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Tetralogy of Fallot

The Tetralogy of Fallot is a disease involving four separate heart defects that exist when a baby is born. These defects change the heart’s structure so that the blood leaving the heart to be carried to the rest of the body is not filled with enough oxygen. As a result, babies born with the Tetralogy of Fallot typically have a blue tint to their skin and under their fingernails. Most of the time, the Tetralogy of Fallot is diagnosed shortly after birth. Rarely the condition is not diagnosed until a child is older. No matter when it’s diagnosed, the Tetralogy of Fallot is a condition someone is born with. It is not common, and it requires surgery and regular monitoring.

Symptoms

Common symptoms include;

  • Getting tired quickly
  • Trouble breathing deeply enough
  • Fast breathing
  • Difficulty gaining weight
  • Fainting
  • Heart murmurs
  • Signs of irritability, like long bursts of crying
  • Bluish colour to the skin
  • Abnormally shaped nail beds

Infants and newborns also experience something called Tet spells. A Tet spell occurs when oxygen levels in the blood dip quickly. Tet spells usually look like a deepening of the blue colour of the skin and possibly fainting.

These occur most commonly in young infants. As children get older, they develop reflexive mechanisms to deal with the symptoms brought on by a Tet spell.

Causes & Risk Factors

The Tetralogy of Fallot is caused by abnormal development of the heart while the baby is still a foetus with no apparent cause.

The four defects that make up the Tetralogy of Fallot are:

  • Narrow lung valve – the valve that separates the heart’s lower right chamber from the pulmonary artery (the main blood supply from the heart to the lungs) becomes smaller, reducing the amount of blood the lungs receive.
  • A hole in the bottom of the heart – the septum divides the right and left ventricles, the heart’s two lower chambers. When a baby has a hole here, also known as a ventricular septal defect, oxygen-poor blood and oxygen-rich blood can mix, making the supply of blood to the body less efficient.
  • Shifted aorta – the aorta is the main artery in the body, and in normal hearts, it comes off of the left ventricle. In a heart with the Tetralogy of Fallot, it’s moved to the right and sits above the hole in the bottom of the heart. This causes the aorta, which delivers blood to the whole body, to receive some blood that has oxygen and some that does not.
  • Thickening of the muscle around the right lower chamber of the heart – the heart works hard in a baby with Tetralogy of Fallot. The body never has enough oxygen, and the heart constantly tries to keep up with the body’s demands. This causes the ventricles, the bottom chambers of the heart that push out blood, to work more than usual, resulting in the thickening of the muscle around the right ventricle. This can cause the heart to become stiff and fail in the future.

Risk factors include contracting a virus during pregnancy, drinking alcohol and not following diet and health guidelines during pregnancy, a mother above 40 years, a parent with Tetralogy of Fallot, and DiGeorge syndrome or Down syndrome in the baby.

Diagnosis & Complications

Tetralogy of Fallot is usually diagnosed as soon as a baby is born.

Some tests a doctor may use to confirm the diagnosis include:

  • Measuring blood oxygen levels
  • Echocardiogram – using sound waves to visualise the heart
  • Electrocardiogram – recording the electrical activity of the heart
  • Chest X-ray
  • Cardiac catheterization – a thin tube is inserted into a blood vessel, usually the femoral artery near the inner thigh, and guided to the heart. A dye is used to help visualise the heart. Cardiac catheterization can help a doctor become more familiar with the heart and inform treatment decisions.

Complications tend to be long-term and can include leakage of blood through the pulmonary valve, irregular heart rhythms, holes in the heart that leak or need to be patched again, one or both ventricles not working well, heart disease, and sudden death due to heart-related issues.

Treatment & Management

Any baby with Tetralogy of Fallot will need open heart surgery to correct the defects. A doctor will advise you when the best time is for your child’s surgery based on their current condition. Some children take medication to manage the symptoms until surgery.

Your doctor may also discuss the option to have a temporary surgery before having complete open heart surgery.

Additional surgeries may be needed in the future as well as medications to manage any heart issues. Patients with Tetralogy of Fallot will need regular check-ups with their doctor for the rest of their life.

 
 
 

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