• Stenosis – Narrowing
• Atresia – Absence of connection between heart chambers or to great arteries that normally communicate
  
• Atrium and Ventricle - There are 4 chambers in the heart; both right and left having two each. The receiving chamber is called an atrium while the blood pumping chamber is called the ventricle. The heart also has four valves that prevent blood flow from leaking backwards. 
  
• Mitral valve – This is the opening between the left atrium and left ventricle in the left side of the heart 
  
• Tricuspid valve – This is the opening between the right atrium and right ventricle in the right side of the heart 
  
• Aortic valve – This is the opening that connects the left ventricle of the heart with the aorta, which is the major blood vessel that delivers oxygenated blood to the body 
  
• Pulmonary valve – The valve in the opening the connects the right ventricle to the vessels that deliver the used, oxygen depleted blood to the lungs 
  
• Septum – The wall that divides the right and left sides of the heart; the wall that divides both the atria is called the atrial septum and the wall that divides both the ventricles is called the  ventricular septum
• The circulation consists of two circuits in series. The left ventricle pumps out oxygenated blood into the body that reach out to the organs, e.g., brain, liver, kidneys, muscles and bowels, etc. Organs take up oxygen and nutrients from the blood flow that is returned to the heart's right atrium via the veins. Next the right ventricle pumps de-oxygenated blood into the pulmonary circuit where blood is replenished with oxygen. Oxygenated blood returns to the left atrium and the circulation starts all over again. 
  

• Congenital aortic stenosis
• Mitral valve stenosis
• Pulmonary valve atresia/stenosis
• Tetralogy of Fallot in which blood flow towards the lungs is obstructed
  
• Coarctation of aorta 
  
• Interrupted aortic arch

2. Left to right shunts

• Antenatal (before a child is born) diagnosis when a fetal cardiologist detects CHD during regular check-ups before the child is born
• If this is the case, plan of management and appropriate treatment can be fully discussed and planned with the parents before labour  
• After the child is born when diagnosis is made soon after birth due to abnormalities detected in routine tests e.g.
• Abnormal (or low) oxygen saturation measurements 
• Presence of heart murmurs during physical examination
  
• Severe symptoms of impaired circulation
  
• Other defects may only be diagnosed later on in the child's life.

• Physical examination
  
• Electrocardiogram (ECG)
• 2-D echocardiogram (ECHO)  - Gold Standard of Imaging 
• Cardiac catheterisation
  
• Computer tomography, CT-angiography
  
• Cardiac magnetic resonance imaging (CMRI)

How does it affect my child’s health? 

• Hours after birth e.g. in total anomalous pulmonary venous drainage
• Within a few days e.g. in transposition of great arteries

In other cases, the operation can be carried out after a few months or years, whilst at times it is to repair the defect(s) in stages. Staged operations typically involve a lifesaving procedure as a neonate, for example, implanting a plastic tube to redirect the blood towards the lungs (i.e. systemopulmonary shunt) so that your child can grow bigger and stronger before carrying out a definitive procedure - e.g. In Tetralogy of Fallot.

Surgery for paediatric congenital heart diseases 

1. Open heart surgery

1.1 Septal defects

1.1.1. Atrial septal defect (ASD): The surgeon closes the defect between the atria

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1.1.2. Ventricular septal defect (VSD): The surgeon closes the defect between the ventricles

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1.1.3. Atrio-ventricular defect: The surgeon seperates the right and left side and repairs abnormal valves

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1.4. Other complex defects

• Your paediatric cardiologist will refer you and your child to a paediatric cardiac surgeon who will discuss with you the planned surgical procedure.
• Depending on the urgency of the surgery, the discussion will either take place in an out-patient clinic or in the ward.
• Once the date for surgery is fixed, your child will be admitted a day prior to the planned procedure for pre-operative preparation such as:

• Taking blood for routine tests
• Chest X-ray
  
• ECG
• The tests rule out any active infection or any other minor conditions that were not detected in previous investigations.
• As a parent, you would need to prepare yourself psychologically. Discuss with your child’s surgeon of any concerns that you may have.
• Should you prefer, you can request for a tour of the paediatrics intensive care unit to be arranged prior to surgery.

What happens during the surgery? 

• You will be allowed to accompany your child into the area in the operating theatre where the anaesthetic doctor will prepare your child to receive general anaesthesia. 
• You child will be injected with or inhale in an anaesthetic agent to put him/her to sleep.
• Once your child falls asleep, you will be asked to leave the operating theatre.
• The anaesthetist will then insert a breathing tube (endotracheal tube) and put in various drips – central venous cathether, arterial cannula, intravenous cannula.
  
• The time taken for the surgery would depend on the complexity of the procedure. It could between an hour to as long as 6 hours.
• Once the procedure is successfully performed, you will be notified by one of the health professionals.
• The surgeon will usually speak to you after the operation.

• Your child will be transferred to the paediatric intensive care unit (PICU) after the operation.
• There will be many tubes connected to your child for various purposes, so don’t be alarmed.
• Your child will be usually be under deep sedation (asleep) usually up to 24 hours or sometimes long after surgery depending on the complexity of the surgery and age of the child.
• It is important to cooperate with the paediatric ICU staff who are well trained, caring and friendly.
• Usually a nurse will be assigned to solely look after your child during his/ her stay in ICU.
• As every patient is different, the staff will discuss your child’s hourly/ daily progress and treatment plans.
• Your child may be treated in the ICU for about 2 days depending on his/her recovery progress.
• When your child is stable, he/ she will then be transferred to a high dependency unit (HDU) for further close monitoring for another 1-2 days until he/she is stable enough to be transferred to the general paediatric ward.
  
• At this stage, most of the tubes and drips will be removed.
  
• Most of the recovery will be taken place in the general ward. Sometimes patients recover quickly and can go home from HDU.
  
• He/ she will be encouraged to move about and will be guided by a physiotherapist.
  
• Depending on the recovery progress, you can expect a period of 5-7 days in the general ward before your child is discharged from the hospital.
  
• A thorough physical examination and various investigations will be carried out prior to discharge. These tests are:

• Blood Tests
• ECG
• Chest X-ray
• 2-D ECHO
• During your child’s recovery period, parents are encouraged to get as much experience as possible. Once the child is discharged from the hospital, you will be solely looking after the child at home. This can be physically demanding and mentally challenging.