Procedures
Some children may need medicine while waiting for surgery to maintain blood flow from the heart to the lungs. Surgery for tetralogy of Fallot involves open-heart surgery to correct the defects (intracardiac repair) or a temporary procedure that uses a shunt.
Self-care
Surgery for tetralogy of Fallot involves open-heart surgery to correct the defects (intracardiac repair) or a temporary procedure that uses a shunt. Most babies and older children have intracardiac repair. This open-heart surgery is usually done during the first year after birth and involves several repairs.
What is the treatment for Tetralogy of Fallot in children?
Tetralogy of Fallot (ToF) is the most common type of cyanotic congenital heart disease. Since the first surgical repair in 1954, treatment has continuously improved. The treatment strategies currently used in the treatment of ToF result in excellent long-term survival (30 year survival ranges from 68.5% to 90.5%).
What is tetralogy of Fallot surgery?
If your child has tetralogy of Fallot, a pediatric cardiologist and cardiac surgeon may be involved in his or her care. A pediatric cardiologist is a doctor who specializes in diagnosing and treating heart problems in children. Cardiac surgeons repair heart defects using surgery.
What is the prognosis of tetralogy of Fallot (TOF)?
What kind of Doctor treats tetralogy of Fallot?
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What is the recommended treatment for tetralogy of Fallot?
All babies who have tetralogy of Fallot need corrective surgery performed by a heart (cardiovascular) surgeon. Without treatment, your baby might not grow and develop properly. Your doctor will determine the most appropriate surgery and the timing of the surgery based on your or your child's condition.
Does tetralogy of Fallot require surgery?
Tetralogy of Fallot must be repaired with open-heart surgery, either soon after birth or later in infancy. The goal of surgery is to repair the four defects of tetralogy of Fallot so the heart can work as normally as possible. Repairing the defects can greatly improve a child's health and quality of life.
How long do tetralogy of Fallot patients live?
Tetralogy of Fallot is a rather common complex cardiac malformation with an incidence of 0.1/1000 live births. Without surgical intervention, patients had a 1 year survival rate of 66%, 49% after 2 years and only 10–15% after more than 20 years [1,2].
Is tetralogy of Fallot serious?
People with untreated tetralogy of Fallot usually develop severe complications over time, which might result in death or disability by early adulthood.
What is the most common complication found in patients with tetralogy of Fallot?
Adults with repaired ToF develop late complications, such as progressive exercise intolerance, arrhythmias, and heart failure [4, 6]. These complications are mainly due to pulmonary regurgitation, which leads to right ventricle dysfunction [7].
Can tetralogy of Fallot cause death?
Background: Sudden cardiac death remains the most common cause of death after repair of tetralogy of Fallot. It has been suggested that sudden cardiac death is related to right ventricular hypertrophy or dilation.
Can tetralogy of Fallot be corrected?
Tetralogy of Fallot can be corrected with surgery. This is generally done during the first year of life, though occasionally it can be done at an older age.
At what age is tetralogy of Fallot repair?
The best survival and physiological outcomes were achieved with primary repair in children aged 3 to 11 months. Conclusions—On the basis of mortality and physiological outcomes, the optimal age for elective repair of tetralogy of Fallot is 3 to 11 months of age.
What is the most common valve problem after fallot repair?
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. Leaky valves are generally repaired surgically, but newer ways of implanting valves without surgery are currently being explored.
What are the complications of tetralogy of Fallot?
The potential complications associated with surgical repair for tetralogy of Fallot include: Electrical disturbances: Putting a patch on the ventricular septal defect can block the ability of the atria to transmit electrical signals to the ventricles. This can be corrected by a pacemaker.
What is the name of the condition that is a cluster of four heart defects?
Named after the physician who first described this condition in 1888, tetralogy of Fallot is the name for a cluster of four heart defects: A large ventricular septal defect: The ventricles are the heart’s two lower chambers, and the septum is the wall that separates the left and right chambers. A ventricular septal defect is a hole in ...
What is the narrowing of the right ventricle?
Pulmonary stenosis is a narrowing of the valve between the right ventricle and the pulmonary artery, or the muscle growth immediately below the valve (subpulmonic stenosis). Often, the narrowing not only involves the valve, but also the muscle directly below it.
How rare is Fallot?
Tetralogy of Fallot is relatively rare, occurring in about 5 of every 10,000 births. Although this heart defect has been recognized for 120 years, the exact cause is still unknown. The defect occurs during the heart's development before birth.
Where is the aorta located in Fallot?
In tetralogy of Fallot, the aorta is positioned between the left and right ventricles, above the ventricular septal defect. This location allows the aorta to receive blood from both sides of the heart, so that oxygen-poor blood from the right ventricle mixes with oxygenated blood from the left ventricle.
What happens when a heart valve leaks?
Leaking valves: Heart valves are designed to allow blood to flow in one direction. 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.
What are the four parts of tetralogy?
These are: Ventricular septal defect: A hole in the wall (septum) between the lower chambers of the heart. Pulmonary stenosis: A narrowed or completely blocked pulmonary valve.
What is fallot surgery?
Tetralogy of Fallot Surgery. Tetralogy of Fallot surgery is typically done in the first year of a child’s life to correct a congenital (something that’s there from birth) heart defect. Tetralogy of Fallot is a heart problem consisting of four related heart defects. These defects include ventricular septal defect, pulmonary valve stenosis, ...
Why does Fallot's cyanosis look blue?
This happens because some of the low oxygen blood that is pumped into the body across the hole (VSD).
What happens if you break a Tet spell?
During a tet spell, the baby may develop intense cyanosis (blue), often following rapid, deep breathing, increased irritability and prolonged crying. A tet spell that does not get better in a few minutes of trying to calm the baby and "break" the spell can be an emergency.
Why do babies get blue spells?
This happens because some of the low oxygen blood that is pumped into the body across the hole (VSD). Infants or young children with tetralogy of Fallot can sometimes develop severe blue spells, called “tet spells.”. During a tet spell, the baby may develop intense cyanosis (blue), often following rapid, deep breathing, ...
What artery do you put a stent in?
The other option that can be done soon after birth in some infants is to place a stent, or mesh tube into the patent ductus arteriosus — a natural bridging artery between the aorta (body) artery and lung artery which every baby has in the womb, but closes a few days after birth.
How many parts are there in tetralogy of fallot?
It may even be detected before birth during a prenatal ultrasound. The word “tetralogy” means a group of four. In tetralogy of Fallot, there are four parts to the heart defects.
How old do you have to be to fix tetralogy of fallot?
All children with tetralogy of Fallot need to have surgery to fix it. Most children have it before they turn 1 year old. It's often done around 6 months of age. A team of heart surgeons will do your child's surgery. To fix TOF your doctor may use a patch to close ventricle septal defect (VSD).
What is the TOF in a child?
Tetralogy of Fallot (TOF) is a group of 4 congenital heart defects. This means that your child is born with them. These 4 problems occur together (tetralogy refers to 4). They are:
What is the aorta in a healthy heart?
The right lower chamber of the heart (ventricle) is bigger than normal. In a healthy heart, oxygen-poor (blue) blood returns to the right chamber of the heart (right atrium) from the body.
Which artery carries blood from the heart to the lungs for oxygen?
The pulmonary artery carries blood from the heart to the lungs for oxygen. Ventricular septal defect. This is an opening in the wall between the 2 lower chambers of the heart (right and left ventricles). Overriding aorta.
How does a cardiac catheterization work?
A cardiac catheterization gives detailed information about the structures inside the heart. In this test, a small, thin, flexible tube (catheter) is put into a blood vessel in your child's groin. Then the healthcare provider guides it to your child's heart. Your child's healthcare provider will inject your child with contrast dye to see his or her heart more clearly. This test measures your child's blood pressure and oxygen in the 4 chambers of the heart. It also measures blood pressure and oxygen in the pulmonary artery and aorta. Your child will get medicine to help relax and prevent pain (sedation).
What does a fallot test show?
This test records the electrical activity of the heart. It also shows abnormal rhythms (arrhythmias or dysrhythmias) and spots heart muscle stress. These issues may be caused by caused by tetralogy of Fallot.
What doctor can check a baby's heart?
Your provider may refer him or her to a heart doctor for children (pediatric cardiologist). Your child's heart doctor will check your baby. He or she will listen to your baby's heart and lungs. The details about your child's heart murmur will also help the doctor make the diagnosis.
What is the goal of Fallot surgery?
The goal of surgery is to repair the four defects of tetralogy of Fallot so the heart can work as normally as possible. Surgery involves widening or replacing the pulmonary valve and enlarging the passage from the right ventricle to the pulmonary artery. This improves blood flow to the lungs.
Why does my baby have a tet spell?
Low levels of oxygen in the blood cause cyanosis. Babies who have unrepaired tetralogy of Fallot sometimes have "tet spells" in response to an activity like crying or having a bowel movement. A tet spell occurs when the oxygen level in the blood suddenly drops. This causes the baby to become very blue.
What is the tetralogy of Fallot?
Tetralogy (teh-TRAL-o-je) of Fallot (fah-LO) is a congenital heart defect. A congenital heart defect is a problem with the heart's structure that's present at birth. This type of heart defect changes the normal flow of blood through the heart. Tetralogy of Fallot is a rare, complex heart defect that occurs in about 5 out of every 10,000 babies.
Why does cytonosis occur?
Cyanosis occurs because the oxygen level in the blood is below normal. Tetralogy of Fallot must be repaired with open-heart surgery, either soon after birth or later in infancy. The timing of the surgery depends on how severely the pulmonary valve is narrowed.
Why do babies with fallot not gain weight?
Babies who have tetralogy of Fallot may not gain weight or grow as quickly as children who have healthy hearts because they tire easily while feeding. Children who have tetralogy of Fallot also may have clubbing. Clubbing is the widening or rounding of the skin or bone around the tips of the fingers.
What are the symptoms of Fallot?
Cyanosis is a bluish tint to the skin, lips, and fingernails. Other signs and symptoms include a heart murmur, delayed growth and development, and clubbing. Clubbing is the widening or rounding of the skin or bone around the tips of the fingers.
What are the long term problems with Fallot?
Teenagers and adults who had surgery to repair tetralogy of Fallot may have long-term heart problems, such as heart function problems, arrhythmias (irregular heartbeats), or problems resulting from the original repair. These problems are treated with medicines, procedures, and surgery.
How is Tetralogy of Fallot diagnosed?
In some cases, Tetralogy of Fallot (TOF) can be diagnosed prenatally (during pregnancy). After birth, a baby might have a heart murmur, lower levels of oxygen in the blood or an abnormal chest X-ray.
How do doctors treat Tetralogy of Fallot?
Surgery is the only treatment for Tetralogy of Fallot (TOF). Most children will need to have surgery around 4-8 months of age. Some children need just one surgery. Others might need more than one.
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What is the tetralogy of Fallot?
Tetralogy of Fallot is a structural heart anomaly characterized clinically by cyanosis, and anatomically by an obstructed right ventricular outflow tract associated with a ventricular septal defect (see Fig. 16); compare left panel with normal anatomy on the right).
What is pulse oximetry?
Pulse oximetry, available in many nurseries, is a helpful initial tool to screen for low blood oxygenation, though not specifically for tetralogy of Fallot as there are many causes of low oxygen saturation in the newborn, including sepsis, lung disease and a variety of CHDs.
What is the deletion of Fallot?
In cases of tetralogy of Fallot, always look for other birth defects and signs of genetic syndromes: A common genetic condition with tetralogy of Fallot (seen in about 15–20 % of cases) is deletion 22q11, a condition in which a small part of chromosome 22 is missing.
What is non invasive newborn screening?
Non-invasive newborn screening via pulse oxymetry, though not perfect, is a helpful screening tool to detect cases that could be missed at birth by clinical examination alone. These babies require a quick diagnosis and treatment (first medical, then surgical). Diagnosis is mainly by echocardiogram.
What is the classic presentation of a newborn?
Clinically, the classic presentation is a newborn who becomes progressively cyanotic (blue tinge most evident in the lips, nose and extremities), either constantly or intermittently, and continues to decompensate. Providing oxygen does not help, as it is not a lung problem.
Is pregestational diabetes a conotruncal condition?
Maternal pregestational diabetes is a modifiable risk factor for tetralogy of Fallot and other conotruncal conditions (e.g. truncus arteriosus).
Is Fallot a tetralogy?
Tetralogy of Fallot is in fact a spectrum of diseases and might be clinically severe or mild, depending on the degree of obstruction in the right ventricular outflow tract. In milder cases, cyanosis might be mild or absent, and might escape detection at birth.
How is tetralogy of Fallot treated?
This repair involves a patch to close the septal defect and enlargement of the pulmonary valve to facilitate blood flow to the lungs. The patch stays in place permanently, though in rare cases it can leak later in life. In contrast, the enlarged pulmonary valve is often regurgitant (meaning blood the heart pumps leaks backward into the heart).
What is the TOF in medical terms?
Ari Michael Cedars, M.D. Tetralogy of Fallot (TOF) is a group of four congenital (present at birth) heart defects that occur together: Pulmonary stenosis (narrowed pulmonary valve) Ventricular septal defect (hole in the wall between the heart’s lower chambers) Overriding aorta (aortic valve positioned halfway between the two ventricles)
What is a congenital heart specialist?
Adult congenital heart specialists can address the unique cardiac needs of adults at every stage of life and determine whether additional treatment may be necessary. They help manage the following issues: Deterioration of childhood heart repairs. Pregnancy.
What is the overriding aorta?
Overriding aorta (aortic valve positioned halfway between the two ventricles) Right ventricular hypertrophy (thickened right ventricle wall) Historically, children born with TOF didn’t live long enough to need care as adults. However, new treatment technologies are making it possible for these children to live full, active lives. ...
Why isn't valve replacement done in childhood?
This surgery isn’t done during childhood because artificial valves don’t grow with a child’s body. An adult congenital heart specialist determines the right time for valve replacement. “Valves only last 10 to 15 years,” says Cedars.
What are the conditions that can develop as you age?
A variety of heart conditions can develop as adults age, including high blood pressure, high cholesterol and coronary artery disease. When someone has a preexisting congenital heart defect like TOF, it is especially important to monitor and treat these conditions.
Can a pregnant woman have a congenital heart defect?
Pregnancy and Congenital TOF. When a woman with a congenital heart defect like TOF is ready to have children, it’s extremely important to monitor her closely. Pregnancy, labor and delivery can put a dangerous amount of strain on an already compromised heart.
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- Usually, tetralogy of Fallot is diagnosed soon after birth. Your baby's skin may appear blue. A doctor might hear an abnormal whooshing sound (heart murmur) when listening to the baby's heart with a stethoscope. Tests to diagnose tetralogy of Fallot include: 1. Oxygen level measure…