What makes up tetralogy of fallot

Heart defects such as tetralogy of Fallot also are thought to be caused by a combination of genes and other risk factors, such as the things the mother or fetus come in contact with in the environment or what the mother eats or drinks or the medicines she uses. During pregnancy, there are screening tests also called prenatal tests to check for birth defects and other conditions. Tetralogy of Fallot might be seen during an ultrasound which creates pictures of the body.

Some findings from the ultrasound may make the health care provider suspect a baby may have tetralogy of Fallot. If so, the health care provider can request a fetal echocardiogram to confirm the diagnosis.

A fetal echocardiogram is an ultrasound of the heart of the fetus. This test can show problems with the structure of the heart and how the heart is working with this defect. Tetralogy of Fallot usually is diagnosed after a baby is born, often after the infant has an episode of turning blue during crying or feeding a tet spell.

However, it is not uncommon for a heart murmur to be absent right at birth. The health care provider can request one or more tests to confirm the diagnosis. The most common test is an echocardiogram. An echocardiogram is an ultrasound of the heart that can show problems with the structure of the heart and how the heart is working or not with this defect. Tetralogy of Fallot is a critical congenital heart defect critical CHD that may be detected with newborn screening using pulse oximetry also known as pulse ox.

Low levels of oxygen in the blood can be a sign of a critical CHD. Newborn screening using pulse oximetry can identify some infants with a critical CHD, like tetralogy of Fallot, before they show any symptoms. Tetralogy of Fallot can be treated by surgery soon after the baby is born.

During surgery, doctors widen or replace the pulmonary valve and enlarge the passage to the pulmonary artery. Then it passes through the mitral valve and into the left ventricle of the heart. Some children with TOF may only have slightly lower than normal oxygen levels in their blood. Other children with TOF will have low oxygen levels in their blood. These children have bluish skin. This is from the low oxygen levels in their blood.

Genes and family history may play a part in TOF. It may also be caused by Down syndrome or 22q Most of the time, this heart defect occurs by chance, with no cause. Symptoms can show up a bit differently in each child. The most common symptom is a bluish color of the skin, lips, and nail beds. This may come on in sudden spells, called TET spells. It happens when blood oxygen level drops quickly. During the spell, babies may have a hard time breathing.

They may also be tired and fussy. In the most severe cases, they may lose consciousness. The symptoms of TOF may be similar to symptoms caused by other problems. Make sure that your child sees a healthcare provider for a diagnosis. Your child will need to see a heart doctor cardiologist. They will check your baby and listen to their heart and lungs. Tests may be done to confirm the diagnosis. 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 TOF. An echo uses sound waves to make a moving picture of the heart and heart valves. Cardiac catheterization: Directly measures pressures in the heart, identifies abnormalities involving the right ventricle, pulmonary valve, pulmonary arteries, left ventricle and aorta.

Cardiac catheterization also provides an opportunity for an intervention to open up narrowed pulmonary arteries, stenotic pulmonary valve, or right ventricular outflow tract. Electrophysiologic study EPS : An invasive test similar to a heart catheterization and evaluates the electrical system of the heart.

The test is able to detect abnormal rhythms from either the upper chambers atria or lower chambers ventricles. This may be done in the EP lab or the operating room, if open heart surgery is pending. The EP lab is the also the place where an ICD or intracardiac defibrillator or pacemaker will be placed. In many instances, the cardiac catheterization and the EPS will be done at the same time. The placement of shunts bypasses the pulmonary narrowing, however the pulmonary stenosis and VSD remain until completely repaired.

The use of early shunt procedures provided blood to the lungs by using the higher pressure arterial supply aorta to the lower pressure lung arteries. Over time, increased pressure and blood flow to the lungs can result in pulmonary vascular disease and high blood pressure in the lungs pulmonary hypertension.

Many adults underwent Blalock-Taussig, Waterston or Potts shunt palliations in childhood. The RVOT refers to the area where blood flows from the right ventricle and out to the main pulmonary artery. If there is narrowing or obstruction of the RVOT, it is resected.

Patients were often left with a non-functioning pulmonary valve which leaks. Previously a routine technique, making a large incision in the right ventricle ventriculotomy and using a large transannular patch to repair TOF, have been abandoned.

Extensive incisions, patches, and suturing stitches lead to the formation of scar tissue in the right ventricle, which is associated with weakness of heart muscle and heart conduction electrical system issues.

Currently, the goal of surgical techniques is to minimize the extent incision or cutting of right ventricle and to maintain the integrity and competence of the pulmonary valve. Despite surgical procedures to augment blood flow to the lungs as well as complete repair operation, some patients may develop long-term complications:. Over time, the muscle of the right ventricle may become thick, bulky, and not pump efficiently.

This extra blood volume causes enlargement stretching of the right ventricle, resulting in a boggy ventricle that does not pump efficiently. This may lead to heart failure, abnormal heart rhythms, and the tricuspid valve leaflets may lose their ability to close properly, resulting in tricuspid valve leaking as well.

Right Ventricular Failure Chronic high-pressure and volume loading on the right ventricle eventually interferes with its ability to pump adequately. Further deterioration in pump function results in right ventricular failure. Symptoms associated with right ventricular failure include exercise intolerance, visible pulsations in the jugular veins, enlargement of the liver sometimes associated with a feeling full after a small amount of food intake , weight gain due to fluid accumulation, and swelling in the hands, feet or legs.

Therefore, it is very important to routinely monitor for heart rhythm problems. Information from multiple sources indicates that the risk for sudden cardiac death in patients with repaired TOF is times greater than in the general population.