Heart Children

Heart Children
Enjoy your children
Li n da Dav ies • M i chelle Ma nn
Proudly printed and sponsored by
Heart Children
A Practical Handbook
for Parents of Children
with Heart Conditions
Linda Davies
& Michelle Mann
Heart Children
A Practical Handbook
for Parents of Children
with Heart Conditions
Linda Davies
& Michelle Mann
©2013 Linda Davies
Revised and updated March 2013
Copyright 1983 Linda Davies and Michelle Mann
1st edition 1983 – 5,000 copies
2nd edition revised 1990 – 6,000 copies
3rd edition revised 1992 – 10,000 copies
4th edition revised 2000 – 4,000 copies
5th edition reprint 2003 – 10,000 copies
6th edition revised 2013 – 6,000 copies
ISBN 0-473-01674-5
Published by:
@Heart Inc.
National Office
Supporting heart kids
through life
PO Box 108034
Symonds street
Auckland 1150
Freephone 0800 543 943
www.heartnz.org.nz
Other publications available from @Heart
Educating Children with Cardiac Conditions by Teresa Kedzlie and Mary Crosbie
Knowing But Not Knowing: Finding Out About Your Baby’s Heart Condition
Before Your Baby Is Born
Contents
Dedication
viii
About the Authors
ix
Foreword
x
Acknowledgements
xi
Introduction
xii
Chapter One: Understanding the Normal Heart
1
Introducing the Normal Heart
1
How the Heart is Formed
2
Foetal Circulation
4
Changes at Birth
6
The Normal Heart
8
Circulation10
The Heart Pacemaker
12
Heartbeat12
Pulse12
Respiration12
Valves14
Chapter Two: Congenital Heart Conditions
15
Introduction15
Heart Murmurs
16
Coarctation of the Aorta
16
Congenital Aortic Stenosis
16
Congenital Pulmonary Valve Stenosis 18
Patent Ductus Arteriosus (PDA)
19
Septal Defects
Atrial Septal Defect Ventricular Septal Defect 20
20
21
Tetralogy of Fallot
Transposition of the Great Arteries
Truncus Arteriosus
Tricuspid Atresia Pulmonary Atresia
Ebstein’s Anomaly of the Tricuspid Valve
Atrioventricular Canal (AV canal)
Hypoplastic Left Heart
Heart Diagram
Transposed heart diagram for illustrating heart conditions
A checklist of items to be discussed with your child’s doctor
If surgery is required
22
23
24
25
26
27
28
29
30
31
32
32
Abnormal Heart Rhythms
33
Wolff-Parkinson-White Syndrome (W-P-W Syndrome)
33
Tachycardia33
Complete Heart Block
34
Acquired Heart Disease 34
Congestive Heart Failure
34
Endocarditis34
Cardiomyopathy35
Congestive Cardiomyopathy
35
Restrictive Cardiomyopathy
35
Hypertrophic Cardiomyopathy 35
Rheumatic Fever 35
Kawasaki Disease
37
Chapter Three: Initial Reactions
39
Shock 39
Denial40
Anger 40
Guilt41
Sadness41
Detachment42
Gradual Acceptance
42
Living on
42
Everyone has Feelings
42
Making sense of it all
45
Chapter Four: Investigations
Blood Pressure (BP)
Electrocardiogram (ECG)
Chest X-Ray
Echocardiography (Echo)
Cardiac Catheterisation (Cardiac Cath)
Electrophysiogical Studies (EP)
Magnetic Resonance Imaging (MRI)
Chapter Five: Medical Procedures
47
47
48
49
50
50
51
51
53
The Fontan Operation
53
Artificial Pacemaker
54
Transplants54
Medications55
Diuretics 55
Ace Inhibitors
55
Digoxin 56
Drugs to Treat Abnormal Rhythms
56
Antibiotics 56
Chapter Six: Your Child in Hospital
59
Waiting59
In Hospital 59
For Parent or Caregiver
60
Who’s Who in Hospital
62
Doctors You May Meet During Your Stay
62
Nursing Staff
62
Other Staff
62
Social Workers
62
Chaplains63
School Teacher
63
Play Specialist
64
Relationships Between Parents and Hospital Staff
64
Chapter Seven: If Your Child Requires Surgery
67
Preparation67
Preparing Your Child
68
Telling Your Child About the Surgery
68
Hospital Stay
68
Pre-operation 69
Operation Day
69
Aftercare70
Return to the Ward
70
Chapter Eight: Feeding Your Heart Baby
73
Baby’s Growth
73
How Much Is Enough?
74
Increasing Calories
74
How Much Weight Gain Is Normal?
74
Medication and Feeding
74
Breastfeeding75
Some Breastfeeding Difficulties
75
Feeding Times
75
Bowel Movements
76
Extra Fluids
76
Mother’s Medication
76
For the Mother Who Wants to Express Her Milk
77
Aids to Milk Expression
77
Take One Day at a Time
77
Bottle Feeding Your Heart Baby
77
Feeding Position
78
Starting on Solid Food
Chapter Nine: Nutrition and Your Heart Child 78
81
Nutrition for Older Children with Heart Conditions
81
Nutrition Basics
82
The Importance of Energy
82
Growth83
How to Achieve a High Energy Diet
83
Boosting Energy
83
Toddlers and Preschoolers
86
Examples87
Teenagers87
Chapter Ten: Realities of Life at Home: Living with a Heart Child
89
Will I cope?
89
How Do I Treat My Special Child?
89
Discipline?90
In What Activities Can My Child Become Involved?
90
Dental Treatment
91
Antibacterial Prophylaxis for dental procedures 91
Hints for Cleaning Your Pre-schooler’s Teeth
92
How Can I Tell If My Child Is Unwell?
92
Speak to Your Doctor If Your Child:
92
Pregnancy92
Grandparents and Other People
94
Dealing with Other People
94
Your Life
94
Sitters95
Cardiac Resuscitation 95
Susceptibility to Other Ailments
95
Effects of Common Childhood Diseases
96
Effects of Congenital Heart Defects on the Brain
96
Growing Up with a Heart Condition
96
Some Children Do Die – Dealing with Your Fears for Your Own Child
97
Your Feelings When Another Heart Child Dies
97
These Feelings Are Normal
98
If Your Child Dies
98
Glossary: Reference for Heart Parents
Support Groups
99
110
@Heart110
Parent to Parent New Zealand
110
La Leche League
111
References
112
Dedication
This handbook was written because in 1980 two beautiful children were born.
Robert Davies
and
Jessica Elizabeth Mann
We would like to thank our husbands Ivor and Graeme for their continued
support over the years and their patience while we were yet again rewriting
this handbook for parents.
Enjoy your children
Peace and best wishes
Linda and Michelle
viii | DEDICATION
About the Authors
Linda Davies M.Ed, (Hons) Post.Grad. Dip Tchg. Grad. Dip. Not-for-Profit
Management; is the mother of five children. Her fourth child Robert was born
with a major congenital heart defect. Robert was born at home, transferred to
National Women’s Hospital and then to Green Lane Hospital, where Linda met
Michelle and Jessica.
Linda’s experience in La Leche League, where information on breastfeeding
children with special needs was available, led her to an expectation that written
information about congenital heart conditions would be available. When it was
not, she set about finding information for herself, and with Michelle Mann, wrote
the first Heart Children book.
Linda and Michelle made contact with families to fill in questionnaires as to
what they wanted in the book, and the book still follows these requests in the
6th edition. A special edition was published in 1994 for the Australian Cardiac
Association Limited to be distributed to families of children with heart conditions
in Australia.
Linda’s work with the establishment of the Parent and Family Resource Centre
Inc., lessened her involvement in Heart Children Inc. A hui was convened it
the mid 1990s to set up a board to oversee the running of Heart Children New
Zealand Inc. This meant that Heart Children Inc. became more organised and
established as a major network of support groups. In 2011 Heart Children NZ
Inc. rebranded themselves as @Heart Inc. to extend their support to the growing
group of adults living with CHD.
Michelle Mann Dip Tchg. is a mother of three children with one grandchild. Her
second child Jessica was born with a major congenital heart defect. Michelle
came to Green Lane Hospital with Jessica and shared a windowless room with
Linda for about six weeks. They got on really well together and while Linda did
the research, Michelle did the editing and corrected the spelling mistakes.
Linda and Michelle both attended the Parent to Parent Basic Helping Skills course
then went on to set up support groups for Heart Children around the country.
ABOUT THE AUTHORS | ix
Foreword
The first edition of this book in 1983 was a great tribute to the commitment and
dedication of two extraordinary mothers, Linda and Michelle. In their experiences
of loving and losing their children, Robert and Jessica, they realised there was a
need for accessible written information and set about to remedy the deficit. To
now achieve the 6th edition is a remarkable accomplishment that will continue
to help parents traverse the difficult journey of living and loving a child with a
heart condition.
Learning that a child has a heart defect either before or after they are born
is distressing and painful for any family. The scramble to make sense of
information and to deal with potential outcomes of treatment possibilities is often
overwhelming at a very difficult time when other major decisions are also being
made. Having clear information that is written by people who have walked their
own path in this journey makes this book a very special resource for all families.
In this day of electronic information there are so many options available but this
book will be accessible for heart families in New Zealand without them having
to worry about internet access and computer skills.
Extra diagrams have been added to this edition. Other sections have reflected
the greater use of fetal echocardiogram and counselling for future pregnancies.
Parents will find an up-to-date resource that provides an excellent starting base
in their search for information and understanding. This useful and practical book
is user friendly, presenting information from a parents’ perspective which gives
it enormous credibility.
This book has been written primarily for the families of heart children but will also
be of immense benefit to health professionals as it contains excellent diagrams
that can be used to identify and demonstrate specific details of each child’s
individual heart condition. Throughout the years I have used and valued each
successive edition of “Heart Children”. It will be a privilege to use the new edition
to support families as they begin, or continue in their journey with their own heart
children.
Heather Spinetto – Paediatric Cardiac Liason Nurse
x | FOREWORD
Acknowledgements
We would like to acknowledge the help, support and encouragement given to
us by the following people:
Heather Spinetto – Paediatric Cardiac Liaison Nurse
Dr. Tom Gentles – Cardiac Surgeon
Julie Neilsen – Branch Coordinator @Heart
Megan Maguire for proof reading
Jo Carter for proof reading
We are very grateful to The Hugh Green Trust in conjunction with @Heart Inc.
without whose financial support this publication would not have been possible.
We would also like to acknowledge and thank The Commonwealth Fund of New
York for the use of illustrations from Helen B. Taussigs M.D.’s 1947 publication
Congenital Malformations of the Heart.
We are also very appreciative of the time and resources donated by DPOD, in
particular Trudy McLauchlan for her invaluable assistance in co-ordinating the
process.
ACKNOWLEDGEMENTS | xi
Introduction
This handbook is for parents.
It sets out to provide answers to some of your questions about what is happening
to your child and also about coping with the day to day care of your child. It is
not meant to be a medical text. For detailed information on your child’s condition
you will need to consult with your General Practitioner, your Cardiologist and the
team of Specialists who will care for your child.
This handbook has been prepared and written by parents, for parents. As many
heart conditions are diagnosed during pregnancy and in the first year of life,
it refers generally to the baby and young child. We hope that parents of older
children will also find it useful.
Copies are free to parents of children with heart conditions however, a donation
to the national office @Heart would ensure that publication of this handbook
continues.
Copies can be obtained from:
@Heart
PO Box 108 034, Symonds Street, Auckland 1150
Phone 0800 543 943
www.heartnz.org.nz
or from regional @Heart branches.
An up-to-date list of your local @Heart branch can be located on the web
page: www.heartnz.org.nz
xii | INTRODUCTION
Chapter One
Understanding the
Normal Heart
Introducing the Normal Heart
The term ‘Congenital heart disease’ refers to an abnormality in the heart, or its
great vessels, developing before birth. In New Zealand almost one baby in one
hundred is born with a heart defect; that is about five hundred a year.
Some of these are very simple, producing no problems throughout life. Others
are more complicated, needing surgery to correct them, and a few cannot be
corrected.
So that you can begin to accept your child’s condition and learn what is involved,
it may be helpful to have a little knowledge of how the heart is formed, how it
functions in the womb, and what changes occur in the heart when your baby
is born.
As you come to understand the complicated series of events required for the
heart to develop and function normally, you will find it easier to see how congenital
abnormalities can occur.
CHAPTER ONE | 1
How the Heart is Formed
The heart begins to develop towards the end of the first month of foetal life. By
the eighth week of pregnancy the fully developed replica of an adult heart has
been formed.
A.
A. The process begins with a group of cells
forming themselves into a hollow tube.
One end of the tube is called venous
and the other arterial.
B. T
he hollow tube grows faster than the
space around it and as it is in a confined
area it begins to bend.
B.
C. The venous end expands and forms
both left and right atria.
C.
2 | CHAPTER ONE
D. The middle section of the original tube
expands into a bag shape, which will
form the left and right ventricles. A
further group of cells forms the mitral
and tricuspid valves.
D.
E. At the arterial end of the original hollow
tube, a partition divides the once single
tube into two channels, the aorta and the
pulmonary artery. Other cells build the
tissue of the aortic and pulmonary valves.
E.
F. E
ight weeks after
conception the foetal
heart looks like a
miniature adult heart.
F.
CHAPTER ONE | 3
Foetal Circulation
Two months after conception the unborn baby’s (foetal) heart looks like an adult
heart, but for the function of circulation, the foetus remains dependent upon its
mother.
1. The taking up of oxygen for the growing baby and the removal of waste
products, occur in the mother’s placenta.
2. Oxygenated blood passes into the foetus through the umbilical cord,
which leads directly to the main vein inferior (vena cava) and on into the
right atrium.
3. Because there is an opening between the atria (foramen ovale), some of
the blood from the right atrium passes to the left atrium.
4. The remaining blood from the right atrium enters the right ventricle ...
5. ... and is pumped into the pulmonary artery.
6. Instead of flowing the length of the pulmonary artery, most of the blood is
directed before it reaches the lungs, into the patent ductus arteriosus; a
blood vessel that connects the pulmonary artery with the aorta.
7. As the lungs do not breathe while inside the womb, they need only enough
blood to nourish the growing tissue.
8. The oxygenated blood flows through the foramen ovale into the left atrium,
reaches the left ventricle and is pumped into the aorta.
4 | CHAPTER ONE
CHAPTER ONE | 5
Changes at Birth
1. When the cord is clamped and the baby begins to breathe, the ductus
arteriosus begins to close, thus closing off the communication between
the pulmonary artery and the aorta; during the following two or three weeks
the unused blood vessel shrinks to form a solid cord of tissue.
2. Now the blood pumped from the right ventricle can flow only into the
lungs.
3. Returning to the left side of the heart through the pulmonary veins.
4. With the closing of the patent ductus arteriosus the pressure in the right
atrium decreases, while the pressure in the left atrium increases. These
changes in pressure cause the foramen ovale to close up and seal, later
becoming fibrosed in this position and forming the intact inter-atrial
septum.
6 | CHAPTER ONE
CHAPTER ONE | 7
The Normal Heart
A full-grown heart is shaped like a blunt cone about the size of a man’s closed
fist. It weighs less than a pound and lies in the centre of the chest slightly to
the left side between the lungs. It is made of muscle enclosed in a protective
membrane called the pericardium.
The heart has four chambers. Two collecting chambers (the right and left atria)
and two pumping chambers (the right and left ventricles). The atria and ventricles
are each divided by a muscular wall called the septum. The atria and the ventricles
are separated by valves which permit only a one-way flow of blood. The aorta is
the main artery from the heart, and the entrance to the aorta is protected by the
aortic valve. The pulmonary artery leads from the heart to the lungs. The entrance
to the pulmonary artery is protected by the pulmonary valve. The superior and
inferior vena cava are veins that return blood from the body back into the heart.
8 | CHAPTER ONE
NORMAL HEART
CHAPTER ONE | 9
Circulation
Circulation is a continuous process.
1. From the left atrium, the blood moves down into the left ventricle.
2. The ventricle pumps it into the aorta, which is the main artery of the body.
3. From branches in the aorta the blood passes through other arteries, and
then through tiny vessels (capillaries), to supply all the organs and tissues
in the body.
4. After its journey round the body, the blood returns along the veins to the
superior and inferior vena cava through which it passes into the right
atrium.
5. From there it passes down into the right ventricle.
6. It is then pumped on its much shorter journey through the pulmonary
artery into the lungs. In the lungs the blood releases the carbon dioxide
it collected on its journey round the body, and picks up a new supply of
oxygen.
7. It then returns through the pulmonary veins to the left atrium, to begin a
new circuit of the body.
10 | CHAPTER ONE
CHAPTER ONE | 11
The Heart Pacemaker
Without an internal control the ventricles would beat at a rate of about 40 beats
a minute, which would be insufficient to meet the needs of the body. The pace
for the heartbeat is set by a special group of cells called nodes, in the wall of
the right atrium. The upper, called the sinoatrial or SA node has a faster natural
rate than the atrio-ventricular or AV node below it, and acts as the pacemaker,
because it sets the pace for the rest of the heart by exciting all the heart muscle.
1. The pacemaker acts like a spark plug for the rest of the heart. The SA node
fires impulses that spread across the two atria, causing them to contract.
2. The AV node passes impulses from the atria to the conductive tissue
known as the Bundle of His.
3. The Bundle of His conducts the impulses down into the ventricles and
causes them to contract, thus bringing the heartbeat to an average rate
of 70 beats a minute.
Heartbeat
If you listen to the heart through a stethoscope, you will find that there are actually
two distinct sounds for each beat, something like ‘lub-dup’ (the ‘lub’ is a longer,
booming sound, while the ‘dup’ is a shorter snapping sound). These two sounds
correspond to different events in the heart as it contracts and relaxes.
The ‘lub’ sound is made by the contraction of the ventricle and the abrupt closing
of the inlet valves between the chambers. The ‘dup’ is made by the closing of
the outlet valves of the aorta and pulmonary artery after each gush of blood is
pumped past them. The heartbeat rate changes with age. In the womb the foetal
heart beats at more than 140 beats a minute. In childhood the rate decreases
to about 90 beats a minute; and the normal resting rate is 70 beats a minute.
Pulse
Pulse rate is the rate at which the heart beats. When taking a pulse at the wrist,
what you feel is the beat of the artery walls in rhythm with the heartbeat.
Respiration
A newborn baby takes about 40 breaths every minute when resting. During
childhood the breathing rate falls and by adult life is down to 13–17 breaths a
minute. A child with a heart condition may have a faster than normal breathing
rate. This may be due to an abnormally high blood flow through the lungs, or to
12 | CHAPTER ONE
CHAPTER ONE | 13
heart failure. The number of breaths a minute gives the doctor an indication as
to the child’s condition.
Valves
The entrances to the aorta and pulmonary arteries and the openings between the
atria and ventricles, are protected by valves. The inlet valves between the atria
and ventricles are called atroventricular valves. They consist of circles of fibre to
which flaps or cusps are attached. The valve between the right atrium and right
ventricle is called the tricuspid valve as it has three flaps. The valve between the
left atrium and left ventricle is called the mitral valve. It is smaller, with only two
flaps. It is stronger and thicker than the tricuspid valve in order to cope with the
left ventricle’s workload of pumping blood through the body.
The free ends of the flaps of the mitral and tricuspid valves are held in place by
fibres. When the heart relaxes, the valves open and the blood flows freely through
the atria into the ventricles. When the ventricles contract, the blood is forced
backward, pushing against the flaps of the valves until they close. The fibres
are just long enough to keep the valves tightly closed, without letting them flap
upward, otherwise blood would leak back into the atria.
The outlet valves that protect the entrance to the aorta and pulmonary artery
are called semilunar valves, because they have three flaps shaped like little
half moons (as in diagram). These valves also allow blood to flow only in one
direction. When the ventricles contract, blood pushes up against the flaps of the
semilunar valves until they open and the blood then flows freely into the arteries.
When the ventricles relax, the flaps of the semilunar valves act like little bowls.
Blood quickly fills them and pushes them downward until they meet and close
off the opening. If the valves stayed open, blood would leak back into the heart
instead of being forced into the arteries.
14 | CHAPTER ONE
Chapter Two
Congenital Heart
Conditions
Introduction
As parents we may find ourselves searching for a reason as to why our child
was born with a heart defect. We may find ourselves checking back through our
family tree, just in case some distant relative happened to have a heart condition.
We may also try to remember back to the first few weeks after conception in the
hope that we can find the cause.
These are normal feelings.
It often seems that it would be easier to accept and come to terms with the defect
if there was a definite cause. But with a congenital heart defect, it is unlikely that
you will find the cause.
Studies are being carried out into the effects of environmental poisons, alcohol,
drugs, etc. on the unborn child. At present there is no evidence indicating
that any substances other than thalidomide or high – dose alcohol cause
heart defects. In some cases a baby’s heart may not have developed properly
because the mother had German Measles (Rubella) during the first few weeks
of pregnancy. A heart defect is usually a chance occurrence in the complex
development of the heart. However in recent years, it has become clear, that
some complicated problems are associated with abnormal genes. But most
children with heart defects have normal genes. Sometimes a child’s heart defect
is part of another condition, for example Down Syndrome, Williams Syndrome,
Marfans Syndrome etc.
CHAPTER TWO | 15
The following section outlines some of the more common defects. No two
children are alike, and you will not find two children with heart conditions that
are exactly the same. The information that follows is intended to give you some
guidelines that will help you as you try to piece together the facts about your
own child’s condition. We have made no attempt to explain the procedure used
in the correction of the defects, or to speculate about the outcome of surgery.
We emphasize that the information and diagrams are meant only as a guideline.
For detailed information on your child’s condition you will need to consult your
child’s doctor. Please read carefully the information on Endocarditis, as it has
relevance for all children with heart conditions.
Heart Murmurs
A doctor listening with a stethoscope can usually hear extra sounds, commonly
called murmurs. A murmur may indicate that a valve is not working correctly, or
that there is a ‘hole in the heart’. Often the kind of sound will give the doctor a
good idea what kind of problem exists.
Sometimes a heart murmur can be heard in a perfectly normal heart. Generally an
experienced doctor can tell the difference between a heart murmur that means
trouble and one that does not mean anything. The meaningless kind of murmur
is often referred to as an ‘innocent’ heart murmur. Many children with innocent
heart murmurs eventually outgrow them and have normal sounding hearts as
adults, though the doctor may want to check every now and then to make sure.
Coarctation of the Aorta
In this condition there is a narrowing of the aorta. Pressure builds up in the left
ventricle, the same way that the pressure builds up in a hose when you turn the
nozzle to a narrow opening. As a result the left ventricle may get thicker walls
and for a while this helps the heart work more effectively. After a certain point
the heart gets less effective and heart failure may occur.
Some infants with coarctation of the aorta develop severe heart failure and must
be operated on immediately, but in many cases the narrowing is not so severe,
and the operation can be delayed.
Congenital Aortic Stenosis
In this type of heart condition there is an obstruction to the flow of blood from
the left ventricle into the aorta.
The obstruction may be due to:
16 | CHAPTER TWO
CHAPTER TWO | 17
1. A partial narrowing of the aortic
valve.
2. A ridge of tissue in the aorta
above the valve; this is called
supravalvular aortic stenosis.
3. A ridge of tissue in the aorta
below the valve; this is called
subvalvular aortic stenosis.
4. A thickening of the muscle in
the left ventricle, just below the
valve. This is called idiopathic
hypertropic subaortic stenosis.
Congenital Pulmonary Valve
Stenosis
Congenital pulmonary valve stenosis consists of a narrowing of the valve of the
pulmonary artery. As with aortic stenosis the narrowing causes a build up of
pressure in the respective ventricles the same way that pressure builds up in
a hose when you turn the nozzle to a narrow opening. As a result the ventricle
may develop thicker walls to help the heart work more effectively. After a certain
point the enlarged ventricle becomes less effective and heart failure may occur. If
the obstruction is severe, heart failure will occur and heart surgery is necessary.
18 | CHAPTER TWO
Patent Ductus Arteriosus (PDA)
The ductus arteriosus is a blood vessel between the aorta and the pulmonary
artery, which shunts blood away from the lungs before birth. Ten to fifteen hours
after birth, the ductus begins to constrict, forming a solid cord of tissue within
two to three weeks. If the blood vessel does not close when supposed to, there
is an open connection between the aorta (which has a higher pressure) and the
pulmonary artery. Some of the oxygenated blood which should be pumped out of
the aorta and on to nourish the body tissue, is sent back into the lungs, circulating
needlessly between the heart and lungs. A child with patent ductus arteriosus
(PDA) may become breathless, have a slower weight gain and tire more quickly.
CHAPTER TWO | 19
Septal Defects
Septal defects are holes in the muscular wall (septum) between the left and right
sides of the heart. If there is a hole between the upper or collecting chambers,
it is known as an atrial septal defect, ASD for short. If it is between the lower
or pumping chambers, it is known as a ventricular septal defect, VSD for short.
Atrial Septal Defect
When an atrial septal defect is present, blood that has just returned from the
lungs leaks from the left atrium (which has as slightly higher pressure) into the
right atrium, so that it is sent back to the lungs again. In this type of septal defect,
the pressure in the pulmonary blood vessels does not usually rise, but the heart
may enlarge and the body may not get enough nourishment. The child with
this condition may tire more quickly and be more prone to chest infections, but
sometimes symptoms do not appear until adulthood.
20 | CHAPTER TWO
Ventricular Septal Defect
If there is a hole in the wall between the ventricles, oxygenated blood will flow
from the left ventricle (which has a higher pressure) back into the right ventricle.
Then when the heart contracts, the oxygenated blood will be sent back to the
lungs, instead of out from the aorta into the body. As a result, the pressure is
raised in the pulmonary blood vessels, and they become thicker than normal.
The heart has to work extra hard to pump enough blood through the body, and
can become enlarged.
A child with a large defect in the ventricular septum may have a slower weight
gain, tire easily and be more prone to chest infections. These problems often start
in infancy. Some septal defects require corrective surgery, while others correct
themselves as the child grows.
CHAPTER TWO | 21
Tetralogy of Fallot
Tetralogy of Fallot is a medical condition named after the doctor who first
described it. It is a combination of four different defects:
1. There is a narrowing of the pathway and valve leading to the pulmonary
artery, so that the flow of blood to the lungs is decreased and pressure
becomes high in the right ventricle.
2. A hole in the septum between the ventricles allows blood to flow from one
ventricle into the other, so that unoxygenated blood flows out of the aorta
again without passing through the lungs.
3. As a result of these defects, the right ventricle becomes much more
muscular.
4. The aorta over-rides both ventricles, and receives blood from both
ventricles. A child with Tetralogy of Fallot receives an inadequate supply
of oxygen and may have a blue tinge to the skin (cyanosis). The cyanosis
gradually or rapidly becomes more severe and surgery is required.
22 | CHAPTER TWO
Transposition of the Great Arteries
Transposition of the great arteries is a condition where:
The aorta rises from the right ventricle instead of the left ventricle and the
pulmonary artery rises from the left ventricle instead of the right ventricle.
This means that:
1. The blood carrying oxygen from the lungs, returns to the left atrium via
the pulmonary veins.
2. It passes on into the left ventricle and is pumped up the pulmonary artery
back into the lungs.
3. The deoxygenated blood returning from the body enters the right atrium
via the vena cava.
4. It passes into the right ventricle and is pumped up the aorta to circulate
around the body again.
At first enough blood passes through the open ductus to maintain adequate
oxygen levels in the blood but as the ductus closes, serious cyanosis occurs.
For the body to receive oxygen from the lungs the child must have to have a septal
defect, which will allow the oxygenated and deoxygenated blood to mix. This
allows some blood carrying oxygen to be pumped out of the aorta and around
the body. A child with transposition of the great arteries receives an inadequate
supply of oxygen and is blue soon after birth, requiring emergency surgery.
CHAPTER TWO | 23
Truncus Arteriosus
In this condition the pulmonary artery arises from the ascending aorta where
the pulmonary blood flow originates. A large ventricular septal defect is always
present. Surgical treatment is usually required in the first few months of life.
24 | CHAPTER TWO
Tricuspid Atresia
Tricuspid Atresia is a condition in which the tricuspid valve has not formed
between the right atrium and right ventricle. Blood is able to circulate only if the
child has septal defects. The right ventricle may be smaller than the left.
1. Blood returning from the body enters the right atrium through the vena
cava.
2. It passes through the atrial septal defect into the left atrium, where it mixes
with blood carrying oxygen.
3. From the left atrium it passes to the left ventricle.
4. Some of the blood then passes through the ventricular septal defect to the
right ventricle, to be pumped into the pulmonary artery and on to the lungs.
5. The remainder is pumped up into the pulmonary artery and on to the body.
The child with Tricuspid Atresia receives an inadequate supply of oxygen and
may have a bluish tinge (cyanosis), to the skin. He may have a slower weight gain,
tire more easily and be more susceptible to chest infections. Cyanosis gradually
becomes more severe and an operation is necessary.
CHAPTER TWO | 25
Pulmonary Atresia
Pulmonary atresia simply means a blockage of the pulmonary valve. Sometimes
a ventricular septal defect is also present, sometimes not. In either case there
is no normal pathway to the lungs and blood can only reach the lungs through
a patent ductus, or through abnormal arteries coming directly from the aorta.
The baby cannot usually live if the ductus closes, but this can be kept open
temporarily with Prostaglandin E1 until a shunt operation is performed. Later
in life it is often possible to carry out a second operation to provide a more
normal circulation.
26 | CHAPTER TWO
Ebstein’s Anomaly of the Tricuspid Valve
Ebstein’s Anomaly is a condition where the tricuspid valve is malformed and is
positioned too low, allowing blood to leak backwards from the right ventricle to
the right atrium.
1. When the foramen ovale is completely closed, the blood from the right
atrium flows into the right ventricle and is pumped through the pulmonary
artery to the lungs, where it is oxygenated.
2. The oxygenated blood is returned by the pulmonary veins to the left atrium.
3. It flows to the left ventricle and is pumped out by way of the aorta on to
the body.
4. It is returned in the normal fashion by the superior vena cavae to the right
auricle.
EBSTEIN’S ANOMALY
CHAPTER TWO | 27
Atrioventricular Canal (AV canal)
This condition is particularly common in children with Down Syndrome.
The atrioventricular canal defect occurs right in the middle of the heart where the
atrial septum meets the ventricular septum, and the mitral and tricuspid valves
form. As a result there is a large hole between the two sides of the heart and the
valves are abnormal.
In the partial type of AV canal defect, the hole between the two sides of the
heart exists only at atrial level, but in the complete form it extends through to
the ventricular level. The valves which are supposed to attach to the missing
area are deformed.
As with any other atrial or ventricular septal defect, blood returning to the left
side of the heart crosses the hole and circulates once more round the lungs.
In addition, the tricuspid and mitral
valves may develop a significant
leak back into the left or right atrium.
The heart pumps a much bigger
volume of blood than normal, the
baby becomes breathless and
develops heart failure.
Sometimes this is prevented
because the baby develops a
protective mechanism. The millions
of tiny arteries and arterioles do not
open up in the usual way, so that the
blood flow through the lungs never
becomes very high. If the valves
are working reasonably, the baby
may be quite well but progressive
damage occurs to these minute
arteries. Generally an operation is
possible for AV canal defect.
If the ventricular septal defect
is large, an operation is usually
needed in the first few weeks of life.
28 | CHAPTER TWO
Hypoplastic Left Heart
In this condition the left ventricle of the heart has not developed properly and is
very small; either the mitral or aortic valve, or both may be narrowed or absent;
the aortic arch may be very narrow. The foetus is not affected by this abnormal
development but at birth the situation changes.
When the baby has to breathe on its own and the pulmonary blood vessels open
to the flow of blood, the underdeveloped left side of the heart is unable to pump
the oxygenated blood around the body.
Survival in this situation for a brief time is possible while the patent ductus
remains open. The aorta receives its blood through the patent ductus, which
permits some blood in the pulmonary artery to flow down the descending aorta
and a smaller portion to flow back up around the malformed aortic arch to the
coronary arteries.
Surgery is now available for babies with this condition. But three operations are
usually required the first in the new born period.
CHAPTER TWO | 29
A checklist of items to be discussed with your child’s doctor
1. Nature of defect.
2. Treatment required for defect.
3. How the condition/treatment will
affect your child.
4. How to contact your local @Heart
group.
If surgery is required
1. The plan for surgical treatment of
the defect.
2. What risks there may be.
3. How you should prepare your
child for going to hospital.
4. What to expect when your child
wakes up after the operation.
5. Recuperation in hospital.
6. Continuing care at home.
NOTES
30 | CHAPTER TWO
The normal heart
Heart Diagram
This is a diagram of the normal heart. The following sketches can be used for
your doctor to illustrate your child’s condition.
CHAPTER TWO | 31
Transposed heart diagram for illustrating heart conditions
32 | CHAPTER TWO
Abnormal Heart Rhythms
Wolff-Parkinson-White Syndrome (W-P-W Syndrome)
This condition is named after the three individuals who first described it.
Wolff-Parkinson-White syndrome is recognized by characteristic changes on
the electrocardiogram, which indicate the presence of an additional pathway
or shortcut from the atria to the ventricles. This abnormal pathway allows the
electrical signal to arrive at the ventricles too soon, and can cause bouts of very
fast heart rates (tachycardia).
Many patients with W-P-W syndrome have no symptoms and have no episodes
of tachycardia. If a child does have episodes of tachycardia these often can be
controlled with medication. However, occasionally such treatment is unsuccessful
and after further tests of the heart’s electrical system, it may be necessary to
have surgery to interrupt the abnormal pathway.
This is now possible using special techniques during cardiac catheterisation and
this treatment is recommended for most children with persistent problems. The
eletrophysiology specialist plots out the abnormal pathway causing the problem
and blocks the pathway by “radio frequency ablation”.
Some patients with W-P-W syndrome can lead normal lives with no restrictions
on their activities. This is true even for those patients who have episodes of
tachycardia.
Tachycardia
Tachycardia is a very fast heart rate. Sometimes the heart can beat at such a
rapid rate that it doesn’t function effectively and medications may be needed to
slow it down to a normal rate. A very fast heart rate may or may not be associated
with a congenital heart defect or W-P-W, but many are not.
The most common type of abnormal tachycardia is called paroxysmal atrial
tachycardia (PAT), sometimes now called supraventricular tachycardia (SVT).
The fast heart rate originates in the upper chambers, or the upper portion of the
electrical conduction system.
Ventricular tachycardia is a fast heart rate in which the beating originates in the
ventricles. This is rare in children. Although the heart may not beat as fast with
ventricular tachycardia as it does with supraventricular tachycardia, function of
the heart is often poorer.
CHAPTER TWO | 33
Complete Heart Block
Heart block means that there is a failure of the heart’s electrical signal to pass
in a normal manner down its conduction system. Heart block may be present
at birth in a baby with an otherwise normal heart, or be associated with certain
types of heart problems, or occur after some surgery.
Complete heart block means that the atria and ventricles beat independently at
their own rate. When present at birth it is called congenital complete heart block.
Most babies grow and develop normally and can participate in normal physical
activity. Later in life the heart rate frequently slows and an artificial pacemaker is
required at some stage, in almost all patients.
Acquired Heart Disease
In contrast to congenital heart disease acquired heart disease is caused by
conditions that occur after birth. Rheumatic fever and endocarditis are such
conditions.
Congestive Heart Failure
This condition does not mean that the heart will stop working, but only that it
is not pumping enough blood to supply the body for normal functioning and
activity. Patients usually show excessive tiring, laboured breathing and/or fluid
accumulation, (oedema).
The fluid can accumulate in the lungs causing laboured breathing or in the rest
of the body where it may create swelling of the legs, abdomen, or eyes. Diuretics
are used to help get rid of any extra fluid and other drugs are used to control
blood pressure and protect the heart. It is also necessary to avoid excessive salt
in the diet and salty foods.
Endocarditis
A child with congenital heart disease, or a child whose heart has been damaged
by rheumatic fever and sometimes a child who has had an operation to correct
a heart defect, may be at risk from endocarditis.
Endocarditis is an infection of the endocardium, (the smooth lining of the heart
which covers the inside of the chambers, the surface of the four valves and the
inside of the great veins and arteries). Endocarditis is serious and difficult to
treat. The serious complication is damage to heart valves, causing heart failure.
34 | CHAPTER TWO
Endocarditis can be caused by bacteria entering the blood stream through the
teeth or gums. The risk can be lessened by taking particular care of your child’s
teeth and by the practice of good oral hygiene. It is also important to take your
child to the dental clinic or dentist regularly.
Cardiomyopathy
This term means that the heart muscle is abnormal. There are three types of
cardiomyopathy; congestive, restrictive and hypertrophic.
Congestive Cardiomyopathy
In this form the heart muscle is damaged, loses some of its contractile power,
and goes “flabby”. The muscle contracts poorly, the heart dilates and heart
failure develops.
The cause is often unknown. Sometimes a biochemical insult, (in adults most
often alcohol), but more often a virus infection, is suspected. Viruses can cause
inflammation of the heart known as myocarditis, and this can produce heart
failure.
Breathlessness and tiredness occur and the heart enlarges. Pain is not usually
a feature. The inflammation burns itself out within a few days or weeks and the
heart usually returns to normal, but sometimes it is permanently damaged.
Restrictive Cardiomyopathy
This is much rarer than congestive cardiomyopathy. The heart muscle suffers
some damage and fibrosis occurs, making it stiff. The heart muscle becomes
hard to fill but doesn’t enlarge. The cause is not always known. Breathlessness
and tiredness secondary to heart failure are the main symptoms.
Hypertrophic Cardiomyopathy
This condition is inherited and it is completely different from congestive or
restrictive cardiomyopathy. The main feature is abnormal thickening (hypertrophy)
of the left ventricular muscle. The heart is hard to fill and the thickened muscle
may bulge into the cavity of the ventricle when it contracts. This may obstruct
the outflow and also tends to interfere with the function of the mitral valve. Many
people have few if any symptoms with this condition but others may become
breathless, dizzy or blackouts may occur, and abnormal rhythms may develop.
CHAPTER TWO | 35
Rheumatic Fever
This illness can affect the heart in two ways. The acute illness, rheumatic fever,
can temporarily involve the heart, but permanent damage is produced by the
subsequent rheumatic heart disease which may follow years later.
Rheumatic fever is primarily a disease of children between the ages of 7 and
14, although cases occur into the 20s. It starts as a throat infection due to a
particular class of bacteria, Streptococcus. Most streptococcal throat infections
respond quickly to penicillin and do not affect other organs, but a few children
are more sensitive to the organism and a more generalised disease, rheumatic
fever, follows a few weeks later. The organism is still confined to the throat but
its effects are felt elsewhere.
Rheumatic fever causes the membrane around the joints and heart valves to
become swollen and inflamed. After a while the swelling goes down and the
joints recover completely. Characteristic skin rashes and uncontrolled jerking
movements may also be present. Abnormal noises (murmurs) may be heard from
the heart valves. Some do persist once the disease is finished and when valve
inflammation is severe, they may be permanently damaged. A period of rest in
hospital is required to settle the inflammation down. Occasionally emergency
surgery is required with a very bad attack.
Long-term treatment with penicillin is required to ensure that there is no further
infection with Streptococcus, as this may cause further rheumatic fever with very
high risk of further valve damage.
The mitral valve is affected most often, followed by the aortic and tricuspid
valves. The pulmonary valve is not affected to any significant extent, however
with careful treatment valve problems can be minimized. Unless the attack of
rheumatic fever is allowed to settle, progressive damage occurs to the valves.
36 | CHAPTER TWO
Kawasaki Disease
Kawasaki Disease is named after Dr. Tomisaku Kawasaki, a Japanese doctor
who identified the disease in 1967. Kawasaki Disease causes high fever, enlarged
lymph glands and swollen blood vessels. It can also cause damage to the heart. It
affects more boys than girls and is usually found in children under five years old.
Kawasaki Disease can affect the heart in the following ways;
• Coronary artery aneurysms
• Leakage of valves
• Accumulation of fluid around the heart (called pericardial effusion)
The most serious of these complications are the coronary artery aneurysms.
The coronary arteries are the blood vessels that take the oxygen rich blood to
the heart muscle. An aneurysm is an area of a blood vessel that has become
dilated and swollen. When an artery is dilated the blood flows through it more
slowly because of the lower pressure. This change in pressure and speed makes
it easier for blood clots to form which can lead to heart attacks.
In time, the coronary artery aneurysms may heal. Problems can also happen
at this stage because the healed sections may be narrower than the rest of the
artery (stenosis).
CHAPTER TWO | 37
Chapter Three
Initial Reactions
The birth of a child with a heart condition has a profound effect on the parents
and indeed on the whole family. It is likely that such an event has significant
implications for each member of the family.
During pregnancy it is probable that you will have been anticipating with pleasure
the birth of a normal child and possibly have imagined various activities that you
might participate in with your child as he grows up.
The process that parents usually go through when told their child has a disability
or special need, is similar to the grieving process that follows a death or any loss.
After the birth of a child with a disability, feelings experienced by parents, have
been reported as shock, denial, anger, sadness, detachment, gradual acceptance
and living on (Gargiulo,1985: Hornby 1987).
Be reassured that it is natural to experience these feelings quite strongly, that it
is not unhealthy or unnatural to have them; that there is nothing to be ashamed
of. Some parents experience these feelings more intensely than others and take
a longer period of time to adjust. More than one reaction or feeling may be
experienced at any particular time.
Shock
The first reaction is usually that of shock. You may have a feeling of numbness at
first, when you are told that your child has a heart defect. It may seem to others
that you do not appear to care. This is your mind’s way of protecting you, and
allowing in, only the amount of pain and upset that you are able to handle at this
time. You might become nauseous, or tearful.
CHAPTER THREE | 39
This is a normal healthy response.
As the shock fades you may experience other feelings.
• you may feel like you are living in a dream
• you may become forgetful and lack concentration
• you may feel that it is difficult to make decisions
• take time with any major decision you may have to make.
At this stage it is important for you to receive accurate information about your
child’s condition. You may need to be told the same information several times.
Denial
The feeling that follows shock is usually denial. The information that has been
received is so disturbing that the inclination is not to accept or acknowledge it.
You may:
• feel disbelief and want to deny the facts
• refuse to accept that your child has a heart condition
• even wish for your child to die and feel guilty for having such thoughts.
The denial reaction is a defence mechanism. The facts are denied because the
truth is so traumatic. Denial gives you time to come to terms with the situation.
Anger
Anger is a common response when disappointment is experienced or hopes are
dashed. The feeling may be “why me, or us – what did I/we do to deserve this?”
It is natural to have feelings of anger with your child or his disability but because
it is unacceptable to express that anger you may take it out on others.
This may take the form of:
• anger with your partner, relations and friends
• anger with hospital staff
• anger with God
• anger with your other children.
It helps if you are able to talk about it and have your feelings acknowledged.
40 | CHAPTER THREE
Guilt
This may be experienced along with, or at the same time, as anger. You may
blame yourself and think that if you had done things differently it would not have
happened. In most cases there is no clear reason why your child was born with
a heart defect, but you may find yourself searching for a reason. You may hear
yourself asking futile questions in an attempt to rationalise it, “Was it medication
I took? Was it the cold virus I had? Is it hereditary?”
When you find this happening, recognise it for what it is – a sense of guilt and the
search for an answer. If you could have prevented the heart defect, you would
have. You and your family are not to blame. It can be helpful to have the support
of another person who can act as a “sounding board”. Someone, who will listen
without judgment and will be there for you.
Sadness
When the feelings of anger pass they are usually replaced by feelings of sadness.
Crying is a good way to release this feeling. You may hold back the tears because
you are afraid that if you start you will be unable to stop, but you will. You may
feel too embarrassed to cry in front of people, or you may be concerned about
their discomfort. Don’t be! It is not only acceptable, but may be helpful to share
your sorrow.
There is a concern that at this stage sadness may lead to depression.
The battle to come to terms with your child’s condition may bring with it bouts
of depression. You may;
• keep thinking “if only I could change places with my child”
• share his pain
• not wish to face another day
• feel unable to cope with what is happening to you and your child.
Remember sadness may be the first sign of acceptance
It can be frightening waiting;
• for a diagnosis
• for results of tests
• until an operation
CHAPTER THREE | 41
• during an operation.
You may find it helpful and reassuring to seek further knowledge of your child’s
condition.
Detachment
Following sadness there may be a period of detachment. You may feel flat and
empty and feel that life has no purpose. At this time you might find it helpful to
make contact with other parents who have children with heart conditions. They
will be able to give you support through listening and sharing their experiences.
Gradual Acceptance
Gradual acceptance of your child’s condition can begin when adequate information
and support has been made available to you. One parent (Featherstone, 1981)
prefers to refer to this stage as coping rather than acceptance.
At this stage you may have to cope with the difficulties of your child’s condition.
You will feel more confident and able to cope if you have been fully involved in
your child’s care and treatment.
Living on
Remember that you don’t need to feel positive or loving towards your child every
moment of the day, just as when parenting a child without a heart condition, you
don’t have to be perfect.
It is still important that you talk through your feelings with your partner, a caring
friend, a member of your local @Heart Support Group, Parent to Parent or similar
support group, your Minister, the Hospital Chaplain or your Doctor.
Everyone has Feelings
Your partner: Most people looking in from outside, think that a sick child brings
partners closer together. Actually the opposite is more often true. You may have
a tendency to blame your partner for your child’s condition. Everything you ever
fought about, disagreed over, disliked in each other, may surface as you work
your way through this distressing time. Be aware that you may both be more
susceptible to a reliance on alcohol or medications to alleviate your fears.
Your own feelings of guilt, anger, shock and sadness may be projected on to your
partner just because he or she is available You may expect your partner to be a
mind reader, assuming that your feelings are naturally known even when you fail
42 | CHAPTER THREE
to share them. You may resent your partner for not sharing his or her feelings.
Remember, you are an individual and everyone copes in his or her own way.
There are times when you will be able to share your feelings, and times when
your feelings call for space and distance. Remember too, the feelings we talked
about earlier will be experienced by your partner as well as yourself.
Your other children: Having known for many months that a baby will be born,
parents are reasonably ready for the experience, and usually have done their best
to prepare their children for the birth of a new brother or sister.
The normal delivery of a healthy baby is a cause for excitement and joy, feelings
that are passed on to the child at home. If the newborn baby has been found
to have a heart condition, and mother and baby have an unexpected stay in
hospital, problems may arise and children may need extra reassurance from
everyone.
When a heart defect is not detected until the baby has been home for some time,
an older child may feel guilty or even responsible, thinking it was something he
did that caused his sibling’s problem.
If children at home are old enough to understand, you can explain that the baby
is sick, and that you may be staying in hospital. Reassure them of your love, and
let them know you would stay with them too, if they should ever be in hospital.
While living in hospital, try to maintain personal contact with your older children
who must remain at home. If the hospital is close, your children can be taken to
visit you both. If distance prevents visiting, communication can be maintained by
letter, postcards addressed to the children, by phone, or e-mail or Skype. Take
lots photos of your baby so that you can either send them home or share them
with your children at home.
When you do come home from the hospital, some jealously may occur, and your
children may cling to you and quarrel with each other more than usual, so again
you may need to reassure them of your love.
Fathers: As a father, your way of coping with the worry of your child’s condition
may be different from that of your partner. Your job may not allow you the
flexibility or freedom to spend as much time as you would like at the hospital
with your child, or accompany him to clinics, doctor’s appointments etc. The
mother usually has the responsibility of these jobs. They can be a great strain,
and you can help your partner by letting her know that you realise this.
Some fathers tend to become so involved in their work and the need to provide
CHAPTER THREE | 43
that they end up having less time for their families than ever before. This need
to work and become involved outside the home is a way of trying to cope with
a difficult situation.
You may find yourself ignoring or avoiding the situation by working too hard, or
continually doing things that give you no time to think of your family or to help
them. You could be depriving them of the support of your presence, when they
need it most.
One of the best ways you as a father can help is by making a point of being with
your child in hospital as often as you can. Visiting time for parents is very flexible
and some fathers have found that evening visits, when they stay until the child
is asleep, fits in well with their working days. You can maintain regular contact
through phone calls, texts, or emails so you know what is happening.
It may be helpful if both parents are present when doctors are doing their ward
rounds. There are times when they would prefer to discuss your child’s condition
with you both. This means you get information first-hand and are able to get
answers to any questions you might have. It also helps you feel that you are not
being left out.
To have a child with a heart condition is not an everyday problem. It is a situation
that is difficult for one person to cope with alone. Even if you feel your partner is
holding up very well, they will appreciate your presence and moral support. You
may be amazed at the apparent strength of your partner in these difficult times.
You may ask yourself if they are doing so well because they have no alternative.
You might ask if there is more that you could do to support them.
Often the mother takes on the role of principal caregiver and you may feel left out.
You may feel you are not needed, that things seem to go along very well without
you. In fact, you are needed by everyone, especially your child and everyone
concerned with the care of your child.
You may find it more difficult than your partner does to accept the fact that your
child has a heart condition. Sometimes, with a new baby, you may feel that you
do not want to become too attached to your child in case he dies. A heart defect
is a rather daunting condition, but nothing to be ashamed of, nor should you
blame yourself or your partner for your child’s condition.
Men have fears and worries too, and if you talk about them it may help ease
the pressure. If you feel left out, admitting this to someone may be the first step
towards finding a solution to the problem. Comparatively few men do share their
feelings. Many seem to feel they should be able to cope alone, but few people
44 | CHAPTER THREE
can go it alone successfully without some outside help. You may like to contact
other fathers through your local @Heart group, Parent to Parent, or there may be
a men’s group in your community that you might find helpful.
Making sense of it all
We have tried to introduce you to some of the feelings and problems that may
come your way as you live with your heart child. You may be familiar with them
already. You may have experienced them along with others and tried to make
sense of it all.
You will have realised that every child’s condition is different. Some conditions
are very minor, while others cannot be corrected. Some children die, while others
with similar conditions live.
You will know by now that there are no easy answers to guide you through this
trying time. We hope you will be aware of your feelings, and acknowledge them.
Moving through the range of emotions will bring you to a point of acceptance
and stability, thus helping you to cope and enjoy the future with your heart child.
Living with and loving a heart child is not easy, but it can become a rich experience.
We hope you will work on the richness and love and enjoy your special child.
CHAPTER THREE | 45
Chapter Four
Investigations
When your child is suspected of having a heart condition, certain tests are
necessary to help find the exact type of heart problem that is present. Not all
children will require all, or even any, of the tests. The decision will depend on
each individual child.
Your child will be given a thorough medical examination to determine which tests
are needed.
Blood Pressure (BP)
Blood pressure is the pressure produced by blood passing through the arteries.
Blood flow pulsates with the heart beat and so does blood pressure.
To measure blood pressure
1. An inflatable rubber cuff is placed around the patient’s arm to constrict
the artery.
2. By squeezing the bulb, the pressure in the cuff is raised and a nurse or
doctor listens through a stethoscope, placed on the arm just below the
cuff.
3. While the pressure in the cuff is lower than in the artery, some blood can
flow through the artery and the sound of its passage can be heard by the
stethoscope.
4. Further pumping on the bulb tightens the cuff enough to shut off the
circulation and the sound stops.
CHAPTER FOUR | 47
5. Gradually air is released from the cuff and the nurse or doctor can hear
the renewed blood flow. This upper level is called systolic and it registers
on the gauge of the sphygmomanometer.
6. The nurse or doctor continue to listen until the sounds disappear. This
lower level also registers on the gauge and is known as the diastolic
pressure.
The average adult blood pressure is about 120/70 ( systolic and diastolic).
Children’s pressures are lower, about 80/60 in infants, and 90–100/65 in older
children.
Abnormally high blood pressure is known medically as hypertension and can be
a strain on the heart and kidneys.
Electrocardiogram (ECG)
An electrocardiogram is a recording of small electrical signals from within the
heart. The heart beat is normally started by a small area of pacemaker tissue
(sinus node) in the right atrium. The electrical activity spreads to the rest of the
heart, triggering a co-ordinated contraction of the atria and ventricles.
The signals are very small, in the region of 1,000th of a volt (a torch battery
often generates 1.5 volts), and so the electricity has to be amplified in an
48 | CHAPTER FOUR
electrocardiograph. A recording is taken by attaching wires to conductor plates
on a person’s arms and legs and over the heart.
The electrocardiogram may reveal whether the heart is going too fast (tachycardia)
or too slow (bradycardia), and is able to illustrate other abnormal rhythms. Further
information as to the state of health of the ventricular and sometimes atrial
muscle may be obtained.
Diseased or overloaded heart muscle becomes electrically abnormal. For
example, a shortage of oxygen in the ventricular muscle produces abnormalities
in the recovery from contraction, which can be seen on the electrocardiogram.
Higher pressure in either the right or left ventricle produces typical changes and
abnormal heart rhythms are readily demonstrated.
Chest X-Ray
X-rays find the solid structure of the heart more difficult to penetrate than the airfilled lungs around it. So the heart shows up as a shadow on the chest X-ray with
the lungs on either side. Abnormalities in the shape or size of this shadow help in
the diagnosis of heart disease. The appearance of the lungs can also be useful.
The size of the heart silhouette is an indicator of heart failure. The ventricles dilate
and their walls thicken in an attempt to maintain cardiac output in heart failure
or where there are leaking valves. This increases the overall size of the heart.
The big heart can be detected by examination but the chest X-ray is more
sensitive. The appearance of the lungs is also helpful in the diagnosis of a failing
left ventricle. The fluid (oedema), that is pushed out of the blood vessels by rising
pressure can be seen in the lungs on X-ray.
Sometimes a sideways (lateral) view will be taken as well as the normal chest
X-ray. This provides a different view of the heart and lungs.
Although the chest X-ray is an important investigation it does have limitations. In
CHAPTER FOUR | 49
most cases it will only reveal the outline of the heart – a silhouette, not a detailed
portrait. The amount of radiation in a chest X-ray is exceedingly low and probably
not significant unless 20 or more are taken in one year.
Echocardiography (Echo)
Echocardiography is a means of visualising the shape and movement of
structures inside the heart. It works by the same principle that sonar depends
on, to find submarines or shoals of fish. High frequency sound waves, well above
the range that can be heard by man or animals, are passed through the heart.
When they hit any change in density, such as the boundary between muscle and
blood, some of the waves are reflected back.
With the help of suitable electronics a picture of the heart can be built up and
displayed on a screen. Similar echo techniques (often referred to as ultra sound
or a scan) are used to visualise an unborn baby, its liver, kidneys or other organs.
The “ultrasound” waves are produced by a special crystal, which is mounted
in a probe and held onto the patient’s chest. The waves travel easily in fluid or
solid structures but are dispersed by air, so a special jelly is applied to ensure
air free contact between the probe and the chest wall. An electrocardiogram is
often recorded simultaneously to time contraction and relaxation of the heart
accurately.
The ultrasound beam moves from side to side like a very fast windscreen wiper
so that a two dimensional pattern is obtained. This method (2D echo) produces
the picture that would be seen by cutting a thin slice from the heart. It is displayed
on a television screen to show the movement of structures. The beam is pointed
in various directions to build up a more complete impression of the heart. In
addition the direction and velocity of blood flow can be shown by computer
processing in black and white and in colour.
The sound waves produce no sensation at all and are too high pitched to be
heard. They are quite harmless. It can be readily done on children. It is especially
useful in the newborn who are difficult to investigate by other means.
Cardiac Catheterisation (Cardiac Cath)
To help sort out the final details, a cardiac catheterisation may be carried out.
To gain entry into the heart, a tube (catheter) is passed into a large artery or vein
and then moved towards the heart. The position of the catheter is followed on an
X-ray screen. By means of the catheter, the pressure can be measured inside the
heart, blood samples can be taken and X-ray opaque fluid (angiocardiography)
50 | CHAPTER FOUR
can be injected to show up the patterns of blood flow.
Angiocardiography, high speed X-ray motion picture photography or video,
records the passage of the dye through the heart and blood vessels, outlining
valve damage or other heart defects and showing up obstructions of blood
vessels. Later, doctors can replay the film or videotape again and again, to study
the problem in detail.
When adults require catheterisation they are mildly sedated, but babies and
children may be unable to lie still and can be frightened by the injection of local
anaesthetic, so the catheter is performed under general anaesthetic.
In general, patients are back to normal within a few hours although it will take at
least until the next day to return to their previous level of activity. There may be
slight bruising around the area where the catheter has been inserted and as a
precaution against any bleeding the patient must rest a while.
In recent years it has become possible to treat a number of conditions during
cardiac catheterisation. The majority of patent ductus can be closed by passing
small coils or a special devise through the catheter into the ductus. Around
50% of atrial septal defects can now be closed with a device delivered through
the catheter, as can a small number of ventricular septal defects. Abnormal
vessels can also be dilated or closed as necessary in complicated anomalies.
With this approach your child may be discharged the day after the procedure,
or sometimes even later on the day of the procedure.
Electrophysiogical Studies (EP)
More detailed investigations are sometimes required for children who have
repeated problems with heart rate and rhythm. This involves inserting several
small fine catheters into different parts of the circulation and recording the
electrical activity directly from the inside of the heart.
Different areas of the heart can also be stimulated by these catheters to assess
the electrical abnormality and abnormal rhythms can be ablated (knocked out)
to cure the problem.
Magnetic Resonance Imaging (MRI)
This involves placing the child in a strong variable magnetic field. Images
are produced looking at the alteration of electrical forces in different parts of
the body. It is possible to see structures clearly and to measure blood flow
accurately. It involves lying still for a long time and is usually done under full
anaesthesia in children.
CHAPTER FOUR | 51
For online information
♥ Log on to www.heartnz.org.nz
♥ Click on “What we do”
♥ Click on the link “about congenital heart disease”
♥ From there you can access a New Zealand approved medical site where
you will be able to obtain more detailed information about the above
investigations.
Chapter Five
Medical Procedures
The Fontan Operation
Some infants are born with a heart condition, which is impossible to correct.
Examples are pulmonary atresia where the right ventricle may be tiny and useless;
aortic atresia where the left ventricle is tiny and useless; or very complicated
anomalies where it is impossible to rebuild the heart, so that one ventricle can
pump blood to the lungs and the second ventricle pump it around the body.
In 1971 Dr Guy Fontan described an operation where the blood from all the veins
bypasses the heart and flows directly to the pulmonary arteries leading to the
lung. The superior vena cava (SVC) is joined to the pulmonary artery from above,
the inferior vena cava (IVC) from below.
Over the last 40 years many improvements have been made to this procedure
allowing many babies to achieve a reasonable life span with only modest
limitations. The first step is now undertaken in the first week after birth and two
further operations may be required over the following five years. This series of
operations obviously carries greater risks than straightforward surgery, but step
by step, the outlook for many babies has been greatly improved.
In embarking on this course it always has to be understood that the expected life
span maybe less than middle age, the baby must go through repeated tests and
operations and that he may never be able to undertake really strenuous activities.
Sometimes a family might decide that they do not wish to follow this course,
particularly when chances of a long-term period of good health are more remote.
On the other hand many young adults are now living a rewarding life and are
grateful for this chance, even though they know that their lives can never be
CHAPTER FIVE | 53
quite the same as the lives of their more fortunate friends and that their longterm outlook is uncertain.
Artificial Pacemaker
If the heart rate becomes too slow to meet the normal requirements of the body,
symptoms such as tiredness, shortness of breath, dizziness or light-headedness
may occur. Tests will be carried out to establish whether an artificial pacemaker
is required.
There are times when an artificial pacemaker could be required after complex
cardiac surgery.
An artificial pacemaker consists of a battery driven electrical circuit. It weighs
about 125gms and is about the size of a match box. The battery can be implanted
under the skin and connected by a fine wire, either to the outside of the heart
(epicardium), or to the inside of the heart (endocardium), using a vein.
Most pacemakers can be programmed. The rate, power supply and timing, can
be altered externally after the pacemaker has been inserted. This can be done
with an electromagnetic “programmer” to suit the pacing needs of the individual.
Some pacemakers are termed “physiological”. This means that they can alter
the rate of pacing in response to exercise. Regular follow-up checks of the
pacemaker are required. In later years it may need to be replaced.
There is very little interference from outside electrical circuits but strong electrical
fields such as dodgem cars, arc welding, etc., should be avoided.
Transplants
The first heart transplants were carried out in the late 1960s, however rejection
was a problem, so very few took place until the late 1970s. By this time much
had been learnt about the control of rejection of the heart and the more powerful
drug, cyclosporine became available. Early transplants were carried out only in
adults but they have become more common in children in the last 25 years or so.
The period spent waiting for a donor, the ordeal of the operation and the weeks
(or months) of postoperative care, place a severe strain on all the family. It is of
course a big decision to go ahead with a heart (or lung) transplant.
The chances of surviving an operation are good but drug treatment is required
throughout life to control rejection. The child’s own body sees the heart as foreign
and without carefully monitored treatment it can damage the cells so that the
heart becomes unable to support life. Monitoring requires repeated blood tests
54 | CHAPTER FIVE
and the taking of samples from the heart through a device passed through the
veins. This makes big demands on the child and the family and a transplant
would only be considered when no other treatment is possible.
There are adult transplant services available in New Zealand, however for a child
(over one year old) to receive a transplant a family would need to relocate to
Australia. This would be considered only after careful and detailed discussions
with the family who must have a clear picture of everything that is involved.
Medications
Your heart child may require medication to help control his condition. The
following section describes some of the medications used in the treatment of a
child with a congenital heart condition.
Diuretics
A diuretic is a substance that increases the output of fluid from the body and
reduces fluid causing congestion. The common diuretics used are Frusemide
or Chlorothiazide.
The use of diuretics may deplete the blood of potassium and in so doing, upset
the delicate balance of salts in the body. Some potassium can be replaced by
increasing the amount of foods high in potassium content in your child’s daily
diet, e.g. bananas, fresh orange juice, wheatgerm and green vegetables.
In some cases, it is necessary for your child to be given a potassium supplement
to maintain adequate levels of the blood. Some diuretics are called potassium
‘sparing’, which means they do not remove potassium from the body, but actually
tend to retain it. When diuretics are taken every day, often Amiloride or Aldactone
are prescribed to help maintain potassium levels.
Ace Inhibitors
ACE inhibitors relax blood vessels so that they lower blood pressure. They
also have a protective effect on heart muscle. The main use in children is the
protection of hearts which have damaged muscle, controlling blood pressure
in children with leaking mitral or aortic valves, or a combination of the two. In
patients with significant valve leaks the heart muscle may be kept healthy and
an operation may be postponed for many years.
Patients with damaged heart muscle and complex lesions may be protected
and kept in reasonable health. Captopril, Cilazapril, and Accupril are the ACE
inhibitors commonly used. Larger doses can impair kidney function and cause
CHAPTER FIVE | 55
the potassium to rise, so that periodic blood checks are needed, particularly
while dosage is being decided.
Digoxin
One of the earlier drugs for the control of heart conditions was originally a folk
remedy. It is over 200 years since William Withering heard that an old lady in
Shropshire was treating people with dropsy (they were over loaded with fluid
because of heart failure) with a tea brewed from the foxglove plant.
The chemical in this brew was digitalis, which is still used today, although diuretics
and ACE inhibitors are now the first choices. Digitalis is also used because of its
ability to control some tachycardias. The dosage must be carefully supervised.
Drugs to Treat Abnormal Rhythms
The most common abnormal heart rhythm in children is a very fast rate triggered
in the atrium, the supraventricular tachycardia. Ventricular tachycardia, which
is generally more dangerous, occurs mostly in adults who have suffered heart
attacks. It is uncommon in children, although it can occur in some families who
have an abnormal ECG with what is called the long QT interval. This is where
the ventricular muscle takes a long time to recover from each electrical impulse.
Ventricular tachycardias may be treated with a Beta blocking drug of which
there are many. Examples are: Propranolol, Atenolol and Celiprolol. These drugs
suppress the activity of the sympathetic system and adrenaline, both of which
stimulate the heart muscle.
Beta blocking drugs may also be used in controlling supraventricular arrhythmias.
Another type of drug is the “Type I” which alters the flow of electricity in heart
muscle. The third type of drug is Amiodarone which has a complicated function
and is used both for supraventricular and ventricular arrhythmias. It is very
potent, but has side effects which restrict its use.
Radio frequency ablation is the ultimate tool for abnormal supraventricular
rhythms which do not respond well to other treatments.
Antibiotics
The word antibiotic is a combination of two Greek words, anti – against and biosis
– life. Some antibiotics act by killing the infecting bacteria, others by stopping
their multiplication and permitting natural defences to fight them successfully.
If a child with a heart condition develops an infection with bacteria, the bacteria
56 | CHAPTER FIVE
can spread through the blood to the heart. Bronchitis, pneumonia, tonsillitis,
infected sores and boils are examples.
A doctor should be consulted to decide if treatment with antibiotics is indicated.
If your child has a minor sniffle or virus, do not insist on an antibiotic, as antibiotic
treatment is ineffective and unnecessary with viral infections. Your doctor may
take tests to determine the best treatment.
If your child is having antibiotic treatment and is not improving, or appears to
be suffering from side effects, tell your doctor so that other treatments can be
considered.
Be sure to check with your pharmacist for the best time to give antibiotics, as
food in the stomach may interfere with the absorption of the drug. Give it round
the clock, do not miss a dose, since its effectiveness depends on keeping the
proper amount of the drug in the system.
If you do miss a dose give it as soon as you remember. Give the next dose at
the normal time. Give all the antibiotic. Do not stop it because your child seems
better after a few days. If you stop giving it too soon, not all the disease-causing
bacteria will be destroyed and re-infection can occur. Keep all liquid containers
tightly closed and follow storage directions carefully.
CHAPTER FIVE | 57
Chapter Six
Your Child in Hospital
Waiting
Some operations (especially in infants) must be done as an emergency, but in
many, some delay may be advantageous to your child if his condition is stable,
because of the benefits of extra growth.
In Hospital
To live in with your child or not?
Having a child go into hospital may cause many disruptions to your normal
way of life. It can be a disturbing experience for the child who must go and
for every member of the family. Each family’s needs and fears are unique, and
consideration will have to be given to your heart child’s needs, those of older
children and those of your partner and yourself.
Other aspects such as finance, travel and the requirements of your household
will influence your decision. Each family must decide, what is best for them. It
is easier if you, as a parent, can go into hospital with your child, but there are
times when neither parent can do this. Hospital staff understand this and seem
to give these children that little extra attention.
If you live close to the hospital but cannot stay with your child at the time, you
will want to decide when it is important to be there. He will most likely need you
on the first night in hospital, on the nights before surgery, and during more painful
or frightening treatments.
Although a preschool child’s needs may be more acute, do not under estimate
the importance of your presence to the older child, or teenager.
CHAPTER SIX | 59
When you leave your child after a visit, expect that he may cry. This is a normal
healthy reaction. Calmly tell him that you are leaving, but that you will be back
that evening, or next day (or whenever it may be). Whatever you tell your child,
be sure it is the truth. You might like to leave something which belongs to you,
for him to take care of until you return.
If it is possible for you to stay in hospital with your child, you will have first-hand
information on his progress and you will always be available to accompany him
for X-Rays, blood tests etc.
If you believe your child needs you while he is having treatment or a test, say so.
Do not be swayed by being told that “children behave better when mother isn’t
there”. Maybe they do some times, but it is only normal for babies and children
to cry when they hurt. Cuddling your child or breastfeeding your baby during,
or immediately following a painful or frightening procedure, can give you both
considerable comfort.
Please be reasonable in your requests as there are some surgical procedures at
which it is just not practical or safe for parents to be present. We are all different;
if you find the tests or procedures distasteful or scary, you may pass on your
distress to your child. If this is the case, it would be far better to leave your child
in the care of the staff. You can always be with him afterwards.
If your child is older, you will be able to plan your trip to hospital in advance.
Talk to him beforehand. Make your explanations simple and truthful. Let your
child know that you will be with him as much as possible. Be sure your child
understands that the doctors and nurses will help to make him well, even if some
things may be frightening or uncomfortable.
Reassure your child about coming home when he is well. If you live close to
the hospital, take your child for a visit beforehand. If your child has a favourite
toy or blanket, take it along when he is admitted. Nurses like to know all they
can about your child, his pet name, special tastes, foods to be avoided, words
for body functions and so on. It is important to mention any medication or food
which may cause an allergic reaction.
For Parent or Caregiver
If you are living in hospital looking after your child, it is very important that you
look after your own health, do not skip a meal because you have not been able
to leave your child. Start the day with a sensible breakfast. Eat a reasonable
amount at lunch time and in the evening. Keep plenty of fresh fruit, nuts etc.
available for in-between snacks. Make sure that you drink enough, particularly
60 | CHAPTER SIX
if you are a breastfeeding mother.
Although you will be doing less than you would at home, take time to rest each
day. When you are under stress and living away from the rest of your family,
you may find it hard to relax and sleep. Worrying about your child is physically
exhausting. Five minutes meditation or quiet time with your feet up may be
beneficial.
While living in hospital, you may find you are not getting the exercise you would
normally get. Take a walk outside every day. Use the stairs and not the lift where
possible If you jog, bring your shoes into hospital and go for a short run. Make
the effort to get exercise each day, as you will feel better and more able to cope.
Sometimes, even though things look bleak in hospital, it is wise to go out for a
couple of hours if practicable. It is amazing how this can put things back into
perspective.
If you are a mother with a newborn baby and have been transferred from a
maternity hospital to a hospital caring for heart children, the same post-natal
checks should be carried out on you, as would be carried out in a maternity
hospital. If not then ask.
CHAPTER SIX | 61
Who’s Who in Hospital
Doctors You May Meet During Your Stay
Anaesthetist
Cardiologist
House Surgeon
Trainee Intern
Paediatrician
Registrar
Surgeon
A doctor who administers anaesthetics.
A doctor who specialises in the diagnosis and
treatment of heart disease.
A doctor who, while working is gaining further
experience.
A medical student gaining further experience.
A doctor who specialises in the diseases of children.
A doctor who, while working is training to be a
specialist.
A doctor who specialises in operations.
Nursing Staff
Charge Nurse
Manager
Nurse Practitioner
Nurse Specialist
Staff Nurse
Student nurse
Enrolled nurse
Hospital Aid
In charge of the ward.
Nurse with advanced clinical skills in Paediatric
Cardiology.
Nurse with advanced knowledge who liaises with
health professionals and/or families.
Registered nurse.
Nurse in training.
NZ registered enrolled nurse.
A person employed for technical tasks.
Other Staff
Physiotherapist
Occupational Therapist
Dietician
Pathologist
Technicians ECG, Pacemaker, ICU Radiologist
etc.
Play Specialist
Hospital School Teacher
Social Workers
Chaplain
Social Workers
Social workers are attached to the hospital and are part of the multi-disciplinary
team of people available to help you. Make use of them. In some hospitals it is
62 | CHAPTER SIX
up to the parent to find out about and approach the social worker.
You can find out from the doctor, clinic or charge nurse how to get in touch with
the social worker, if he does not approach you.
The social worker can be a great help to almost any parent. He is really an inbetween person, trained to help parents in all kinds of ways. He knows what
services are available and can help with all kinds of problems. These may be of
the practical everyday kind, such as transport, or financial assistance. He can
give you information about any services available to assist you. He can put you
in touch with them and advocate on your behalf if necessary.
He is available to listen, if that is what you need and can also help with personal
things. If you are having trouble managing your heart child, or any of your other
children, or if you have problems with your relationship, the social worker is a
good person to approach.
His job is to be available and to help you sort things out. If you need more
specialised assistance he will help you to get it.
Very few people can come through a major crisis, such as having a child with
a heart condition, without needing some kind of assistance from outside the
family. Feeling that you just can not cope alone is nothing for you to be ashamed
of and it can be of great benefit to you and your family if you ask for help when
you feel you need it.
You will meet many medical people during your stay in hospital, each will have a
special job to carry out, as one of the team of specialised people who will care
for your child.
Chaplains
A Chaplain is always available and willing to support you and your family in times
of stress.
School Teacher
The hospital teacher provides a therapeutic programme designed to give support
and reassurance about schoolwork. Where possible, lessons take place in the
hospital classroom but children can receive assistance at their bedside.
The teacher is a link with the “outside world”. He encourages communication
between the child and the child’s usual school, through phone calls, text, emails,
faxes, cards etc. If your child’s hospital stay is a long one, the teacher can assist
with a programme at home during convalescence and re-entry to school.
CHAPTER SIX | 63
Play Specialist
The goal of the hospital play specialist is to help children and their families cope
effectively with health care experiences by:
• providing developmental and therapeutic play suited to the ages of the
children
• imparting information about the healthcare setting, its personnel and its
environment
• preparing children for treatments and procedures using various play
techniques
• establishing supportive relationships.
Many play experiences are provided on the ward, both in the playroom and at
the bedside. The play specialist caters for all ages from new born babies to
teenagers and includes siblings of patients in the programme.
Relationships Between Parents and Hospital Staff
Relationships between parents and hospital staff may at times be difficult and
rather strained. This is not surprising, as the emotions involved are very strong
ones of loving, caring and hoping. Living in hospital with your child for a long
period of time may be stressful.
Parents and staff need to work together to make relationships as pleasant as
possible. It may take you a while to feel comfortable with some members of the
hospital staff and feel that you can trust them with the care of your child.
The hospital staff do their best to make your child well. There are very few
doctors and nurses who are not totally committed to the children in their care.
They try very hard to understand your feelings and work with you to provide the
best possible care. Like yours, theirs is a difficult job and understanding on both
sides is not always easy.
Occasionally parents and hospital staff may have a conflict of personality or
ideas. If a staff member has upset you, stay calm and make a time to talk with
them about it. It may be a simple matter which you can resolve together. However,
if you are unable to resolve the issue, discuss the problem with the charge nurse,
doctor, social worker, chaplain, or patient advocate.
Complaining is a difficult area (not that there is anything wrong with complaining)
but a parent may feel at a disadvantage, be too shy to say anything, or
64 | CHAPTER SIX
feel that nobody will listen. The staff always seem to have so much control
that you may feel powerless and that your child is no longer yours, but the
property of the hospital. Remember, you are the child’s parent. If there
is something worrying you or your child, do not pretend that all is well.
If you are worried or angry, give yourself time to cool off, as emotion is likely to
complicate the situation. Choosing the time and place to discuss a problem is
important. In private is better than in public. If you can wait, make an appointment
to see the person concerned.
Any complaint is more effective if your partner, a relative or friend is present. If
the situation is delicate, talk it over in confidence with someone you trust. If you
feel you would like to talk to someone outside the hospital, contact your @Heart
Support person, Parent to Parent or the Patient Advocate.
CHAPTER SIX | 65
Chapter Seven
If Your Child
Requires Surgery
Preparation
When the time comes for your child to have surgery, it is best for you to be as
well prepared as possible.
The hospital staff will inform you as to the procedures in the hospital and you
will be required to sign a consent form for the operation. It is not unusual to have
doubts about giving your consent; it is a normal response when faced with a
very difficult decision.
The doctors will outline what they are going to do so that you have a good
understanding of what to expect. They will also make you aware of any possible
problems and risks that may be involved.
The hospital will inform you as to the procedure in Intensive Care. They will show
you photographs of children in the unit and what they will expect your child to
look like. They will also explain the equipment that will be used to monitor your
child’s condition. You should be able to visit Intensive Care before your child
has his operation.
If the hospital staff have not shown you the photographs, or taken you to visit
Intensive Care, or instructed you as to what to expect prior to your child’s
scheduled surgery, speak to the nurse. This preparation is very important for
every parent.
CHAPTER SEVEN | 67
Preparing Your Child
After the decision has been made to proceed with surgery, it will be scheduled
according to the urgency of the operation and the availability of an opening in
the surgery schedule. In most cases your child will be admitted one day before
his operation. Bring his favourite toys or other familiar possessions as reminders
of home. It is very important that your child be in optimum health at the time
of the operation.
If there is any evidence of a cough or cold, you should contact the hospital, to
decide if the operation should be delayed.
Occasionally, the operating schedule may be disrupted because of an
emergency operation. Both you and your child should be prepared for a possible
postponement of the operation. Out-of-town families need to be aware that they
may have to go home and return at a later date.
Telling Your Child About the Surgery
What you tell your child about the operation will depend upon his age, ability to
understand and his emotional make up. The truth is usually less frightening to a
child, than trying to hide the facts and leaving him to his imagination and fears.
You may not be able to do away with those fears completely but you can help
him deal with them.
Your own outlook will be reflected in your child’s attitude both before and during
surgery. Explain honestly and simply what is wrong, and tell him what must
be done to repair his heart defect. This will help develop a sound and healthy
attitude. Your child need only be told as much as he can understand for his age,
and as much as is necessary to prepare him for the immediate future.
Hospital Stay
During your child’s hospital stay he may turn to you constantly for emotional
support, and this is especially important in preparing him for the time when he
will be in the operating room, then in intensive care. He needs to be reassured
that you will be nearby, waiting to be with him as soon as the doctors feel that
the time is right.
If your child is young he may be afraid that you are leaving him and he may be
hurt in some way. He may also feel that this is a punishment for being naughty
or disobedient, so it is important to give reassurance as to the reason for the
operation.
68 | CHAPTER SEVEN
Pre-operation
Your child will be admitted from one to three days prior to the operation so that
various tests, such as blood tests, X-rays, ECGs, echocardiograms, urine tests
etc., may be carried out. These tests are important, as they give the doctors
information on which to base post-operative treatment.
Prior to the operation you will meet with the surgeon and the anaesthetist. They
will explain what they plan to do, using diagrams if necessary.
Operation Day
On the day of the operation, your child will not be given any food or fluid for five
or six hours before surgery is due to commence. Before going to the operating
theatre he may be given pre-operative medication to calm him.
The time that your child is in theatre, will probably seem the longest hours of
your life. You might like to take some time out from the hospital. Some families
have spent the day with friends, or if they have other children, have taken them
to visit the zoo or museum.
If you are going to be away, give the hospital your phone number in case you are
needed. It is possible that the operation will take five or more hours; there are
sometimes delays and this does not mean that anything has gone wrong. When
this time is spent with other caring people it goes much faster than it would if
you sat around at the hospital.
The operation: It is very difficult to operate on a living, beating heart. It moves
constantly and is filled with blood, so the surgeon cannot see what he is working
on.
Yet, if the heart is not beating, blood does not circulate through the blood vessels,
so the body’s tissues do not receive oxygen and nutritional materials necessary
to sustain life. Some of the tissues such as the brain, are especially sensitive.
Deprived of their oxygen supply for too long, they start to die and cannot be
replaced.
With some of the simpler defects, surgery can be carried out while the heart is
still beating, but for any operation inside the heart, it is necessary to stop the
heart and empty it of blood, to enable the surgeon to repair the defects.
Heart-Lung Machine: During surgery the heart-lung machine is used to carry
out the job of the heart and lungs. Ties are placed on the aorta and main veins
(vena cava). The blood from the vena cava is diverted to the heart lung machine
CHAPTER SEVEN | 69
where carbon dioxide is removed and oxygen is added.
A pump returns the oxygenated blood to the aorta above the tie, to circulate
through the body, thus giving the brain and kidneys a sufficient supply of healthy
blood. With the heart bypassed, the surgeon can work on the now empty, still
heart.
Sometimes the surgeon must stop blood flow for short periods. In the case of a
baby, it is first cooled to a low temperature by cooling the blood going through
the heart-lung machine, so that a period without flow is possible with minimal
risk.
Aftercare
Regardless of the kind of heart surgery, the post-operative care of all patients
follows the same general pattern. Convalescence begins in the Intensive Care
Unit to which your child will be transferred immediately after the operation and
may remain for about a day.
Specially trained nurses, respiratory therapists and other skilled professionals
are at hand. A mechanical ventilator supports your child’s lungs until he is
awake and able to breathe satisfactorily. The staff, with the aid of electronic
monitors and electrocardiograms, watch arterial blood pressure. Precautionary
measures, including the use of antibiotics, will be given to minimise the possibility
of infection.
You are welcome to sit with your child for as long as you wish, as there is no
restriction on the visiting time for parents. Some parents like to stay for a few
hours at a time while others prefer short frequent visits. However, if you are asked
to leave at any time, do so without question.
If you are living in at the hospital and are having difficulty sleeping, do not hesitate
to visit during the night. Seeing your child may help you to relax, so that when
you return to bed you are able to sleep.
The time in Intensive Care is critical and very worrying for a parent. Try to consider
each hour that passes as an hour closer to your child’s recovery.
Return to the Ward
In most instances after a time in Intensive Care, your child will have recovered
enough to return to the ward. There you will be able to participate actively in his
care. A physiotherapist may visit, to help with breathing exercises, coughing and
chest massage. Physiotherapy helps prevent lung complications.
70 | CHAPTER SEVEN
After a few days, emotional reactions in your child may become evident. He may
refuse to co-operate and even appear to be angry with you. This is a normal
response and you will need to reassure him of your love. Nightmares can also
occur, but again, your presence and reassurance will help through this difficult
time.
Following surgery, some children may have a fever for a while. This may be
a reaction to the surgery. If the fever is prolonged, tests and X-rays will help
determine the cause and the best course of treatment.
When your child is eating well, has no fever and doctors are satisfied that recovery
is progressing satisfactorily, it will be time to go home. When discharged from
hospital, your doctor will discuss with you medications, diet, etc. and give you
information relevant for the recovery of your child.
Plans are usually made at this time for follow-up examinations at regular intervals
to check on progress. The hospital doctor will contact your doctor with details
of the surgery and plans for future care.
CHAPTER SEVEN | 71
Chapter Eight
Feeding Your
Heart Baby
Feeding your baby is a rewarding responsibility of parenting. Feeding not only
provides nutrition for your baby’s growth, it also affects growth and social
development.
When your baby gains weight steadily, is content and thriving, you will feel
reassured and happy. But if your baby has a heart condition, the feeding
experience can become frustrating and disheartening because your baby may
fail to gain weight in spite of your persistent efforts.
Baby’s Growth
A baby with a heart condition may show delayed growth. The degree varies
according to the type and severity of the condition. Some factors related to heart
disease that can interfere with growth include: poor appetite, increased energy
needs, decreased food intake because of rapid breathing, frequent respiratory
infections, poor absorption of nutrients from the digestive tract, and decreased
oxygen in the blood (hypoxia) in a very blue baby.
A baby’s failure to grow may also be due to hereditary or genetic conditions or
additional non cardiac conditions. The most common reason for poor growth may
be the baby is not taking enough volume and therefore calories and nutrients.
Even if your baby seems to drink enough, he may still gain weight very slowly,
for reasons still not fully understood.
CHAPTER EIGHT | 73
How Much Is Enough?
Most normal babies drink 120–180mls of breast milk or formula for each 1kg
of body weight. By six weeks of age a baby may be taking up to 600–900mls
of milk each day, increasing to 750–1000mls per day by the age of six months,
then back down to 600–800mls by the age of one year.
Increasing Calories
Each 300mls of milk provides approximately 200 calories. A baby who is not
gaining weight may require as much as 150 calories per kilogram (or 70 calories
per pound) of body weight for daily growth.
A breastfed baby can be given calories in the form of glucose polymers which
can be dissolved in expressed breast milk and given as a paste. This will need to
be discussed with your baby’s doctor or dietician. A formula fed baby can also
be given extra calories by giving a formula with a higher calorific content, or by
the addition of glucose polymer to the formula. Again you will need to discuss
this with your baby’s doctor or dietician.
How Much Weight Gain Is Normal?
A healthy baby usually doubles their birth weight by six months of age. Your
doctor should be able to tell you what the appropriate weight gain for your baby
is. An acceptable weight gain for a heart baby may be less than 500gms (a
pound) per month. It is not necessary for your baby to be weighed frequently at
home, as babies gain and lose weight from day to day, since their appetites vary
just as an adult’s does. Remember your baby’s weight can also be influenced by
medication. Your Plunket Nurse will check your baby’s weight regularly to make
sure growth is steady.
When babies are too sick to be fed from the breast or a bottle, they can be tube
fed, by having a tube inserted through the nose into the stomach. If your baby
has to be fed by this method, it is still important for you to hold and cuddle him.
Medication and Feeding
It is preferable to give your baby his medication before feeding as all babies do
occasionally spill up at the end of a feed.
If he vomits immediately after the medication has been given it may be necessary
to repeat that dose of medicine. Medicine should not be repeated regularly
without discussion with your doctor.
74 | CHAPTER EIGHT
Breastfeeding
You may have already breastfed one or more of your babies, or if this is your
first child, you may have decided prior to the birth to breastfeed. The following
section is for you.
If your child has a heart defect, conditions for establishing a good breastfeeding
relationship are not ideal. But it can be done. Breastfeeding your heart child may
require extra knowledge and often, more patience and commitment. However,
many women who have breastfed in this situation have found that the special
benefits of breastfeeding become more important. They have enjoyed the
emotional closeness and reassurance that comes in this unique relationship.
Breastfeeding lets mother and baby get to know one another in spite of the
complications. In addition, breast milk’s nutritional and immunological benefits
are especially important. If your baby can have any liquid or food by mouth, or
even by a tube inserted through the nose into the stomach, be confident that
your milk is the best.
Well meaning friends, relatives and occasionally, medical personnel, may lack
confidence in breast milk because of their inexperience with breastfeeding. They
may recommend substitute liquids.
Nature intends that a baby should be breastfed for at least the first six to eight
months of life. A baby requires a complete food, one that supplies all that is
necessary, in the ideal proportions and agrees with the digestive system.
It may take time to learn the techniques and gain the confidence required to
bring about successful breastfeeding. However, your positive attitude is a most
important factor, and one that doctors take into consideration.
Some Breastfeeding Difficulties
A baby with a heart defect may have difficulty sucking and tire easily. You
may need to coax your baby to stay awake and encourage him to nurse more
frequently, i.e. every 2–3 hours on demand, with a longer stretch at night. Frequent
breastfeeding or expressing will help build up your supply. Expressing milk after
each feed will also help to increase your supply. Remember the more milk your
baby drinks or you express, the more you will make.
Feeding Times
Heart babies may take longer to feed, as they often take a number of breaks
during a feed. Then too, they often fall asleep after a feed and wake a short time
CHAPTER EIGHT | 75
later, wanting another top up before going back to sleep. This is due to their
special condition. Do not try to hurry your baby or it may make him spill up.
Smaller, more frequent feeds are more easily digested.
If your heart baby is having between six and eight good wet nappies a day, you
can be assured that he is getting enough to drink and that your milk supply is
adequate. A heart baby may not gain weight as rapidly as other babies of the
same age. Discuss with your doctor what weight gains you can expect. Test
weighing a breastfed baby to get an indication as to the amount of milk he is
taking, is only satisfactory if carried out over a period of 24 hours because the
amount of milk taken at different feeds will vary greatly. (If test weighing is going
to be done over the 24 hour period it is important that the same person does the
weighing each time and the scales for the test are not used for any other baby
during the test period.)
Bowel Movements
A breastfed baby’s bowel movement is usually quite loose and unformed, often
the consistency of pea soup. It is yellow, to yellowish green, to brownish in colour.
Sometimes it is only a stain on a nappy.
The frequency of bowel movements varies from once a day to once every few
days, or every nappy every day. This is quite normal. The odour should be mild
and not unpleasant. The medication your baby is given may cause a variation in
your baby’s bowel movements.
Extra Fluids
Giving your baby extra fluid in a bottle after a feed is something which you will
need to discuss with your doctor, as additional bottle feeds will dull his appetite
and desire to suckle. The sucking reflex is so automatic that many babies will
obligingly take a little more of anything that is offered, needed or not.
Mother’s Medication
If you are breastfeeding, be sure that your doctor is informed if you are taking any
medication. Some medications are not suitable for the breastfeeding mother. The
La Leche League International Professional Advisory Board does not recommend
the use of hormonal contraception by lactating mothers, as the hormones are
secreted in the mother’s milk.
76 | CHAPTER EIGHT
For the Mother Who Wants to Express Her Milk
When? As with breastfeeding, little and often seems to produce the best results.
You will find that the frequency and duration of hand pump or breast milker
expression will be dictated by the time you have. Try to work out a schedule,
e.g. 5–10 minutes each side, every 2–3 hours. However, don’t fret if you miss
the occasional session on a ‘bad’ day when you cannot seem to catch up. You
can make up on one of your better days.
Aids to Milk Expression
An electric breast milker may be available for hiring. Check with La Leche League
New Zealand or Parent to Parent Centres throughout New Zealand.
A variety of expressing and feeding bottles are available from your local chemist.
The expressing and feeding bottle is an excellent aid to mothers who need to
express their milk. They are small, very portable and most effective. Please don’t
allow the business of obtaining your breast milk to become an obsession or a
burden upon yourself. For some lucky women it is very easy. For most of us,
it is hard work. So try to regard each few mls of your expressed milk as a very
special gift of love, which you have been able to give your baby.
Take One Day at a Time
The amount of milk which you get will fluctuate from day to day depending on
how relaxed you are. You may have to supplement your breast milk with formula.
We all know that breast milk is the perfect food for babies, but it won’t harm
your baby if you have to put him on formula. For instructions and suggestions on
expressing and storing breast milk, contact your local La Leche League Support
Group or lactation consultant.
Mother to Mother help: The support and help of another woman who has
successfully breastfed her baby can help you overcome many problems and
worries. No book on breastfeeding can equal talking to an experienced nursing
mother. We hope that you will join with other mothers as we did and find the same
reinforcement and support that we have come to know. For more information,
contact your local La Leche League group listed in the phone book. Books on
breastfeeding are listed in the bibliography.
Bottle Feeding Your Heart Baby
We realise that some women will not wish to breastfeed their baby, or that the
circumstances surrounding them, their families and babies will make bottle
CHAPTER EIGHT | 77
feeding the most satisfactory way to meet their needs. We hope you will feel
good about whatever decision you make. The most important thing is that you
love and enjoy your special baby.
Some milk formulas are more suitable that others and your doctor, or Plunket
Nurse will be able to help you choose one. When preparing the formula, follow
the manufacturer’s directions with care.
Feeding Position
Hold your baby in a semi-up-right position with the head well above the stomach.
Swallowing and breathing will be easier in this position. It also helps prevent
choking which can occur when formula falls back into the baby’s breathing
passage.
Holding your baby in this position makes it more likely for the air he swallows to
rise above the milk in the stomach and escape more easily with burping. Many
babies do not need to burp until they have finished a feed. If your baby is sucking
happily, he should be allowed to continue feeding.
After the feed, to make burping easier, hold your baby upright against your
shoulder.Sometimes he may not need to burp and can simply enjoy the cuddle
and being held close. Keeping your baby in an infant seat for a few minutes after
each feed or placing them on their stomach or right side may also make them
more comfortable.
What kind of teat is best? If your baby has difficulty sucking from a regular teat,
try a softer one, or one designed for a premature baby that has a hole large
enough to allow easy flow of formula. When you tip the bottle upside down, the
formula should come out at a rate of several drops per second. If the hole in
the teat is too small, your baby will have to suck too hard and can swallow air.
Too much air in the stomach results in spilling up the formula. You can enlarge a
teat hole that is too small with a red-hot needle or a round tooth pick. Then boil
the teat for 5 minutes or soak in sterilising solution.
For information on bottle feeding consult your Plunket Nurse.
Starting on Solid Food
A baby with a heart condition often breathes too fast, interfering with his ability
to suck. In this case, your doctor may suggest adding solids to the diet in the
78 | CHAPTER EIGHT
first few months. He may also suggest the introduction of solids if your baby has
a low weight gain due to other factors.
If your baby is very young, it may be advisable to discuss with your Plunket Nurse
or dietician what to introduce, as some foods can cause allergies.
The first few meals should be given only as an introduction, not to fill your baby
up, as the digestive system is not geared for solids until about six months of age.
Choose a time when your baby is most relaxed and happy to introduce a new
food. Babies like the plain taste of milk, so first foods also need to be plain. Do
not add extra salt, sugar or honey to food you make for your baby.
Give the milk feed first then offer solids as a “top-up”. Try one teaspoon first
and gradually increase until your baby is having about three to four teaspoons
at a meal.
First foods need to be soft and smooth. Use a blender or push food through a
sieve with a wooden spoon. You can add breast milk, formula or water to obtain
a suitable consistency.
Hold your baby while you feed, or sit him in a high chair. Use a small teaspoon
and put the food in the middle of the tongue.
A suitable first food is mashed ripe banana, followed by other mashed ripe fruit.
You can follow fruit with cereal, such as baby rice, vegetables, and lastly meat.
New foods should be introduced one at a time. This means a single food, not
mixed foods like soup or stew. If your baby does not like it the first time, leave
it for a few days and try again with a small amount. You should not add salt to
your baby’s diet as it may cause fluid retention.
Give more variety as your baby grows. Change the type of food offered, how
much you give and the thickness of the mixture.
CHAPTER EIGHT | 79
Chapter Nine
Nutrition and Your
Heart Child
Nutrition for Older Children with Heart Conditions
The nutritional information in this section was taken from a paper written by
Cherie Mellows, a NZ Registered Dietician.
This information is not intended for the normal healthy child, but for the child
with a heart condition, who has a slow weight gain. Food is essential to life as it
provides nutrients and energy to keep our bodies functioning so that we can be
active and healthy, both mentally and physically.
With heart disease the type and energy value of food eaten is very important.
Nutritional status influences both the ability to fight infections as well as overall
well-being. Good eating habits are important for everyone. Everyone needs the
same nutrients for health and well-being, but in different amounts.
Since everyone differs in health, as well as food likes and dislikes, it is impossible
to design a uniform ‘diet’. Nutrition in heart disease must be individualised and
must focus on higher than normal energy and protein requirements.
Nutritional needs however are not so unusual that different foods must be
prepared at every meal, although some extras will be needed. Keep family meal
planning in mind. Think of how your child’s dietary needs can be incorporated
into the overall nutritional plan for the family and how the meat, milk, vegetables,
fruit and cereals you serve the family can be adapted to the heart child’s diet.
CHAPTER NINE | 81
Nutrition Basics
Nutrients are the basic building blocks of food, which the body uses for growth,
activity and health. The main nutrients are protein, fat, carbohydrate, vitamins
and minerals. Protein, fat and carbohydrates are the nutrients that supply the
body with energy (which is measured in calories). Energy is especially important
for growth and activity.
Protein is used for growth and to build, maintain and repair body tissue. It is
found in foods such as meat, fish, chicken, eggs, milk, milk products, nuts, seeds
and legumes (e.g. beans, lentils). Protein can also be used for energy.
Carbohydrates are used mainly as a ready source of energy. They are found as
starch in foods such as bread, flour, rice, pasta, some vegetables and as sugar
in fruit, table sugar, honey, soft drinks, sweets and jelly.
Fat is also important for energy as well as other body functions. Foods such as
butter, lard, and vegetable oils contain little else except fat. However, many of
the protein foods mentioned before are also good sources of fat.
Vitamins and minerals are needed for the regulation of a wide range of body
functions. No single food will supply these nutrients in the right amount. However,
choosing carefully from a wide range of foods each day is the best way to get
the nutrients we need for health and well-being.
The Importance of Energy
Your child’s nutritional status and his ability to fight infection and stay as healthy
as possible, are closely related. This means focusing on a high energy diet. A
child with a heart defect may require more calories.
This means, just to keep growth and development at a normal pace, the energy
intake must be higher than that needed by a child without a heart condition of
comparable age, height and weight.
Thus, your child should have 100% of normal energy needs plus extra to replace
the energy used in dealing with the heart problem. There is no dietary restriction
for the child with a heart condition. He needs a high energy diet with as large a
range of foods as possible.
If the body takes in more energy than it needs that day, the extra energy is stored
in the form of body fat. This stored energy can be advantageous, especially
during periods when he needs extra fuel to run his body machinery and fight
infection. It is important to encourage a high energy intake, even when your
child is well, as a type of insurance against the times when he is sick and likely
82 | CHAPTER NINE
to lose weight.
Because of problems with coronary heart disease in New Zealand, there is much
emphasis on reducing the amount of fat in our diet. This does not apply to infants
however, who need a large number of calories for growth, especially if they have
heart problems.
Fat is a very high energy source, providing nine calories per gram, which is more
than double that generated by an equal measure of protein or carbohydrate. Fatrestrictive diets are not needed by children with heart problems.
Growth
How do you know whether your child is eating enough food to grow properly?
Getting your baby or child weighed regularly is the best way to tell whether
growth is normal.
A child who is not eating enough will fail to gain weight properly and will eventually
begin to lose weight. It is a good idea to get your child weighed regularly to check
that his growth is normal for a child with a congenital heart condition.
How to Achieve a High Energy Diet
Encourage the child to choose meals and snacks that contain high concentrations
of energy (calories). Small volumes of these foods will be easier to eat and still
provide plenty of energy.
Eating time and bulk are also important in planning high energy meals and snacks
for a child who tires easily or becomes restless when having to eat a large amount
of food.
If the food is not too bulky, there will be more room for other high energy foods,
make the best use of stomach space.
The following are ideas for boosting energy intake using high energy foods. After
cooking the family meal, you can add one or more boosters to a child’s portion.
In this way, the child will be eating the same meat and vegetables or other foods
that the family members have, but will still benefit from an energy boost.
Boosting Energy
A. Protein foods: Protein foods are generally high in energy.
1. Milk Powder – One or more tablespoons can be added before serving to
cream or casserole dishes, sandwich fillings, white sauce and gravies,
salad dressing, soups, puddings, milk shakes, mashed potatoes and
CHAPTER NINE | 83
porridge. You can add the milk powder before cooking to many items,
such as biscuits or cakes, meat loaves, omelettes, scrambled eggs,
custards, pancakes and batters.
2. Milk – Is a favourite for most children. Take advantage of every glass
consumed, by increasing its energy and protein value with milk powder.
You can add a tablespoon or more to an individual serving, or prepare a
whole litre of this strengthened milk mixture. (1.5 cups powder to 1 litre
milk). Use this fortified milk in food preparation. If you find using full cream
milk powder causes digestive problems for your child, or it is too rich, try
using skim milk powder. Remember, it has a lot lower energy value. There
are a number of commercial milk-based energy supplements available.
Your chemist will give you information on these and how to use them.
3. Yoghurt – Is a food which is high in protein and energy. Natural yoghurt
can be added to casseroles, soups and sauces. Flavoured yoghurt can
be added to custards and milk puddings or used to top fruit for a dessert,
or as a snack. Try blending yoghurt into fruit juice for a refreshing change,
and serve icy cold.
4. Egg – Is another versatile protein and energy booster. The whole egg or yolk
and whites, separately, either boiled or raw, can be used to supplement
many foods. Try adding eggs when preparing meat loaf, custards, milk
shakes, mashed potato and white sauces. Extra eggs can often be added
to recipes for cakes, biscuits and pancakes, without any changes to the
finished product. Try blending an egg into an orange juice and serve icy
cold. The finely chopped cooked egg can easily be added to salads, meat
dishes and fried rice.
5. Cheese is another supplement high in calories and protein as well as
fat. Grated cheese can be added to meat loaf, mince dishes, mashed
potatoes, tinned spaghetti and baked beans, scrambled eggs, omelettes
and pasta. Add extra grated cheese to cheese sauce, pizza, sandwiches,
cheese on toast and topping for casseroles.
B. Carbohydrate Foods: Foods high in carbohydrate also pack a lot of
energy into a small volume and can be easily digested.
1. Bread, pasta, rice and noodles – can be added to soups and casseroles.
They also form the basis of high energy dishes such as macaroni cheese,
spaghetti bolognaise, pizza, fried rice and puddings. Breadcrumbs with
grated cheese make a great crunchy topping for casseroles.
84 | CHAPTER NINE
2. Sugar, jam and honey are pure carbohydrate. They can be used to add
energy, but are low in other nutrients, therefore they should only be used
as treats, rather than replacing more nutritious foods.
3. Dried Foods are mainly sugar and can be added to breakfast cereals,
curries, casseroles, cakes and biscuits, or just enjoyed as a snack. Fruit
and vegetables are nutritious but low in energy. Therefore, use them with
energy boosters as much as possible, e.g. fruit and cheese, or juice and
yoghurt.
C.Fat: food’s fat content cannot be judged by appearance. To qualify as
high fat, a food does not have to be “gooey” with icing or chocolate, or
battered fish, or swimming in grease. The fat content of food is often
invisible, hidden in the marbling of meat or just not apparent, as in the
egg yolk, the avocado or the olive.
1. Meat and other protein foods contain a variable amount of fat. The fat
content of red meat depends on the cut. Fish, seafood and chicken are
relatively low in fat, although chicken skin is quite high. Some pork is high
in fat especially bacon and ham. Other meat products which are quite high
in fat are sausages and small goods e.g. salami and luncheon meat. Nuts
and products such as peanut butter are high in fat.
2. Milk and dairy products – contain fat, for example, ice cream and cheese
are both quite high. Many products have skim or low fat versions, for
example milk or yoghurt. Cottage cheese or ricotta cheese are also quite
low in fat and therefore lower in energy
3. Butter, margarine, lard, vegetable oils and cream contain very little else
except fat. They are very useful for adding energy. Besides the type of food
chosen, another major contributor to the fat content of a meal or snack is
its preparation. Food when it is fried or roasted has a higher energy value.
For example, one medium boiled potato contains about 70 calories and no
fat. The same weight of chipped potatoes has 240 calories and 13 grams
of fat. So, fried meat, eggs, rice and vegetables are all higher in fat, and
of course energy.
Most “take away” foods are fried and therefore high in fat. These include
fish and chips, fried chicken, and fish cakes etc. Others which are not fried
but are also quite high in fat are for example, pizza, some Chinese food,
pies, pastries, sausage rolls and sweet pastries.
CHAPTER NINE | 85
Butter and margarine can be added to vegetables, rice and noodles and
used for spreading on bread, biscuits and plain or fruit cake. Cream can
be mixed into milk drinks, white sauces, soups, casseroles and desserts
and used on fruit and jelly. In the interest of healthy eating for the rest of
the family, fried foods should only be used in moderation.
D. Desserts and Sweets
Many New Zealanders have a sweet tooth. We know people who need to lose
weight should avoid desserts that are high in fat and sugar, but these foods can
be an excellent source of energy for your child. Desserts such as ice cream,
doughnuts, mousses, pastries, tarts and pies are high in fat and of course,
energy. Those desserts which are relatively low in fat, such as jelly, fruit, meringue,
sponge cake, pancakes and custard, still provide energy and you can add cream
if you want to boost calories.
E.Snacks
Snacks are a very important way of increasing energy and protein. Be adventurous
with snacks, virtually any food can be a snack. Sometimes offering different and
unusual foods can tempt a decreasing appetite.
Try dried fruit with nuts and cheese, Weetbix with butter and jam, cereal with
milk, crumpets, muffins, scones, raisin toast, leftover chops or sausage, chunks
of luncheon meat, cheese, or health bars. A nibble box in the fridge to which
the child has free access may help with snacking. A milk shake or carton of
flavoured milk is a valuable addition to any snack, and much better energy and
protein value than cordial or soft drink. Sensible snacking is a habit and should
be encouraged from an early age. Don’t just rely on three meals per day.
Infants – the dietary requirements of a baby with a heart defect and an infant
without illness differ in several respects. Refer to the section on Breast Feeding
and Bottle Feeding.
Toddlers and Preschoolers
As soon as feasible you should include the child at the family meal table and
introduce him to family food. Solid food should be served in the typically
breakfast/lunch/dinner pattern. Thus, as the child grows older, his eating pattern
will be similar to the rest of the family.
Now that your child is not growing as rapidly as he was during infancy his appetite
may not be as big as it seemed then. Not being hungry, but being more aware
86 | CHAPTER NINE
and active, pre-school age children typically lose interest in eating. They have
better things to do, at least in their view.
Parents should realize that it is normal for children at this age to rebel against
eating regular meals and prefer a single food (such as toast or vegemite
sandwiches). During this stage you walk a tight rope, being firm with your child
and not being overly indulgent of food likes and dislikes. You will not want to
show too much concern, which could encourage your child to rebel even more
with eating habits.
Although you will naturally worry that high energy and protein requirements are
not being met, it is better in the long run for your child to eat less, than to be
force-fed during this difficult eating period. Because children at this age are easily
distracted at meal times and do not eat well if overly tired, it is better to offer
small servings and provide second portions, rather than pile plates full of food.
This is the time to introduce the habit of healthy snacking. It is important to
make sure that snacks are nutritious and not too close to meals as to spoil the
appetite for the meal. If your child tires easily from eating, your best bet may
be to concentrate on foods which in small amounts contain a lot of energy and
protein, and those foods which require less work, that is, chewing. ‘Chewy ‘
food, like salads, (also low in energy), should be served in very small portions.
Meat, which can be hard for a child to eat, can be served as a patty or mince
or sausages, or made less dry by gravy or sauce. Milk shakes and egg flips are
a very useful way of getting some energy and nutrients into a child who is too
tired or grumpy to eat.
Another hint – appeal to your child’s playfulness and preference for small amounts
by preparing ‘finger foods’ in interesting shapes, soft foods and foods that have
been well chopped. The use of energy boosters can help pack in the energy.
Examples
• Soup with additional milk powder.
• Add eggs and milk powder to custards and milk puddings.
• Grated cheese in canned spaghetti or baked beans and extra grated
cheese and butter in mashed vegetables.
Teenagers
During the early teens your child will experience another growth spurt, which will
use up large amounts of energy and protein. If food intake does not meet the
CHAPTER NINE | 87
body’s nutritional needs, development will be slower than normal.
For example, if weight gain does not keep pace with height increase, the child will
become very thin. A good energy intake is absolutely crucial during adolescence
to maintain adequate growth.
Teenagers spend much of their time away from home. While at school with
friends ‘peer pressure’ has an important and possibly unhealthy impact on their
diet.
Adolescence is a period when emotional ‘ups and downs’ are common. To
establish independence (as much to themselves as to parents), they may do
exactly the opposite of what is asked or advised.
How can parents cope with these factors? Stay calm and remember that rebellion
is normal for this stage and that teenagers can and should be making their own
decisions about eating habits.
It is helpful sometimes to adapt family meals to foods, such as hamburgers and
pizza, typically enjoyed by this age group.
Snacking is an important part of the adolescent lifestyle. The use of energy
boosting is particularly important for the teenager who has little time or interest
in eating.
For online information
♥ Log on to www.heartnz.org.nz
♥ Click on “What we do”
♥ Click on the link “about congenital heart disease”
♥ From there you can access a New Zealand approved medical site where
you will be able to obtain more detailed information about the above
investigations.
88 | CHAPTER NINE
Chapter Ten
Realities of Life
at Home: Living with a
Heart Child
Will I cope?
Children with heart conditions are all different and the day to day management
of these children will vary greatly. If your child has been in hospital for a length
of time, it is a great feeling to have him back with you and your family. This may
be a difficult time for you as until now the hospital has taken responsibility for
your child and you may sometimes feel it is difficult to cope.
You may find yourself worrying unnecessarily when your child is out of sight for
a moment and checking constantly during the night to make sure that he is all
right. These very strong feelings are quite normal and after a while you will find
that you relax as you gain confidence with him.
How Do I Treat My Special Child?
There will be times when you are worried, upset or angry and these feelings may
influence the way you treat your child. Treat him as you would any of your other
children with no special concessions, as he needs to feel part of the family and
not different from his siblings.
Keeping your child as emotionally and physically healthy as possible is hard
work and at times you may be tempted to try and protect him from any pain,
CHAPTER TEN | 89
discomfort, worry and upset. As parents, you will soon become aware that this
cannot be done, as the very act of living means a risk of danger and pain and
over protecting your child will be no fun for either of you.
Discipline?
There is often the complaint that children, who have a heart condition, or for that
matter any other illness, have complete disregard for rules, order and authority.
As parents we are perfectly capable of teaching our children rules for personal
safety. However, difficulty may be encountered with rules for social behaviour.
All children need guidelines and limits. Parents may tend to avoid reprimanding
their heart child for fear of causing illness or unhappiness. However, for the child’s
sake, management must be the same as with other children and parents may
need to look at alternative methods of discipline e.g. withdrawal of privileges
and time out.
The aim of discipline is to help children change, so that they become able to live
with others as well as themselves. The child who knows no discipline should
be having the time of his life. Actually, such a child is never happy; knowing no
limits, he can be full of fears. Discipline brings contentment to children as well
as safety to our world.
The key to good discipline is a good relationship between parents and their
children and if you would like to read a book on discipline which may suit the
needs of a heart child, check the bibliography list at the back of this book.
In What Activities Can My Child Become Involved?
Young children with heart conditions usually know their limits, more so than adults
and almost never overtax themselves. It is therefore unnecessary to restrict their
activities. However, some older children, with major heart problems, may have to
avoid over-strenuous exertion so it may be necessary to guide your child towards
activities which are less strenuous.
Competitive athletics and/or team rugby are generally unwise, but heart children
can usually play socially in the playground and often enjoy non-competitive
tennis, physical education and swimming. Your child’s doctor may suggest
activities which are suitable.
The aim is to permit as full and normal a life as possible and unnecessary
restraints should never be imposed. No restrictions at all need to be put on
children with minor defects.
90 | CHAPTER TEN
Dental Treatment
With any dental treatment where there is a possibility of bleeding, ie. extractions,
or other procedures which affect the gums or bones, your child may need
antibiotic cover. The antibiotic may be supplied by your child’s doctor or dentist
and should be arranged before any dental work is due to commence.
If your child is already on a course of antibiotic treatment he may require a further
antibiotic, and the first dose should be taken not more than one hour prior to
work commencing. This is to ensure that the antibiotic will be present in the
blood stream in sufficient strength to be capable of combating any new release
of bacteria from the mouth.
Following treatment a second dose should be given. Where possible all dental
work should be completed in one visit or alternatively, visits should be a month
apart and each visit covered by antibiotics.
Antibacterial Prophylaxis for dental procedures
(The Cardiac Society of Australia and New Zealand)
Cardiac conditions for which Endocarditis Prophylaxis is recommended:
• Prosthetic heart valves (bio or mechanical)
• Rheumatic valvular heart disease
• Previous endocarditis
• Unrepaired cyanotic congenital heart disease (including palliative shunts
and conduits)
• Surgical or catheter repair of congenital heart disease within six months
of repair procedure.
Heart children are often required to take medication regularly and unfortunately
most medication given to children is sweetened, which can lead to tooth decay.
This problem can be lessened by brushing your child’s teeth after medication
is given.
It is very difficult to clean the teeth of a child under the age of two years, but if
your child is having medication, it would be advisable to try brushing with a very
soft baby brush to remove any of the sticky syrup which may adhere to the teeth
and lead to decay.
CHAPTER TEN | 91
Hints for Cleaning Your Pre-schooler’s Teeth
• Stand slightly behind your child and support his head by holding it against
you.
• Move his lips back, so that you can see what you are doing with the tooth
brush.
• Support the chin with the rest of your fingers.
• Try to clean all sides of the teeth, the chewing surface, and the sides by
the tongue and cheek.
• Use a soft brush with a small head.
PLEASE discuss antibiotics and dental care with your doctor.
How Can I Tell If My Child Is Unwell?
As you become more confident in caring for your heart child, you will become
very aware of any changes in their health. The following changes are guidelines
only – you will need to discuss your child’s condition in detail with your doctor.
Speak to Your Doctor If Your Child:
• Breathes more rapidly than normal
• Suffers frequent chest infections or persistent coughing
• Goes off food or begins vomiting
• Seems unusually pale or sweaty
• Is blue or turns blue after exertion
• Develops other unusual symptoms.
Pregnancy
A heart defect is usually a chance occurrence in the complex development of the
heart and it is uncommon to have more than one child in the family with a defect.
Although it is unlikely that heart defects are inherited, the chances of a second
child having a heart defect increases from 1 in 100 to 1 in 50. Although there is
no evidence that any drugs other than thalidomide or high dose alcohol cause
heart defects, it is recommended that you do not take any prescription or non
prescription drug if you suspect you may be pregnant, without first consulting
your doctor.
92 | CHAPTER TEN
If you already have a heart child, or have a sibling with a heart condition and are
considering having a baby you may be concerned about the chances of your
baby having a heart condition. Discuss your feelings and possible fears with your
doctor, who can, if necessary, refer you to a genetic counsellor at a city hospital.
Care during pregnancy will include regular ultrasounds to check on a baby’s
development. A study has shown that 55% of major defects of the heart and
arteries can be detected by ultra sound between 10 and 14 weeks of pregnancy.
An ultrasound produces sound waves that are beamed into the body causing
return echoes that are recorded to “visualize” structures beneath the skin. The
ability to measure different echoes reflected from a variety of tissues allows a
shadow picture to be constructed. The technology is especially accurate at
seeing the interface between solid and fluid filled spaces. These are actually
the same principles that allow sonar on boats to see the bottom of the ocean.
A transducer, or probe, is used to project and receive the sound waves and the
return signals. A gel is wiped onto the patient’s skin so that the sound waves are
not distorted as they cross through the skin.
There is now screening available for families who have a history or are at higher
risk for complications from a congenital heart defect (like a previous baby with
a defect). Usually a simple ultrasound can be done as a general screening for
women and their babies. If this indicates further testing, foetal echocardiograms
are available.
The foetal echocardiogram is a non-invasive procedure, much like a regular
ultrasound. It can be done in 20 to 60 minutes, depending on the positioning of
the baby. It is done with sound waves. This test may be ordered if you have a
family history, a baby who has an abnormal heart rate during pregnancy, or any
other marker for potential complications. The scan is very similar to a normal
ultra sound scan, except that it will concentrate on examining your baby’s heart.
This usually involves checking the atria and ventricles. This examination will help
to identify any problems with the aorta and pulmonary arteries as they leave
the heart. This examination will help to identify any problems with the structure,
function or rhythm of your baby’s heart.
Once your baby is diagnosed, what happens really depends on the type of
defect. If it is a murmur you would be monitored closely. If the condition is more
serious it may mean that your baby will need to be delivered at a hospital with
the facilities to manage his condition. It could mean that you will need to spend
the last few weeks of pregnancy living close to the hospital where your baby is
to be delivered. For further information check out “Knowing But Not Knowing”
CHAPTER TEN | 93
available from @Heart.
Make contact with your local @Heart group or Parent to Parent support group.
They will be able to put you in touch with other families who have had a similar
experience. They will also have suggestions and information that could make
your stay away from home more manageable.
Grandparents and Other People
Your child may get a lot of attention and gifts from grandparents and others
close to the family. This may cause jealousy and upset your other children. It is
difficult, because people feel they want to reach out and comfort and this is a
very understandable human feeling. Some extra attention will not do any harm
as it shows that people care and brothers and sisters can usually cope if they are
made to feel they are still a very special part of the family and are given plenty
of love and reassurance.
Dealing with Other People
Some people who have never had a sick child find it very difficult to understand
the feelings involved. They may expect you to react in a certain way, or they may
offer advice on what they think is best for you and your family. Although well
meaning, such advice may be ill-considered. Nothing you can say or do will help
such people understand, so you are best to ignore them.
You do not have to discuss your child or your feelings with other people if you
do not want to. However, it is good to confide in at least one person who will
understand and act as a “sounding board” for you. This may be a relative, a close
friend or a parent from an @Heart group, or Parent to Parent, whose child has
been through a similar experience.
Your Life
Making your heart child the centre of your world is as unwise as rejecting him.
Your life involves other people, who will suffer if they are suddenly cut out. You
will also suffer if you exclude everyone from your life.
The time may come when you will welcome the support and love of others, so
don’t become so involved in your child that you have no room for anyone else.
Keep up the interests you had before. Do not refuse invitations or resign from
clubs, unless you are in a position where it is impossible to do otherwise.
Having a heart child may mean that you have to make some sacrifices, but do
not make any that are unnecessary. Cutting yourself off from everything and
94 | CHAPTER TEN
everyone is not good for you or your child, as it may make him feel different
from other children.
Sitters
If you wish to go out without your child, do not be put off by what other people
may think or say. Leave your child with a sitter; the break will do you good. Leave
a phone number as you would when leaving any other child, so that you can be
contacted if anything does go amiss.
Sometimes it may be difficult to find someone who is confident enough to look
after your child. You may have to help them gain confidence and get to know
him, so perhaps your first few outings could be only for an hour or so.
Cardiac Resuscitation
Cardiac resuscitation is used for the treatment of cardiac arrest (complete
cessation of the pumping of the heart). Cardiac arrest can occur anywhere and all
sorts of people may be called upon to react to the emergency that has happened
in front of them.
The National Heart Foundation of New Zealand believes that it is the responsibility
of every adult New Zealander to learn the technique of Cardiac Pulmonary
Resuscitation (CPR). @Heart also recommends that the adult members of a
family with a heart child learn CPR.
The best way to learn about cardiac resuscitation is to attend a short teaching
session. A variety of organisations hold them from time to time and reasonable
proficiency can be obtained within two to three hours. Plastic models are used
for practice.
Your local @Heart group may be able to arrange a teaching session for you
through the National Heart Foundation of New Zealand or St. Johns’ Ambulance
Association.
You may feel that your child’s condition is not serious, so you don’t need to learn
CPR, but CPR may also be needed for other emergencies, such as breathing
difficulties or choking attacks; so don’t just think about it, do it.
Susceptibility to Other Ailments
There is no evidence that a congenital heart defect increases the chances of
developing other heart trouble, such as rheumatic heart disease or coronary
heart disease in later life. However, congenital heart defects do make patients
CHAPTER TEN | 95
more susceptible to bacterial endocarditis.
Effects of Common Childhood Diseases
The child with a congenital heart defect can usually cope with most common
childhood illnesses as safely as children with normal hearts, although children
with some kinds of congenital heart defects can be subject to recurrent chest
infections.
Most children with a congenital heart defect can receive the standard
immunizations at the ages recommended, after discussion with their doctor.
However, if your child has Heterotaxy syndrome they will require an individual
immunization plan.
Effects of Congenital Heart Defects on the Brain
When a child with a congenital heart defect also has an intellectual disability, this
usually means the child was born with two major defects and not that the heart
affected the brain. Parents of heart children often ask if brain tissue is harmed by
the low oxygen content of the blood. When the oxygen content of the blood is
low, the brain is usually able to get all the oxygen it needs. Sometimes a normally
bright child tires easily as a result of a heart defect and will fall behind in school
work, but after successful heart surgery the child usually catches up at school.
Growing Up with a Heart Condition
If you were born with a heart condition you may have to take a little more care
with your health and life style. Diet is important. Try to maintain a good balanced
diet. Avoid too much fat, salt and sugar (refer to the section on nutrition).
Obesity in adolescents and adults causes the heart to work harder. The fatter
you are, the harder it has to work and you are more likely to have high blood
pressure, which causes damage to the artery walls.
Alcohol in excess is not good for anyone. Small amounts cause no problems in
adults.
Smoking is hazardous to health. It will damage the normally smooth lining of
the arteries allowing clots to form. Blocked arteries, as a result of these clots,
produce heart attacks and strokes. Smoking also causes cancer of the lungs,
mouth and oesophagus. Passive smoking, that is regular breathing in of other
people’s exhaled smoke, causes similar problems to smoking itself.
Contraception should be discussed with your doctor to determine if special
96 | CHAPTER TEN
precautions are necessary. For women, in most cases a low dose combined
contraceptive pill can be taken. However, women with polycycthaemia (thick
blood), high blood pressure, poor circulation or who are on anti-coagulants may
need to use other forms of contraception.
Women with mild heart defects that do not require correction can have children.
Women who have had successful heart operations can also have children after
discussion and with monitoring by their doctor.
With some serious conditions, pregnancy is dangerous and should be avoided.
A woman should discuss both contraception and pregnancy with her doctor to
determine if special precautions are necessary.
Some Children Do Die – Dealing with Your Fears for Your Own
Child
When you have a child who has congenital heart disease it is likely that you will
acknowledge, even if only to yourself, the fear that your child might die. It is often
difficult to voice this fear or find someone who will listen to you and understand
your need to talk about it.
Some people are afraid to talk about death for fear of upsetting you. They may
worry that you have given up hope and may try and reassure you if you introduce
the subject. Many people feel uncomfortable talking about death. It is normal to
have these strong feelings of fear for your child’s life.
If you feel you would like to talk about your fears and you don’t have someone
who is able to understand your need to talk, you might like to contact your
nearest @Heart group. If you are in hospital the hospital chaplain and social
worker are available to talk it over with you.
Your Feelings When Another Heart Child Dies
If you are staying in hospital you may develop strong bonds of friendship with
other parents who have children with heart conditions and if a child dies, the
effect on everyone within the hospital system is shattering.
Nursing staff may react strongly. They may not wish to talk about the child for
fear of upsetting other parents and they may somehow feel they have failed. You
may feel a great loss.
You may feel guilty, as your child is still alive. You may be afraid, fearing for your
own child’s life and be unable to approach the other child’s parents, even though
you had been very close.
CHAPTER TEN | 97
These Feelings Are Normal
The parent whose child has died may feel afraid to approach you, as they may
feel that their loss will upset you and your child. Go to the bereaved parent
and tell them you are sorry and sad that their child has died. This may seem
inadequate to you, but may be of more comfort to them than you realise.
If Your Child Dies
We have included this section because Robert and Jessica, our heart children,
both died.
You will experience again all of the feelings you felt when you were told that your
child had a heart condition but, with an intensity that words cannot describe.
Grief is unbearable.
Sometimes you may actually feel a physical pain in your chest almost as if
someone had punched you there. This is a normal physical response and will
be accompanied by extreme anguish.
There is nothing we can tell you or any short cuts that you can take to help you
through this terrible time. But with love and support of family and friends you will
survive and one day you will be able to recall the good times and the love you
shared with your child. The love you feel for your child will remain long after the
deep pain and sadness have faded.
Sometimes we felt that this would never happen, but now we look back at the
time we shared with Robert and Jessica with pleasure instead of pain and it is
because of them that we have written this book for you.
98 | CHAPTER TEN
Glossary
Reference
for Heart Parents
Parents’ Dictionary
This dictionary of heart terms was written to give clear definitions of technical
terms in non-technical language. The dictionary contains words from medicine,
anatomy, physiology, instrumentation, pharmacology and some special terms
used by certain related professionals in dealing with heart conditions in children.
If there are any other terms used relating to your child that you do not understand,
please ask members of the team involved with your child’s care to explain.
Acidosis A metabolic condition in
which the acid content of the blood or
body tissues is too great; it may result
from failure of the lungs to remove
carbon dioxide ( respiratory acidosis)
or from an over production of acid
substances in the body’s tissues
(metabolic acidosis).
Aetiology The causes of a disease.
Anaemia Reduction in the red blood
cell count.
Anaesthetic A chemical that
produces loss of consciousness.
Aneurysm A spindle-shaped or
sac-like bulging of the wall of a vein
or artery, due to weakening of the
wall by disease or to an abnormality
present at birth.
Angiocardiography X-ray
examination of the heart and great
blood vessels that follows the course
of an opaque fluid which has been
injected into the blood stream.
Angioplasty Stretching of a narrow
artery by a balloon catheter.
GLOSSARY | 99
Anomalous Pulmonary Venous
Return Oxygenated blood returning
from the lungs is carried abnormally
to the right side of the heart by one
or more pulmonary veins emptying
directly, or indirectly through venous
channels, into the right atrium. Partial
anomalous return of the pulmonary
veins to the right atrium results in
the same problem as an atrial septal
defect (some blood drains to the
wrong side of the heart). In complete
anomalous return of the pulmonary
veins, an interatrial communication is
necessary for survival.
Anoxia Literally, no oxygen. This
condition most frequently occurs
when the blood supply to part of
the body is completely cut off. This
results in the death of the affected
tissue. For example, a specific area
of the heart muscle may die when the
blood supply (and hence the oxygen
supply) has been blocked, as by a
clot in the artery supplying that area.
Anticoagulant A drug which
delays clotting of the blood. When
given in cases where a blood vessel
is plugged up by a clot, it tends to
prevent new clots from forming, or
the existing clots from enlarging, but
does not dissolve an existing clot.
Examples are heparin and warfarin.
Aorta Main artery which receives
blood from the left ventricle of the
heart. It originates at the base of the
heart, arches up over it and passes
down through the chest and abdomen
in front of the spine. It gives off many
100 | GLOSSARY
lesser arteries which take blood to all
parts of the body except the lungs.
Aortic Arch The part of the aorta
leaving the heart, which curves up like
a cane handle over the top of it.
Aortic Valve The valve at the
junction of the aorta and the left
ventricle of the heart. Formed by three
opposing cup-shaped membranes,
it allows the blood to flow from the
heart into the aorta and prevents a
back flow.
Aortography X-ray examination
of the aorta (main artery conducting
blood from the left pumping chamber
of the heart to the body) and its main
branches. This is made possible
by the injection of a fluid which is
opaque to X-rays.
Apex The blunt rounded end of the
heart, directed downward, forward
and to the left.
Arrythmia An abnormal rhythm
of the heartbeat. Sometimes
during surgery the natural pacing
mechanisms in the heart become
swollen resulting in an irregular or
slow heartbeat. It may be necessary
to use a temporary external
pacemaker or on rare occasions
a permanent pacemaker may be
required. Occasionally the heart beats
very fast and medication is used to
slow it down.
Arterial Blood Oxygenated blood.
The blood is oxygenated in the lungs
and passes from the lungs to the
left side via the pulmonary vein. It is
then pumped by the left side of the
heart into the arteries which carry it
to all parts of the body (see “Venous
Blood”).
Banding An artificial narrowing of
the pulmonary artery with a “band” to
reduce blood flow to the lungs.
Arterioles The smallest arterial
vessels (about 0.2mm or 1/125 inch
in diameter) resulting from repeated
branching of the arteries. They
conduct the blood from the arteries to
the capillaries.
Bifurcation Division into two
branches.
Artery Blood vessels which carry
blood away from the heart to the
body. They usually carry oxygenated
blood, except for the pulmonary
artery which carries unoxygenated
blood from the heart to the lungs for
oxygenation.
Artifical Valve Artificial valves are
used to replace valves which do
not function. The choice of valve
replacement will depend on the
child’s condition. Artificial valves
will not grow, therefore may need
replacing as the child grows.
Ascites Fluid in the abdomen.
Atheroma A deposit of fat and other
substances in the inner lining of the
artery wall which causes narrowing
and a reduction of blood flow, on to
which clots may form.
Atrium One of the two upper
chambers of the heart. The right
atrium receives unoxygenated blood
from the body. The left atrium receives
oxygenated blood from the lungs.
Autopsy Medical examination of the
body after death.
Biopsy Removal of a small piece of
tissue.
Blood Pressure. The pressure
produced by blood passing through
the arteries. Blood flow pulsates with
the heart beat and so does blood
pressure.
Blue Babies Babies having a
blueness of the skin (cyanosis)
caused by insufficient oxygen in the
atrial blood. This often indicates a
heart defect, but may have other
causes such as premature birth with
impaired respiration.
Bradycardia Abnormally slow heart
rate.
Brain Damage A complication
which on rare occasions may occur
post-operatively if the brain does not
receive an adequate blood supply.
This may cause unconsciousness
or convulsions but is usually short
lived and most children make a full
recovery. However in rare cases there
may be lasting problems.
Capillaries Extremely narrow
tubes forming a network between
the arterioles and the veins. The
walls are composed of a single layer
of cells through which oxygen and
nutrition pass out to the tissues and
carbon dioxide and waste products
GLOSSARY | 101
are admitted from the tissues into the
blood stream.
Carbon Dioxide Waste product
of chemical reactions in the cells. It
passes from the cells to the blood
which eventually releases it in the
lungs to be breathed out.
Cardiac Pertaining to the heart.
Sometimes describes a patient who
has heart disease.
Cardiac Cycle One total heart beat,
i.e. one complete contraction and
relaxation of the heart. In man, this
normally occupies about 0.85 second
– about 37,000,000 beats per year.
Cardiac Output The amount of
blood pumped by the heart per
minute.
Cardiomyopathy Disease of the
heart muscle.
Cardiopulmonary Bypass A
machine with a pump and an
oxygenator to maintain blood supply
to the body while the heart’s action is
stopped.
Cardiovascular Pertaining to the
heart and blood vessels.
Carotid Arteries The left and
right common carotid arteries are
the principle arteries supplying the
head and neck. Each has two main
branches, external carotid artery and
internal carotid artery.
Catheter A thin flexible tube which
can be guided into body organs.
A cardiac catheter is inserted into
a vein or artery (usually an arm or
102 | GLOSSARY
leg) and gently threaded into the
heart. Its progress can be watched
on a fluoroscope. It can be used for
diagnosis (to take samples of blood
or pressure readings in the chambers
of the heart), treatment (administer a
drug), or to carry out a procedure.
Cholesterol A fat- like substance
found in animal tissue.
Chordae Tendineae Fibrous chords
which serve as guy ropes to hold the
mitral and tricuspid valves secure
when the ventricles contract. They
stretch from the cusps of the valves
to muscles called papillary muscles in
the walls of the ventricle.
Chorea Involuntary irregular
twitching of the muscles some times
associated with rheumatic fever. Also
called St Vitus Dance or Sydenham’s
Chorea.
Circulatory Pertaining to heart,
blood vessels and the circulation of
the blood.
Clubbed Fingers Fingers with a
short broad tip and overhanging nail
somewhat resembling a drumstick.
This condition is sometimes seen in
children with heart conditions.
Coarctation Literally a pressing
together or narrowing of a blood
vessel.
Collaterals Circulation of the blood
through nearby smaller vessels when
a main vessel has been blocked.
Compensation A change in
the circulatory system made to
compensate for some abnormality. An
adjustment of size of heart or rate of
heart beat made to counterbalance a
defect in structure or function. Often
used specifically to describe the
maintenance of adequate circulation
in spite of the presence of heart
failure.
Conduit An artificial tube that
carries blood and usually has a valve
within it. Conduits are used when
it is impossible to repair a valve, to
replace a completely missing valve, or
bypass a severe narrowing that can
not be corrected.
Congenital Anomaly An
abnormality present at birth.
Congestive Heart Failure When the
heart is unable to adequately pump
blood fast enough to meet the body’s
needs, pressure builds up in the veins
leading to the heart. Congestion or
accumulation of fluid in various parts
of the body (lungs, legs, abdomen,
etc.) may result.
Convulsion A fit or seizure.
Coronary Arteries Two arteries,
arising from the aorta, arching down
over the top of the heart and carrying
blood to the heart muscle.
Coroner An official who inquires
into unnatural death, e.g. sudden,
unexpected or those related to
procedures or operations.
Cor Pulmonale Heart disease
resulting from disease of the lungs
and blood vessels in the lungs. This
is due to resistance to the passage of
blood through the lungs.
Cyanosis Blueness of the skin
caused by insufficient oxygen in the
blood. Oxygen is carried in the blood
by haemoglobin, which is bright red
when saturated with oxygen, but
becomes purple without oxygen.
Decompensation Inability of
the heart to maintain adequate
circulation, usually resulting in a
water logging of tissues. A person
whose heart is failing to maintain
normal circulation is said to be
“decompensated”.
Defibrillator A device which
produces a timed electric shock,
which stops an inco-ordinate
contraction of the heart muscle and
restores a normal heart beat.
Dextrocardia A condition in which
the heart lies in the right (instead of
the left) side of the chest. The heart
may be normal otherwise, or may
have additional congenital defects.
Dilation Stretching or enlargement
of the heart blood vessels.
Diaphragm Important muscle of
breathing that separates the chest
from the abdomen.
Diastole The phase of the heart’s
cycle during which it relaxes and fills
its chambers with blood.
Diastolic Blood Pressure The
lower of the blood pressure readings.
Drain A tube used to move fluid or
air from the body.
GLOSSARY | 103
Drip A means of getting nutrients
and drugs into the body through a
vein.
Dysphagia Difficulty with
swallowing.
Dyspnoea Difficult or laboured
breathing.
Echocardiogram A picture of
the heart and blood vessels using
reflected high frequency sound
waves.
Electrocardiogram ECG. A graphic
record of the electrical activity of the
heart.
Electrodes Fine wires that carry
electrical activity from or into the
heart.
Embolus A clot or other matter
which travels through the blood
stream to lodge in a small vessel
and cause an obstruction to the
circulation.
Endocardium The thin, smooth
lining on the inside of the heart which
is in contact with the blood.
Extrasystole A contraction of the
heart which occurs prematurely and
interrupts the normal rhythm.
Familial Runs in families.
Femoral Artery The main blood
vessel supplying blood to the leg.
Fibrillation Unco-ordinated
contractions of the heart muscle
occurring when the individual muscle
fibres take up independent irregular
contractions. With ventricular
104 | GLOSSARY
fibrillation the heart effectively stops
(cardiac arrest).
Foramen Ovale An oval hole in the
septum of the foetal heart between
the right and left atrium, which
normally closes shortly after birth.
Gallop Rhythm An extra, clearlyheard heart sound which, when the
heart rate is fast, resembles a horse’s
gallop. It occurs in heart failure but is
also heard in many normal children.
Gene An inherited characteristic, a
part of a chromosome.
Haematoma A localised collection
of blood outside a vessel (bruise).
Haemodynamics Blood flow and
the forces involved.
Haemoglobin The oxygencarrying red pigment of the red blood
corpuscles. When it has absorbed
oxygen in the lungs, it is bright red
and called oxy-haemoglobin. After it
has given up its oxygen in the tissues,
it is purple in colour and is called
reduced haemoglobin.
Haemorrhage Loss of blood
from a blood vessel. In external
haemorrhage, blood escapes from the
body. In internal haemorrhage, blood
passes into the tissues surrounding
the ruptured blood vessel.
Heart Block A condition in which
the electrical impulse, which travels
through the heart and triggers the
heart beat, is slowed or blocked.
This causes a disturbance to the
rhythms of the upper and lower heart
chambers.
Heart Lung Machine A machine
through which the blood stream is
diverted for pumping and oxygenation
during heart surgery.
Incompetent Valve Any valve which
does not close tight and leaks blood
back in the wrong direction. Also
called valvular insufficiency.
Heparin A chemical substance
which tends to prevent blood from
clotting. Sometimes used in cases
of an existing clot in an artery or vein
to prevent enlargement of the clot
or the formation of new clots. An
anticoagulant.
Infarct The area of tissue which
is damaged or dies as a result of
receiving an insufficient blood supply.
Hypertension Commonly called
high blood pressure. An unstable or
persistent elevation of blood pressure
above the normal range, which may
eventually lead to increased heart
size, kidney damage and heart failure.
Hypertrophy The enlargement of
a tissue or organ due to increase
in the size of its constituent cells.
This may result from demand for
increased work in the heart. It refers
to thickening of the walls, allowing a
more powerful contraction.
Hypotension Commonly called low
blood pressure. Blood pressure below
the normal range. Most commonly
used to describe an acute fall in blood
pressure, as occurs in shock.
Hypoxia Less than normal content
of oxygen in the organs and tissues
of the body. At very high altitudes a
healthy person suffers from hypoxia
because of insufficient oxygen in the
air that is breathed.
Immunization A method of
increasing patient’s defence against
infection.
Infusion Fluid or medication given
slowly into a vein.
Innominate Artery One of the large
branches of the aorta. It rises from the
arch of the aorta and divides to form
the right common carotid artery and
the right subclavian artery.
Intubation Passage of a tube into
the windpipe to assist with breathing.
Ischaemia A local, usually
temporary deficiency of blood in
some part of the body, often caused
by a constriction or obstruction in the
blood vessel supplying that part.
Isotope A term applied to one
of two elements, chemically
identical, but differing in some other
characteristic, such as radioactivity.
Radioactive isotopes are often
used in medicine to trace the fate of
substances in the body.
Jaundice Yellow colouring of
skin and eyes as a result of liver
dysfunction or red cell breakdown.
Jugular Veins Veins which return
blood from the head and neck to the
heart.
Keloid A hard, lumpy scar from
excess fibrous tissue.
GLOSSARY | 105
Leucocyte White blood cell that
fights infection.
the inner layer (endocardium) and the
outer layer (epicardium).
Lumen The passageway inside a
tubular organ. Vascular lumen is the
passageway inside a blood vessel.
Neonate Baby in the first month of
life.
Lymph Body fluid running in
channels, drains fluid and particularly
fats from the bowel back into the
circulation.
Mediastinum Space in the chest
between the lungs, heart and great
vessels.
Mitral Insufficiency Incomplete
closure of the mitral valves. When the
ventricle contracts, some blood leaks
back to the left atrium. Sometimes
this is the result of scar tissue forming
after rheumatic fever infection.
Mitral Stenosis A narrowing of the
valve (called bicuspid or mitral valve)
opening between the upper and
lower chamber in the left side of the
heart. Sometimes the result of scar
tissue forming after rheumatic fever
infection.
Mitral Valve Two-cusped valve
between the left atrium and the left
ventricle.
Murmur Noise produced by blood
flow in the heart and vessels.
Myocardial Insufficiency An
inability of the heart muscle
(myocardium) to maintain normal
circulation.
Myocardium The muscular wall of
the heart. The thickest of the three
layers of the heart wall, it lies between
106 | GLOSSARY
Node Area of specialized cell that
controls the rhythm of the heart.
Oedema Swelling due to
abnormally large amounts of fluid in
the tissues of the body.
Oliguria Too little urine.
Oximeter A machine to measure
oxygen.
Oxygen Part of the air that is
needed by all animal cells for normal
working.
Oxygenator An artificial machine
that delivers oxygen into the blood.
Pacemaker Electrical control of the
heart.
Pacemaker (Artifical) An electrical
device which can substitute for a
natural pacemaker and control the
beating of the heart by a series of
rhythmical electrical discharges.
If the electrodes which deliver the
discharges to the heart are placed on
the outside of the chest it is called an
external pacemaker. If they are placed
within the chest wall it is called an
internal pacemaker.
Palpitation A fluttering of the heart
or abnormal rate or rhythm of the
heart.
Papillary Muscles Small bundles of
muscles in the wall of the ventricles
of the heart to which cords leading
to the cusps of the valves (chordae
tendineae) are attached. When the
valves are closed, these muscles
contract and tighten the cords which
hold the valve firmly shut.
Parenteral Medicines given by
injection.
Paroxysmal Tachycardia A period
of rapid heart beats which begins and
ends suddenly.
Pediatric Sometimes spelt
“paediatric” – word to describe
science of medical problems in
children.
Percussion Tapping the body as
an aid in diagnosing the condition of
parts beneath by the sound obtained,
much as one taps on the barrel to
detect its fullness.
Pericarditis Inflammation of the thin
membrane sac (pericardium) which
surrounds the heart.
Pericardium A thin membrane sac
which surrounds the heart and roots
of the great vessels.
Peripheral Resistance The
resistance offered by the arterioles
and capillaries to the flow of blood
from the arteries to the veins (the
narrower the vessel the higher the
resistance). An increase in peripheral
resistance causes a rise in blood
pressure.
Platelets Small particles in the
blood which are important for blood
clotting.
Plasma The cell-free liquid portion
of uncoagulated blood. It is different
from serum which is the fluid portion
of the blood obtained after clotting.
Pleura Covering layer of the lungs
and the inside of the chest.
Pneumothorax Air outside the lung
and within the chest cavity.
Polycythaemia Abnormal condition
of the blood characterised by an
increased number of red blood cells.
Prognosis An estimation of outlook
for the patient’s particular problem.
Prophylaxis Preventive treatment.
Pulmonary Pertaining to the lungs.
Purkinje Fibres Specialised
muscular fibres forming a network in
the walls of the ventricles involved in
conducting electrical impulses. These
electrical impulses are responsible for
contractions of the heart.
Regurgitation The backward flow
of blood through a defective valve.
Sedative A drug which depresses
the activity of the central nervous
system, thus having a calming effect.
Examples are barbiturates and choral
hydrate.
Shunt A passage between two
blood vessels or between the two
sides of the heart, as in cases
where a hole exists in the wall which
normally separates them. In surgery,
the operation of forming a passage
between blood vessels to divert blood
from one part of the body to another.
GLOSSARY | 107
Sodium A mineral essential to life,
found in nearly all plant and animal
tissue. Table salt (sodium chloride)
is nearly half sodium. In some types
of heart disease the body retains an
excess of sodium and water, and
therefore sodium intake is restricted.
Stokes-Adams Syndrome Sudden
attacks of unconsciousness,
sometimes with convulsions, which
may accompany heart block with a
sudden fall in heart rate.
Sympathetic Nervous System A
part of the autonomic nervous system
or involuntary nervous system, it
regulates tissues not under voluntary
control, e.g. glands, heart, blood
vessels and smooth muscle.
Syncope A faint. One cause for
syncope can be an insufficient blood
supply to the brain.
Syndrome A set of characteristics
which occur together and are
therefore given a name to indicate
that particular combination.
Transplantion Replacement of a
failing organ with a healthy one, from
one person to another.
Tricuspid Valve Three leaflet valve
between the right atrium and right
ventricle.
Truncus Single vessel arising from
the heart that divides into aorta and
lung artery.
Vaccination Used generally for
immunization.
Vaccine A liquid of weak or killed
micro-organisms, or their proteins
that can be used to prevent diseases.
Vagus Nerves Two of the nerves
of the parasympathetic nervous
system which extend from the brain,
through the neck and thorax into the
abdomen. Known as the inhibitory
nerves of the heart, they slow the
heart rate when stimulated.
Valve Structure that allows blood
flow in one direction and prevents
leakage.
Systemic Circulation The
circulation of the blood through all
parts of the body except the lungs,
the flow being from the left lower
chamber of the heart (left ventricle)
through the body, back to the right
upper chamber of the heart (right
atrium).
Valvoplasty Stretching of a narrow
valve often with a balloon catheter.
Thrombosis The formation or
presence of a blood clot (thrombus)
inside a blood vessel or cavity of the
heart.
Vascular Relating to blood vessels.
108 | GLOSSARY
Valvotomy Cutting or stretching of a
narrow valve.
Valvular Insufficiency Incomplete
closure of a valve, permitting back
flow of blood in the wrong direction.
Vasoconstrictor The
vasoconstrictor nerves are part of the
involuntary nervous system. When
these nerves are stimulated they
cause the muscles of the arterioles
to contract, thus narrowing the
arteriole passage, increasing the
resistance to the flow of blood and
raising the blood pressure. Chemical
substances which stimulate the
muscles of the arterioles to contract
are called vasoconstrictor agents
or vasopressors. An example is
adrenalin or epinephrine.
Venous Blood Unoxygenated
blood. The blood, with haemoglobin
in the reduced state, is carried by the
veins from all parts of the body back
to the heart and then pumped by the
right side of the heart to the lungs
where it is oxygenated.
Ventricle Pumping chamber of
heart.
Vein (Vena) Thin walled vessel
carrying blood towards the heart.
GLOSSARY | 109
Support Groups
Do make contact with a support group. Support groups can help with practical
things and information about the day to day care of a child with a heart condition.
@Heart
@Heart is the only organisation in New Zealand dedicated to providing lifelong
support for all those affected by childhood heart conditions.
Started in 1980 by two heart mothers due to a need for parental information and
support, the network continued to grow and formalised itself as Heart Children
Incorporated in 1984 and subsequently changed its name to Heart Children New
Zealand Incorporated.
With an increasing number of adults surviving childhood heart conditions, the
organisation has responded by extending its support to heart children throughout
their lives, not just in their younger years. It changed its name to @Heart Inc in
2010 to better represent all those affected by childhood heart conditions. The
organisation serves Kids@Heart, Teens@Heart, Adults@Heart and Families@
Heart.
@Heart (www.heartnz.org.nz) now stretches across the nation providing kids,
teens, adults and families affected by childhood heart conditions with free access
to its 50+ services. The charity is a non-government organisation and is not
affiliated with the Heart Foundation. Charity Registration CC20102.
Parent to Parent New Zealand
Parent to Parent New Zealand Inc. is a national, not-for-profit organisation set
up in 1983 by a small group of parents and professionals who believed in the
value of parents supporting parents when faced with the challenge of parenting
a child with a disability, health impairment, or special need. 110 | SUPPORT GROUPS
Parent to Parent operates through a network of regional groups, which are
affiliated to the National Body. Currently, Parent to Parent has over 500 trained
volunteer support parents who provide information, advice and emotional support
to families. They also participate in regional committees providing additional local
family support activities.
Parent to Parent believe in Empowering parents, caregivers and whanau to get
the greatest life for their children and family members.
National Office – Hamilton
Location: 8 Liverpool Street; Hamilton, 3204
Postal: PO Box 234, Waikato Mail Centre, 3240
Phone: 07 853 8491 or 0508 236 236
Email: [email protected]
www.parent2parent.org.nz
La Leche League
Many people think La Leche League is only for when mothers have breastfeeding
problems. You can get great information, ideas and support before your baby is
born, as well as after. La Leche League is run by mothers for mothers. Whether
you’re pregnant or already breastfeeding your heart baby, you’ll find great
support and ideas at La Leche League.
www.lalecheleague.org.nz
La Leche League New Zealand Inc.
PO Box 50780 Porirua 5240
Phone : 04 471 0690
Email: [email protected]
SUPPORT GROUPS | 111
References
Brazelton, T.B. (1990), Families, Crisis and Caring. Perseus Books. ISBN
020192646.
Debakey, M. and Gotto, A. (2012), Living Heart in the 21st Century. NY:
Prometheus Books.
Featherstone, H. (1981), A Difference in the Family: Living with a Disabled
Child. Harmondsworth: Penguin.
Gargiulo, R.M. (1985), Working With Parents of Exceptional Children: a Guide
for Professionals. Boston: Houghton Mifflin Co.
HeartLine Association in England (1982), Heart Children. A helpful source of
additional information for this addition of Heart Children.
Hornby, G. (1987), Families with exceptional children, in D.R. Mitchell and
N.N. Singh, Exceptional Children in New Zealand. Palmerston North:
Dunmore Press.
Horby, G., Murray, R. and Davies, L. (2000), Parent to Parent: Basic Helping
Skills: Leaders Manual. Auckland: Auckland College of Education.
Jelliffe, D. and Jelliffe, P. (1987), Human Milk in the Modern World:
Psychological, Nutritional, and Economic Significance. Oxford:
Oxford University Press.
Johnson, J. and Johnson, M. (1992), Children Die Too. Nebraska: Centering
Corporation.
Klaus, M.H., Kennell, J.H. and Klaus, P.H. (1996), Bonding: Building the
Foundations of Secure Attachment and Independence. Boston: AddisonWesley Publishing Company.
Kroth, R. and Edge, D. (2007), Communicating With Parents of Exceptional
Children. Denver, CO: Love Publishing Company.
Kubler-Ross, E. (1969), On Death and Dying. New York: Macmillan.
Mackintosh, A. (1984), The Heart Disease Reference Book. London: Harper
and Rowe Ltd.
Neal, S. (1992), Cleft Lip and Palate: Handbook for Parents. Auckland.
Parker, M. and Mauger, D. (1979), Children with Cancer. Australia: Cassell
Australia Ltd.
112 | REFERENCES
Sears, W. (1987), Creative Parenting: How to Use the Attachment Parenting
Concept to Raise Children Successfully from Birth Through Adolescence.
Ottawa: Optimum Publishing International Inc.
Silverstein, A., Silverstein, V. and Silverstein Nunn, L. (2006), Heart Disease.
Minnesota: 21st Century Books.
Wiessinger, D., West, D. and Pitman, T. (2010), The Womanly Art of
Breastfeeding. US: Ballantine Books, Random House.
For online information
♥ Log on to www.heartnz.org.nz
♥ Click on “What we do”
♥ Click on the link “about congenital heart disease”
♥ From there you can access a New Zealand approved medical site where
you will be able to obtain more detailed information about the above
investigations.
We Support Kids, Teens, Adults and Families @Heart
We Provide Practical and Emotional Support
We Fund Equipment
We Educate
We Inform
We Fund Research
We Listen, We Learn, We Act
We Make a Difference
REFERENCES | 113
Supporting heart kids
through life