Heart Rhythm Disorders: History, Mechanisms, and Management Perspectives

485 Pages·2020·41.525 MB·English

by  J. Anthony Gomes

J. Anthony Gomes
Heart Rhythm Disorders
History, Mechanisms, and Management
Perspectives
123
Heart Rhythm Disorders
J. Anthony Gomes
Heart Rhythm Disorders
History, Mechanisms,
and Management Perspectives
J. Anthony Gomes
Icahn School of Medicine
Mount Sinai Hospital
New York, NY
USA
ISBN 978-3-030-45065-6 ISBN 978-3-030-45066-3 (eBook)
https://doi.org/10.1007/978-3-030-45066-3
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To my wife, Margarita Suren; and to the memory of Dr. Anthony N. Damato.
Foreword
This is a remarkably well balanced and beautifully written book with evocative photography
of pioneers and their important contributions figurately represented in electrocardiographic/
electrophysiologic tracings. It also has a collection of excellent chapters taking the reader
through the history of diagnosing and treating cardiac arrhythmias from ancient times to the
present. It is written by a single author, who clearly devoted several years to the project, nar-
rating history eloquently of what transpired in the past and the progress we have made in the
management of cardiac arrhythmias.
The book describes how in the first half of the twentieth century several investigators, using
cardiac anatomy, animal experiments, and deductive reasoning to the surface electrocardio-
gram (ECG), contributed to the knowledge of cardiac impulse generation and conduction,
construing hypotheses on mechanisms of arrhythmogenesis and heart block. Thereafter, in the
second half of the twentieth century, the introduction of advanced diagnostic extra- and intra-
cardiac techniques resulted in rapid evolution of our understanding of the physiology and
pathophysiology of cardiac arrhythmias that in turn resulted in the development of several new
modalities of treatment.
An important moment, as discussed in Chap. 3, was the birth of clinical cardiac electro-
physiology as a new subspecialty in Cardiovascular Medicine. I had the good fortune to be
present at the start of that new era. It happened in Amsterdam in 1966. By positioning catheters
inside the intact human heart for intracardiac pacing, a new method was born to study rhythm
disturbances. That initiative came after Professor Dr. Dirk Durrer showed during epicardial
mapping in a patient with the Wolff-Parkinson-White (WPW) ECG who required surgical
closure of an atrial septal defect that the patient had an extra atrioventricular connection. It
started the discussion in our group: If there are two pathways between the atrium and ventricle
in WPW syndrome, can we create unidirectional block in one AV pathway followed by retro-
grade conduction over the anterogradely blocked pathway inducing the supraventricular tachy-
cardia which is often present in WPW patients? For that, we needed catheters to pace inside the
heart with a safe, reliable stimulator. Our first patient was a young woman suffering from fre-
quent palpitations and we could show that it was possible to initiate and terminate her tachy-
cardia by appropriately timed intracardiac stimuli during pacing. This new approach was
called programmed electrical stimulation of the heart (PES).
Of interest, at about the same time, independent from the ongoing work in Amsterdam, Dr.
Philippe Coumel and coworkers in Paris reported on the use of programmed stimulation in a
patient with a narrow QRS tachycardia.
In 1969, another major milestone occurred. Drs. Benjamin Scherlag, Anthony N. Damato,
Sun H. Lau, and associates, at the United States Public Health Service Hospital in Staten
Island, New York, reported on a catheter technique to reproducibly record a His bundle elec-
trogram. This was another seminal achievement, since it made it possible to localize the site of
a conduction disturbance and to separate supraventricular from ventricular rhythms.
Initially, programmed electrical stimulation together with His bundle recordings was used
to localize the site of block and the origin of different types of arrhythmias and/or the tachy-
cardia pathways. Undoubtedly, these studies resulted in a much better interpretation of the
vii
viii Foreword
12-lead ECG by correlating the new information from intracardiac studies with ECG changes
and ushered the specialty of clinical cardiac electrophysiology.
Thereafter, as scholarly discussed in the book, programmed electrical stimulation of the
heart moved from an investigational tool to development of new therapies. The ability to local-
ize the arrhythmia, to differentiate supraventricular from ventricular arrhythmias by recording
activity from the His bundle and other structures from the cardiac conducting system, and to
obtain insights into the mechanism of arrhythmias opened the door to surgical excision of the
arrhythmia substrate or interruption of a critical part of the tachycardia circuit. Programmed
electrical stimulation was also used for selecting antiarrhythmic drug therapy in the past,
before the era of the internal defibrillator. Its role became clear in supraventricular tachycar-
dias, but less so in ventricular arrhythmias. This was related to the observation that surviving
Purkinje-muscle fibers in ventricular scar could potentially result in different tachycardia cir-
cuits with variable responses to antiarrhythmic drug therapy.
Cooperation between cardiac electrophysiologists and industry resulted in the introduction
of novel catheter systems, 3-dimentional mapping systems, and sophisticated pacing devices
for brady- and tachycardias.
An important and revolutionary development was Dr. Michel Mirowski’s introduction of
the implantable defibrillator, a device capable of converting life-threatening ventricular
arrhythmias to sinus rhythm regardless of the underlying mechanism. The saga of Michel
Mirowski, his trials and tribulations during the development of the internal defibrillator, is
effectively and elegantly described by the author. It was followed by catheter ablation of car-
diac arrhythmias ranging from supraventricular tachycardias and atrial fibrillation to ventricu-
lar tachycardias using advanced imaging techniques and accurate cardiac 3- dimensional
activation-propagation mapping. Another more recent development was resynchronization of
abnormal ventricular activation by cardiac pacing in patients with heart failure, and the intro-
duction of leadless pacemakers, and left atrial appendage occlusion devices.
In this book, each of the different types of heart rhythm disorders has received a separate
chapter as to their history, diagnosis, mechanism and treatment. Other chapters review our cur-
rently used diagnostic and therapeutic techniques always with a critical analysis of their value
and limitations.
The author also discusses the future of cardiac arrhythmia management. Despite the enor-
mous advances made during the last 50 years most of our interventions are still palliative. They
make the patient live longer with a better quality of life, but few offer a real cure, except in
catheter ablation of the accessory pathway in patients with the WPW syndrome, and in most
supraventricular tachycardias.
We still have a long way to go to reach a cure and prevention of cardiac arrhythmias and
sudden cardiac death. What will be the contribution of genetic information coupled with arti-
ficial intelligence to improve risk stratification and management?
I want to close by congratulating and thanking Dr. Gomes for his unique review of the past,
present and future of heart rhythm disorders. This is an important and uniquely well written
book. It deserves a wide audience among the health professionals taking care of patients with
cardiac arrhythmias.
Hein J. J. Wellens, MD
Emeritus Professor of Cardiology, Maastricht University
Maastricht, The Netherlands
Preface
For while knowledge defines all we currently know and understand, imagination points to all we might
yet discover and create. — Albert Einstein
In 1967 and 1969, two monumental studies would change the course of heart rhythm disor-
ders, ushering a new field in cardiovascular medicine, that of clinical cardiac electrophysiol-
ogy. This book is about the diagnosis, mechanisms, and treatment of Heart Rhythm Disorders
that plague the old as well as the young affecting their quality of life and often resulting in
premature death.
I have been fortunate to have lived to see and partake in the astronomical advances in car-
diovascular medicine over the last five decades and, moreover, to have been at the birth of my
specialty, of clinical cardiac electrophysiology. The contributions to the field of cardiac elec-
trophysiology have come over several decades from a host of individuals often working inde-
pendently in different institutions mostly in Europe and the United States, and from device and
drug companies here and abroad. While in the past, the advancement in medical science was
often the domain of pioneering individuals (The ECG—Willem Einthoven; Angioplasty—
Andreas Roland Grüntzig; Internal Defibrillator— Michel Mirowski etc.), more recently, the
developments have often come from pharmaceutical, technological, and device companies
with physician collaboration. Moreover, the field has kept on advancing at a rapid pace in the
basics and genetics of cardiac arrhythmias as well as in the introduction of novel catheters and
mapping systems.
During my teaching rounds to the Cardiology as well as Electrophysiology Fellows, I came
to recognize their lack of knowledge as well as an unmitigated hunger for the history of car-
diovascular medicine, including heart rhythm disorders, specifically, how the field started, the
evolution of the specialty, and the contributions of several pioneers who made the field what it
is today. This observation and my own profound interest in history prompted me not only to
give a perspective of the diagnosis, mechanisms, and current treatment of Heart Rhythm
Disorders in this book but also to write about its history. For in the words of Isaac Asimov:
“There is not a discovery in science, however revolutionary, however sparkling with insight,
that does not arise out of what went before.” “If I have seen further than other men,” said Isaac
Newton, “it is because I have stood on the shoulders of giants.”
And so, in my historical musings, I have tried my very best to abide by the standards of
Miguel de Cervantes: “Historians ought to be precise, faithful and unprejudiced; and neither
interest nor fear, hatred nor affection, should make them swerve from the way of the truth.”
However, I’m cognizant of the fact that I might have erred at times and at other instances left
out the contributions of some individuals inadvertently, and others I could not reach. To them
I extend my sincere apologies and a depth of gratitude.
New York, NY, USA J. Anthony Gomes, MD
ix
Acknowledgments
I have many people to offer my gratitude. To begin with, I would like to thank Richard Lansing,
Editorial Director for Clinical Medicine at Springer, for accepting my book proposal after a
mere 4 days, and to Michael Griffin of Springer Nature for his editorial assistance. To my wife,
Margarita, for her patience and encouragement as I spent endless hours on the computer
researching and writing.
I would like to express my most sincere appreciation to a host of clinicians and clinician-
scientists for their overt enthusiasm for this book and for providing their photographs and
electrocardiographic/electrophysiological tracings, their time, and effort, some more than oth-
ers, in communicating their own contributions to the field of cardiac electrophysiology and
those of their colleagues. These physicians include: Dr. Andre d’ Avilla; the late Dr. Masood
Akhtar; Drs. Pedro Brugada, Srinivas Dukkipati, John D. Fisher, and John J. Gallagher; the
late Dr. Mark Josephson; and Drs. Warren Jackman, Frank Marchlinski, Rahul Mehra, Robert
Myerburg, Eric Prystowski, Carlo Papone, Pratap Reddy, Vivek Y. Reddy, Jeremy Ruskin,
Benjamin Scherlag, Melvin N.  Scheinman, Peter Schwartz, Nabil L.  Sherif, William
G. Stevenson, Hein J.J. Wellens, Albert Waldo, Stephen Winters, and Douglas P. Zipes.
I am indebted to my electrophysiology colleagues at The Mount Sinai Medical Center and
The Leona M. and Harry B. Helmsley Charitable Trust Center for Cardiac Electrophysiology,
specifically to Drs. Subharao Choudhry, Srinivas Dukkipati, Jacob Koruth, Marc Miller, Vivek
Y. Reddy, and William Whang, for providing me with some of the illustrations of cases done
in our EP laboratories, as well as providing their precious time in reading and commenting on
some of the chapters. Among these colleagues, a very special thanks to Dr. Marc Miller for a
range of technical assistance while penning this book, and to Dr. William Whang for promptly
reviewing some of the chapters. A note of appreciation to Dr. Umesh Gidwani for his com-
ments on the chapter on Hypothermia. I would also like to recognize my ex-associates Drs.
Marie Noelle Langan and Davendra Mehta, and the CT surgeons Drs. Arisan Ergin and Jorge
Cammunas.
Finally, I want to express my gratitude to the fulltime cardiovascular faculty at Mount Sinai
Medical Center, specifically to Drs. Valentin Fuster, Samin K. Sharma, Jonathan L. Halperin,
Annapoorna S. Kini, and Martin E. Goldman, as well as to the voluntary faculty for their trust
in administering to their patients and their consistent support and friendship over the last
36 years.
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Contents
Part I T he Enlightenment Period in Cardiovascular Dynamics
1 Discovery of the Circulatory System . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 3
Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 3
Egyptian View of the Heart . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 3
The Asian View: The Pulse . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 5
The Ancient Greek View of Circulation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 5
Galen’s View of the Circulatory System . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 5
The Islamic View . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 5
Ibn al-Nafis and the Discovery of Pulmonary Circulation . . . . . . . . . . . . . . . . . . . . . 6
Leonardo da Vinci’s View of the Heart . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 6
Contribution of Michael Servetus and Realdo Colombo . . . . . . . . . . . . . . . . . . . . . . 7
William Harvey and the Ultimate Discovery of Circulation of Blood . . . . . . . . . . . . 8
References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 9
2 The Road to Unearthing the Conducting System of the Heart . . . . . . . . . . . . . . . . 11
Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 11
Jan Evangelista Purkyně and the Discovery of Purkinje Fibers . . . . . . . . . . . . . . . . . 12
Wilhelm His Jr. and the Discovery of the His Bundle . . . . . . . . . . . . . . . . . . . . . . . . 12
The Contribution of Walter Gaskell . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 12
Sunao Tawara and the Discovery of the AV Node . . . . . . . . . . . . . . . . . . . . . . . . . . . 13
Arthur Keith and Martin Flack and the Discovery of the Sinus Node . . . . . . . . . . . . 13
Modern Concepts of Impulse Generation and Transmission . . . . . . . . . . . . . . . . . . . 13
References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 17
3 Birth of Clinical Cardiac Electrophysiology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 21
Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 21
The Electrocardiogram . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 21
Programmed Electrical Stimulation of the Heart . . . . . . . . . . . . . . . . . . . . . . . . . . . . 22
Recording of Intracardiac Electrical Activity: Dr. Benjamin Scherlag, PhD . . . . . . . 22
The Anthony N. Damato School of Cardiac Electrophysiology . . . . . . . . . . . . . . . . . 24
The Dutch School of Cardiac Electrophysiology . . . . . . . . . . . . . . . . . . . . . . . . . . . . 27
The Mushrooming of EPS Laboratories . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 28
The Heart Rhythm Society . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 28
References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 30
4 Molecular Basis of Impulse Generation and Propagation . . . . . . . . . . . . . . . . . . . 41
Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 41
The Action Potential . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 41
Sodium Channels . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 43
Voltage-Gated Calcium Channels . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 43
Voltage-Gated Potassium Channels . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 44
The Funny Current of the Sinus Node . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 45
xiii