Tetralogy of Fallot is a congenital heart defect (a problem with the heart's structure that's present at birth). Congenital heart defects change the normal flow of blood through the heart. This rare and complex heart defect occurs in about 5 out every 10,000 babies. It affects boys and girls equally.
The heart has a wall that separates the chambers on its left side from those on its right side. This wall is called a septum. The septum prevents blood from mixing between the two sides of the heart.
A VSD is a hole in the part of the septum that separates the ventricles-the lower chambers of the heart. The hole allows oxygen rich blood to flow from the left ventricle into the right ventricle instead of flowing into the aorta, the main artery leading out to the body.Know More.
Pulmonary Stenosis
This is a narrowing of the pulmonary valve and the passageway through which blood flows from the right ventricle to the pulmonary arteries. Normally, oxygen-poor blood from the right ventricle flows through the pulmonary valve into the pulmonary arteries and out to the lungs to pick up oxygen. In pulmonary stenosis, the heart has to work harder than normal to pump blood, and not enough blood can get to the lungs.
Right Ventricular Hypertrophy
This is when the right ventricle thickens because the heart has to pump harder than it should to move blood through the narrowed pulmonary valve.
Overriding Aorta
This is a defect in the location of the aorta. In a healthy heart, the aorta is attached to the left ventricle, allowing only oxygen-rich blood to go to the body. In Tetralogy of Fallot, the aorta is between the left and right ventricles, directly over the VSD. As a result, oxygen poor blood from the right ventricle can flow directly into the aorta instead of into the pulmonary artery to the lungs.
Together, these four defects mean that not enough blood is able to reach the lungs to get oxygen, and oxygen-poor blood flows out to the body.
Figure A shows the normal structure and blood flow in the interior of the heart. Figure B shows a heart with the four defects of tetralogy of Fallot.
Babies and children with tetralogy of Fallot have episodes of cyanosis, which is a bluish tint to the skin, lips, and fingernails. 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.
What Causes Tetralogy of Fallot ?
Doctors don't know what causes most cases of tetralogy of Fallot and other congenital heart defects.
Having some conditions or factors during pregnancy may raise your risk for having a child with tetralogy of Fallot.
These conditions and factors include : -
German measles (rubella) and some other viral illnesses
Poor nutrition
Overuse of alcohol
Age (being older than 40)
Diabetes
Heredity may play a role in this heart defect. An adult with tetralogy of Fallot may have an increased chance of having a baby with this defect.
Children with genetic disorders, such as Down syndrome and DiGeorge syndrome, often have congenital heart defects, including tetralogy of Fallot.
What Are the Signs and Symptoms of Tetralogy of Fallot ?
An important symptom of tetralogy of Fallot is cyanosis, or a bluish tint to the skin, lips, and fingernails. Low levels of blood in the oxygen cause this symptom.
Babies with 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.
The baby may also : -
Have a hard time breathing
Become very tired and limp
Not respond to a parent's voice or touch
Become very fussy
Lose consciousness
Another common symptom of Tetralogy of Fallot is a heart murmur. A heart murmur is an extra or unusual sound that a doctor can hear while listening to the heart during a physical exam. When a heart defect causes an abnormal flow of blood through the heart, it will make a certain sound.
Normal growth and development depend on a normal workload for the heart and normal flow of oxygen-rich blood to all parts of the body. Babies who have tetralogy of Fallot may not gain weight or grow as quickly as children with healthy hearts because they tire easily while feeding.
Children with this heart defect also may have "clubbing," an abnormal, rounded shape to the skin or bone around the fingernails
How Is Tetralogy of Fallot Diagnosed ?
Doctors perform a physical exam on the baby and order medical tests to diagnose tetralogy of Fallot. The signs and symptoms usually appear during the first weeks of life. Your infant's doctor may see the signs or symptoms during a routine checkup. Some parents also notice cyanosis (a bluish tint to the skin, lips, and fingernails) or poor feeding and bring the baby to the doctor.
Specialists Involved
Doctors who specialize in heart problems are called cardiologists. Pediatric cardiologists take care of babies and children who have heart problems. Other specialists who treat heart defects include cardiac surgeons (doctors who repair heart defects using surgery).
Physical Exam
During the physical exam, the doctor : -
Listens to your baby's heart and lungs with a stethoscope.
Looks for signs and symptoms, such as bluish color of skin and lips and rapid breathing.
Looks at general appearance. Some children with tetralogy of Fallot have a characteristic facial appearance because they have DiGeorge syndrome.
Diagnostic Tests and Procedures
The doctor will order several tests to diagnose tetralogy of Fallot.
These tests will help the doctor determine the exact nature of the defects and how serious they are.
Echocardiogram : - This test, which is harmless and painless, uses sound waves to create a moving picture of the heart. During an echocardiogram, ultrasound waves bounce off the structures of the heart. A computer converts the sound waves into pictures on a video screen. The test allows the doctor to clearly see any problem with the way the heart is formed or the way it's working.
ECG / EKG (Electrocardiogram) : - An ECG detects and records the electrical activity of the heart. This simple and painless test is used to assess the heart rhythm. An EKG shows how fast the heart is beating and whether the heart's rhythm is steady or irregular. This test also can help determine whether the right ventricle is enlarged (ventricular hypertrophy).
Chest X ray : - A chest x ray takes a picture of the heart and lungs. It can show whether the heart is enlarged or whether the lungs have extra blood flow or extra fluid, which can be a sign of heart failure.
Pulse Oximetry : - Pulse oximetry shows how much oxygen is in the blood. A sensor is placed on the fingertip or toe (like an adhesive bandage). The sensor is attached to a small computer unit that displays a number that tells how much oxygen is in the blood.
Cardiac Catheterization : - During cardiac catheterization, a thin, flexible tube called a catheter is put into a vein in the arm, groin (upper thigh), or neck and threaded to the heart. A dye that can be seen on an x ray is injected through the catheter into a blood vessel or a chamber of the heart. This allows the doctor to see the flow of blood through the heart and blood vessels on the x-ray image.
Cardiac catheterization also can be used to measure the pressure inside the heart chambers and blood vessels. It can determine whether blood is mixing between the two sides of the heart.
How Is Tetralogy of Fallot Treated ?
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 defects so the heart can work as normally as possible. Achieving this goal can greatly improve a child's health and quality of life.
Your baby's heart doctor and heart surgeon will determine when to do the surgery. Their decision will be based on the baby's health and weight, how severe the defects are, and how severe the baby's symptoms are.
Sometimes, teenagers or adults who had the tetralogy of Fallot defects repaired in childhood need additional surgery to correct heart problems that develop over time.
Types of Surgery
Complete Intracardiac Repair
Surgery to repair tetralogy of Fallot in India is done to improve blood flow to the lungs and to make sure that oxygen-rich and oxygen-poor blood flows to the right places.
The surgeon will : -
Widen the narrowed pulmonary blood vessels. The pulmonary valve is widened or replaced and the passageway from the right ventricle to the pulmonary arteries is enlarged. These procedures improve blood flow to the lungs, allowing the blood to get enough oxygen to meet the body's needs.
Close the ventricular septal defect (VSD). A patch is used to cover the hole. This patch stops oxygen-rich and oxygen-poor blood from mixing between the ventricles.
Fixing these two defects resolves problems caused by the other two defects. When the right ventricle no longer has to work so hard to pump blood the lungs, it can return to a normal thickness. Fixing the VSD means that only oxygen-rich blood will flow out of the left ventricle into the aorta.
The incision from the surgery usually heals in about 6 weeks.
Temporary or Palliative Surgery
It was common in the past to do a temporary surgery during infancy to improve blood flow to the lungs, and then do a complete repair later in childhood. Now, most babies with tetralogy of Fallot have their defects fully repaired in infancy.
However, some babies are too weak or too small to have the full repair. They must have temporary surgery first. This surgery improves oxygen levels in the blood and gives the baby time to grow and get strong enough for the full repair.
In the temporary procedure, the surgeon places a tube called a shunt between a large artery branching off the aorta and the pulmonary artery. One end of the shunt is sewn to the artery branching off the aorta, and the other end is sewn to the pulmonary artery. The shunt creates an additional pathway for blood to travel to the lungs to get oxygen. The shunt is removed when the baby's heart defects are fixed during the full repair.
After temporary surgery, your baby may need medicines to keep the shunt open while waiting for the complete repair. These medicines are stopped after the shunt is removed.
Living With Tetralogy of Fallot
Caring for Your Child at Home
Babies with tetralogy of Fallot can tire while nursing or feeding. Small, frequent meals may be easier for your baby to handle. He or she also may need extra nutrition. A supplement or an extra feeding can give the baby more calories, vitamins, or iron. Your child's doctors will decide what extra nutrition your baby may need.
Lowering your baby's anxiety or stress can help prevent "tet spells" and save the baby's energy. For example, picking up your baby slowly and speaking in a soothing voice can avoid startling him or her, which may prevent or lessen crying.
Tet Spells
Talk to your doctor about how you can manage your baby's or child's tet spells. Your doctor may suggest that you:
Bring the baby's or child's knees up tight against his or her chest (this is called the knee-chest position) or have your child squat down.
> This will increase blood flow to the lungs.
Try to calm your child.
Call for emergency care if the symptoms don't improve right away.
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