Heart Children. A Practical Handbook for Parents of Children with Heart Conditions. & Michelle Mann. Enjoy your children

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1 Enjoy your children Proudly printed and sponsored by Heart Children Linda Davies Michelle Mann Heart Children A Practical Handbook for Parents of Children with Heart Conditions Linda Davies & Michelle Mann

2 Heart Children A Practical Handbook for Parents of Children with Heart Conditions Linda Davies & Michelle Mann

3 2013 Linda Davies Revised and updated March 2013 Copyright 1983 Linda Davies and Michelle Mann 1st edition ,000 copies 2nd edition revised ,000 copies 3rd edition revised ,000 copies 4th edition revised ,000 copies 5th edition reprint ,000 copies 6th edition revised ,000 copies ISBN Published Inc. National Office PO Box Symonds street Auckland 1150 Freephone Supporting heart kids Other publications available 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

4 Contents Dedication About the Authors Foreword Acknowledgements Introduction viii ix x xi 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 Circulation 10 The Heart Pacemaker 12 Heartbeat 12 Pulse 12 Respiration 12 Valves 14 Chapter Two: Congenital Heart Conditions 15 Introduction 15 Heart Murmurs 16 Coarctation of the Aorta 16 Congenital Aortic Stenosis 16 Congenital Pulmonary Valve Stenosis 18 Patent Ductus Arteriosus (PDA) 19 Septal Defects 20 Atrial Septal Defect 20 Ventricular Septal Defect 21

5 Tetralogy of Fallot 22 Transposition of the Great Arteries 23 Truncus Arteriosus 24 Tricuspid Atresia 25 Pulmonary Atresia 26 Ebstein s Anomaly of the Tricuspid Valve 27 Atrioventricular Canal (AV canal) 28 Hypoplastic Left Heart 29 Heart Diagram 30 Transposed heart diagram for illustrating heart conditions 31 A checklist of items to be discussed with your child s doctor 32 If surgery is required 32 Abnormal Heart Rhythms 33 Wolff-Parkinson-White Syndrome (W-P-W Syndrome) 33 Tachycardia 33 Complete Heart Block 34 Acquired Heart Disease 34 Congestive Heart Failure 34 Endocarditis 34 Cardiomyopathy 35 Congestive Cardiomyopathy 35 Restrictive Cardiomyopathy 35 Hypertrophic Cardiomyopathy 35 Rheumatic Fever 35 Kawasaki Disease 37 Chapter Three: Initial Reactions 39 Shock 39 Denial 40 Anger 40 Guilt 41 Sadness 41 Detachment 42 Gradual Acceptance 42 Living on 42 Everyone has Feelings 42 Making sense of it all 45

6 Chapter Four: Investigations 47 Blood Pressure (BP) 47 Electrocardiogram (ECG) 48 Chest X-Ray 49 Echocardiography (Echo) 50 Cardiac Catheterisation (Cardiac Cath) 50 Electrophysiogical Studies (EP) 51 Magnetic Resonance Imaging (MRI) 51 Chapter Five: Medical Procedures 53 The Fontan Operation 53 Artificial Pacemaker 54 Transplants 54 Medications 55 Diuretics 55 Ace Inhibitors 55 Digoxin 56 Drugs to Treat Abnormal Rhythms 56 Antibiotics 56 Chapter Six: Your Child in Hospital 59 Waiting 59 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 Chaplains 63 School Teacher 63 Play Specialist 64 Relationships Between Parents and Hospital Staff 64 Chapter Seven: If Your Child Requires Surgery 67 Preparation 67 Preparing Your Child 68 Telling Your Child About the Surgery 68 Hospital Stay 68 Pre-operation 69

7 Operation Day 69 Aftercare 70 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 Breastfeeding 75 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 78 Chapter Nine: Nutrition and Your Heart Child 81 Nutrition for Older Children with Heart Conditions 81 Nutrition Basics 82 The Importance of Energy 82 Growth 83 How to Achieve a High Energy Diet 83 Boosting Energy 83 Toddlers and Preschoolers 86 Examples 87 Teenagers 87 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

8 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 Pregnancy 92 Grandparents and Other People 94 Dealing with Other People 94 Your Life 94 Sitters 95 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 99 Support Groups 110 Parent to Parent New Zealand 110 La Leche League 111 References 112

9 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

10 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 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

11 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

12 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 Megan Maguire for proof reading Jo Carter for proof reading We are very grateful to The Hugh Green Trust in conjunction 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

13 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 would ensure that publication of this handbook continues. Copies can be obtained PO Box , Symonds Street, Auckland 1150 Phone or from branches. An up-to-date list of your branch can be located on the web page: xii INTRODUCTION

14 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

15 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. The 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

16 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. Eight weeks after conception the foetal heart looks like a miniature adult heart. F. CHAPTER ONE 3

17 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 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

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19 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

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21 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

22 NORMAL HEART CHAPTER ONE 9

23 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

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25 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 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

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27 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

28 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

29 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

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31 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

32 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

33 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

34 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

35 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

36 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

37 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

38 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

39 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

40 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

41 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

42 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

43 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 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. The normal heart NOTES 30 CHAPTER TWO

44 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

45 Transposed heart diagram for illustrating heart conditions 32 CHAPTER TWO

46 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

47 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

48 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

49 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

50 Kawasaki Disease Kawasaki Disease is named after Dr. Tomisaku Kawasaki, a Japanese doctor who identified the disease in 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

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52 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

53 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

54 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