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What Is Tetralogy of Fallot?

A team of congenital heart specialists from the National University Heart Centre, Singapore (NUHCS) and the department of paediatrics at National University Hospital (NUH) in Singapore performed a procedure in August 2016 to treat a form of congenital heart defect known as Tetralogy of Fallot (or in short TOF) without open heart surgery.

During the procedure, doctors inserted a thin, hollow tube containing a “specially designed heart valve” into the vein and push it up into the heart. The valve is crimped into a small size but expands with the help of a balloon once it is in the right position. This procedure takes a few hours and helps improve heart function so that the need for open heart surgery can be delayed.

Being a defect that affects the functioning of heart valve, TOF is the most common types of congenital heart defects that refers to 4 heart defects present from the time of birth: a ventricular septal defect (hole between the ventricular chambers that allows blue and red blood to mix), pulmonary stenosis (narrowing that makes it difficult for blue blood to reach the lungs), right ventricular hypertrophy (thickening of the heart muscle from pumping blood past the narrowing), and an overriding aorta.

Such defect also affects the normal functioning of the pulmonary heart valve, which controls blood flow from the right chamber of the heart to the lungs. Obstruction in the pulmonary valve prevents the normal amount of blood from being pumped to the lungs. Sometimes the pulmonary valve is completely obstructed (pulmonary atresia). Babies with TOF often have cyanosis (blue-tinged color of the skin) resulting from lack of oxygen-rich blood.

Genetic factors or environmental or a combination of both are thought to play a role in patients with TOF. Statistics show that approximately 25 percent of untreated patients with TOF die within the first year of life, 40 percent by 4 years, 70 percent by 10 years, and 95 percent by 40 years. Complete repair that requires open-heart surgery tends to be done early in life, usually when the child is between 6 months and 2 years old. However, should the child be too weak to go through surgery, a shunt procedure is done first. During the procedure, the surgeon makes a surgical cut in the left side of the chest to move blood from one area to another. Once the child is older, the shunt is closed and the main repair in the heart is then performed. Sometimes a synthetic tube with a man-made or biological valve in it is placed between the right ventricle and the pulmonary artery.

Man-made valves last longer than biological valves and usually do not have to be replaced. Nevertheless, patients with man-made valves need to take medicines to prevent blood clots from forming on the valve that may lead to heart attack or stroke. Man-made valves also raise your risk of infective endocarditis. Biological valves are made from pig, cow, or human heart tissue and may have man-made parts as well. These valves are specially treated, so patient would not need medicines to stop the body from rejecting the valve. Biological valves, however, usually have to be replaced after about 10 to 12 years as they are prone to become a leaky or narrowed due to wear and tear. As a result, a repeat surgical operation must be carried out every 10 to 12 years to replace the valve.

With the new procedure mentioned above, patient does not feel pain and can resume normal activities that include playing badminton after the procedure, and it reduces the psychological stress of repeat surgery for the patient’s family, too. The procedure was first performed in London in 2000. Studies have so far shown good results, and the valves of almost all patients who went through the procedure were free of leakages for up to 5 years.

Cost about S$30,000 (US$21,400), the new device is certainly not cheap. But with a shorter hospitalization, it may still be cheaper than a long-term hospitalization plus expensive surgery and manpower to look after the patient post-operatively.




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