Tetralogy of Fallot (TOF) is one of the most common congenital heart disorders (CHDs).

TOF is classified as a cyanotic heart disorder because the condition results in an inadequate flow of oxygenated blood to the systemic circulation. Patients with TOF initially present with cyanosis shortly after birth, thereby attracting early medical attention. The congenital heart defect called tetrology of Fallot is a complex combination of four birth defects in the heart. The four heart defects are; 1. Pulmonary stenosis ( a narrowing of pulmonary valve and the outflow tract creating a blockage or obstruction of the blood flow from the right ventricle to the pulmonary artery) 2. A large ventricular septal defect ( a hole in the wall of the septum of the ventricle) 3. An overriding aorta (the aortic valve is enlarged and it appears to arise from both the right and left ventricles instead of only the left ventricle as usually occurring in the normal hearts) and, 4. A right ventricular hypertrophy ( an enlargement of the right ventricle and the thickening of the muscular walls of the right ventricle, which occurs because the right ventricle is pumping blood at a high pressure).

The cause of most cases of tetralogy of Fallot is not known. Heredity may play a role. In rare cases, more than one child in a family is born with a congenital heart defect like tetralogy of Fallot. Also, parents who have a congenital heart defect may be more likely than other parents to have a child with tetralogy of Fallot. Babies who have other birth defects, such as DiGeorge syndrome or Down's syndrome, are also more likely to have tetralogy of Fallot. Other factors that increase the risk of a mother's having a child with tetralogy of Fallot are: Using alcohol or street drugs during pregnancy Taking medications to control seizures during pregnancy Having a condition called phenylketonuria Having viral infections, such as German measles (rubella), during pregnancy

Louis Arthur Fallot, after whom the name tetralogy of Fallot is derived, was not the first person to recognize the condition. Stensen first described TOF in 1672; however, it was Fallot who first accurately described the clinical and complete pathologic features of the defects.

He wrote a paper: Contribution a l'anatomie pathologique de la maladie bleu (cyanose cardique). Marseille med, 1888, 25,77-93. The "tetralogy of Fallot." He gave an important, but not the first, account of this condition. The first written description of ToF was by Niels Stensen [Stenonius, Nicolaus] 1638-1686. In a paper called Embryo monstro affinis Parisiis dissectus. Acta med. Philos. Hafniensa, 167172,1, 202-03. Niels was a Danish Physician, natural scientist, bishop and later Saint Nicolaus Stenonius. Fallots description was from 1888 based on the symptoms of two persons he observed with the bluish color to the skin and with a survey of 50 other patients previously noted. There are at least forty descriptions of this condition before Fallots description.

Another description is by Sandifort, Eduard born 1742 died in 1814. Sandifort wrote in a paper, Observationes anatomicae-pathologicae. Vol.1 Lugduni Batavorum,P.v.d.Eyk&D. Vygh, 1791. First English translation.

One Thomas Bevill Peacock born 1812 and died in 1882 wrote in a paper called; On malformations, etc, of the human heart. London, J. Churchill, 1858. This includes an account of the "tetralogy of Fallot". Peacock's book was "the first comprehensive study covering the whole field" (Maude Abbott)

The illustration shows a cross-section of a healthy heart and its inside structures. The blue arrow shows the direction in which oxygen-poor blood flows from the body to the lungs. The red arrow shows the direction in which oxygen-rich blood flows from the lungs to the rest of the body. Heart Chambers The heart has four chambers or "rooms." The atria are the two upper chambers that collect blood as it comes into the heart. The ventricles are the two lower chambers that pump blood out of the heart to the lungs or other parts of the body. Heart Valves Four valves control the flow of blood from the atria to the ventricles and from the ventricles into the two large arteries connected to the heart. The tricuspid valve- is in the right side of the heart, between the right atrium and the right ventricle.

The pulmonary) valve is in the right side of the heart, between the right ventricle and the entrance to the pulmonary artery, which carries blood to the lungs. The mitral valve is in the left side of the heart, between the left atrium and the left ventricle. The aortic valve is in the left side of the heart, between the left ventricle and the entrance to the aorta, the artery that carries blood to the body. Valves are like doors that open and close. They open to allow blood to flow through to the next chamber or to one of the arteries, and then they shut to keep blood from flowing backward. When the heart's valves open and close, they make a "lub-DUB" sound that a doctor can hear using a stethoscope. The first soundthe lubis made by the mitral and tricuspid valves closing at the beginning of systole (SIS-toe-lee). Systole is when the ventricles contract, or squeeze, and pump blood out of the heart. The second soundthe DUBis made by the aortic and pulmonary valves closing at beginning of diastole. Diastole is when the ventricles relax and fill with blood pumped into them by the atria. Arteries The arteries are major blood vessels connected to your heart. The pulmonary artery carries blood pumped from the right side of the heart to the lungs to pick up a fresh supply of oxygen. The aorta is the main artery that carries oxygen-rich blood pumped from the left side of the heart out to the body. The coronary arteries are the other important arteries attached to the heart. They carry oxygen-rich blood from the aorta to the heart muscle, which must have its own blood supply to function. Veins The veins are also major blood vessels connected to your heart. The pulmonary veins carry oxygen-rich blood from the lungs to the left side of the heart so it can be pumped out to the body. The vena cava is a large vein that carries oxygen-poor blood from the body back to the heart.

Due to structural defects, there is right to left heart shunt causing cyanosis. The most vital abnormalities are pulmonary stenosis and VSD (generally perimembranous membranes). Obstruction of blood flow from the right ventricle due to pulmonary valve stenosis results in shunting of deoxygenated blood through the VSD into the left ventricle, then to the aorta causes cyanosis. Degree of the cyanosis depends upon the degree of right of ventricular outflow tract of obstruction and the size of the VSD. Outflow obstruction can also occur due to infundibular hypertrophy and supravalvular stenosis. Right ventricular hypertrophydevelops due to obstruction. Finally the condition is complicated by persistent arterial unsaturation, poor pulmonary vascularity, polcythemia to compensate cyanosis and increased blood viscosity resulting thrombophlebitis and formation of emboli. Minimum right to left shunt in small obstruction causes mild form of TOF.

Tetralogy of Fallot symptoms vary, depending on the extent of obstruction of blood flow out of the right ventricle and into the lungs. Signs and symptoms may include: A bluish coloration of the skin caused by blood low in oxygen (cyanosis) Shortness of breath and rapid breathing, especially during feeding Loss of consciousness (fainting) Clubbing of fingers and toes an abnormal, rounded shape of the nail bed Poor weight gain Tiring easily during play Irritability Prolonged crying A heart murmur Tet spells Sometimes, babies with tetralogy of Fallot will suddenly develop deep blue skin, nails and lips after crying, feeding, having a bowel movement, or kicking his or her legs upon awakening. These episodes are called "Tet spells" and are caused by a rapid drop in the amount of oxygen in the blood. Toddlers or older children may instinctively squat when they are short of breath. Squatting increases blood flow to the lungs. Tet spells are more common in young infants, around 2 to 4 months old.

Chest X-ray. A typical sign of tetralogy of Fallot on an X-ray is a "boot-shaped" heart, because the right ventricle is enlarged. Plain Film Normal to slightly enlarged cardiac silhouette "Boot-shaped" heart or Coeur-ensabot appearance (due to 1) RVH --> uplifting of cardiac apex and 2) small pulmonary arteries --> concave main pulmonary artery segment) Large aorta (because almost all blood flow from both ventricles is delivered to the aorta) Right aortic arch - 25% of patients Decreased pulmonary vasculature Pulmonary vasculature may be asymmetric (secondary to left pulmonary artery stenosis)

Blood test. Your child will need a test that measures the number of each type of cell in the blood, called a complete blood count. In tetralogy of Fallot, the number of red blood cells may be abnormally high (erythrocytosis) as the body attempts to increase the oxygen level in the blood. Oxygen level measurement (pulse oximetry). This test uses a small sensor that can be placed on a finger or toe to measure the amount of oxygen in the blood. Echocardiography. Echocardiograms use high-pitched sound waves, inaudible to the human ear, to produce an image of the heart. Sound waves bounce off your baby's heart and produce moving images that can be viewed on a video screen. This test helps diagnose tetralogy of Fallot because it allows the doctor to see whether there is a ventricular septal defect, if the structure of the pulmonary valve is normal, if the right ventricle is functioning properly, and if the aorta is positioned properly. Electrocardiogram. An electrocardiogram records the electrical activity in the heart each time it contracts. During this procedure, patches with wires (electrodes) are placed on your baby's chest, wrists and ankles. The electrodes measure electrical activity, which is recorded on paper. This test helps determine if your baby's right ventricle is enlarged (ventricular hypertrophy) and if the heart rhythm is regular.

Cardiac catheterization. During this procedure, your doctor inserts a thin flexible tube (catheter) into an artery or vein in your baby's groin and threads it up to his or her heart. A dye is injected through the catheter to make your baby's heart structures visible on X-ray pictures. The catheter also measures pressure and oxygen levels in the chambers of the heart and in the blood vessels.

Palliative shunts (symptomatic but poor surgical candidates) Blalock-Taussig: any surgical connection between the subclavian artery and the pulmonary artery. Total corrective repair: VSD closure, removing infundibular muscle causing obstruction, and inserting either a transannular patch or external conduit to relieve obstruction between right ventricle and main pulmonary artery.

The goals of tetralogy of Fallot (TOF) therapy are to reduce the ventilatory drive, decrease systemic venous return, and increase peripheral venous return. Analgesics These agents reduce ventilatory drive. Pain control ensures patient comfort and promotes pulmonary toilet. Most analgesics have sedating properties, which are beneficial for patients who are having hypercyanotic episodes. Morphine sulfate (Duramorph, Astramorph, MS Contin) DOC for narcotic analgesia because of its reliable and predictable effects, safety profile, and ease of reversibility with naloxone. Administered IV, may be dosed in number of ways and commonly titrated until desired effect obtained. Alpha-adrenergic Agonist These agents improve hemodynamic status by improving myocardial contractility and increasing heart rate, resulting in increased cardiac output. Peripheral resistance is increased by vasoconstriction. Increased cardiac output and increased peripheral resistance increase blood pressure. Phenylephrine (Neo-Synephrine) Strong postsynaptic alpha-receptor stimulant with little beta-adrenergic activity; produces vasoconstriction of arterioles, increasing peripheral venous return.

Alert to childs condition. because of the of the roght-to-left shunt through the VSD, treat IV lines like arterial lines. ** a clot dislodged from a catheter tip in a vein can across the VSD can cause cerebral embolism . The same complication can occur if air enters the venous lines. Explain TOF to the parents. Teach parents to recognize serious hypoxic spells, which can cause signs such as dramatically increased cyanosis: deep, sighing respirations, and loss of consciousness. Tell them to place their child in the knee chest position and to report such spells immediately. Warn parents to keep their child from the persons with infection to prevent infective endocarditis and other infections. After corrective surgery, explain to parents to prophylactic antibiotics to prevent infective endocarditis will still be required.