Most patients are diagnosed with tetralogy of Fallot as infants or young children. Patients with unrepaired tetralogy of Fallot are usually blue (cyanotic). This is true in infants, children and adults with unrepaired tetralogy of Fallot. Most adults with tetralogy of Fallot have had it repaired in childhood. Many people are symptom free but may have residual or recurrent problems. These include valve leakage of blood into the heart's right side, blockage of blood leaving the heart's right side and heart rhythm problems. Some patients with these problems may have limited exercise tolerance and may require medicines, repeat operations and/or a special pacemaker to lower the risk of heart rhythm problems.
A is a painless test that creates pictures of the structures in the chest, such as the heart and lungs. This test can show whether the heart is enlarged or whether the lungs have extra blood flow or extra fluid, a sign of .
Special dye is injected through the catheter into a blood vessel or one of the heart's chambers. The dye allows the doctor to see the flow of blood through the heart and blood vessels on an x-ray image.
In some patients, a operation may be done first to provide more blood flow to the lungs. This is not open-heart surgery and doesn't fix the inside of the heart. The shunt is usually a small tube of synthetic material sewn between a body artery (or the aorta) and the pulmonary artery. The shunt is removed when a complete intracardiac repair is done later. This is usually done in babies to allow them grow big enough to have a full repair but is occasionally done in adults if a complete repair isn't an immediate option. Some adults had shunts as children, but never got a complete repair. Those patients may still be able to get a complete repair, even as adults.
The doctor also can use cardiac catheterization to measure the pressure and oxygen level inside the heart chambers and blood vessels. This can help the doctor figure out whether blood is mixing between the two sides of the heart.
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.
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).
An is a simple, painless test that records the heart's electrical activity. The test shows how fast the heart is beating and its rhythm (steady or irregular). An EKG also records the strength and timing of electrical signals as they pass through the heart.
There are two main kinds of valves—tissue valves and mechanical valves. Tissue valves can come from human cadavers (homograft valves) or from pig or cow heart tissue (heterograft valves). Mechanical valves are not used very often for PVR. A person with a mechanical valve will need to be on medicines to prevent blood clots since there is a higher risk of developing dangerous clots. Each kind of valve has its own pros and cons. Talk with your doctor about what kind of valve is best for you heart. Newer valves, such as the Melody or Edwards SAPIEN valves, can be placed with a catheter going from the leg into the heart. Right now there are strict standards for which patients can have a PVR done by catheter.
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.
Taussig soon began to study the cardiac manifestations of disease, and then her interest turned to congenital heart disease. Eventually she came to the realization that the major physiological problem in tetralogy of Fallot (the blue-baby syndrome) was lack of blood flow to the lung.
The right time to replace a pulmonary valve will vary from one person to the next. Your ACHD heart doctor will decide when the right time is based on your symptoms and on the results of heart tests. Since valve replacements do not last forever, your doctor might want to wait as long as possible (without hurting your heart) to replace a valve. About 20%-30% of patients will need to have another PVR within ten years.
Tetralogy of Fallot is 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.