Tetralogy of Fallot (TOF)
Tetralogy of Fallot (TOF) is a complex congenital heart malformation consisting of an opening in the wall between the lower heart chambers, a narrowing of the pulmonary valve and the muscular area just beneath it, thickening of the right ventricle, and abnormal position of the great artery (aorta). The combination of these four heart defects comprises the condition called tetralogy of Fallot. The result is that blue blood from the right ventricle is partially blocked from getting to the lungs. Instead, some blood can go through the VSD and out the overriding aorta to the body.
Treatment for TOF :-
If the branches of the pulmonary artery are close to normal size, this operation can be done in one step. If, however, the pulmonary arteries of the baby are too small, an additional procedure may be needed first to help the blood vessels grow to a more normal size. Complete repair of TOF is usually done in the first year of life. It consists of patch closure of the VSD so that the aorta arises solely from the left ventricle, as well as enlarging the right ventricle outflow tract, pulmonary valve, and branch pulmonary arteries as much as possible.
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 :
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.
What causes it?
In most cases, the cause isn’t known although in some patients, genetic factors play a role. It’s a common type of heart defect. It may be seen more commonly in patients with Down syndrome (in association with AV canal defects) or DiGeorge syndrome. Some patients can have other heart defects along with tetralogy of Fallot.
Tetralogy of Fallot is made up of 4 heart defects:
A hole in the wall between the lower chambers (the ventricles), which lets oxygen-poor blood mix with oxygen-rich blood. This is called a ventricular septal defect.
A narrowed outlet to the pulmonary artery, usually combined with an abnormal pulmonary valve. This can block blood flow from the lower-right chamber (the right ventricle) into the lungs.
An aorta that straddles the wall (septum) between the lower chambers (the ventricles). This lets oxygen-poor blood flow into the aorta (the main blood supplier to the body).
Thickened and enlarged heart muscle tissue in the lower-right chamber (the right ventricle).
How does it affect the heart?
Normally the left side of the heart only pumps blood to the body, and the heart’s right side only pumps blood to the lungs. In a patient with tetralogy of Fallot, blood can travel across the hole (VSD) from the right pumping chamber (right ventricle) to the left pumping chamber (left ventricle) and out into the body artery (aorta). Obstruction in the pulmonary valve leading from the right ventricle to the lung artery prevents the normal amount of blood from being pumped to the lungs. Sometimes the pulmonary valve is completely obstructed (pulmonary atresia).
What can be done about tetralogy of Fallot?
Tetralogy of Fallot is treated with two kinds of surgery. One provides temporary improvement by a shunt to give more blood flow to the lungs. The other is a complete repair of the two most important abnormalities that make up tetralogy of Fallot. Patients might have one or both surgeries in their lifetime.
Tetralogy of Fallot involves four defects : -
A large ventricular septal defect (VSD)
Right ventricular hypertrophy
An overriding aorta
Ventricular Septal Defect :
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.
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.
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