This chapter discusses preparing for two possibilities: 1) if your child needs surgery, and 2) if there is a health emergency.

Getting ready if surgery is needed

The cardiologist may recommend surgery for your child to either repair the CHD or to reconstruct the heart so it can function as well as it can with its defect.

For some children, it may be possible to repair the heart completely with a single surgery. For other children, surgery may be needed to relieve a problem or help the child gain weight before definitive surgery. This is called palliative surgery. Many children with complex CHDs require staged surgeries to get the best result.

The decision to have surgery

Before any surgery happens, the cardiologist will first talk to your family about the best type of surgery and the best time for it to take place. The information you and your child give to your cardiologist and nurse clinician about your child’s current health status is a very important part of deciding when a surgery should happen. Your child will be included in this conversation if they are old enough.

Then, your cardiologist will present your child’s case to the health care team in a meeting. The team consists of cardiologists, surgeons, radiologists, nurses, and other specialists. After reviewing your child’s health status, diagnostic test results, and other information as a group, the health care team decides whether or not the operation is the best treatment at this time.

Your cardiologist will contact you with the final decision about surgery. If your child has been diagnosed before birth, the team will perform an echocardiogram on your newborn and the results will be discussed with you. (See echocardiogram in Appendix A for a description of this diagnostic test and what to expect.) The knowing-but-not-knowing period will change into a knowing period with a definite diagnosis and plan for your newborn.

Meeting the surgical team

Once the decision is made for surgery, you and your child will be scheduled for a pre-admission appointment that takes several hours and includes meetings with several health care professionals and some testing.

Sometimes this pre-admission appointment is held weeks before the surgery, and other times it is held the day prior. Each case is individual and depends on the timing or urgency of the surgery.

At the appointment you will meet your surgeon, nurse practitioner, and cardiac surgery nurse clinician. A psychologist, child life specialist—who has experience in preparing children of all ages for surgery—and/or other specialists may be involved but not for every child. Your anaesthetist will either see you and your child at the pre-admission appointment or on the morning of the surgery.

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I have to say, it is extremely difficult to be taking her back into the hospital, even though we have known since she was born that this is only one of many operations that she will need. We will hope and pray that after her surgery she will be that much stronger and more than ready to conquer new things. —PARENT

At the pre-admission appointment, the surgeon will discuss

  • the reason for the surgery,
  • the risks and benefits of having the surgery performed,
  • the surgical procedure involved,
  • the expected length of the hospital stay.

Ask any questions you have and discuss your concerns. Repeat information back to make sure you understand it correctly. For example, if the surgeon has just explained the type of surgery to you, you could say, “So, what you are planning to do is . . .”

In addition, you may be asked to sign the informed consent for surgery. (See “Giving an informed consent for surgery” in this section.)

You will also be sent a package of helpful information from the surgeon’s office and the contact information for the cardiac surgery nurse clinician at the hospital. The package contains everything you need to know about the pre-operative day.

Giving an informed consent for surgery

An important part of the meeting is signing a document called an informed consent. An informed consent says you have been told about the type of surgery and the risks involved for your child, you understood this information, you had a chance to ask questions, and you agree to the surgery.

Be sure to read the consent completely before signing. A legal guardian must sign the form. Children aged 18 years may sign their own consent forms.

Some families do not feel comfortable asking about the surgical procedure. However, it is not possible to give an informed consent without an understanding of what will happen to your child. The information given in your pre-admission appointment will be helpful.

I explained to him and the other kids that his heart was like a jigsaw puzzle that was missing pieces. The surgeon could put the puzzle back together and make it right. —PARENT
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Preparing for the appointment with the surgical team

In the time leading up to the appointment, think of several questions and concerns you would like to talk about with the team. Write them down so you feel as prepared as possible for the meeting. It is a good idea to write down the answers during the meeting so you can review them later. If you want to do some research to learn about the procedure, please ask your nurse for a credible website.

Here are examples of questions you may want to ask:

  • What will this surgery do for our child if it is successful?
  • What is the best we can hope for?
  • What is the worst that might happen?
  • Is there anything that could be done instead of the operation that would get the same results?
  • What will happen if we do nothing?
  • What are the most common complications?
  • How often do those complications occur?
  • What is the most serious complication?
  • How often does that complication occur?
  • What are the possible risks and complications of the anaesthetic, if any?
  • Who will perform the operation
  • What experience has the surgeon had with this type of procedure?
  • How successful have those other procedures been?

In some emergencies, you may not have time to ask anything. The health care team may have to act immediately to prevent further complications arising.

Bringing a support person to the appointment with the surgical team

Families often report feeling overwhelmed by the amount of information they receive during the appointment with the surgical team. It can help to bring a family member or friend to the appointment. That person can help you by taking notes, remembering the details, and making sense of the information you are given.

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I know we will make it through all the hurdles that come our way, thanks to all of your prayers, good thoughts, and good wishes. I look forward to one day sharing with Izumi all the inspirational emails that we have received on both of her hospital stays. —PARENT

The risks of surgery

The risk involved in your child’s heart surgery depends on many factors, including your child’s age, health, history of previous surgeries, and the details of the planned operation, such as whether it is an open-heart or closed-heart procedure. Open-heart surgery means that a heart-lung machine (often called a cardiopulmonary bypass) is needed. Closed-heart surgery does not use a heart-lung machine.

Although heart surgery for babies and children has improved a great deal over the past twenty years, it still carries some risk. There are two types of risk: morbidity (complications during or following surgery) and mortality (loss of life). The level of risk depends on the procedure your child needs. The surgeon will talk to you about the specific risks for each procedure.

In general, complications after cardiac surgery are rare. In the unusual case that complications occur, the most common ones include

  • bleeding after surgery,
  • heart rhythm problems,
  • the need for a permanent pacemaker in future,
  • infection, which may be minor or severe.

The risk of kidney damage, liver damage, or stroke following cardiac surgery is now extremely low.

Your surgeon will be happy to address your questions about surgical complications during your pre-admission appointment.

Blood products and their safety

Your child may need blood or blood products for surgery, especially if it is open-heart surgery. For example, when the heart-lung machine is used in babies and small children, blood products are used to fill the machine’s tubing. If other fluids are used instead, the child’s blood can become diluted and the cells of the body do not get enough oxygen.

If you have spiritual beliefs about the use of blood products, please discuss your concerns with your health care team.

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Open-heart surgery means that a heart-lung machine (often called a cardiopulmonary bypass) is needed.
Closed-heart surgery does not use a heart-lung machine.

Planning for health emergencies

Prepare for an emergency. Know what to do if an emergency arises. It is like planning for a house fire or earthquake—you hope one will never happen but being prepared will improve the outcome.

Your plan should include

  • a medical identification (ID) necklace or bracelet for your child (if required);
  • family members’ and other caregivers’ involvement;
  • the signs to watch for, such as symptoms or drug reactions;
  • information on how and where to get emergency help;
  • information to give to paramedics or emergency department staff.

Medical ID jewellery

Many families wonder if their child should wear a medical ID necklace or bracelet. While it is not needed in most cases, medical ID jewellery is a good idea for children with certain heart conditions, medications, or implantable medical devices. Examples of these situations include

  • single-ventricle heart defects;
  • inherited arrhythmias, such as long QT syndrome (LQTS), catecholaminergic polymorphic ventricular tachycardia (CPVT), or arrhythmogenic right ventricular cardiomyopathy (ARVC);
  • specific anticoagulation therapy, such as warfarin;
  • life-threatening allergies that can cause anaphylaxis and for which your child carries an EpiPen;
  • implanted pacemakers or implantable cardioverter defibrillators (ICDs);
  • transplant patients.

For children in these situations, medical ID jewellery can be very helpful when a parent or caregiver is not with them and the child cannot speak for themselves, e.g., during camp or school trips.

How does medical ID jewellery work?

Organizations, like MedicAlert Foundation Canada, provide a range of medical ID options. Many companies offer engraved jewellery with a one-time cost. Each family should consider what type of medical ID will work best for their child.

The medical ID organization keeps your child’s medical information in a database and charges a monthly subscription fee.

It is a good idea to talk with your nurse or cardiologist about whether medical ID jewellery is recommended for your child and if so, what information should be included on the ID.

Family members and caregivers

Family members, teachers, babysitters, and other caregivers should recognize if your child is having serious problems.

Complete the form “My Child’s Important Medical Information” in Appendix B. Make sure you provide a copy to anyone taking care of or supervising your child. Ask them to read it and check if they have any questions.

Encourage everyone to take an infant/child cardiopulmonary resuscitation (CPR) course. Your family can also help look after your other responsibilities if you are going to be away from home. Some of the things they can help with are taking care of children, livestock, and pets, and calling your workplace.

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These few days while I have just sat at the bedside have been the most exhausting days of my life. —PARENT

Monitoring your child’s health: signs and symptoms

It is important for each of us to listen to our bodies. As children with CHD grow and develop, they learn to spot small changes in their health. Parents of infants or younger children often are the first ones to notice changes in their child’s health.

Symptoms are the body’s way of telling us something is wrong with it. Just as a cough and a stuffy nose are the body’s way of telling us we have a cold, there are symptoms we can learn that tell us how our hearts are working.

Each child growing up with heart disease has a unique heart and, therefore, a unique set of indicators for a problem. It is important that you ask your child’s cardiologist at each follow-up appointment what symptoms you should be watching for as your child grows and develops.

Different symptoms will have different degrees of significance, depending on your child’s unique heart condition and medical history. Discuss with your cardiologist the level of urgency for your child and the plan of action should your child develop any of the following symptoms:

  • gradual increased effort of breathing
  • decrease in exercise performance or tolerance
  • increasing pallor (pale skin) or cyanosis (blue skin) at rest
  • increasing tiredness or lethargy
  • decrease in appetite or food intake
  • significant weight gain or weight loss over a short period of time
  • severe nausea or vomiting
  • change in bowel patterns (diarrhea, constipation)
  • dizziness or faintness with exertion or at rest
  • anxiety or restlessness
  • chest pains, particularly with exercise or activity (This is often normal and related to muscles and bones changing and growing but can also indicate that the heart is needing to work harder.)
  • palpitations or pounding in the chest that may indicate an abnormal heartbeat (Some children use different words, such as “I have butterflies in my chest” or “My heart is racing,” to describe the sensation.)

Ask your cardiologist if there are any other symptoms that you should watch for and who you should call if you notice them.

Signs your child needs urgent medical help

Some symptoms require urgent medical attention.

Get help immediately if your child experiences any of the following symptoms:

  • difficulty breathing (panting, gasping, or wheezing)
  • seizures
  • palpitations associated with dizziness, chest pain, or loss of consciousness (fainting)
  • dizziness or faintness
  • change in alertness, including confusion, extreme lethargy, or unresponsiveness

Ask your cardiologist if there are any other symptoms that indicate your child needs urgent medical help.

Steps to follow if your child needs urgent medical help

If your child appears to require urgent medical help, follow these steps:

  1. Have your child rest and stop activities.
  2. Give any emergency medications as instructed previously by your doctor in preparation for emergencies.
  3. Stay calm and act at once. Do not delay getting to the hospital. Avoid packing a suitcase or notifying family or friends at this time.
  4. Call 911 or your local emergency number if your child needs urgent medical attention. Unless you are minutes away from the hospital and can get there safely, you are better off calling an ambulance. Although it may seem to take more time than going yourself, it is important that your child be monitored and given any necessary treatment during transport.
  5. If an ambulance is not available, drive carefully to your nearest hospital emergency. Any doctor or emergency department can contact the cardiologist on call 24 hours a day, 7 days a week.
  6. Contact your pediatric cardiologist, your family doctor/pediatrician, and/or any other health specialists your child has. (Use the “Health provider contact information” in the “My Child’s Important Medical Information” form in Appendix B.)

Information for paramedics and emergency staff

Use the “My Child’s Important Medical Information” form in Appendix B to record your child’s critical data. Always have this form on hand. Store copies on your phone, in your wallet, car, diaper bag, and your child’s backpack. Make sure caregivers have one or know where the copies are.

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Order medical ID jewellery for your child (if required).
Include family members and other caregivers in the plan.
Learn what to watch for, such as symptoms or drug reactions.
Know how and where to get help.
Prepare information to give to paramedics or emergency staff.

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» Go to Appendix A: Diagnostic Tests