What Is Tetralogy of Fallot? Tetralogy (teh-TRAL-o-je) of Fallot (fah-LO) is a congenital heart defect.

A congenital heart defect is a problem with the heart's structure thats present at birth. This type of heart defect changes the normal flow of blood through the heart. Tetralogy of Fallot is a rare, complex heart defect that occurs in about 5 out of every 10,000 babies. It affects boys and girls equally. To understand this defect, it's helpful to know how a healthy heart works. The Diseases and Conditions Index How the Heart Works article describes the structure and function of a healthy heart. The article also has animations that show how your heart pumps blood and how your heart's electrical system works. Overview Tetralogy of Fallot involves four heart defects:

A large ventricular septal defect (VSD) Pulmonary (PULL-mon-ary) stenosis Right ventricular hypertrophy (hi-PER-tro-fe) An overriding aorta

Ventricular Septal Defect The heart has a wall that separates the two chambers on its left side from the two chambers 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 from the left ventricle to mix with oxygen-poor blood from the right ventricle. Pulmonary Stenosis This defect is a narrowing of the pulmonary valve and the passage through which blood flows from the right ventricle to the pulmonary artery. Normally, oxygen-poor blood from the right ventricle flows through the pulmonary valve, into the pulmonary artery, 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 reaches the lungs. Right Ventricular Hypertrophy This defect occurs if 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 aorta, the main artery that carries oxygen-rich blood to the body. In a healthy heart, the aorta is attached to the left ventricle. This allows only oxygen-rich blood to flow 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 flows directly into the aorta instead of into the pulmonary artery to the lungs. Outlook 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. Normal Heart and Heart With Tetralogy of Fallot

Figure A shows the structure and blood flow in the interior of a normal heart. Figure B shows a heart with the four defects of tetralogy of Fallot. Babies and children who have tetralogy of Fallot have episodes of cyanosis (si-a-NO-sis). This 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.

Over the past few decades, the diagnosis and treatment of tetralogy of Fallot have greatly improved. As a result, most children who have this heart defect survive to adulthood. However, theyll need lifelong medical care from specialists to help them stay as healthy as possible. What Causes Tetralogy of Fallot? Doctors don't know what causes most cases of tetralogy of Fallot and other congenital heart defects. Certain conditions or factors that occur 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 causing tetralogy of Fallot. An adult who has tetralogy of Fallot may have an increased chance of having a baby with the condition. Children who have certain genetic disorders, such as Down syndrome and DiGeorge syndrome, often have congenital heart defects, including tetralogy of Fallot. Scientists continue to search for the causes of tetralogy of Fallot and other congenital heart defects.

What Are the Signs and Symptoms of Tetralogy of Fallot? An important sign of tetralogy of Fallot is cyanosis. Cyanosis is a bluish tint to the skin, lips, and fingernails. Low levels of oxygen in the blood cause cyanosis. Babies who have unrepaired 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 also may:

Have a hard time breathing Become very tired and limp Not respond to a parent's voice or touch Become very fussy Lose consciousness

In years past, when tetralogy of Fallot wasn't treated in infancy, children would get very tired during exercise and could faint. This heart defect is now repaired in infancy to prevent symptoms like this.

Another common sign of tetralogy of Fallot is a heart murmur. A heart murmur is an extra or unusual sound that doctors may hear while listening to the heart. The sound occurs because the heart defect causes abnormal blood flow through the heart. However, not all heart murmurs are signs of congenital heart defects. Many healthy children have heart murmurs. 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 who have healthy hearts because they tire easily while feeding. Children who have tetralogy of Fallot also may have clubbing. Clubbing is the widening or rounding of the skin or bone around the tips of the fingers. How Is Tetralogy of Fallot Diagnosed? Doctors diagnose tetralogy of Fallot based on a babys signs and symptoms, a physical exam, and the results from tests and procedures. Signs and symptoms of the heart defect usually occur during the first weeks of life. Your infant's doctor may notice 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 If your child has tetralogy of Fallot, a pediatric cardiologist and cardiac surgeon may be involved in his or her care. A pediatric cardiologist is a doctor who specializes in diagnosing and treating heart problems in children. Cardiac surgeons repair heart defects using surgery. Physical Exam During a physical exam, the doctor may:

Listen to your baby's heart and lungs with a stethoscope. Look for signs and symptoms, such as a bluish tint to the skin, lips, or fingernails and rapid breathing. Look at your babys general appearance. Some children who have tetralogy of Fallot have characteristic facial traits because they have DiGeorge syndrome.

Diagnostic Tests and Procedures

Your childs doctor may recommend several tests to diagnose tetralogy of Fallot. These tests can provide information about the four heart defects that occur in tetralogy of Fallot and how serious they are. Echocardiography Echocardiography (echo) is a painless test that uses sound waves to create a moving picture of the heart. During the test, the sound waves (called ultrasound) bounce off the structures of the heart. A computer converts the sound waves into pictures on a screen. Echo allows the doctor to clearly see any problem with the way the heart is formed or the way it's working. Echo is an important test for diagnosing tetralogy of Fallot because it shows the four heart defects and how the heart is responding to them. This test helps the cardiologist decide when to repair these defects and what type of surgery is needed. Echo also is used to check a child's condition over time, after the defects have been repaired. EKG (Electrocardiogram) An EKG is a simple, painless test that records the hearts electrical activity. The test shows how fast the heart is beating and its rhythm (steady or irregular). It also records the strength and timing of electrical signals as they pass through each part of the heart. An EKG also can help the doctor determine whether the right ventricle is enlarged (ventricular hypertrophy). Chest X Ray A chest x ray 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 heart failure. Pulse Oximetry For this test, a small sensor is attached to a finger or toe (like an adhesive bandage). The sensor gives an estimate of how much oxygen is in the blood. Cardiac Catheterization During cardiac catheterization (KATH-e-ter-i-ZA-shun), 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. Special dye is injected through the catheter into a blood vessel or a chamber of the heart. The dye allows the doctor to see the flow of blood through the heart and blood vessels on an x-ray image.

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 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 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. The pediatric cardiologist and cardiac surgeon will decide the best time to do the surgery. Their decision will be based on your baby's health and weight, how severe the defects are, and how severe your baby's symptoms are. Sometimes, teenagers or adults who had tetralogy of Fallot repaired in childhood need additional surgery to correct heart problems that develop over time. See Living With Tetralogy of Fallot for more information. Types of Surgery Complete Intracardiac Repair Surgery to repair tetralogy of Fallot 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 passage from the right ventricle to the pulmonary artery is enlarged. These procedures improve blood flow to the lungs. This allows 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 in the septum. 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 to the lungs, it will 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 (cut) that the surgeon makes to reach the heart usually heals in about 6 weeks. The surgeon or a hospital staff member will explain when it's okay to give your baby a bath, pick him or her up under the arms, and take your baby for his or her regular shots (immunizations). Temporary or Palliative Surgery

It was common in the past to do temporary surgery during infancy in babies who had tetralogy of Fallot. This surgery improved blood flow to the lungs. A complete repair of the four defects was done later in childhood. Now, most babies who have 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. It also gives the baby time to grow and get strong enough for the full repair. In the temporary surgery, 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. 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 full repair. These medicines are stopped after the shunt is removed. Management: Medical: 1. Antibiotic prophylaxis before tooth extraction or GIT and urinary procedures. 2. Treatment of cyanotic spells: -Oxygen therapy.

Give morphine sulfate. -I.V. beta- adrenergic inhibitor. -If acidosis is present give I.V. sodium bicarbonate.

Surgical: palliative and total corrective surgery is being done on infants and children of all ages.

Diagnostic Evaluation for Heart Diseases: A variety of invasive and noninvasive tests may be used in the diagnosis of heart disease. 1. Electrocardiography: Measures electrical potential generated from the heart muscle. 2. Echocardiography: Short pulses of ultrasounds transmitted through heart bounce off heart structure reflex test on a screen. 3. Ultrasonography: Similar to echocardiography is synchronized with the E.C.G. to provide a three dimensional recording of heart structure.

4. Roentgenography; Fluoscopy: Provides direct observation of heart size, position, contour and relationships. Radiography: Provides permanent record of heart size, configuration. Angiocardiography: Opaque media injected into circulatory system outlines blood flow through heart and vessels, performed in conjunction with cardiac catheterization. 5. Cardiac Catheterization: Opaque catheter introduced into heart chambers via large peripheral vessels is observed by fluoroscopy or image intensification, pressure managements and blood samples provide additional sources of information. 6. Digital Subtraction Angiography (D.S.A): Opaque media injected into circulatory system provides computerized image as vessels and tissue containing dye subtracts all tissue dont containing dye.

-Put the child in knee- chest position.