What Is Tetralogy of Fallot?

Tetralogy of Fallot or ToF is one of the congenital heart disease. The disorder that affects the structure of the heart causes the pumping of blood that no longer contains oxygen ( deoxygenated blood ) from the heart to the entire body.

ToF is a rare disorder and is generally detectable at the time of newborn. This disorder always consists of a combination of four sets of conditions as follows:
  • Pulmonary stenosis. This disorder causes the pulmonary valve to not open completely so that the amount of blood that reaches the lungs is reduced. Pulmonary valve is a valve that becomes the dividing door between ventricular chambers or the heart chambers with blood vessels leading to the lungs.
  • Ventricular Septal Defect (VSD). In this disorder, there is a hole formed in the septum or wall separating the right and left ventricle. This disorder causes oxygen-rich blood mixing from the left ventricle with oxygen-poor blood from the right ventricle.
  • Hypertrophy or abnormal thickening of the right ventricle wall muscle. This condition occurs as a result of narrowing of the opening of the pulmonary valve.
  • Abnormal aortic position, between the left and right ventricle (just above VSD), resulting in poor oxygenated blood flowing into the aorta. In the normal heart, the main aorta or artery should come out of the left ventricle containing oxygen-rich blood.

Symptoms of Tetralogy of Fallot
There are various symptoms of ToF. This diversity depends on the severity of the disturbances in the bloodstream. A number of symptoms experienced by people with ToF include:
  • Shortness of breath and rapid breathing (such as wheezing), especially during activity or throughout the meal.
  • The skin, lips, and mucous membranes lining the inner surface of the nose and mouth are blue (cyanosis) due to poor blood circulation of oxygen.
  • Basic nails of hands and feet are round and convex (clubbing of fingers) due to enlargement of bone or skin around the nail.
  • Urgent or fussy.
  • Fainting.
  • Bad growth.
  • Difficulty in gaining weight.
  • Easily tired.
Infants with ToF generally have cyanosis at 8 to 16 weeks of age. These symptoms sometimes appear when the baby is eating, crying, or anxious. This is because oxygen levels in the blood suddenly decline sharply. In toddlers and older children will suddenly squat when they experience shortness of breath.

Immediately take your child to the hospital if cyanosis is accompanied by grumpy, convulsive, unconscious, and difficulty breathing. During an ambulance or on the way to the hospital, tilt your child's body and pull his knee toward his chest. This position will help increase blood flow to the lungs.

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Causes and Risk Factors Tetralogy of Fallot
The cause behind Tetralogy of Fallot is not known for certain, but there are a number of factors that are thought to increase the risks to a baby. Some of these risk factors are:
  • Age of pregnant women over 40 years.
  • Viral infections during pregnancy, such as rubella (German measles).
  • Malnutrition during pregnancy.
  • Have one or both parents with the same disorder.
  • Alcoholic addiction during pregnancy.
  • Diabetes to the mother.
  • The presence of birth defects is a baby suffering from Down Syndrome or DiGeorge Syndrome.
Tetralogy of Fallot occurs since the fetus is still in the forming stage, precisely when the process of heart formation.

Diagnosis Process of Tetralogy of Fallot
Tetralogy of Fallot is generally detected soon after the baby is born until the age of the toddler. However, based on the severity and symptoms, there is also a new ToF realized when people have passed the toddler.

The main symptom that becomes the reference is the blue skin. If your baby shows this sign, the doctor will recommend a series of further tests to confirm the diagnosis. The check step includes:
  • Blood tests to check blood cell count and hemoglobin.
  • Echocardiogram , which checks the performance and structure, thickness, and motion of each heartbeat through ultrasound.
  • Electrocardiogram (EKG) to check the electrical activity of the abnormal heart muscle.
  • Chest X-rays to check the heart shape and aortic position.
  • Cardiac catheterization that serves to examine the heart's structure as well as pressure and oxygen levels in the hall or ventricular chambers of the heart, and blood vessels. This is an important examination that can confirm the presence of heart disorders Tetralogy of Fallot.
  • Pulse oximetry , the process of measuring the levels of oxygen in the blood.
In addition, ToF is also sometimes detectable during pregnancy through an ultrasound process. If there is an alleged ToF, the doctor will recommend a fetal echocardiogram to confirm the diagnosis.

Step Handling Tetralogy of Fallot
Surgery is the only effective treatment for tetralogy of Fallot. This procedure is generally done immediately after the baby is born or at the age of several months. This choice will be determined by your doctor based on your baby's condition.
In general, the patient's surgery aims to widen the vascular door to the lungs (narrow pulmonary valve) and close the hole due to VSD. This surgery will help smooth the flow of blood into the lungs, so that the oxygen levels in the patient's blood will increase, and the symptoms experienced will be reduced.

However, there are also some patients who sometimes require shunt- mounting operations prior to undergoing the major surgical procedures. For example in premature infants or who have pulmonary arteries that are not fully developed. The shunt will connect the aorta to the pulmonary artery and will increase blood flow to the baby's lungs. Shunt will then be released in the next operation.

Diagnosis and surgery done as early as possible will increase the chance of people with ToF to live a normal life. However, people still have to undergo regular health checks to monitor the condition and its development.

Risk of Postoperative Complications
Generally, infants who have undergone surgery can live a normal life. However, surgery remains at risk of triggering complications that include irregular heartbeats or arrhythmias, enlargement of the base of the main artery (aorta), blood reverses back into the right ventricle via a leaking pulmonary valve, or infection.

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