NURSING CARE OF PATIENTS WITH TETRALOGY OF FALLOT I.

INTRODUCTION In the embryo, the heart begins to beat at 4 weeks of age, even before its nerve supply has been established. If a person lives to be 80 years old, his or her heart continues to beat an average of 100,000 times a day, every day for each of those 80 years. Imagine trying to squeeze a tennis ball 70 times a minute. After a few minutes, your arm muscles would begin to tire. Then imagine increasing your squeezing rate to 120 times a minute. Most of us could not keep that up very long, but that is what the heart does during exercise. A healthy heart can increase its rate and force of contraction to meet the bodys need for more oxygen, then return to its resting rate and keep on beating as if nothing very extraordinary had happened. In fact, it isnt extraordinary at all; this is the job the heart is meant to do. The primary function of the heart is to pump blood through the arteries, capillaries, and veins. As we learned in the previous lectures, blood transports oxygen and nutrients and has other important functions as well. The heart is the pump that keeps blood circulating properly. ANATOMY & PHYSIOLOGY OF THE HEART The heart is a four-chambered, muscular organ that lies in the chest cavity, under the protection of the ribs, slightly to the left of the sternum. The heart sits within a loose, fluid-filled sac, called the pericardium. The four chambers of the heart include the left and right atria and the left and right ventricles. The atria sit next to each other above the ventricles. The atria and ventricles are separated from each other by one-way valves. The right and left sides of the heart are separated by a wall of tissue called the septum. There is normally no mixing of blood between the two atria, except during fetal life, and there is never mixing of blood between the two ventricles in a healthy heart. Connective tissue surrounds all chambers. The heart is extensively innervated. The left side of the heart pumps blood through the systemic circulation, which reaches all cells of the body except those involved with gas exchange in the lungs. The right side of the heart pumps blood through the pulmonary circulation, which delivers blood only to the lungs to be oxygenated. As blood passes each cell of the body in the systemic circulation, carbon dioxide and other cellular waste products are added to the blood, while oxygen and nutrients are delivered from the blood to the cells. In the pulmonary circuit, the opposite occurs: carbon dioxide is eliminated from the blood and oxygen is added. By continual cycling of the blood through the pulmonary and systemic circulations, oxygen supply and waste removal are ensured for all cells. (Black, Hawks & Keene, 2002-p.1362; Marieb & Hoehn-2007)

II. TETRALOGY OF FALLOT (TOF) A. BACKGROUND Tetralogy of Fallot (TOF) is a congenital heart defect which is classically understood to involve four anatomical abnormalities. It is the most common cyanotic heart defect, representing 55-70%, and the most common cause of blue baby syndrome. 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. TOF occurs in approximately 400 per million live births. Its cause is thought to be due to environmental or genetic factors or a combination. It is associated with chromosome 22 deletions and diGeorge syndrome. Specific genetic associations include:JAG1, NKX2-5, ZFPM2, VEGF. It occurs slightly more often in males than in females. Embryology studies show that it is a result of anterior malalignment of the conal septum, resulting in the clinical combination of a VSD, pulmonary stenosis, and an overriding aorta. Right ventricular hypertrophy results from this combination, which causes resistance to blood flow from the right ventricle (American Heart Association, 2010). B. DEFINITION OF RELATED TERMS: 1. Stenosis is a constriction or narrowing of a duct, passage, or opening in the body. 2. Aorta is the main artery in mammals that carries blood from the left ventricle of the heart to all the branch arteries in the body except those in the lungs. 3. Hypertrophy is a growth in size of an organ through an increase in the size, rather than the number, of its cells 4. Valve is a membranous structure in a hollow organ or vessel such as the heart or a vein that prevents the return flow of fluid passing through it by folding or closing. (Sommers & Beery, 2007)

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ANATOMIC MORPHOLOGY 1. Primary four malformations "Tetralogy" denotes a four-part phenomenon in various fields, including literature, and the four parts the syndrome's name implies are its four signs. As such, by definition, tetralogy of Fallot involves exactly four heart malformations which present together: a. 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. b. 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. Ventricular Septal Defect (VSD) 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. 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.(Medscape, 2009)

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Other anomalies In addition, tetralogy of Fallot may present with other anatomical anomalies, including: stenosis of the left pulmonary artery, in 40% of patients a bicuspid pulmonary valve, in 40% of patients right-sided aortic arch, in 25% of patients coronary artery anomalies, in 10% of patients a foramen ovale or atrial septal defect, in which case the syndrome is sometimes called a pentalogy of Fallot an atrioventricular septal defect partially or totally anomalous pulmonary venous return forked ribs and scoliosis Tetralogy of Fallot with pulmonary atresia (pseudotruncus arteriosus) is a severe variant in which there is complete obstruction (atresia) of the right ventricular outflow tract, causing an absence of the pulmonary trunk during embryonic development. In these individuals, blood shunts completely from the right ventricle to the left where it is pumped only through the aorta. The lungs are perfused via extensive collaterals from the systemic arteries, and sometimes also via the ductus arteriosus (Medscape, 2010).

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PATHOPHYSIOLOGY & SYMPTOMS Tetralogy of Fallot results in low oxygenation of blood due to the mixing of oxygenated and deoxygenated blood in the left ventricle via the VSD and preferential flow of the mixed blood from both ventricles through the aorta because of the obstruction to flow through the pulmonary valve. This is known as a right-to-left shunt. The primary symptom is low blood oxygen saturation with or without cyanosis from birth or developing in the first year of life. If the baby is not cyanotic then it is sometimes referred to as a "pink tet". Other symptoms include a heart murmur which may range from almost imperceptible to very loud, difficulty in feeding, failure to gain weight, retarded growth and physical development, dyspnea on exertion, clubbing of the fingers and toes, and polycythemia. Children with tetralogy of Fallot may develop "tet spells". The precise mechanism of these episodes is in doubt, but presumably results from a transient increase in resistance to blood flow to the lungs with increased preferential flow of desaturated blood to the body. Tet spells are characterized by a sudden, marked increase in cyanosis followed by syncope, and may result in hypoxic brain injury and death. Older children will often squat during a tet spell, which cuts off circulation to the legs and therefore improves blood flow to the brain and vital organs (Budd & Gardiner, 1999-pp. 111-112). MEDICAL & SURGICAL MANAGEMENT/TREATMENT 1. Emergency management of tet spells Prior to corrective surgery, children with tetralogy of Fallot may be prone to consequential acute hypoxia (tet spells), characterized by sudden cyanosis and syncope. These may be treated with beta-blockers such as propranolol, but acute episodes may require rapid intervention withmorphine to reduce ventilatory drive and a vasopressor such as epinephrine, phenylephrine, or norepinephrine to increase blood pressure. Oxygen is effective in treating spells because it is a potent pulmonary vasodilator and systemic vasoconstrictor. This allows more blood flow to the lungs. There are also simple procedures such as squatting in the knee-chest position which increases aortic wave reflection, increasing pressure on the left side of the heart, decreasing the right to left shunt thus decreasing the amount of deoxygenated blood entering the systemic circulation. 2. Palliative Surgery The condition was initially thought untreatable until surgeon Alfred Blalock, cardiologist Helen B. Taussig, and lab assistant Vivien Thomas at Johns Hopkins University developed a palliative surgical procedure, which involved forming an anastomosis between the subclavian artery and the pulmonary artery. It was actually Helen Taussig who convinced Alfred Blalock that the shunt was going to work. This redirected a large portion of the partially oxygenated blood leaving the heart for the body into the lungs, increasing flow through the pulmonary circuit, and greatly relieving symptoms in patients. The first Blalock-Thomas-Taussig shunt surgery was performed on 15-month old Eileen Saxon on November 29, 1944 with dramatic results. The Potts shunt and the Waterston-Cooley shunt are other shunt procedures which were developed for the same purpose. These are no longer used. Currently, Blalock-Thomas-Taussig shunts are not normally performed on infants with TOF except for severe variants such as TOF with pulmonary atresia (pseudotruncus arteriosus). Total Surgical Repair The Blalock-Thomas-Taussig procedure was the only surgical treatment until the first total surgical repair was performed in 1954. Between 1944 and when total repair became available at major surgical centers in the early 1960s, many infants and children were treated palliatively with Blalock(-Thomas)-Taussig procedures. This first total repair was performed by C. Walton Lillehei at the University of Minnesota in 1954 on a 11 year old boy. The first successful total repair on an infant at one years of age, was performed in 1991, on Brittany Leska, now Brittaney Seaber. Total repair initially carried a high mortality risk which has consistently improved over the years. Surgery is now often carried out in infants 1 year of age or younger with a <5% perioperative mortality. The surgery generally involves making incisions into the heart muscle, relieving the right ventricular outflow tract stenosis by careful resection of muscle, and repairing the VSD using a Gore-Tex patch or a homograft. Additional reparative or reconstructive work may be done on patients as required by their particular anatomy. (Lange, 2006)

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NURSING CARE 1. Assessment a. History Ask for possible causes of heart diseases: History of heart disease in other family members (parent/sibling) Contact with known teratogens, such as rubella, during pregnancy Presence of chromosomal abnormality, such as Down syndrome

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Poor weight gain and/or feeding behavior Frequent respiratory infections Prior murmurs Respiratory difficulties, such as tachypnea, dyspnea and shortness of breath Cyanosis Recent streptococcal infection of the child Exercise intolerance of the child (e.g., during feeding) Physical Examination Inspection Nutritional State-Failure to thrive or poor weight gain is associated with heart disease. Color-Cyanosis is a common feature of CHD, and pallor is associated with poor perfusion. Chest deformities-An enlarged heart sometimes distorts the chest configuration. Unusual pulsations-Visible pulsation of the neck veins are seen in some patients. Respiratory excursion-This refers to the ease or difficulty in respiration (e.g., tacypnea, dyspnea, presence of expiratory grunt). Clubbing of fingers-This is associated with cyanosis. Palpation and Percussion Chest-These maneuvers help discern heart size and other characteristics (e.g. thrills) associated with heart disease. Abdomen-Hepatomegaly and/or splenomegaly may be evident. Peripheral pulses-Rate, regularity, and amplitude (strength) may reveal discrepancies. Auscultation Heart rate and rhythm-Listen for fast heart rate (tachycardia), slow heart rate (bradycardia), or irregular rhythms. Character of heart sounds-listen for distinct or muffled sounds. ((Wong & Hockenberry-Eaton, 2001 p.933) Diagnostic Evaluation By using several tests, the doctor can confirm the diagnosis. 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. Complete blood count. This is a test to measure the number of each type of cell in the blood. 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. 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 is used to diagnose tetralogy of Fallot by assessing whether a ventricular septal defect is present, the structure of the pulmonary valve, and the function of the right ventricle and the position of the aorta. 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 in the chambers of the heart and in the blood vessels. (MayoClinic-2009)

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Nursing Diagnoses & Corresponding Management

All diagnoses used for children with left to right shunts can be applied to the child with right to left shunts. The nursing diagnosis and subsequent plan of care must be individualized for each patient. 1. Altered tissue perfusion related to hypercyanotic episodes Expected outcome: The child will remain free of signs of decreased tissue perfusion, as evidenced by absence of profound cyanosis, normal activity level or affect for child, and normal respiratory status and oxygen saturation for child. Intervention Rationale Keep child as stress-free as possible by anticipating and meeting the infants feeding and comfort needs properly. Monitor for signs of hypercyanosis and notify physician if noted. Administer propanolol as ordered, ensuring correct dosage. Monitor glucose level every 4-6 hours if the child is NPO and receiving propanolol. Notify physician if glucose level Crying and/or agitation may precipitate hypercyanotic episodes.

Severe hypercyanotic episodes can result in metabolic acidosis and can terminate limpness, syncope, seizures, cardiovascular accidents and death. Propanolol, a beta-blocker, is used for palliative treatment of hypercyanotic episodes. Propanolol can cause hypoglycemia if given when patient is NPO or hypovolemic.

is less than 60 mg/dL. If hypercyanotic episode occurs, place child in knee-chest position and notify physician.

Administer other therapies as ordered, such as oxygen and medication, ensuring correct dosage, route and effectiveness. Place child in safe environment (with parent). Instruct patient or family on the signs and symptoms of hypercyanotic episodes.

Knee-chest position is thought to increase pulmonary blood flow by increasing systemic vascular resistance and to improve systemic arterial oxygen saturation by decreasing venous return so that smaller amounts of highly saturated blood reach the heart. Toddlers and other children with unrepaired TOF may squat to obtain this position and relieve chronic hypoxia. However, most defects are corrected by this age. Oxygen is needed to improve oxygenation and prevent profound hypoxemia. Morphine may be used as a calming agent, and sodium bicarbonate may be used to correct metabolic acidosis. Decreases stress and prevents further deterioration due to stress. These episodes may occur at home, and parents need to able to recognize them and have a plan of action should they occur.

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Risk for impaired gas exchange related to inadequate ventilator support, pulmonary hypertension, atelectasis, and CHF Expected outcome: The child maintains adequate gas exchange, as evidenced by normal respiratory status and oxygen saturation for child. Intervention Rationale Monitor respiratory status every 1-2 hours, assessing respiratory rate, breath sounds, and chest expirations, use of accessory muscles, nasal flaring and retractions. Notify physician of significant changes. Assess/verify ventilator settings every shift and as needed with changes in the childs condition. Ensure endotracheal tube security. Restrain hands of intubated children. Verify endotracheal tube position by chest auscultation every 1-2 hours or by chest x-ray, as needed. Suction endotracheal tube every 2-4 hours, as required. Administer sedation/paralytics, as ordered, ensuring correct dosage and effectiveness. Early signs of inadequate gas exchange are exhibited by respiratory difficulty (i. e., an increase in breaths per minute, nasal flaring, retractions, and use of accessory muscles.

To ensure that settings are as ordered and that ventilator adjustments are made with changes in respiratory status To avoid inadvertent dislodgement of the tube To avoid inadvertent dislodgement/removal of the tube Proper ventilator support is dependent on correct tube placement.

To ensure patency and remove unwanted secretions Medications are often necessary with children receiving mechanical ventilation. Sedatives can ease pain and anxiety; paralytics will prevent movement.

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Risk for decreased cardiac output related to CHF Expected outcome: The child maintains adequate cardiac output, as evidenced by absence of sinus tachycardia and diaphoresis, warm extremities free of mottling, urine output greater than 1-2 ml/kg/hr, normal respiratory status and oxygen saturation for child, absence of jaundice, and nonpalpable liver. Intervention Rationale Obtain vital sign parameters for each child and set monitor limits accordingly. Vital signs that may be normal for children without cardiac disease may not be normal for the child with cardiac disease. Obtain vital signs every 1-4 hours, as ordered, and document. Notify physician outside prescribed parameters. Assess for signs and symptoms of increasing heart failure every 2-8 hours. Note any edema in extremities, trunk, head and neck. Obtain body weight every 12-24 hours using same scale at approximately same time of day, and alert physician if excessive weight gain (>50 g/day in infants; >200 g/day in children). Children with CHF will experience changes in their breathing patterns and other vital signs due to increasing at-rest fluid retention. Baseline vital signs with appropriate monitoring parameters will assist in determining if patient status improves or deteriorates. Normals for these children may fall outside the range of expected normals. Same as above.

CHF can progress fairly rapidly, so subtle changes are important. Because of decreased venous return, fluid pools in the lower extremities. Fluid retention may not always be evident by visual inspection. Weight changes may indicate the bodys inability to get rid of excess fluid. Weighing at approximately the same time of day ensures consistency. Children with CHF will not wet as many diapers or will urinate less due to fluid retention.

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Maintain accurate input and output records. Notify An imbalance between the fluid intake and output is important because it gives clues as to physician if urine output is <1 ml/kg/hr for 4-8 hours or if how much fluid is being retained. blood is present in urine. Obtain urine specimen gravity once/twice a shift and document. Diuretics will help to get the fluid out of the body. The response to diuretic administration Administer diuretics/afterload reducers, as ordered, is very important. ensuring correct dosage and effectiveness. Noting subtle changes in respiratory status may assist in preventing CHF. The earlier the Assess respiratory effort every 2-4 hours. Notify physician assessment findings are noted, the earlier the interventions can be implemented. if retracting, flaring or grunting is a new finding or has Pulmonary edema can develop very rapidly and, in fragile infants, can be manifested by increased severity. decreased breath sounds rather than crackles, crackles indicate fluid in the alveoli. Auscultate breath sounds every 2-4 hours. Notify Elevation of the head and upper trunk promotes fluid shifting from the lungs. The less physician if breath sounds decrease or crackles are fluid in the lungs, the less respiratory effort is required. present. If respiratory effort is increased, place child in reverse Trendelenburg position (elevate head and upper body) to An increase in the oxygen level in the blood helps to decrease the bodys requirement for facilitate respiratory effort and decrease work of cardiac output. breathing. Administer humidified oxygen as ordered and during times An increase in liver size is an indication of right-sided heart failure. This size increase of exertion and stress, ensuring effectiveness by oxygen makes the liver more palpable below the rib cage. saturation measurement. Palpate liver every 4-12 hours. If palpable, document how many centimeters below right costal margin. Notify physician of any new developments. (Ashwill & Droske, 1997 pp. 928-930; Swearingen-2004; Doenges, et al.-2006) Home Care and Follow-Up Instruct the parent to follow these tips to make the baby/child more comfortable: Feed your baby slowly. Also give him or her smaller, more frequent meals. Remain calm if your baby has a Tet spell. This will reduce your child's anxiety. Give yourself a break. Ask other family members or friends to help you take care of your child. As your child grows, you may have some concerns about how best to care for your child, including: Preventing infection. A child with severe heart defects may need to take preventive antibiotics before certain dental and surgical procedures. Your doctor will help you learn if this is necessary. Maintaining good oral hygiene and getting regular dental checkups are excellent ways to help prevent infection. Exercising and play. Parents of children with congenital heart defects often worry about the risks of rough play and vigorous activity even after successful treatment. Although some children may need to limit the amount or type of exercise, many can lead normal or near-normal lives. Your doctor can advise you about which activities are safe for your child. To help coordinate your child's care, you may wish to prepare a brief note with your child's diagnosis, medications, surgeries and their dates, and your cardiologist's name and number. This note will provide necessary information to others who may care for your child and will help any new doctor understand your child's health history. (MayoClinic, 2009) Prognosis The operative mortality for total corrections of TOF is 5%. With improved surgical techniques, there is a lower incidence of dysrhythmias and sudden death; surgical heart block is rare. CHF may occur postoperatively (Wong & Hockenberry-Eaton, 2001 p.924). Untreated, tetralogy of Fallot rapidly results in progressive right ventricular hypertrophy due to the increased resistance on the right ventricle. This progresses to heart failure (dilated cardiomyopathy) which begins in the right heart and often leads to left heart failure. Actuarial survival for untreated tetralogy of Fallot is approximately 75% after the first year of life, 60% by four years, 30% by ten years, and 5% by forty years. Patients who have undergone total surgical repair of tetralogy of Fallot have improved hemodynamics and often have good to excellent cardiac function after the operation with some to no exercise intolerance (New York Heart Association Class III). Surgical success and long-term outcome greatly depends on the particular anatomy of the patient and the surgeon's skill and experience with this type of repair. Ninety percent of patients with total repair as infants develop a progressively leaky pulmonary valve as the heart grows to its adult size but the valve does not. Patients also often have damage to the electrical system of the heart from surgical incisions, causing abnormalities as detected by EKG and/or arrhythmias. Long-term follow up studies show that patients with total repair of TOF are at risk for sudden cardiac death and for heart failure. Therefore, lifetime follow-up care by an adult congenital cardiologist is recommended to monitor these risks and to recommend treatment, such as interventional procedures or re-operation, if it becomes necessary. Antibiotic prophylaxis is indicated during dental treatment in order to prevent infective endocarditis. The use of antibiotics is no longer required by cardiologists and varies from case to case (MayoClinic, 2009).