CONGENITAL HEART MALFORMATIONS IN...

Visit the link below to download the full version of this book:

https://medipdf.com/product/congenital-heart-malformations-in/

Click Download Now

Published by
Imperial College Press
57 Shelton Street
Covent Garden
London WC2H 9HE

Distributed by
World Scientific Publishing Co. Pte. Ltd.
P O Box 128, Farrer Road, Singapore 912805
USA office: Suite IB, 1060 Main Street, River Edge, NJ 07661
UK office: 57 Shelton Street, Covent Garden, London WC2H 9HE

Library of Congress Cataloging-in-Publication Data

Congenital heart malformations in mammals : an illustrated text / editors, Magnus
Michaelsson, Siew Yen Ho.
p. cm.
Includes bibliographical references and index.
ISBN 1-86094-158-3 (alk. paper)
1. Veterinary cardiology. 2. Mammals—Diseases. 3. Congenital heart disease in
animals. I. Michaelsson, Magnus. II. Ho, Siew Yen.

SF811.C66 2000
636.089'612-dc21 00-039673

British Library Cataloguing-in-Publication Data

A catalogue record for this book is available from the British Library.

Copyright © 2000 by Imperial College Press

All rights reserved This book, or parts thereof, may not be reproduced in any form or by any means, electronic or
mechanical, including photocopying, recording or any information storage and retrieval system now known or to be
invented, without written permission from the Publisher.

For photocopying of material in this volume, please pay a copying fee through the Copyright Clearance Center, Inc.,
222 Rosewood Drive, Danvers, MA 01923, USA. In this case permission to photocopy is not required from the
publisher.

Printed in Singapore.
 Abbreviations

A Aortic (anterior) leaflet of mitral valve

Ao Aorta
AAo Ascending aorta
AS Antero-superior leaflet
AtS Atrial septum
BT Brachiocephalic trunk
CS Coronary sinus
CX Circumflex artery
D Arterial duct
DAo Descending aorta
IB Inferior bridging leaflet
ICV Caudal (inferior/posterior) caval vein
LA Left atrium
LAA Left atrial appendage
LC Left-facing aortic sinus
LCA Left coronary artery
LPA Left pulmonary artery
LSC Left subclavian artery
LV Left ventricle
M Mural leaflet
MLA Morphologically left atrium
MS Membranous septum
NC Non-facing aortic sinus
OS Outlet septum
PT Pulmonary trunk
RA Right atrium
RAA Right atrial appendage
RC Right-facing aortic sinus
RCC Right common carotid artery
RCA Right coronary artery
RPA Right pulmonary artery
RV Right ventricle
S Septal leaflet
SB Superior bridging leaflet
SCV Cranial (superior/anterior) caval vein
TV Tricuspid valve
VS Ventricular septum
VSD Ventricular septal defect

The scales shown on the photographs are in mm (fine lines) and cm (thick lines) divisions.
V
This page is intentionally left blank
 FOREWORD

This work is authored by a paediatric cardiologist with vast experience and a deep interest in
comparative cardiology, in collaboration with an internationally recognised expert in cardiac
morphology. It is an extremely thorough and informative presentation of congenital heart
malformations in mammals and, to our knowledge, unique. Previously published works are reviewed
and presented together with the authors' own observations of new findings in the normal heart
and many new cases of malformed hearts. The section describing a method of sequential segmental
analysis provides a very useful tool for clarifying anomalies in the cardiovascular system. The
last chapter on comparative aspects of malformations in different species is especially valuable.
The illustrations, of high quality and accompanied by lucid descriptions, make a didactic
presentation. In summary, this work is of great interest to anyone active in the field of cardiology
and it provides an excellent aid in clinical work, teaching and research.

Clarence Kvart (DVM, PhD, Diplomate E.C.V.I.M)

&
Jens Haggstrom (DVM, PhD, Diplomate E.C.V.I.M)

Department of Animal Physiology

Faculty of Veterinary Medicine
University of Agricultural Science
Uppsala, Sweden

vu
This page is intentionally left blank
 PREFACE

At a paediatric cardiology meeting decades ago, the veterinarian D.F. Patterson gave a presentation
on canine cardiac defects. At that time, human cardiologists knew very little about congenital
heart malformations in animals. Until today, textbooks in paediatric cardiology include only a
few lines, if at all, on isolated observations in other mammals. An attempt to bridge the gap
was made in two articles by the late Helen B. Taussig — outstanding clinician, teacher and
the author of Congenital Malformations of the Heart published in 1947. Dr Taussig launched
a concept of evolutionary (as opposed to teratogenic), origin of isolated cardiac malformations.
Her idea came up as a result of deductive reasoning based on published reports and on her
own experimental work in mammals and birds.
In order to provide a better understanding of the malformations in mammals, I thus decided
to produce a book that, hopefully, will prove to be useful. R.H. Anderson, friend and co-worker,
encouraged me to proceed with the plans for the book. Linking his senior associate to the project
was a prerequisite for quality and insight.
The first step in preparing this book was to familiarise myself with previously published work.
It is clear from the list of references in Appendix 1 that there are a number of important
contributions covering parts of the field. Surprisingly, no attempts have been made in recent
years to present a more collected view on the subject. Ancient publications cannot be retrieved
with computer-assisted literature search. I have had the privilege of full access to old veterinary
books and scientific journals kept buried in the libraries of the Swedish University of Agricultural
Sciences and of the National Veterinary Institute in Uppsala. The lists on published cases, old
and new are certainly not complete, but the librarians did their best. Their help is gratefully
acknowledged.
One requires more than just experience in human paediatric cardiology to be able to describe
congenital defects in animals. It was clear that the dominant part of this work had to be based
on post-mortem studies. However, it did feel natural, as a first step, to learn about living animals.
My teachers of clinical veterinary cardiology — Clarence Kvart, Jens Haggstrom and Heikki
Sateri, generously welcomed me as a regular guest at their outpatient clinic service. This admittance
resulted in friendship, new knowledge and novel scientific studies. Echocardiography and Doppler
examination are not very different from that in human paediatric cardiology but I would certainly
not like to be without the experience of listening, for instance, to the slow Doppler signals from
the equine aortic regurgitation. Estelle Agren is to thank for opening the doors to the Department
of Clinical Radiology at the Swedish University of Agricultural Sciences. She has selflessly
provided radiological help with those tricky ruminant heart bones.
Personal experience in post-mortem studies of fresh normal and abnormal hearts was judged
to be an essential part of the learning curve. Access to the post-mortem rooms in the Departments
of Pathology at the National Veterinary Institute and at the Swedish University of Agricultural

IX
X Congenital Heart Malformations in Mammals — An Illustrated Text

Sciences was obtained through the Heads — Professors Claes Rehbinder and Lennart Jonsson,
and by Torsten Momer — Head of the Unit for Wild Animals at the National Veterinary Institute.
The rounds with the daily education offered by the veterinary pathologists for more than a year
have greatly contributed to the knowledge on cardiac and extra-cardiac diseases. The generous
and patient attitude of all the staff, heads as well as technicians in the departments, is gratefully
acknowledged. Special thanks are due to Carl Hard af Segerstad, Lennart Jbnsson, Erika Karlstam,
Claes Rehbinder and Erik Agren. Clarence Kvart kindly allowed us to reproduce many of the
pictures in his personal collection.
About 60 congenital heart defects were identified, almost exclusively in domestic animals.
This is a screening study on a selected autopsy material and it can hardly be used for calculations
of incidence and prevalence figures. In our view, it can be looked upon as a guidance for studies
on mammalian heart defects — where to look and what to find. We were impressed by the
frequent occurrence of subvalvar aortic stenosis, not only as an isolated defect, but often in
combination with other malformations of the heart. It so happened that many of die malformations
found in this study are representative of the prevalence in different species, such as double outlet
right ventricle in cattle, subaortic stenosis in pigs and bicuspid atresia in the horse. The fact
that no severe defects were found in wild animals is hardly conclusive, although it can be
tempting to speculate about effects of inbreeding and selectivity as explanations to differences
between domestic and wild animals.
Congenital heart malformations in mammals is a vast topic. We have tried to generalise the
text although it was often tempting to go into more details. A detailed account would have
destroyed the original idea of presenting a reasonably short, illustrated text on our experiences
as well as those of others. We hope that the reading will stimulate further studies — either
on the clinical and pathological aspects of heart disease in various species or on animal models
of human heart defects. Numerous questions in this relatively young field remain to be answered.
Or, if we may borrow the words of Winston Churchill from 1942: "This is not the end, it is
not even the beginning of the end but it is the end of the beginning".

Magnus Micbaelsson
Department of Women's and Children's Health
Section for Paediatrics
Uppsala University
Sweden
December, 1998
 Preface XI

Having majored in Zoology whilst at University, I was delighted when Magnus, a long-standing
friend and collaborator, approached me with the idea of producing a book on congenital heart
malformations in mammals. Through Magnus, I was able to re-aquaint myself with the animal
kingdom and marvel, yet again, at the great diversity within this group. It is neither possible
nor practical to include in this book all the subgroups. We have, therefore, focussed on the more
common examples. The effort in this production is largely Magnus. His driving force behind
this project, and the encouragement given by Professor R. H. Anderson, my mentor, were essential
for me to complete this task. Magnus and I are both indebted to our many colleagues who brought
cases to our attention and shared our enthusiasm. With my colleague, Leon Gerlis, I have also
had many discussions on the variations of the aortic arch. My colleagues, Zarlasht Nejam, Satta
Goba, Vi-Hue Tran, Karen McCarthy helped with printing the photographs while Peacelyn
Jeyaratnam helped with the typing. They all provided assistance with a smile. The finishing
touches of the manuscript could not have been achieved without their cheerful willingness.

Any deficiencies within this book, which undoubtedly there will be, are our full responsibility.

Siew Yen Ho
Reader in Cardiac Morphology
Paediatrics
National Heart & Lung Institute
Imperial College
United Kingdom
December, 1998
This page is intentionally left blank
 CONTENTS

Abbreviations v

Foreword vii

Preface ix

Chapter 1 Introduction: Normal Hearts — A Comparison 1

Chapter 2 Sequential Segmental Analysis 19

Chapter 3 Definition, Causes, Frequency of Occurrence and Prevalence 29

of Congenital Cardiovascular Defects

Chapter 4 Horses 43

Chapter 5 Cattle 57

Chapter 6 Pigs 69

Chapter 7 Sheep and Goats 77

Chapter 8 Dogs 81

Chapter 9 Cats 97

Chapter 10 Mice, Rats and Rabbits 109

Chapter 11 Other Animals 113

Chapter 12 Comparative Aspects 119

Appendix 1 Key References 151

Appendix 2 Methods of Preparation and Dissection 153

This page is intentionally left blank
 -CHAPTER 1~

INTRODUCTION: NORMAL HEARTS —

A COMPARISON

Since understanding of the malformed heart requires a prior knowledge of normality, we begin
by reviewing the normal heart. Although all mammalian hearts have four chambers, there are,
nevertheless, subtle differences between the major groups. More than 3000 animal hearts have
been studied, varying in weight from 50 mg in a shrew to 15 kg in a female Indian elephant.
The material includes domestic, laboratory and wild terrestrial and aquatic mammals, 50 different
species in all. The ten most common species were dogs (755), cats (208), horses (292), cattle
(351), pigs (511), sheep (149), lynxs (146), red foxes (94), roe deer (84) and elks (54). Most
of the animals were grown-up with the exception of pigs, where piglets, aged two to three weeks,
were most frequent. About 60 congenital heart defects were identified, predominantly in domestic
animals.

Table 1.1 Heart mass as related to body mass and heart rate.

Heart Weight/Body Weight Ratio (%) Heart Rate at Rest

Mean Range Range
Baboon 0.43-0.51 95-140
Bat 1.38-1.44 375-600
Bottleaose dolphin 70-85
Cat 0.40 0.28-1.42 110-140
Cow 0.48 0.30-0.87 50-80
Dog 0.71 0.43-1.66 70-140
Elephant 0.33-0.52 22-40
Ermine 1.18 350
European hare 0.77 60-70
Goat 0.46 0.26-0.66 70-120
Guinea pig 0.42 0.26-0.51 130-325
Hedgehog 0.38-0.67 200-325
Horse 0.69 0.45-1.20 25-50
Man, male, 21-25 yrs 0.55 70
Mole 0.76 250-350
Mouse 0.61-0.79 450-750
Rabbit, domestic 0.29-0.34 170-280
Rat 0.36-0.40 250-450
Seal 0.92 60-70
Sheep 0.41 0.17-0.82 70-120
Shrew 1.35 600-1320
Swine 0.40 0.23-0.48 70-120
Whale 0.51-0.52

1
2 Congenital Heart Malformations in Mammals — An Illustrated Text

The table on heart mass as related to body mass and on heart rate (Table 1.1) is compiled
from a number of sources 5~7, '• n - 14 ~ 17,23,25,27,29,32 and is presented with some reservations. The
number of animals from which the figures are derived varies between species. The conditions
during which the data have been collected is usually not clearly stated. The age of the animal
is rarely mentioned. According to the study of Bezndk, the relative heart weight in rats is
decreasing with increasing body weight in young and in adult animals. Rats weighing 75 g had
a ratio of 0.40% and when reaching 270 g, the rats had a ratio of 0.28%.4 The technique of
weighing the hearts is rarely described. In Schubert's study from 1909, dog hearts with residual
blood had ratios of 0.9-2.2% and the empty hearts showed a ratio of 0.85-1.4%.27 Another
source of error is pericardia] and epicardial fat which can be abundant especially in ruminants.
The wide span of heart rates "at rest" can partly be explained by variations in environmental
conditions and the degree of excitement of the animal. In a description of feline ECG data,
a mean value of 197 was given with a range between 120 and 240,31 and in other studies the
range of normal heart rates was between 110 and 130 beats per minute29.
Keeping these deficiencies in mind, some undisputable observations could be noted. The
smallest animals, the bat and the shrew, have the relatively heaviest hearts and the fastest heart
rates. Large animals like elephants have low heart rates and a relatively low heart weight.
Allometric equations have been proposed to express heart mass and heart rate as a function
of the body mass. The allometric constant for heart weight is given as 0.0058 x body mass
in kg ° 98 and for rate the formula is 241 x body mass in kg ~°-25 8 The allometric analysis,
however, has serious drawbacks. Examples of deviations from the theoretically derived figures
are a comparison between horses and cattle of roughly equal body mass. The heart rate of cattle
is twice that of the horse. Another example is a comparison between leporids. The relative mass
of the domestic rabbit was 0.24% and that of the wild rabbit 0.28% as compared to the European
hare with a ratio of 0.7715. The heart rate of the hare is said to be 60-70 and that of the rabbit
200-325. Thoroughbred horses and Greyhounds have big hearts, probably explained not only
by heavy physical activity but also by genetic predisposition. Many wild animals have a high
heart mass/body mass ratio. It could be of interest to study if this ratio is present at birth or
if it is explained only by less access to food and a higher physical activity as compared to
domestic animals.

Morphology and Function of the Heart and Great Vessels — General Aspects
Information from the literature 2,n " 13,28 supplemented with own experience form the basis for
the following description. Not surprisingly, the structure and function of mammalian hearts are
strikingly similar. There are two suctioning and compressing muscular parts, the ventricles,
assisted by the atria and with "back-water valves" preventing backflow in systole to collecting
compartments, the atria and the veins and in diastole of blood pumped to peripheral vessels.
The thin-walled right ventricle is adopted to great variability in volume and the left ventricular
muscle is a high pressure pump allowing less beat-to-beat variations in volume.
A pressure gradient all along the vascular system is the prerequisite for the convection of
blood from the heart through the capillary system and back through the veins, atria and ventricles.
 Introduction: Normal Hearts — A Comparison 3

The blood pressure is regulated by the flow from the ventricles, the peripheral vascular resistence,
the amount of blood in the vascular system, the elasticity of the vessel walls and the viscosity
of the blood.
The venous blood draining the body enters the right atrium through the two caval veins and
the azygous veins and is pumped to the lungs by the right ventricle widi its two backflow valves,
the tricuspid leaflets with tendinous cords and papillary muscles and the pulmonary leaflets.
The oxygenated blood passes through the pulmonary veins to the left atrium and is pumped
into the aorta and its branches by the left ventricle with its two valve systems, the mitral leaflets
with tendinous cords and papillary muscles and the aortic leaflets. The heart itself is perfused
through the two coronary arteries and the venous heart blood empties into the right atrium via
the coronary sinus.
Automatic activity of the specialised pacemaker cells in the sino-atrial node initiates the heart
beat. Propagation of the impulse to the atrioventricular node then activates the conduction through
the bundle of His with its branches and further via the Purkinje fibers to the myocardium. The
existence of internodal pathways has been controversial largely due to misinterpretations of
original descriptions of the conduction system. The concept of preferential spread of activation
from the sinus node to the atrioventricular node through ordinary myocardium and not through
"specialised internodal pathways" is sound and proven.
Neurologic control of the heart action is mediated through parasympathetic and sympathetic
branches of the autonomous nervous system.

Pericardium, position and shape of the heart

The heart is located in the lower ventral part of the mediastinum in all mammals and it is covered
by lungs apart from a small area ventrally. The organ is suspended within the pericardial cavity.
The pericardial sac is fixed to the root of the great arteries and the cranial vein and is attached
to the sternum in all animals but the fixation to the diaphragm varies between species. The
attachment to the central tendinous aponeurosis of the diaphragm is firm and broad in man but
in carnivores a ventral ligament is the only attachment between the pericardium and the diaphragm
and in horses and ruminants the caudal part of the pericardium is attached through the
sternopericardial ligaments only. The human type of fixation was noted in pigs, seals, phocids
and beaver.
Compared to man, quadrupeds have less pronounced left-sided orientation and a more ventrally
tilted long axis of the heart. The difference in the in vivo orientation of the heart has caused
some confusion in the naming of cardiac structures, especially of the leaflets of the aortic valve.
A simple way to avoid misunderstanding is to describe the aortic leaflets (and sinuses) as "facing"
and "non-facing". Always, in a valve with three leaflets, two of the leaflets are nearest to the
pulmonary valve. Imagining oneself in the aortic valve, looking toward the pulmonary valve,
the left and right coronary arteries can then be described as arising from the left- and right-
facing sinuses, regardless of the location of the aorta relative to the pulmonary trunk. The third
sinus, furthest from the pulmonary valve is non-coronary and non-facing. Ruminants have a
relatively larger right lung than other quadrupeds which causes displacement of 3/5 of the heart