Tetralogy of Fallot (fah-LO) is a mix of problems triggered by a birth defect in the structure of the heart that alters the method blood streams through the heart.
Tetralogy of Fallot, which is among the most common congenital heart conditions, consists of right ventricular (RV) outflow tract obstruction (RVOTO) (infundibular stenosis), ventricular septal flaw (VSD), aorta dextroposition, and RV hypertrophy (see the image below). The mortality rate in untreated patients reaches 50% by age 6 years, however in the present period of cardiac surgery, children with basic types of tetralogy of Fallot enjoy excellent long-term survival with an exceptional quality of life.
Tetralogy of Fallot in Children
Each year, 4 from every 10,000 babies born in the United States have the condition, which was named after the French doctor who first described it in the late 1800s, Étienne Fallot. About 10% of all babies born with a heart issue have tetralogy of Fallot (TOF).
The four associated problems that together comprise tetralogy of Fallot are:
- Ventricular septal problem (VSD), which is a hole in the septum, or wall, separating the two lower chambers, or ventricles, of the heart. The septum serves as a barrier that avoids blood from both sides of the heart from blending together. However when there is a VSD, blood high in oxygen from the left ventricle can blend with blood low in oxygen from the right ventricle. VSD is the defect that can lead to the other problems connected with TOF.
- Pulmonary stenosis, a constricting or thickening of the valve that links the right ventricle to the pulmonary artery, a blood vessel that carries low-oxygen blood from the heart to the lungs, where the blood gets more oxygen then goes back to the heart. With pulmonary stenosis, the heart has to work more difficult than regular to pump blood to the lungs. Typically, the amount of blood reaching the lungs is below normal.
- Right ventricular hypertrophy (hi-PER-truh-fee), which is a thickening of the muscular wall of the right ventricle. The thickened wall can obstruct the flow of blood through the lung valve, which allows blood from the heart to stream into the lungs.
- An “overriding aorta,” which implies the artery that brings high-oxygen blood to the body is out of location and emerges above both ventricles, instead of simply the left ventricle, as in a healthy heart. This permits some blood that is low in oxygen to flow into the aorta and out to the body, instead of to the pulmonary artery, which would generally take it to the lungs to pick up oxygen.
As a result of these problems, insufficient blood goes to the lungs. The low-oxygen blood then circulates to the remainder of the body and too little oxygen reaches the body tissues.
If tetralogy of Fallot goes untreated, a child may have:
- lightheadedness, fainting, or seizures
- a greater risk of establishing endocarditis, an infection of the inner layer of the heart
- high pressure in the right side of the heart that can cause an irregular heart beat, called an arrhythmia
A child whose TOF is not fixed may need to restrict involvement in competitive sports and other physical activities. Many babies who have surgery to fix the flaw do extremely well, take part in regular kid activities, and live to their adult years.
Researchers have not yet recognized a particular cause for tetralogy of Fallot in all cases, however they believe genetics play a role. Someone born with TOF is more likely to have a child or sibling with it.
Mothers who get rubella (German measles) or other viral diseases during their pregnancies are at a higher risk of giving birth to babies with TOF. Other risk factors and conditions include poor nutrition, alcohol abuse, unchecked diabetes, and the mother’s age (over 40).
Most of the time, a child with TOF does not have other birth defects. But children who have specific congenital diseases, such as Down syndrome, often have genetic heart defects, consisting of TOF.
Specific environmental factors, such as air pollution, likewise might increase a mother’s opportunities of having a baby with TOF.
One of the most common signs of tetralogy of Fallot is cyanosis (a blue or purple tint to the baby’s skin, lips, and fingernails). Healthy babies can sometimes likewise have a bluish complexion around the mouth or around the eyes from popular veins under the skin. This is completely normal. If a baby’s lips and tongue look pink, you usually do not need to be worried due to the fact that babies who have low oxygen levels in the blood normally have blue lips and tongues in addition to bluish skin.
A child with TOF might have sudden episodes of deep cyanosis, called “Tet spells,” during sobbing or feeding. Older children who have Tet spells will typically intuitively squat down, which assists to stop the spell.
Other signs consist of:
- heart whispering
- simple tiring with exertion
- trouble breathing
- fatigue (tiredness)
- quick heartbeat (palpitations)
- ” clubbing,” where the skin or bones around the suggestions of fingers are expanded or rounded
Your doctor might use numerous tests to learn if your child has tetralogy of Fallot, including:
- pulse oximeter: a small sensing unit that clips onto the fingertip, toe, or ear and determines how much oxygen is in the blood.
- electrocardiogram (or EKG): a test that records the electrical activity of the heart.
- echocardiogram, or “echo”: an ultrasound picture of the heart structures (chambers, walls, and valves). It tapes the motion of the blood through the heart and can determine the direction and speed of blood flow within the heart structures.
- chest X-ray.
- heart catheterization: a thin, flexible tube called a catheter is placed into the heart, normally through a vein in the leg or arm, and supplies details about the heart structures in addition to blood pressure and blood oxygen levels within the heart chambers. Often a device will be placed into the heart or blood vessels through the heart catheter.
Tetralogy of Fallot is fixed through open-heart surgery not long after birth or later in infancy, depending on the baby’s health and weight and intensity of defects and symptoms.
The two surgical alternatives are:
- Total repair: the surgeon broadens the passageway in between the right ventricle and the lung artery to improve blood flow to the lungs. The ventricular septal flaw is covered to stop the blending of high-oxygen blood with low-oxygen blood between the ventricles.These repair works also fix the two remaining flaws (bypassing aorta and right ventricular hypertrophy). Since the right ventricle does not need to work as hard to pump blood into the lungs, the thickness of the ventricle wall will decrease. And the covered VSD avoids blood with low oxygen from streaming into the aorta.
- Temporary or palliative surgery: small repair works are made to enhance blood circulation to the lungs. This generally just occurs when the baby is too weak or small to undergo complete surgery. In the temporary surgery, the surgeon develops a secondary path for blood to travel to the lungs for oxygen. This is done by positioning a little tube, called a shunt, in between a big artery branching off the aorta and the lung artery.
Later on when the baby grows more powerful, the complete repair is carried out.
A lot of babies born with tetralogy of Fallot do effectively and endure to the adult years, however require regular follow-up with a heart expert.
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