, in a place in which
confederacy would be rendered impossible, the medium having been
previously searched, would be a demonstration that it was the work
of no human agency, whatever else that agency might be. The
Siècle, and other Parisian newspapers immediately published their
suspicions that these two professional and very clever gentlemen
had become the confederates of the spiritists!
Professor Pepper, director of the Polytechnic Institute of London,
invented a clever apparatus to produce spiritual appearances on the
stage, and sold his patent in 1863, in Paris, for the sum of 20,000
francs. The phantoms looked real and were evanescent, being but
an effect produced by the reflection of a highly-illuminated object
upon the surface of plate-glass. They seemed to appear and
disappear, to walk about the stage and play their parts to perfection.
Sometimes one of the phantoms placed himself on a bench; after
which, one of the living actors would begin quarrelling with him, and,
seizing a heavy hatchet, would part the head and body of the ghost
in two. But, joining his two parts again, the spectre would reappear, a
few steps off, to the amazement of the public. The contrivance
worked marvellously well, and nightly attracted large crowds. But to
produce these ghosts required a stage-apparatus, and more than
one confederate. There were nevertheless some reporters who
made this exhibition the pretext for ridiculing the spiritists—as though
the two classes of phenomena had the slightest connection!
What the Pepper ghosts pretended to do, genuine disembodied
human spirits, when their reflection is materialized by the elementals,
can actually perform. They will permit themselves to be perforated
with bullets or the sword, or to be dismembered, and then instantly
form themselves anew. But the case is different with both cosmic
and human elementary spirits, for a sword or dagger, or even a
pointed stick, will cause them to vanish in terror. This will seem
unaccountable to those who do not understand of what a material
substance the elementary are composed; but the kabalists
understand perfectly. The records of antiquity and of the middle
ages, to say nothing of the modern wonders at Cideville, which have
been judicially attested for us, corroborate these facts.
Skeptics, and even skeptical spiritualists, have often unjustly
accused mediums of fraud, when denied what they considered their
inalienable right to test the spirits. But where there is one such case,
there are fifty in which spiritualists have permitted themselves to be
practiced upon by tricksters, while they neglected to appreciate
genuine manifestations procured for them by their mediums.
Ignorant of the laws of mediumship, such do not know that when an
honest medium is once taken possession of by spirits, whether
disembodied or elemental, he is no longer his own master. He
cannot control the actions of the spirits, nor even his own. They
make him a puppet to dance at their pleasure while they pull the
wires behind the scenes. The false medium may seem entranced,
and yet be playing tricks all the while; while the real medium may
appear to be in full possession of his senses, when in fact he is far
away, and his body is animated by his “Indian guide,” or “control.” Or,
he may be entranced in his cabinet, while his astral body (double) or
doppelganger, is walking about the room moved by another
intelligence.
Among all the phenomena, that of re-percussion, closely allied
with those of bi-location and aërial “travelling,” is the most
astounding. In the middle ages it was included under the head of
sorcery. De Gasparin, in his refutations of the miraculous character
of the marvels of Cideville, treats of the subject at length; but these
pretended explanations were all in their turn exploded by de Mirville
and des Mousseaux, who, while failing in their attempt to trace the
phenomena back to the Devil, did, nevertheless, prove their spiritual
origin.
“The prodigy of re-percussion,” says des Mousseaux, “occurs
when a blow aimed at the spirit, visible or otherwise, of an absent
living person, or at the phantom which represents him, strikes this
person himself, at the same time, and in the very place at which the
spectre or his double is touched! We must suppose, therefore, that
the blow is re-percussed, and that it reaches, as if rebounding, from
the image of the living person—his phantasmal[578] duplicate—the
original, wherever he may be, in flesh and blood.
“Thus, for instance, an individual appears before me, or, remaining
invisible, declares war, threatens, and causes me to be threatened
with obsession. I strike at the place where I perceive his phantom,
where I hear him moving, where I feel somebody, something which
molests and resists me. I strike; the blood will appear sometimes on
this place, and occasionally a scream may be heard; he is wounded
—perhaps, dead! It is done, and I have explained the fact.”[579]
“Notwithstanding that, at the moment I struck him, his presence in
another place is authentically proved; ... I saw—yes, I saw plainly the
phantom hurt upon the cheek or shoulder, and this same wound is
found precisely on the living person, re-percussed upon his cheek or
shoulder. Thus, it becomes evident that the facts of re-percussion
have an intimate connection with those of bi-location or duplication,
either spiritual or corporeal.”
The history of the Salem witchcraft, as we find it recorded in the
works of Cotton Mather, Calef, Upham, and others, furnishes a
curious corroboration of the fact of the double, as it also does of the
effects of allowing elementary spirits to have their own way. This
tragical chapter of American history has never yet been written in
accordance with the truth. A party of four or five young girls had
become “developed” as mediums, by sitting with a West Indian
negro woman, a practitioner of Obeah. They began to suffer all kinds
of physical torture, such as pinching, having pins stuck in them, and
the marks of bruises and teeth on different parts of their bodies.
They would declare that they were hurt by the spectres of various
persons, and we learn from the celebrated Narrative of Deodat
Lawson (London, 1704), that “some of them confessed that they did
afflict the sufferers (i. e., these young girls), according to the time
and manner they were accused thereof; and, being asked what they
did to afflict them, some said that they pricked pins into poppets,
made with rags, wax, and other materials. One that confessed after
the signing of her death-warrant, said she used to afflict them by
clutching and pinching her hands together, and wishing in what part
and after what manner she would have them afflicted, and it was
done.”[580]
Mr. Upham tells us that Abigail Hobbs, one of these girls,
acknowledged that she had confederated with the Devil, who “came
to her in the shape of a man,” and commanded her to afflict the girls,
bringing images made of wood in their likeness, with thorns for her to
prick into the images, which she did; whereupon, the girls cried out
that they were hurt by her.”
How perfectly these facts, the validity of which was proven by
unimpeachable testimony in court, go to corroborate the doctrine of
Paracelsus. It is surpassingly strange that so ripe a scholar as Mr.
Upham should have accumulated into the 1,000 pages of his two
volumes such a mass of legal evidence, going to show the agency of
earth-bound souls and tricksy nature-spirits in these tragedies,
without suspecting the truth.
Ages ago, the old Ennius was made by Lucretius to say: