What are the 4 problems in tetralogy of Fallot?

Tetralogy of Fallot - What it is

Tetralogy of Fallot (TOF) consists of four defects.

  1. A hole (ventricular septal defect) between the two lower heart chambers (ventricles).
  2. There is an obstruction from the right ventricle to the lung caused by thick muscle (infundibular stenosis) and/or narrowing of the pulmonary valve (pulmonary stenosis).
  3. The aorta, which is the major artery that supplies oxygen-rich blood to the body, lies over the ventricular septal defect (overriding aorta).
  4. The muscle of the right ventricle becomes thick (right ventricular hypertrophy).

Less oxygenated ‘blue’ blood returning to the right side of the heart is mixed with the oxygenated ‘red’ blood from the left side of the heart through the hole and into the overriding aorta. Babies with unrepaired TOF are often blue. Sometimes, the pulmonary valve is also completely obstructed (pulmonary atresia).

Associated problems can include an atrial septal defect (hole between the two upper heart chambers) and abnormalities of the coronary arteries.

Common problems which can remain after repair can include:

  1. Residual pulmonary valve narrowing or leaking (Pulmonary stenosis or regurgitation)
  2. Branch pulmonary artery narrowing (Pulmonary artery stenosis)
  3. Abnormal heart rhythms (Arrhythmias)
  4. Aortic valve leaking (Aortic regurgitation) 

Tetralogy of Fallot - Symptoms


​You will need to come back earlier to see your doctor if you develop breathlessness, palpitations (abnormal sensation of the heart beating), lower limb swelling, syncope (fainting), giddiness or chest pain.

Tetralogy of Fallot - How to prevent?

Tetralogy of Fallot - Causes and Risk Factors


Risk factors

Activity

You may need to restrict your activity especially if your heart function is weak or there is presence of abnormal heart rhythm. Your cardiologist will determine the limitations.

Prevention of endocarditis

You will need antibiotics to prevent endocarditis (infection of the inner layer of the heart) before any dental procedures if there are residual problems after operation. Antibiotics may also be required if you should go for body piercing or tattooing. Please consult your cardiologist.

Family planning and pregnancy 

There are some associated risks with pregnancy even after TOF repair is done. Please consult your cardiologist for advice.

Additional Information

In the long term, your heart muscle function may be weakened and you may need medications. After the TOF repair, you may develop abnormal heart rhythm that can cause giddiness or fainting. Occasionally, you may need to go for another procedure to eliminate the abnormal heart rhythm if the symptoms are not well controlled with medications.

Tetralogy of Fallot - Diagnosis

Diagnosis normally starts with a physical examination by a doctor, assessing your medical history and routine tests. Other tests recommended may include:

  • Chest X-ray
  • Electrocardiogram (ECG)
  • Echocardiogram
  • Exercise testing
  • Magnetic Resonance Imaging (MRI) scan of the heart
  • Cardiac catherisation

Tetralogy of Fallot - Treatments

In early infancy, babies with TOF may need a procedure to temporarily increase blood flow to the lung (Blalock-Taussig shunt). Total surgical repair will be done later when the baby is older. In adulthood, there may be residual obstruction from the thick muscle in the right heart chambers or from the narrowed pulmonary valve. Sometimes, the pulmonary valve may become leaky (does not close properly) after the initial childhood repair. Thus, further intervention (pulmonary valve replacement) may be necessary during adulthood.

Options for pulmonary valve replacement are surgical replacement or percutaneous pulmonary valve implantation. You may be suitable for percutaneous pulmonary valve implant if you have a previous pulmonary valve replacement or conduit. Percutaneous pulmonary valve implantation (PPVI), also referred to as transcatheter pulmonary valve implantation, is a minimally invasive procedure to correct severe pulmonary valve and conduit stenosis (narrowing) or regurgitation (leaking). This procedure is performed in the invasive cardiovascular laboratory. You will be given a general anaesthetic (GA) by a qualified anaesthetist. You will lie on an X-ray table and an X-ray camera will move over your chest during the procedure.


You may need to take medicine after your operation to strengthen your heart muscle contraction and to control any abnormal heart rhythm. There are various tests that may be required during follow-up appointment before decisions can be made on the need and the timing of further procedures or operations.

Tetralogy of Fallot - Preparing for surgery

Tetralogy of Fallot - Post-surgery care

Tetralogy of Fallot - Other Information

What is the most common complication found in patients with tetralogy of Fallot?

When a valve leaks, blood can flow back into the chamber. Patients with tetralogy of Fallot are also at risk for developing aneurysms of the ascending aorta. The most common valve problem after tetralogy of Fallot repair is a leaking pulmonary valve, but the aortic and tricuspid heart valves can leak also.

How many heart defects are present in tetralogy of Fallot?

Tetralogy of Fallot is made up of the following four defects of the heart and its blood vessels: A hole in the wall between the two lower chambers―or ventricles―of the heart. This condition also is called a ventricular septal defect. A narrowing of the pulmonary valve and main pulmonary artery.

What happens in tetralogy Fallot?

Tetralogy of Fallot (fah-LO) is a congenital (present at birth) heart defect. In tetralogy of Fallot (TOF), four related heart defects change the way blood flows to the lungs and through the heart. TOF is repaired through open-heart surgery soon after birth or later in infancy.

How does tetralogy of Fallot affect the heart?

In a tetralogy of Fallot heart, the aorta is located between both the left and the right ventricle. This causes oxygen-poor blood from the right ventricle to flow into the aorta instead of the pulmonary artery. If infants with tetralogy of Fallot are not treated, the symptoms usually become progressively more severe.