Tetralogy of Fallot

By: Marica, Jan Rouel Mata, Bernice Mayor, Marvin Mella, Ferie Mendoza, Janine Menguez, Jerold Minoc, Julian Marie Miranda, Eunice Avelette Miranda, Marie Joy

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. It is the most common cyanotic heart defect, and the most common cause of blue baby syndrome. 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.

It was described in 1672 by Niels Stensen, in 1773 by Edward Sandifort, and in 1888 by the French physician tienne-Louis Arthur Fallot, for whom it is named.

Niels Stensen

tienne-Louis Arthur Fallot

Tetralogy of Fallot involves four heart defects:  V - entricular septal defect (VSD)  O - verriding aorta  R - ight ventricular hypertrophy  P - ulmonary stenosis

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.

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.

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.

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.

What Causes Tetralogy of Fallot? Certain conditions or factors that occur during pregnancy may raise the 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.

RISK FACTORS  Mother infected with Rubella virus  Maternal alcoholism  Poor nutrition  Mother older than 40  Parent who had TOF  Child with Downs syndrome

Anatomy & Physiology

The heart is made up of 4 chambers: 2 upper chambers called atria and 2 lower, larger chambers called ventricles. Each atrium is separated from its paired ventricle by a valve. The heart has a left and a right side. The left and right sides of the heart are separated by a wall called septum. The right side of the heart receives oxygen-depleted or blue blood returning by veins (superior vena cava and inferior vena cava) from the body. The blood flows from the right atrium through the tricuspid valve into the right ventricle, which pumps it through the pulmonic valve into the pulmonary artery, the main artery to the lungs.

In the lungs, the blood absorbs oxygen and then returns to the left atrium through the pulmonary veins. From the left atrium, the blood is pumped through the mitral valve to the left ventricle. The left ventricle pumps the blood out of the heart into the circulatory system via a large artery known as the aorta. The blood moves throughout the body, supplying oxygen and nutrients to organs and cells. Organs cannot work properly if they do not receive enough oxygen-rich blood.

Superior & Inferior Vena Cava

Circulatory System

Aorta

R Atrium

L Ventricle

Blood Flow
R Ventricle L Atrium

Pulmonary Artery

Lungs

Pulmonary Veins

Signs & Symptoms

An important sign of tetralogy of Fallot is cyanosis. Babies who have unrepaired tetralogy of Fallot sometimes have tet spells" in response to an activity like crying or having a bowelmovement. A tet spell occurs when the oxygen level in the blood suddenly drops. This causes the baby to become very blue.

The baby may also: 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. 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.

Irritability Exertional dyspnea relieved by squatting or knee-chest position Convulsion Poor eating

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Diagnostic Tests
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 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.

Nursing intervention Obtain consent and explain the procedure to the pt. Tell the pt. that the procedure is painless. Tell the pt. that the procedure last for 30-45 mins. The pt. will ask to turn to left lateral side and hold breath.

ECG (Electrocardiogram) An ECG 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).

Nursing intervention Before: Expose the chest, ankles & wrists Assist pt. in supine or semifowlers position During: Place lead properly. After: Disconnect the equipment, remove the electrodes & Remove the gel w/ a moist cloth towel

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. Nursing intervention Explain the procedure to the pt. The pt. is ask to take a full inspiration(deep breath)

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. Nursing intervention Explain the procedure to the pt. and relatives.

Cardiac Catheterization During cardiac catheterization (KATHe-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.

Nursing intervention Before: Consent form should be signed before the procedure. Patients may be required to be admitted to the hospital the night before the procedure. For some patients, overnight stay is not required. Nothing to eat or drink by mouth 6-8 hours before the test. The doctor should be informed of allergies to medications, iodine or food. It should also be documented legibly in the patients chart. Some blood tests and electrocardiogram (ECG) will be performed before the procedure. During: A mild sedative will be given orally or intravenously to comfort the patient and relieve anxiety. After: The patient is asked to refrain from any exercise or exertion. They are not to lift heavy load or involve themselves in strenuous exercise. Care should be taken of the incision site so as to prevent a sore or swelling. It is also advisable that the patient does not drive for 24hours post procedure.

jerold

Surgical Management

Blalock-Taussig shunt (BT shunt) this procedure is temporarily used to direct blood flow to the lungs and relieve cyanosis while the infant is waiting for corrective or palliative surgery. One branch of the subclavian artery or carotid artery is separated and connected with the pulmonary artery. The lung receives more blood with low oxygenation from the body.

Advantages of BT shunt (1)preservation of the subclavian artery (2)suitability for use on either side (3)good relief of cyanosis (4)easier control and closure at time of primary repair (5)excellent patency rate (6)decreased incidence of iatrogenic pulmonary/systemic artery trauma

This image shows completed blocking with a Taussig shunt.

This image shows a closed ventricular septal defect and closure of right ventriculotomy with Gore-Tex.

Gore-Tex is used for complete closure of right ventriculotomy

Remember that there will be no BP or PR in the left arm after the BlalockThomas-Taussig shunt since the subclavian artery has already been anastomosed.

Complete TOF repair - This is an open-heart procedure.

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:

1. 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.

2. 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.

Nursing Care Plan

ASSESSMENT O > (+) DOB > (+) cyanosis > (+) clubbing on fingers > (+) nasal flaring > (+) use of accessory muscles > (+) restlessness > (+) irritability >cold, clammy skin > RR of 75 cpm > ABG Results of: pH 7.30 PaCO2 50 mm Hg PaO2 75 mm Hg HCO3 20 mEq/L

DIAGNOSIS Ineffective Cardiopulmonary perfusion r/t O2 blood transport across alveolar and capillary membrane 2 to TOF

PLANNING Short Term Goal >After 8 of nursing intervention the patient will be able to demonstrate behaviors to improve circulation. >(-) DOB >(-) cyanosis >(-) nasal flaring >(-) use of accessory muscles >(-) restlessness >(-) irritability > warm to touch > RR within normal range of 30-60 cpm

Long Term Goal >After 72 of nursing intervention the patient will be able to demonstrate increase perfusion. > (-) clubbing of fingers > ABG within normal range of: pH 7.35-7.45 PaCO2 35-45 mm Hg PaO2 80-95 mm Hg HCO3 22-26 mEq/L

NURSING INTERVENTIONS >Monitor Vital signs >Monitor heart sounds >Maintain quiet and restful atmosphere. >Caution SO to avoid patient doing activities that would cardiac workload. >Provide comfort measures such as touching and giving pillows. >Encourage SO to avoid smoking near the patient. >Administer O2 therapy

EVALUATION After 8 of nursing intervention the patient was able to demonstrate behaviors to improve circulation. >(-) DOB >(-) cyanosis >(-) nasal flaring >(-) use of accessory muscles >(-) restlessness >(-) irritability > warm to touch > RR within normal range of 30-60 cpm

ASSESSMENT
O > (+) DOB > (+) cyanosis > (+) daiphoresis > (+) nasal flaring > (+) use of accessory muscles > (+) restlessness > (+) irritability >cold, clammy skin > PR of 145 bpm > RR of 75 cpm > ABG Results of: pH 7.30 PaCO2 50 mm Hg PaO2 75 mm Hg HCO3 20 mEq/L

NURSING DIAGNOSIS Impaired gas exchange r/t ventilation perfusion imbalance

PLANNING Short Term Goal >After 8 of nursing intervention the patient will be able to participate with the treatment regimen together with SO. >(-) DOB >(-) cyanosis >(-) nasal flaring >(-) use of accessory muscles >(-) restlessness >(-) irritability > warm to touch > RR within normal range of 30-60 cpm > PR within normal range of 75-120 bpm

Long Term Goal >After 72 of nursing intervention the patient will be able demonstrate to improved ventilation and adequate oxygenation as evidenced by ABG results within normal range. >(-) respiratory distress

NURSING INTERVENTIONS >Monitor Vital signs >Position pt. in moderate HBR >Instruct SO to reposition the pt. frequently >Caution SO to avoid patient doing activities that would cardiac workload. >Provide comfort measures such as touching and giving pillows. >Encourage SO to avoid smoking near the patient. >Maintain calm and restful environment >Administer O2 therapy >position the pt in knee-chest position whenever episodes DOB takes palce

EVALUATION After 8 of nursing intervention the patient was able to participate with the treatment regimen together with SO. >(-) DOB >(-) cyanosis >(-) nasal flaring >(-) use of accessory muscles >(-) restlessness >(-) irritability > warm to touch > RR 45 cpm > PR 114 bpm

Drug Study

Drug name

Dosage

Action

Pharmacoki netics

Pharmacod ynamics

Indication

Contraindic ation

Adverse reaction

Nursing intervention s -assess pain: location, type, character, intensity, before therapy and regularly thereafter to monitor drug effectivenes s. Give dose before pain becomes extreme. -monitor allergic reactions. -monitor for respiratory depression after administrati on. -monitor input and output. -assess patients and familys knowledge

Morphine (hizon morphine sulfate, Relimal CR)

Inj:0.1- 0.2 mg/dose to be repeated every 4 hrs. to a max. of 5 mg. Epidural: 2-5 mg/ dose every 10-12 hrs.

Binds with opiod receptors in the CNS causing alteration of both perception of and emotional response to pain through unknown mechanism.

Absorption: Variable (PO); well absorbed (IM, SC, rectal), complete (IV) Distribution: Widely distributed Metabolism: Liver Excretion: Liver and Kidneys Half Life: 2-3 hrs.

Onset PO- 1hr PO-EXT REL Unknown IM 10-30 mins. SC 10-30 mins. RECTAL 20-60 mins. IV <5 mins. IT 15-60 mins. Duration PO 4-12 hrs. PO-EXTREL Unknown IM 4-5 hrs

Relief of moderate to severe acute and chronic pain. Renal and Intestinal colic. Cancer. Myocardial pulmonary congestion. Antitussive. Narcotic, hypnotic, sedative.

Known hypersensiti vity to morphine or to one of the other excipients, existing intestinal obstruction (ileus) and pregnancy. Asthma, shortage of oxygen, shallow breathing, brain damage, epilepsy, simultaneou s treatment with antidepress ants, chronic alcohol abuse, immediately after bile duct surgery, anastamose

Allergic reaction. Gastrointest inal disturbance s, urinary retention. Depression, delirium and convulsion. Nausea, xerostomia, emesis, anorexia, dysguesia, facial flushing, chill, palpations, general asthenia, syncope, cardiac failure, respiratory depression, sedation of different degree, headache, perspiration , perceptive disorders,

Drug name

Dosage

Action

Pharmac okinetics

Pharmac odynami cs
SC 4-5 hrs. RECTAL 4-5 hrs. IV 4-5 hrs. IT 24 hrs. Peak PO 1-2 hrs. PO-EXT REL Unknown IM 30-60 mins. SC 50-90 mins. RECTAL 20-60 mins. IV 20 mins.

Indicatio n

Contrain dication

Adverse reaction

Nursing interventi ons

Drug name

Dosage

Action

Pharmacoki netics

Pharmacod ynamics

Indication

Contraindic ation

Adverse reaction

Nursing intervention s

Phenlyephri ne (mydfrin)

1-2 drops according to purpose of application.

Powerful and selective alpahaadrenergic receptor agonist in the sympathetic nervous system that cause contraction of blood vessels and vasocontrict ions.

Absorption: well(IM), complete (IV), minimal (nasal, ophthalmic) Distribution: Unknown Metabolism: Liver Excretion: Kidney Half-life Unknown

ONSET IV Immediate IM 10-15 mins. SC 10-15 mins. NASAL 15-20 mins. OPHTH 2.5%- 15-60 10% 10-90 mins. PEAK IV Unknown IM Unknown SC Unknown NASAL Unknown OPHTH

Vasoconstri ctor, decongesta nt and mydriatic in ophthalmic conditions and procedures.

Narrowangle galaucoma. Infant, elderly with severe arterioscler otic CV or cerebrovasc ular disease. Disturbed corneal apithelial barrier. Hypersensit ivity.

-assess patients condition before starting theraphy and regularly thereafter to monitor drug effectivenes s. -monitor blood pressure frequently. -monitor for hypersensiti vity reaction. -monitor input and output. -note for paresthesia s and coldness of extremities to assess decreased peripheral

Lowering stress Lowering your baby's anxiety or stress can help prevent tetralogy "spells" and save the baby's energy. You can try to predict what your baby needs to stop or to lessen crying. For example, picking up an infant slowly and speaking in a soothing voice can avoid startling a baby.

Drug name

Dosage

Action

Pharmacoki netics

Pharmacod ynamics

Indication

Contraindic ation

Adverse reaction

Nursing intervention s

DURATION IV 15-20 mins. IM - 2 hrs. SC 50-60 mins. NASAL 1/2 -4 hrs. OPHTH 2.5%-3hrs. 10%-5-7 hrs.

Nutrition Babies with tetralogy of Fallot can tire while nursing or feeding. You may need to feed your baby more often. Some babies also need extra nutrition. This is given as a supplement or an extra feeding. These feedings usually are formulas that give the baby extra calories. Many babies need extra vitamins or iron. Your child's doctors will decide what extra nutrition your baby might need.

Treating tetralogy spells If your baby has a tetralogy spell, you should: Bring the baby's knees up tight against the baby's chest (the knee-chest position). Attempt to calm the baby. Call 911 (in the US) if your baby's symptoms do not immediately improve. Even if the baby's symptoms improve at home without emergency treatment, you should still contact your doctor to report the spell.

Lowering stress Lowering your baby's anxiety or stress can help prevent tetralogy "spells" and save the baby's energy. You can try to predict what your baby needs to stop or to lessen crying. For example, picking up an infant slowly and speaking in a soothing voice can avoid startling a baby.

After surgery After temporary surgery, your baby may need medicines to keep the shunt open while waiting for the corrective surgery. These medicines are stopped after the shunt is removed during the corrective surgery. It is rare to need medicines after corrective surgery unless there are other problems. The scar from surgery usually heals in about 6 weeks. Your surgeon or another member of the hospital staff will tell you when: You can give your baby a bath You can pick the baby up under the arms Your baby should get his or her regular shots (immunizations) Your baby may need to take medicines to prevent a serious respiratory infection called respiratory syncytial virus (RSV). Because of having the heart defect, your baby may be more prone to get this infection. Your pediatrician or family doctor will decide if your baby needs any medicines to prevent RSV.

Living with Tetralogy of fallot For parents Parents of children born with tetralogy of Fallot often think that they did something to cause the problem. It is important that parents know that they did not cause their child's illness. Treatment and care for your child can be costly. The cost of surgery and hospital stays is very high. Your health insurance may not pay for everything. Many parents need help to cover medical bills. You can get information from your doctor and hospital about how to apply for financial aid. It is important that you keep your health insurance current. If you think about changing jobs, check to see if the health insurance at the new job will cover a child who has tetralogy of Fallot. Some health insurance plans may not cover some medical conditions that you or your family had before joining the new plan

It is also very important for your child to have health insurance as adulthood approaches. Review your current health insurance plan. Find out how coverage can be extended beyond age 18. Some policies may allow you to keep your child on your plan if he or she remains in school or is disabled. The care and treatment of a child with a complex congenital heart condition like tetralogy of Fallot can be very stressful for families. This is especially true during the period surrounding the surgery. Support is very important. Talk to your doctor or hospital social worker about a parental support group in the area. In these groups, you can talk with other parents who are going through the same thing. Counseling can also be very helpful.

Thank You & God bless!