What is Tetralogy of Fallot?
Tetralogy of fallot is a rare condition caused by a combination of four congenital heart defects. These defects, which affect the structure of the heart, cause oxygen-deficient blood to flow from the heart to the rest of the body. Babies and children with Tetralogy of fallot usually have blue skin because their blood does not have enough oxygen.
Tetralogy of fallot is often diagnosed in childhood or shortly thereafter. However, depending on the severity of the defects and symptoms, tetralogy of fallot may not be detected later in life in some adults. With proper surgical treatment after an initial diagnosis, many children and adults with tetralogy of fallot will lead relatively normal lives, although they may need regular medical care for the rest of their lives and may have exercise limitations.
How does it affect the heart?
Usually, the left side of the heart only sends blood to the body and the right side of the heart only sends blood to the lungs. In children with tetralogy of fallot, blood can travel from the right pumping chamber (right ventricle) to the left pumping chamber (left ventricle) and into the body artery (aorta) through a port (VSD). The obstruction of the pulmonary valve leading from the right ventricle to the pulmonary artery prevents the normal amount of blood from being pumped into the lungs. Sometimes the pulmonary valve is completely blocked (pulmonary atresia).
How does tetralogy of fallot affect my baby?
Infants and young children with unrepaired tetralogy of fallot are often blue (cyanotic). The reason is that some oxygen-free blood is pumped into the body through a hole in the wall between the right and left ventricles without reaching the lungs.
Will my children’s activities be limited?
Your child may need to limit physical activity, especially for competitive sports, if there are residual blockages or leaks in the pulmonary valve, which is normal after repair. Children with decreased heart function or rhythm disturbances may need to limit their activity further.
If the tetralogy is repaired surgically and there are no obstructions or leaks in the pulmonary valve, your child can participate in normal activities without much risk. Your pediatric cardiologist will help you determine if your child needs restrictions in physical activity.
What does my baby need in the future?
If your child has a tetralogy of fallot repair, she may need regular follow-up with a pediatric cardiologist. As an adult, your baby may need to follow a life-long routine with a cardiologist who is specially trained in congenital heart defects.
Some chronic problems include residual or exacerbated blockages between the right pumping chamber and the pulmonary arteries. Children with repaired tetralogy of fallot are more likely to have a heart rhythm disorder known as arrhythmias. Sometimes these can cause dizziness or fainting. In general, the long-term outlook is good, but some children may need medicine, cardiac catheterization, or more surgery.
Symptoms of tetralogy of fallot
The tetralogy of follow-up symptoms varies depending on the obstruction of blood flow from the right ventricle to the lungs. Signs and symptoms can include:
- The blue color of the skin due to a lack of oxygen (cyanosis) in the blood.
- Shortness of breath and rapid breathing, especially when dieting or exercising
- Loss of consciousness (epilepsy)
- Drumstick fingers and toes: unusual, rounded shape of the nail bed
- Little weight gain
- You tire easily during play or exercise.
- Prolonged crying
- Heart murmur
- Occasionally, children with tetralogy of phlegm suddenly develop deep blue skin, nails, and lips that cry or become anxious after eating.
These episodes are called tet episodes and are caused by a rapid drop in the amount of oxygen in the blood. TET episodes are most common in young children 2 to 4 months old. Young or older children can naturally squat when breathing. Squatting increases blood flow to the lungs.
Causes of tetralogy of fallot
Tetralogy of fallot causes low oxygen levels in the blood. This leads to cyanosis (the pale blue color of the skin). The classic form has four defects of the heart and its main blood vessels:
- Ventricular septal defect (hole between the right and left ventricles)
- Low-flow pulmonary tract narrowing (valve and artery that connect the heart to the lungs)
- The aorta (the artery that carries oxygen-rich blood to the body) is transplanted into the right ventricle and ventricular septum defect, leaving only the left ventricle.
- A thick wall of the right ventricle (right ventricular hypertrophy)
- Tetralogy of Fallot is very rare, but it is the most common form of congenital heart disease. It occurs equally in men and women. Other congenital abnormalities are also more common in those with a tetralogy of fallot.
The cause of most congenital heart defects is unknown. There seem to be many elements to this. Factors that increase the risk of developing this condition during pregnancy are:
- Alcoholism in the mother
- Mother over 40
- Poor nutrition during pregnancy
- Rubella or other viral diseases during pregnancy
- Children with tetralogy of Fallot are more likely to have chromosomal abnormalities such as Down syndrome, Alzheimer’s syndrome, and Digeorge syndrome (heart defects, low calcium levels, and poor immune function).
How is the tetralogy of fallot (TOF) diagnosed?
Doctors can perform several tests to find out if the baby has tetralogy of fallot and get more details about the baby’s heart and blood vessels:
- Pulse oximeter: A small sensor that measures the amount of oxygen in the blood by placing it on a fingerprint, thumb, or ear. In many nurseries, all babies have a pulse oximeter test before going home to make sure their oxygen level is normal.
- Electrocardiogram (EKG): A test that records the electrical activity of the heart.
- Echocardiogram (Echo): An ultrasound image of the heart. It records the movement of blood through the heart and can measure the direction and speed of blood flow.
- Chest x-ray
- Cardiac catheterization: A thin, flexible tube called a catheter is inserted into the heart, usually through a vein in the leg or arm, and provides information about the heart, as well as blood pressure and blood oxygen levels. Sometimes a device is inserted into the heart or blood vessels through a heart catheter.
Treatment of tetralogy of fallot
Surgery is the only effective treatment for tetralogy of fallot. Surgical options include a temporary procedure with intracardiac repair or bypass. However, most children and older children undergo intracardiac repair. You or your pediatrician will determine the most appropriate surgery and timing of surgery based on your / your child’s condition. In some cases, your baby may need medicine to keep the two large blood vessels in the heart open. Helps maintain blood flow from the heart to the lungs prior to intracardiac repair.
This open-heart surgery is usually performed in the first year after birth and involves several repairs. Adults with tetralogy of fallot rarely undergo this procedure unless they were surgically repaired as children. The surgeon places a patch over the VSD to close the hole between the lower chambers (ventricles) of the heart.
He or she repairs or replaces the narrowed pulmonary valve and dilates the pulmonary arteries to increase blood flow to the lungs. Since the right ventricle does not have to struggle to pump blood after this procedure, the right ventricle wall returns to its normal thickness. After intracardiac repair, the oxygen level in the blood increases, and the symptoms decrease.
Sometimes children may need temporary (palliative) surgery before intracardiac repair to improve blood flow to the lungs. This procedure may be done if your baby is born prematurely or has underdeveloped (hypoplastic) pulmonary arteries.
In this procedure, the surgeon creates a shunt between a large artery that separates it from the aorta and the pulmonary artery. When your child is ready for intracardiac repair, the surgeon will remove the shunt during the intracardiac repair process.
Although most children and adults function well after an intracardiac repair, chronic problems are common. Problems can include:
- Chronic pulmonary regurgitation, in which blood leaks back through the pulmonary valve into the pumping chamber (right ventricle)
- Other heart valve problems, such as back blood through the tricuspid valve
- Holes in the wall between the ventricles (ventricular septal defects) may leak after repair or may need repair
- An enlarged right ventricle or left ventricle that is not working properly
- Irregular heartbeat (arrhythmia)
- Coronary artery disease
- Dilation of the aortic root, in which the ascending aorta expands
- Sudden cardiac death
Problems in childhood, adolescence, and adolescence can persist in those with tetralogy of Fallot. Most adults with a repaired tetralogy of fallot will need another approach or intervention throughout their lives. It’s important to have regular follow-up with a trained cardiologist who can diagnose it and look for people with congenital heart disease (pediatric cardiologist or adult congenital cardiologist) who will determine the appropriate time for another intervention or procedure.
Blood flow to the lungs can sometimes be limited after intracardiac repair. Babies, children, or adults with these problems may need additional surgeries. Most commonly, there is a leak through a repaired pulmonary valve. Most adults with repaired lamina tetralogy can have a pulmonary valve leak (regurgitation) and the pulmonary valve must be replaced during their lifetime. Your cardiologist will determine the appropriate time for this procedure.
Arrhythmia after the repair is common and can be treated with medication, a special pacemaker that treats arrhythmia (ablation), or malignant heart rhythms (implantable cardioverter-defibrillator). Also, as with any surgery, there is a risk of infection, unexpected bleeding, or blood clots.
Lifelong care with a cardiologist trained in treating congenital heart disease (adult congenital cardiologist or pediatric cardiologist) is required for you or your baby after surgery, including routine follow-up appointments to ensure the initial operation or procedure is successful and to monitor anything. New problems.
You or your pediatrician may perform routine and physical exams at regular exam appointments to evaluate and monitor your / your child’s condition. The doctor might also recommend that you or your child limit strenuous physical activity, particularly if there’s any pulmonary valve leakage or obstruction, or arrhythmias.
Sometimes, antibiotics are recommended during dental procedures to prevent infections that might cause endocarditis — an inflammation of the lining of the heart. Antibiotics are especially important for those who have had prior endocarditis, have artificial valves, or have had repair with prosthetic material. Ask the doctor what’s right for you or your child.