Tetralogy of Fallot

Tetralogy of Fallot is a rare disorder that is caused by the combination of four heart defects that manifest at the time of birth (congenital).

These deficiencies can alter the heart’s structure causing blood with low oxygen levels to drain from the heart to other parts of our body. Children and infants with the Tetralogy of Fallot generally have blue-tinged skin due to the fact that their blood does not have enough oxygen.

Tetralogy of Fallot is usually diagnosed when the baby is in the infant stage or shortly after. It is possible, based upon the seriousness of problems and symptoms the tetralogy of Fallot is not discovered until later in life.

All children with Tetralogy of Fallot require corrective surgery. Patients with tetralogy of Fallot require regular medical check-ups throughout their lives and could be restricted from activities¹.

 

Symptoms

The Tetralogy of Fallot symptoms differs, based on the blood flow that’s being blocked. The symptoms and signs could include:

• A blue-colored skin is caused by the low levels of oxygen in the blood (cyanosis)
• Breathing is short and quick breathing, particularly during meals or exercising
• Poor weight gain
• It is easy to get tangled up in the gym or during exercise
• Irritability
• The crying continues for hours
• Heart murmur
• Fainting
• An unusual, rounded, shape on the surface of the nail the toes, and fingers (clubbing)

Tet spells

Sometimes, babies with the tetralogy of Fallot can suddenly have deep blue nails, skin, and lips following crying, feeding, or even when they are stressed.

These are also known as Tet spells. Tet spells result from a rapid reduction in the oxygen levels in the blood. Tet-related spells are more common in infants who are two to four months of age. Children who are older or in their toddler years may naturally squat when they’re feeling short of breath. Squatting can increase blood flow to the lungs.

When is the best time to visit a doctor?

Consult a doctor if observe that your child is suffering from the following symptoms or signs:

• Trouble breathing
• The skin is a bluish discoloration
• Passing out or seizures
• Weakness
• Unusual irritability

If your baby is blue (cyanotic) Place your baby on his or her side, and bring your baby’s knees to the chest of your baby. This increases blood flow to the lungs. Make sure to dial 911 or the local emergency number right away.

Causes

Tetralogy of Fallot is a condition that occurs when the baby’s heart develops during the pregnancy. Usually, the reason is unidentified.

Tetralogy of Fallot has four deficiencies:

• The lungs are narrowed valve (pulmonary valve stenosis). Narrowing of the valve that divides the left chamber in the lower part of the heart (right ventricle) from the blood vessel that carries blood to the lung (pulmonary arterial) restricts the flow of blood to the lung. The narrowing may affect the muscles beneath the valve that forms the pulmonary. Sometimes the pulmonary valve fails to develop correctly (pulmonary atresia).
• A gap between the lower chambers of the heart (ventricular septal defect). A ventricular septal defect (VSD) is a defect in the heart’s wall (septum) that divides the two chambers below the heart (left and right ventricles). The defect causes the oxygen-deficient blood from the right ventricle, to join with blood rich in oxygen from the left ventricle. This leads to inefficient blood flow, which reduces the flow of oxygen-rich blood for the entire body. The problem can eventually weaken the heart.
• shifts of the main artery in the body (aorta). Normally the aorta runs off of the left ventricle. In the tetralogy of Fallot, the aorta may be placed in the wrong place. It is shifted to the right and is directly over an opening in the wall of the heart (ventricular septal defects). This means that the aorta gets a mix of oxygen-rich blood and oxygen-poor blood coming from both the left and right ventricles.
• The right lower chamber of the heart (right hypertrophy of the ventricular wall). When the heart’s pumping actions are overworked by the muscular part of the right ventricle gets thick. In time, this can make the heart stiff up, weaken and eventually end up failing.

A few children or adults with tetralogy of Fallot might also have other heart problems, such as a gap between the upper chambers of the heart (atrial septal defects) or an arch of the right aortic or issues with coronary arteries.

Risk factors

The exact reason for the tetralogy of Fallot is not known, certain factors could increase the chance of having a child with this disorder. The risk factors for tetralogy Fallot are:

• An illness caused by a virus during pregnancy, like rubella (German measles)
• Drinking alcohol during pregnancy
• Poor nutrition during pregnancy
• Mothers who are older than 40
• A parent who is a Tetralogy of Fallot
• It is possible to detect Down syndrome, or the DiGeorge syndrome can be seen in the newborn baby

Complications

A potential complication of the Tetralogy of Fallot is the infection of the lining inside the heart valve or the heart, due to a bacterial infection (infective endocarditis). The doctor who treats your child might suggest taking antibiotics prior to certain dental procedures in order to avoid the development of infections that can trigger this type of infection.

Patients with untreated tetralogy Fallot typically experience severe complications over time. This could lead to disabilities or death before young adulthood.

Complications of tetralogy from Fallot surgery

Although the majority of children and adults recover well following open-heart surgery for repair of the tetralogy Fallot problems (intracardiac repair) However, complications that last for a long time are quite common. These complications could be:

• Leaking valves in the pulmonary system (chronic regurgitation pulmonary) where blood leaks out of the valve, and then back to the pumping chamber (right ventricle)
• Leaking in the tricuspid valve
• There are holes in the walls between ventricles (ventricular septal defects) that persist to leak even after repair, or could require to be repaired again.
• A large left or right ventricle that’s not functioning in a proper way
• Insane heartbeats (arrhythmias)
• Coronary arterial disease
• The aorta’s ascending area is enlarged (aortic root dilation)
• Sudden cardiac death

It’s vital to have regular checks with a heart specialist who is trained to care for patients with congenital heart disease (pediatric cardiac surgeon or adult congenital cardiologists).

 

Diagnosis

Usually, it is the case that the Tetralogy of Fallot is detected shortly after the birth. The skin of your baby may appear blue. The doctor may hear an odd noise (heart murmur) when they listen to the heart of the infant via a Stethoscope.

Tests to determine if you have tetralogy Fallot are:

• Oxygen levels measurement (pulse oxygen measurement). A small sensor that is placed on the finger or toe, measures the level of oxygen in the blood.
• Echocardiogram. An echocardiogram uses sound waves to produce images of the heart’s motion. An echocardiogram will show the structure, location, and functions of the heart’s wall chambers of the heart, the heart, and the pulmonary valves as well as the aorta.
• Electrocardiogram (ECG or EKG). An electrocardiogram is a way to record the electrical activity of the heart when it contracts. In this process, it is possible to stick sticky patches of wires (electrodes) are placed on the wrists, chests and ankles. The wires are connected to a computer that shows the heart’s beat. An ECG It can be used to determine if the heart chambers are too large and also if there’s an abnormal rhythm (arrhythmia).
• Chest Xray. A chest X-ray can reveal the structure of the heart as well as the lung. A typical sign of tetralogy Fallot on an X-ray would be an oval-shaped heart due to the right ventricle being larger.
• The procedure is known as cardiac catheterization. Doctors may use this test to determine the heart’s structure and determine the best surgical approach. The doctor inserts an extremely thin and malleable tubing (catheter) into a blood vessel, which is usually in the groin region, and then guides it towards the heart. The catheter is filled with dye to make the heart’s structures more visible through X-rays. The doctor will be able to gauge oxygen levels and pressure in the heart’s chambers as well as blood vessels throughout the procedure.

Treatment

All babies diagnosed with the tetralogy of Fallot require corrective surgery by a cardiac (cardiovascular) specialist. If your child is not treated, he or she could not grow or develop appropriately. Your doctor will decide on the best procedure for your child and the date of the procedure depends on your child’s health.

Certain children might require medicine in the interim between surgery and keep blood flow flowing through the heart the lungs.

Other procedures or surgeries

The treatment for tetralogy of Fallot requires an open-heart procedure to repair the heart defect (intracardiac fixation) or an interim procedure using an infusion. A majority of babies and children are treated with the intracardiac repair.

Repair of the intracardiac

The open-heart procedure is typically performed in the first year following birth and requires several surgeries. Adults who suffer from tetralogy of Fallot are not often able to undergo this procedure even if they didn’t undergo surgery as children.

During an intracardiac procedure surgery, the surgeon:

• Patch on the defect in the ventricular septal to seal the gap in the heart’s lower chambers (ventricles).
• Replace or repair the pulmonary valve that is narrowed to improve the flow of blood to the lungs.

Since the ventricle in the right side won’t have to exert as much effort to pump blood following this procedure the ventricle’s wall will return to its normal size. Following the intracardiac repair procedure, the oxygen levels in the blood rise, and symptoms diminish.

Shunt surgery for temporary conditions

Sometimes babies have to undergo an interim (palliative) procedure prior to having an intracardiac surgery to increase blood flow to the lung. This procedure is possible in the event that the baby is born too early or has lung arteries that aren’t developed (hypoplastic).

In this operation, surgeons create an artery bypass (shunt) between an arterial branch that is a part of the aorta as well as the pulmonary artery.

If your baby’s ready to undergo an intracardiac procedure surgically, the surgeon will remove the shunt in the process to repair the intracardiac area.

After surgery

The survival rates over the long term for those who’ve undergone tetralogy Fallot surgery continue to rise.

But, in some cases, circulation of blood to the lung could remain restricted following the tetralogy Fallot surgery. Further surgeries could be required. Adults with a treated tetralogy of Fallot could be suffering from an open pulmonary valve (pulmonary valve regurgitation) and might require the pulmonary valve replaced in the future.

Heart rhythm issues (arrhythmias) occur frequently following the tetralogy Fallot repairs. Your doctor may recommend medications, a procedure to treat the arrhythmias (ablation) or a special pacemaker that treats life-threatening arrhythmias (implantable cardioverter-defibrillator).

Ongoing care

Patients with tetralogy of Fallot require ongoing care from an adult or pediatric congenital cardiologist to ensure the procedure’s success as well as to check for potential complications. The checkups usually contain imaging tests to assess how well the treatment is working.

 

Lifestyle and home remedies for home

After tetralogy Fallot treatments, your physician may suggest lifestyle changes and suggestions to help take care of your child’s condition. This could include:

• The prevention of the spread of infection. A child, adult, or teenager with significant heart defects may have to take preventive antibiotics prior to certain dental procedures or operations. Your doctor or the doctor of your child will inform you whether this is required. Maintaining a healthy oral hygiene routine and getting regular dental checks are essential ways to avoid infection.
• Restricting certain types of exercises. The doctor might suggest to you and your kid restrict the amount of physical exercise that is strenuous, especially in the case of heart rhythm issues (arrhythmias) or obstruction or leakage of the pulmonary valve. Exercise decisions should be taken on an individual basis. Discuss with your child’s doctor regarding which exercises are safe for your child.

In the case of an adult suffering from an inherited heart condition, then you may be concerned about other things, such as:

• employment. If you have severe heart rhythm issues or the possibility of life-threatening complications, a visit to specialists is necessary to assess your danger, and provide therapy as well as provide advice on the possibility of employment.
• The pregnancy. A severe heart abnormality or arrhythmia could increase the chance of complications during pregnancy. If you suffer from congenital heart disease, you should discuss the family planning process with your physician. The doctor might suggest you seek treatment during your pregnancy by specialists who specialize in congenital heart diseases, high-risk obstetrics, and genetics. Certain heart medications aren’t suitable in pregnancy and could have to be cut off or modified prior to becoming pregnant.