The child’s cardiovascular system has one overwhelming purpose – to provide an adequate supply of oxygen and nutrients to all the tissues. The heart is the muscular organ of the circulatory system that constantly pumps blood throughout the body. It is approximately the size of a clenched fist. Two functions: 1. Pulmonary circulation: Provides for the oxygenation of blood in the lungs. The pulmonary circulatory loop transports oxygen-poor blood from the right side of the heart to the lungs and then to the left side of the heart, which enables the blood to pick up oxygen and get rid of carbon dioxide. 2. Systemic circulation: Provides for the distribution of the oxygenated blood to the body tissues and removal of metabolic waste products. The systemic circulatory loop transports oxygen-rich blood from the left side of the heart to all the body’s tissues and then to the right side of the heart.
The heart has four separate compartments or chambers. In the heart, there are four separate compartments or chambers - just like the rooms of a house. The upper chamber is called an ATRIUM. There are two upper chambers, one on the right and one on the left. The lower chamber is called a VENTRICLE. Again, there are two ventricles, right and left. The atrium on each side of the heart receives and collects the blood coming to the heart. It then delivers blood to the powerful ventricle, which pumps blood away from the heart through powerful, rhythmic contractions.
Each ventricle has a one-way inlet valve and a one-way outlet valve. The tricuspid valve opens from the right atrium into the right ventricle, and the pulmonary valve opens from the right ventricle into the pulmonary arteries. The mitral valve opens from the left atrium into the left ventricle, and the aortic valve opens from the left ventricle into the aorta. The inlet valves (tricuspid and mitral) are called the atrioventricular valves. The outlet valves (pulmonary and aortic) are called the semilunar valves.
The heart is a muscular pump powered by electricity. The electrical system: causes the heart to beat. controls the heart rate (the number of beats per minute). has special pathways (conduction pathways) that carry the electrical signals at lightning speed through the heart, triggering a heartbeat. These conduction pathways are a complex network of cells and fibers: In a healthy heart, each heartbeat begins in the sinoatrial node (the heart’s natural pacemaker), which is located in the right atrium. The electrical signal from the sinus node (sinoatrial or SA node) starts an electrical chain reaction that spreads across both atria, much like ripples on the calm surface of a pond. This causes the atria to contract and pump blood into the ventricles. This electrical chain reaction continues from the atria through an area between the atria and ventricles called the atrioventricular (AV node or AV junction). The AV node acts as an electrical gateway to the ventricles. The AV node connects to conduction pathways that relay the signal to the ventricles. The ventricles then contract and pump blood to the body.
Cardiac muscle tissue is very strong and able to contract and relax rhythmically throughout a person&apos;s lifetime. The human heart is actually two pumps in one. The right side receives oxygen-poor blood from the various regions of the body and delivers it to the lungs. In the lungs, oxygen is absorbed in the blood. The left side of the heart receives the oxygen-rich blood from the lungs and delivers it to the rest of the body.
The heart&apos;s pumping action depends on the proper functioning of the heart valves. The heart has four valves, or tissue flaps, that open and close, allowing blood to move between the heart&apos;s four chambers. The four valves of the heart are the tricuspid valve, the pulmonary valve, the mitral valve, and the aortic valve. Each valve controls the flow of blood, causing it to flow forward into the next chamber. The closing of the valves is responsible for the sounds made by the beating heart. When the tricuspid and mitral (atrioventricular) valves close-the first &quot;lubb&quot; sound is audible (S1). The second &quot;dubb&quot; sound occurs when the pulmonary and aortic (semilunar) valves close (S2).
Cardiac output is the product of the stroke volume (amount of blood pumped) times the heart rate. Until late school-age or early adolescence, cardiac output in the child is RATE DEPENDENT, not stroke volume dependent, making heart rate more rapid.
Preload—amount of “stretch” in the ventricle caused by blood volume. Stretchability of heart and ventricles. Too much preload (stretch) can lead to cardiomegaly (enlarged heart). Central venous pressure is the pressure of blood in the right atrium. Helpful in determining the amount of circulating blood volume (venous blood).
Afterload—the amount of pressure that the ventricles have to build up to eject the blood out. Afterload is the major workload of the heart – the greatest expenditure of oxygen.
A positive inotropic agent is one that increases the strength of the heart beat.
Fetal circulation differs from neonatal circulation in three areas: the process of gas exchange, the pressures within the systemic and pulmonary circulations, and the existence of anatomic structures that assist in the delivery of oxygen-rich blood to vital organ systems.
In fetal circulation, gas exchange occurs in the placenta; the lungs are nonfunctional with very little blood flow. The placenta provides oxygen and nutrients for the fetus, and removes carbon dioxide and other waste products. The mother and fetus’ blood vessels intertwine but do not join (no mixture of blood). The exchange of oxygen, nutrients, and waste materials between the mother and fetus occurs by diffusion. The umbilical cord connects the fetus to the placenta, and contains two small arteries and one large vein, providing for the transport of blood to and from the fetus and placenta. Oxygenated blood from the placenta flows by way of the umbilical vein to the inferior vena cava.
Resistance in the pulmonary circulation is higher than resistance in the systemic circulation.
A shunt is a detour that directs blood flow in a different direction. In utero, the fetus receives blood carrying oxygen and nutrients from the placenta through the umbilical vein. The liver is bypassed along the way, by the first fetal shunt—the ductus venosus. Blood routed to the ductus venosus bypasses the liver and is routed to the inferior vena cava. Only a small amount of blood circulates to hepatic tissue. Liver function is minimal in the fetus, so very little blood supply is required.
Oxygenated blood from the placenta flows into the right atrium. Two-thirds of the oxygenated blood flows directly to the head, heart, and upper extremities. The brain and coronary arteries receive the blood with the highest oxygen concentration. In fetal circulation, the pressures on the right side of the heart are greater than pressures on the left side. The foramen ovale is an opening between the atria (top chambers of the heart) that allows blood flow from the right atrium directly to the left atrium. This is the second fetal shunt. Most of the blood received from the inferior vena cava is shunted through the foramen ovale and into the left atrium. From the left atrium, the blood is pumped through the left ventricle into the aorta. The direction of the blood flow as well as the high blood pressure in the right atrium propels this blood through the foramen ovale into the left atrium. Most of the lungs are bypassed, supplying the heart and the brain with oxygenated blood. These are the two most oxygen-needy organ systems.
The remainder of the less oxygenated blood now returns from the upper body, head, neck, and arms, and travels from the superior vena cava into the right atrium, where it mixes with some of the oxygen-rich blood coming up from the inferior vena cava. Because of the direction of the blood flow, the right ventricle pumps most of the blood returning from the superior vena cava. This blood travels through the tricuspid valve and the right ventricle into the pulmonary artery. A very small portion of this blood flows from the right atrium into the right ventricle and out the pulmonary artery into the nonfunctioning lungs. The pressure in the pulmonary circulation is very high. The lungs are collapsed and filled with fluid. Because of the high pressure, most of the blood bypasses the lungs. Most of the blood pumped out by the right ventricle bypasses the lungs by flowing through the third fetal shunt, the ductus ateriosus, into the descending aorta. This supplies blood to the lower portions of the body. The ductus arteriosus is a conduit between the pulmonary artery and the aorta that shunts blood away from pulmonary circulation. Blood then flows from the descending aorta to the other organ systems, then back to the placenta for gas exchange by way of the two umbilical arteries.
With the neonate’s first breath, gas exchange is transferred from the placenta to the lungs. This means that the baby must use his/her lungs to take in oxygen and get rid of carbon dioxide. With the initiation of respirations, the Pa02 levels are increased and PaC02 levels are decreased. Inspired oxygen dilates the pulmonary vessels, and pulmonary vascular resistance is decreased. Because resistance in the lungs has decreased, blood flow into the lungs increases.
After the first breath, resistance to pulmonary blood flow decreases and a marked increase in pulmonary blood flow follows. Decreased pulmonary resistance (after the first breath) allows for the increased pulmonary blood flow. Resistance in the systemic circulation increases.
Umbilical cord clamped = less blood flow to right side of the heart. Pressures on the left side of the heart become greater than pressures on the right side. Systemic vascular resistance becomes greater than pulmonary vascular resistance. The right heart receives poorly oxygenated blood from the body, while the left heart receives highly oxygenated blood from the lungs. Deoxygenated blood flow starts flowing into the right heart and lungs.
Because of these favorable pressure changes, more blood flows to the lungs, where it is oxygenated.
After the neonate’s first breath, the fetal shunts close in response to pressure changes and increased Pa02 levels in the pulmonary and systemic circulations. Clamping of the umbilical cord causes blood to the ductus venosus to fall instantly. The absence of blood will cause the ductus venosus to close and it will eventually turn into a ligament. There is dramatically less blood flowing to the right side of the heart after the umbilical cord is cut. This causes an immediate drop in blood pressures on the right side of the heart. At the same time, the pressure in the left side of the heart builds as the greatly increased blood flow from the lungs enters the left atrium. The systemic vascular resistance increases because of more blood flowing to the left side of the heart. The foramen ovale opens only from right to left. The decreased pressure on the right side of the heart, along with increased pressure on the left side of the heart stimulates the closure of the foramen ovale. The change in circulation patterns causes the ductus arteriosus to close. The closure of this shunt causes blood to flow from the pulmonary artery into the lungs for oxygenation. The increased pulmonary blood flow enhances the left ventricular output. Resistance in the systemic circulation increases. In some neonates, it may take several days for the fetal shunts to completely close.
Health history—family history of congenital heart disease; maternal illnesses, infections, and medications taken during pregnancy.
Feeding difficulties infants, frequency of respiratory infections, poor weight gain, fatigue, exercise intolerance. Poor weight gain with failure to thrive is a common sign of congenital heart disease.
Best way to check for pediatric cardiovascular status is color and respiratory rate.
Related to hemoglobin level and oxygen saturation.” The hemoglobin molecule carries oxygen. The oxyhemoglobin gives the skin the pink color. In the absence of oxyhemoglobin, the skin color darkens.
Order of auscultation: “A(P)TM machine” PMI—found at 3rd – 4th ICS in infants; 4th ICS in young children and the 5th intercostal space in children older than 7 years. PMI more than 2cm below—enlarged heart, congestive heart failure. S1 best heard at the apex of the heart. S2 is heard best at base (right and left of sternum at 2nd intercostal space). The younger the child, the higher the pulse rate. Chest wall of infants and young children is thinner—may be able to see the PMI and it is easier to hear innocent murmurs. Murmurs are caused by turbulent blood flow. Murmurs are described according to location, timing within cardiac cycle, intensity, and pitch
Temperature—compare temperature of trunk with extremities. Pulses—weak or absent pulses in the lower extremities may indicate coarctation of the aorta. Blood pressure—discrepancies between upper and lower extremity may indicate cardiac disease. Capillary refill—normal &lt; 2 seconds. Chest—point of maximal impulse, thrills, friction rubs.
Correct answer: D
The child’s bone marrow responds to chronic hypoxemia by producing more red blood cells to increase the amount of hemoglobin available for oxygenation. This increase is known as polycythemia. A hematocrit value of 50% or higher is common in children with cyanotic heart defects.
Correct answer: A
Mild hypoxemia: 90-95% Moderate hypoxemia: 85-90% Severe hypoxemia: &lt; 85%
Noninvasive. Child needs to be quiet and cooperative. Displays heart rate and rhythm.
Noninvasive. Provides information about heart size, blood flow to lungs, position of stomach, liver, and heart.
An echocardiogram (&quot;echo&quot;) is a type of ultrasound test that uses high frequency sound waves (not radioactive) for viewing the heart. It is a safe and painless procedure that helps doctors diagnose heart problems. Best done while the child is quiet and cooperative. Pictures of the child&apos;s heart can be viewed on a small monitor while the procedure is being performed. It is a non-invasive test (no probes or needles) and everything is done from the outside of the body. Echocardiograms are generally the best tests to demonstrate the structure of the components of the heart. The echocardiogram is used for measuring the size and thickness of the heart chambers, how the heart is handling the pumping of blood through the chambers, and blood flow through the heart valves. The echocardiogram can detect structural abnormalities of the heart (holes between the chambers, fluid around the heart, mass inside the heart, etc.) and show valve shape, motion, narrowing or leaking. There are many different types and methods of “echos.”
This is both an invasive diagnostic procedure and an interventional and therapeutic procedure as well. Cardiac catheterization is an invasive test in which a specially trained cardiologist places thin, flexible tubes (catheters) through the blood vessels (usually the right femoral artery or vein) and guides them inside the heart and major vessels around the heart. This is done using special x-ray equipment. The catheters allow cardiologists to obtain information about pressures in the heart and blood vessels, blood oxygen levels, and blood flows. This is usually performed to help in providing a diagnosis of heart problems.
Interventional catheterization is a type of cardiac catheterization where actual treatments can be performed by use of specialized catheters. These specialized catheters include balloon catheters that can open narrowed valves or arteries and also catheters where devices can be deployed which can close extra vessels or certain &quot;holes&quot; in the heart.
Discharge instructions for a child following cardiac catheterization should include: sponge baths only for the first 3 days at home; change the bandage over the insertion site daily for 3 days; observe for inflammation, bleeding or swelling at catheter insertion site. Observe for warmth, color on affected extremity.
“B” is correct.
In infants, poor sucking and swallowing may be early indications of heart defects. The goal should be to promote as normal life as possible for the child. The child needs to have social interaction, discipline, and appropriate limit setting.
Failure to thrive (FTT) is defined as a child with deficiencies in weight and height as compared to age related normals. This includes children whose weight and height are less than the 3rd percentile or whose weight or height have decreased more than 2 major percentiles (ex. 50 to 3rd percentile on their growth charts). Children with complex heart problems have a 40% rate of failure to thrive. Corrective surgery usually allows catch-up growth.
Provide periods of uninterrupted rest The child needs to be well rested before feeding. The child’s needs should be met to minimize crying. The nurse must organize care to decrease energy expenditure. Smaller meals require less initial effort, as well as less digestive effort. Limit bottle feedings to 20 to 30 minutes. Use a nipple that the infant can comfortably adjust for flow rate and energy to express milk. May need a soft, large-hole nipple. Feed smaller volumes of concentrated formula (24 to 27 cal/oz) every 3 hours. May require 120 to 150 kcal/kg/day for adequate weight gain. Time feedings to allow for adequate rest—every 3 hours is optimal. Maintain a neutral thermal environment – This is an environment in which the child can maintain a stable body temperature without an increase in metabolic rate or oxygen use.
Feed the infant or child slowly in a relaxed environment with frequent, small feedings. Anticipate the child&apos;s needs and fears, and help to reduce anxiety, sadness (i.e., crying) and anger. Place the infant on his or her right side after feeding. Maintain HOB elevation of 30 - 45º.
Gavage feedings decrease energy expenditure and allow calories consumed to be used for growth. Can be used in conjunction with timed nipple periods to maintain feeding skills.
NG feeds may need to be implemented if the infant or child tires before the recommended amount of feedings is consumed, takes longer than 30 minutes to feed, experiences increased fatigue during or after feeding, or demonstrates poor weight gain on adequate caloric intake.
Correct answer is “B”
Polycythemia—too many red blood cells. This is the compensatory response of the body to chronic hypoxia. H & H as a compensatory response to chronic hypoxemia. The body is compensating for tissue hypoxia by increasing red blood cell production. At greater risk for blood clots: “thick” blood can cause thrombi and organ ischemia. Cyanotic children are often smaller than their peers and may demonstrate clubbing. Cyanotic children experience frequent respiratory infections and miss more school days.
Fingers and toes Caused by chronic hypoxemia (long-term lack of oxygen in the arterial blood supply)
“D” is correct.
Congestive heart failure is a condition in which the heart cannot pump enough oxygenated blood to meet the body’s needs. The heart keeps pumping, but not as efficiently as a healthy heart. Usually, the loss in the heart&apos;s pumping action is a symptom of an underlying heart problem. This can be due to either extra volume that a normally working heart needs to pump because of a structural problem, or it can be a result of a weak heart muscle&apos;s inability to pump the normal amount of blood it is expected to pump. The severity of the condition and symptoms depends on how much of the heart&apos;s pumping capacity has been affected. The body diverts blood away from less vital organs, particularly muscles in the limbs, and sends it to the heart and brain.
When the right side of the heart begins to function less efficiently, it is unable to pump much blood forward into the vessels of the lungs. Because of the congestion in the right side of the heart, blood flow begins to back up into the veins. As blood flow out of the heart slows, blood returning to the heart through the veins backs up. This causes backup of blood and fluid into the liver and veins leading into the heart. Swelling is noticed in the feet, ankles, eyelids, and abdomen due to fluid retention. The kidneys are less able to dispose of sodium and water, also causing fluid retention in the tissues. Right side: Excessive weight gain Enlarged liver or spleen (hepatosplenomegaly) Peripheral and periorbital edema Ascites Neck vein distention (JVD) The digestive system receives less blood, causing problems with digestion.
When the left side of the heart fails, it is unable to pump blood forward to the body efficiently. Blood begins to back up into the vessels in the lungs, and the lungs become stressed. Breathing becomes faster and more difficult. Also, the body does not receive enough blood to meet its needs, resulting in fatigue and poor growth. Blood &quot;backs up&quot; in the pulmonary veins (the vessels that return blood from the lungs to the heart) because the heart can&apos;t keep up with the supply. This causes fluid to leak into the lungs. Left side: Respiratory problems Cough Nasal flaring in infants Grunting Intercostal retractions Wheezing Tachypnea
It is not uncommon for both sides of the heart to fail at the same time and cause backup into both systems simultaneously. To &quot;make up for&quot; the loss in pumping capacity, the heart beats faster. Cardiomegaly: An enlarged heart.
The symptoms of poor growth -- difficulty with feeds and fast breathing -- will gradually appear during the third or fourth week of life with congenital heart defects.
With these nursing diagnoses, minimizing energy expenditure is a priority!
With these nursing diagnoses, minimizing energy expenditure is a priority!
It is very important for the nurse to recognize the early signs that the child is going into congestive heart failure. Early treatment will prevent complications to a more severe state. Tachypnea is one of the early signs that should be identified. Tachypnea at rest, dyspnea, retractions, poor feeding, and activity intolerance are physical signs and symptoms. When a child is going into heart failure, they will also get really sweaty. There will usually be decreased urinary output. When you notice these things on assessment, notify the physician. Handled at the same time by using Lasix and dig together.
C. Pulmonary edema
The aim of all cardiac medications are to increase oxygenation to the heart by decreasing the cardiac workload (cutting down on energy expenditure) and increasing the cardiac output. The management of a child with CHF involves correcting the underlying problem as soon as it is feasible to do so. Medications are often helpful in treating heart failure initially. Eventually, medications may lose their effectiveness and many heart defects will need to be repaired surgically. Medications may also be used after surgery to help the heart function during the healing period. Digoxin and Lasix are the most commonly used medications. Supplemental oxygen may also be used, as well as nutritional supplements.
Drug therapy usually begins with digoxin—most common pediatric cardiac drug. Digoxin is a medication that helps strengthen the heart muscle, enabling it to pump more efficiently (positive inotropic agent). It helps strengthen the force of ventricular contractions. It also slows down the heart rate (negative chronotropic effect). Effectiveness depends on achieving and maintaining a therapeutic serum drug level. Because digoxin is such a potentially toxic drug to children, there is a very narrow margin for safety to children taking digoxin. The book states 0.8 – 2.0, but can vary from institution to institution. Signs of dig toxicity in children: frequent vomiting, child refuses to eat (anorexia). Need to be monitoring electrolytes, because most of the time the child will be on Lasix also (which can drop the electrolytes) and low potassium levels can cause a child to become dig toxic. Hypokalemia, hypomagnesemia, and hypercalcemia can all potentiate dig toxicity.
The most common drug that you will be teaching parents about is digoxin. Parents need to be well-educated before administering digoxin. Monitor HR for one full minute before giving and if it is below 100 in infants/toddlers or below 70 in older children, notify doctor before giving.
Not appropriate to use a teaspoon/dropper because the dose drawn up needs to be very accurate so it needs to be drawn up in a 1-ml syringe. No infant should ever receive more than 1 ml of a drug. Never give parents anything greater than 1-ml syringe for drawing up meds. Tell them to always take the cap off first (the cap could pop off in the child&apos;s mouth and could choke the child). If you notice, there are no caps on the syringes on 2 West A. Instruct parents that if the child experiences nausea, vomiting, listlessness, or anorexia, the physician must be notified. These are all signs of dig toxicity.
Diuretics help the body get rid of excess fluid. They do this by helping the kidneys excrete excess fluid. Their effectiveness is evaluated from the urine output, either by measuring the amount of urine, or by weighing the diapers. Monitor electrolytes, urinary output, blood pressure. Furosemide (Lasix) is a diuretic used to eliminate excess water and salt to prevent reaccumulation of the fluid. Using the same scale, weigh the child daily at approximately the same time. Excessive weight gain is the earliest indication of excess fluid volume. Notify physician of weight gain of more than 50g/day in infants and more than 200g/day in children.
Potassium-sparing diuretics - helps the body retain potassium, an important mineral that is often lost when taking diuretics.
ACE (angiotensin-converting enzyme) inhibitors - dilates the blood vessels, making it easier for the heart to pump blood forward into the body. Decreases the production angiotensin II, a potent vasoconstrictor, resulting in peripheral vasodilatation Does this by relaxing smooth muscles in peripheral blood vessels, causing vasodilation. Reduces afterload. Monitor BP before and after giving this drug. You need to observe for hypotension. Notify the doctor if the BP is low.
“D” is correct.
“B” is correct.
Hypertension—most of us know it as high blood pressure, when the force of blood pushing against the walls of our arteries is too high, causing the heart to work harder than it should. It’s the most common cause of heart attack and stroke. There is another type of hypertension that is little known and far less common. Yet, it is very serious, difficult to diagnosis and can become progressively worse—even fatal. It’s called pulmonary hypertension (PH). Pulmonary hypertension is a condition in which pressure in the blood vessels in the lungs is higher than normal. The small arteries of the lung narrow throughout the lungs, causing greater resistance to blood flow. As a result of the increased workload caused by this resistance, the right side of the heart becomes enlarged and right-sided heart failure develops. Left untreated, the left side of the heart eventually fails, with reduced cardiac output and hypotension. By definition, pulmonary hypertension is diagnosed when the blood pressure in the heart to lung circulation exceeds 25 mm Hg at rest or 30 mm Hg with exercise. Most common causes: lung disease (such as chronic lung disease of infancy (BPD) resulting from immature lungs in premature babies, or airway obstruction from meconium aspiration syndrome), and some congenital heart diseases with excessive blood flow to the lungs or blockage of blood flow from the lungs back to the heart.
Pulmonary hypertension is the narrowing of the pulmonary arterioles within the lung. The narrowing of the arteries creates resistance and an increased work load for the heart. The heart becomes enlarged from pumping blood against the resistance. Some symptoms include chest pain, weakness, shortness of breath, and fatigue. Untreated, the disease usually develops into congestive heart failure.
Infants display symptoms of failure to thrive and congestive heart failure. Children with PH usually present with nonspecific and progressively worsening symptoms: fatigue, chest pain, difficulty breathing with activity, episodes of fainting or nearly fainting.
Inhalation of nitric oxide helps dilate the vessels in the lungs. Sildenafil, taken orally, helps the blood vessels in the lungs to relax so blood and oxygen can flow more freely.
Two types: Congenital One or more heart structure abnormalities that develop before birth or persistence of a fetal structure after birth. Acquired Any cardiac condition that was not present at birth; Develops over time and may be seen both in children with normal hearts and in those with congenital heart disease. In a congenital condition, the problem is present before or shortly after birth thus causing problems for the newborn infants. Nurse’s role is very important—astute and vigilant monitoring of a child with potential cardiovascular alterations. The degree of change in acuity and the speed of decompensation are much greater in infants and children than in any other age group. The skill required of the pediatric nurse in assessing, monitoring, and delivering prompt treatment is an unparalleled nursing challenge.
Six out of every 1000 babies born in the United States has a congenital (present at birth) heart defect - a problem that occurred as the baby&apos;s heart was developing during pregnancy, before the baby is born. Congenital heart defects are the most common birth defects. Seen more commonly with: Family history(can run in families) Infants of diabetic mothers Mothers contracting rubella in the first trimester Infants with Down syndrome. Women who have babies after 35. Mothers who took dilantin or lithium during pregnancy. Children with true congenital heart disease have a lot of respiratory problems and have growth problems. They are those organic failure-to-thrive children. Birth defects are the leading cause of death, excluding prematurity, during the first year of life. Height and weight are tale-tell signs of congenital heart disease in a child.
The current approach to congenital heart disease is to return the child to normal or as-near-to-normal-as-possible anatomy and physiology as soon as possible. Corrective and palliative procedures are now performed earlier in life because of a number of advances in surgical techniques and medical management.
Acyanotic: the congenital heart defects that don’t start with a “T.” You will see left-to-right shunting. Most children with congenital heart defects have acyanotic conditions. The common forms of acyanotic CHD are those where there is a defect in one of the walls separating the chambers of the heart, or obstruction to one valve or artery.
Acyanotic heart lesions are lesions that INCREASE pulmonary blood flow. Left to right shunt. Shunt—abnormal blood flow from one side of the heart to the other. Systemic pressure is greater than pulmonic pressure. Oxygenated blood mixes with deoxygenated blood, and the extra blood volume overloads the pulmonary system. No deoxygenated blood (venous blood) enters systemic arterial circulation. All of the blood returning to the right side of the heart passes through the lungs and pulmonary circulation.
Most of the time, no cyanosis (bluish or purplish hue). Clinical signs not always apparent at birth (may take awhile to develop). Increased pulmonary blood flow (pulmonary hypertension) due to backwashing of blood to right side of the heart and overloading the pulmonary system. More blood to right side of the heart—right heart enlarges (hypertrophy) and sometimes left heart also. One of the major consequences of left-to-right shunting heart defects is congestive heart failure. Other consequences include pulmonary hypertension, pulmonary vascular disease, and frequent upper and lower respiratory infections that can progress to respiratory failure. Signs of respiratory distress include dyspnea, tachypnea, and intercostal retractions. Child will exhibit poor growth. May also see enlarged liver.
This is an abnormal communication between the ventricles. A ventricular septal defect allows blood to flow between the heart’s left and right lower chambers. In the normal heart, the right and left chambers are completely separated from each other by a wall called a septum. The most common type of congenital heart defect. 25% of all congenital heart disease. Often accompanied by other cardiac defects. VSD is an opening between the left and right ventricle (it is like a hole in the heart in the lower chambers). May vary in size from a miniscule hole to complete absence of the septum between the two ventricles.
Direction of shunting is from the high pressure left side to the lower pressure right side. Ordinarily, the resistance or pressure on the heart&apos;s left side is much higher than the pressure on the heart&apos;s right side. When there is a large defect, the blood takes the &quot;path of least resistance&quot; and goes back to the right ventricle instead of out to the body. This results in pulmonary overcirculation and extra work for the right side of the heart. When the heart is unable to meet this extra work load, symptoms of congestive heart failure develop including excessive sweating (a cold, clammy sweat often noted during feeding), poor feeding, slow weight gain, irritability or lethargy, and/or rapid breathing. Infants are usually not symptomatic at birth but become symptomatic within the first few weeks of life (usually when the child is feeding). Large defects: CHF accompanied by poor feeding, poor weight gain, and failure to thrive.
Many (20-60% of VSD’s) close spontaneously during the first year of life, so a lot of children won’t need surgery. Usually, ventricular septal defects diagnosed in infancy get smaller with time and even large defects can close completely. If the child develops congestive heart failure, treatment is needed. This involves the use of medications to decrease the work of the heart and increase the strength of the heart beat. Medications that may be used include Lanoxin and Lasix. These medications often control the symptoms until the child gets bigger and the defect gets smaller or closes altogether. If the medicines aren&apos;t effective, then surgery is usually recommended.
Patients are instructed to use antibiotics before dental work and surgeries for the rest of their lives. Often penicillin for at least 10 days. May also need antibiotics if there is a cut finger or break in the skin. If they don’t, they may get a heart infection that can cause valvular disease.
“A” is correct.
Surgical closure is recommended for any defect that is big enough after the first year or two of life to allow excessive pulmonary blood flow. This is to prevent a very serious permanent complication called pulmonary vascular obstructive disease. The arteries going to the lungs become &quot;stiff&quot; and cause more and more resistance to blood flow to the lungs, causing irreversible damage to the lungs and heart, cyanosis with clubbing. There will also be longterm respiratory problems such as rapid breathing or coughing. There are times when a VSD cannot be closed. This usually occurs when there are multiple septal defects, unusually located VSD’s, very small infants in whom open-heart surgery cannot be performed, or other associated heart defects or medical problems making simple VSD closure difficult or impossible. In these cases, a pulmonary artery band is applied which reduces the amount of blood flowing to the lungs. Pulmonary banding is a closed heart surgery which consists of placing a tie around the pulmonary artery and constricting it. It is sort of a noose, and decreases the amount of blood flow to the lungs and the pressure in the lungs, thereby decreasing the chance of pulmonary vascular disease.
Surgical repair of a ventricular septal defect involves open heart surgery and placement of a prosthetic patch, sutured in placed, that covers the defect. The patch may be a synthetic piece of fabric (Dacron) or the patient’s own tissue (pericardium) and it is secured with fine sutures. The heart tissue grows over the patch so the heart never &quot;outgrows&quot; it. This type of surgery requires a sternotomy (cracking open the sternum) and the use of cardiopulmonary bypass.
“B” is correct. Don’t want to go in blind.
Patent ductus arteriosus—when the ductus arteriosus fails to close after birth; accounts for 10% of all congenital heart disease. Persistence of the ductus beyond 10 days after birth is considered abnormal. Failure of the ductus to close is quite common in premature babies but fairly rare in full-term babies. In most full-term infant the ductus closes spontaneously in the first hours following birth as the baby takes its first breath. It remains open more frequently in premature infants with low birth weight. PDA is the most common acyanotic lesion that you will deal with in the NICU. A PDA allows blood to flow from the aorta (high pressure) to the pulmonary artery (low pressure). Oxygenated blood from the aorta mixes with the de-oxygenated blood in the pulmonary artery. The shunt is left to right because the pressures are higher in the aorta than in the pulmonary artery and blood flows to the path of least resistance. Blood ends up flowing from the aorta back into the lungs and left side of the heart, instead of to the body. You see in the diagram a big circuit involving the pulmonary artery, lungs, left atrium, left ventricle, and aorta. The lungs and left heart get overloaded, not the right ventricle as in other types of left-to-right heart lesions. If the PDA does not get fixed, it will cause severe pulmonary disease from the increased pulmonary congestion. The left side of the heart now needs to work harder and pump more blood, since much of it is flowing back into the lungs and returned to the left atrium and left ventricle. The left ventricular output can be two to three times that of normal. Over time, this increase in work causes the left side of the heart to hypertrophy and congestive heart failure may result. The characteristic in PDA babies—a machinery-type murmur.
A PDA may vary in size from small to large. Smaller PDA&apos;s will generally cause no symptoms or ill effects in the young child. A child with a large PDA might have poor growth and frequent respiratory infections. In some infants, usually those that are premature, the PDA can be closed with a drug called indomethacin. Indomethacin is successful in closing the PDA most of the time. Cardiac catheterization or surgical repair is recommended if the PDA still remains open by three months of age. Cardiac catheterization is less invasive and has become the corrective means of choice. If cardiac catheterization is not possible, the surgeon can close the ductus arteriosus by tying it or clipping it, without opening the heart (ligation of the PDA) through an incision in the baby&apos;s chest (left thoracotomy). In some cases this surgery can be performed laparascopically. Children with untreated PDA&apos;s require penicillin prior to any dental work or surgery on the mouth, bowel, or bladder. This helps prevent the uncommon, but possible, occurrence of bacterial infection affecting the heart near the defect or heart valves (bacterial endocarditis).
Indomethacin acts by blocking the effects of PROSTAGLANDIN, which is the natural substance that keeps the ductus arteriosus open. When prostaglandin is blocked, the ductus closes. Indomethacin is a nonsteroidal anti-inflammatory agent (NSAID) and can cause bleeding. Several doses of indomethacin may be necessary to totally close the PDA, but with each successive dose, there is the danger of bleeding into the brain tissue (intraventricular hemorrhages) so many PDA&apos;s cannot be closed by this method. Big danger – BRAIN BLEEDS. Monitor renal function (BUN/ creatinine and bleeding). Contraindications include low platelets (thrombocytopenia), renal insufficiency, and GI bleeding. You never give Toradol to a pregnant woman, because Toradol is also an NSAID and can close the ductus arteriosus of the fetus in utero. Can’t be used for management of pain in labor and delivery.
ASD is a hole or opening that exists between the heart&apos;s two upper chambers (the atria). 5-10% of all congenital heart disease. The opening lets some blood from the left atrium (oxygenated blood that&apos;s already been to the lungs) return via the hole to the right atrium. Pressure in the left atrium exceeds that in the right atrium, causing blood to flow left to right through the defect. If the opening is large enough, the increase in blood flow leads to enlargement of the right atrium and right ventricle, problems with the way the right heart functions, or rhythm abnormalities. Pulmonary blood flow is increased and may lead to lung injury (pulmonary vascular obstructive disease).
Most children with ASD have few, if any, symptoms, dependent on the size. A very small defect or patent foramen ovale (PFO) may have only a very small amount of blood flow across it. Most people with an ASD have no symptoms in childhood as symptoms usually do not occur for decades. When symptoms do occur, they are due to the development of pulmonary vascular disease, right heart failure, and rhythm abnormalities (palpitations, atrial dysrhythmias). There is also the danger of blood clots forming in the atria and causing a stroke. The diagnosis of ASD is suspected by abnormal findings such as shortness of breath, difficulty with exercise, poor growth, rapid breathing, palpitations, and a systolic heart murmur. The diagnosis is confirmed with an echocardiogram. If asymptomatic, the child will be followed by a cardiologist. Will need bacterial endocarditis prophylaxis before invasive procedures. Treatment (if needed): “pericardial patch” done by cardiac catheterization or open heart surgery (cardiopulmonary bypass) when the child is 2 to 5 years of age.
Simply put, this is a large hole in the center of the heart. This septal defect involves both upper and lower chambers: a combination of ASD and VSD. Also, the tricuspid and mitral valves that normally separate the upper from the lower chambers of the heart are not formed as individual valves. Instead, a single large valve forms which crosses the defect. ASD accounts for 2-5% overall of congenital heart disease, but is present in 15-20% of newborns with Down syndrome.
The large opening in the center of the heart allows oxygen-rich blood from the left side of the heart to pass freely into the right side of the heart. The right heart has to pump an additional amount of blood back to the lungs and may become enlarged. Symptoms may occur at any time from birth to several months of age. Most infants with an atrioventricular canal do not grow normally and may become undernourished. Because of the large amount of blood flowing to the lungs, high blood pressure may occur there and damage the blood vessels.
If not treated in time, increasing pressures in the lung tissues can cause deoxygenated blood to permanently “shunt” through septal openings from right to left causing cyanosis.
Symptomatic infants with atrioventricular septal defects may improve with medicine, but in all cases corrective heart surgery will be necessary. Medical treatment of infants with atrioventricular septal defects is usually used to relieve symptoms and allow the baby to get big enough to undergo surgical repair with lower risks. Medicines commonly used to treat congestive heart failure from left-to-right shunts in infants include diuretics such as lasix (furosimide), ACE inhibitors such as captopril, and digoxin. These type of defects will never close on their own and will always require corrective surgery for treatment. This usually occurs at 3-4 months for infants with a complete atrioventricular septal defect. During the operation, the surgeon closes the large hole with one or two patches. The patch will become a permanent part of the heart as the heart lining grows over it. The surgeon also divides the single valve between the upper and lower chambers of the heart and constructs two separate valves to resemble normal valves as nearly as possible. The most common later problem that is seen following surgery is a leaky mitral valve which may require reoperation in up to 10 percent of patients, but most become medication-free and are able to lead essentially normal lives. Follow-up visits with the cardiologist are important to assess valve and heart muscle function and continued antibiotic prophylaxis for endocarditis is recommended.
Cardiac volume overload. Congestive heart failure may occur when the amount of blood passing from left to right side of the heart overloads the pulmonary system.
When a child has pulmonary stenosis, the area where blood exits the heart&apos;s lower right chamber is too narrow. Usually, the pulmonary valve itself is affected. This problem is often caused by fusion of the valve leaflets. Pulmonary stenosis is relatively common and accounts for about 25-30% of heart defects diagnosed during childhood. It can occur in children with otherwise normal hearts or along with other congenital heart defects such as atrial septal defect or Tetralogy of Fallot.
The health effects of pulmonary stenosis are related to the severity of the narrowing and the presence of other heart defects. Most children are asymptomatic and require very little intervention, other than antibiotic prophylaxis before invasive procedures. Severe or critical pulmonary stenosis requires either dilation by balloon angioplasty (a type of cardiac catheterization) or surgical correction by open-heart surgery.
Aortic stenosis is a term used to describe congenital heart defects that cause obstruction of blood flow from the heart to the body. This obstructive congenital disorder is relatively uncommon and occurs more often in boys. Detrimental health effects of aortic stenosis are related to the degree of the narrowing, valve leaks, and if there are other heart defects.
The more severe the problem, the harder the left ventricle (left-sided pumping chamber) has to work. Over time, if the problem is not treated, this overwork causes a thickening of the heart muscle called ventricular hypertrophy. Eventually, the muscle becomes damaged resulting in left-sided heart failure and congestive heart failure.
Aortic stenosis is classified as mild, moderate, severe, or critical. In a baby born with critical aortic stenosis, the opening is so small that the heart cannot pump enough blood to meet the baby&apos;s needs. Unless the problem is treated early, the baby will develop problems with shock and congestive heart failure. Mild: continue to monitor, antibiotic prophylaxis Severe or critical: aortic balloon valvuloplasty (cardiac catheterization), surgical valvotomy May need aortic valve replacement.
8 – 10% of congenital heart disease. Occurs when the aorta is pinched or narrowed, obstructing blood flow to the body.
5 – 10% of congenital heart disease. The narrowing results in high blood pressure before the point of coarctation and low blood pressure beyond the point of coarctation. Most commonly, there is high blood pressure in the upper body and arms and low blood pressure in the lower body and legs. The left ventricle has to pump harder because the pressure is high. Because of this, the heart may enlarge. There is a disparity in the pulses and blood pressures and frequently the femoral pulses are weak or absent. This is one that is found in the newborn nursery because the nurse will not find a pulse in the femoral area or the infant’s BP will be much higher in the arms than in the legs. Before it is fixed, the child may complain of weakness, coldness or tingling in the lower extremities, and muscle cramps. After this has been fixed, the child will often complain of belly pain because they have not had good blood flow through the femoral artery and they haven’t had good blood supply to the mesenteric gut.
In the children without symptoms, in who a coarctation is diagnosed on routine examination, repair of the coarctation, either surgically or using balloon angioplasty at a cardiac catheterization is usually recommended by 18-24 months of age. In the newborn or infant with coarctation who presents in congestive heart failure, initial treatment consists of stabilizing the infant with medications. If the infant is less than 2 weeks of age the baby will receive a medicine to open the ductus arteriosus, prostaglandin, and the most critically ill babies will require the use of a ventilator. After a brief period of stabilization, infants with coarctation and congestive heart failure require surgical repair. The surgical repair of aortic coarctation is done through an incision on the left chest below the armpit (left thoracotomy). The correction is done either by inserting a patch in the area or removing the narrowing and sewing the two ends of the aorta back together (anastomosis). Children with coarctation are at risk for an infection within the aorta or the heart valves and should get antibiotics before dental work and certain surgeries (bacterial endocarditis prophylaxis is required for life).
The most common congenital heart defects are the cyanotic ones. Examples of cyanotic defects are tetralogy of Fallot, transposition of the great arteries. Start with a “T.” Can make a child turn blue.
Cyanotic heart birth defects are more complex than acyanotic defects, and have a combination of defects. With cyanotic defects, remember CPR: Cyanotic heart defect Pulmonic pressure greater than systemic Right to left shunt A right-to-left shunt bypasses the lungs and delivers venous (unoxygenated) blood from the right side of the heart directly into the arterial circulation. There is an obstruction on the right side of the heart. Pressures from obstructed blood in the right side of the heart exceed those in the left. The unoxygenated venous blood flows directly from the right sided chambers to the left-sided chambers (right to left shunt) while bypassing the lungs. Unoxygenated blood (venous blood) enters the systemic circulation and there is mixing of pure oxygen-rich blood with venous blood.
Since a lot of the blood never got oxygenated in the lungs, the person will get cyanosis. Infants suffering from cyanotic conditions usually have blue nail beds and lips due to the excess unoxygenated blood in their systems. The term &quot;blue babies&quot; is often applied to these infants.
The most common of the cyanotic heart defects. Tetralogy babies are born with 4 defects in the heart that lead to a reduced blood flow to the lungs, cyanosis, and shortness of breath: Ventricular septal defect (opening between the left and right ventricles). The VSD allows blood to seep from the left ventricle into the right ventricle. This results in too much blood flow to the right side of the heart, which causes the right ventricle to work harder to compensate for the problem. Pulmonic stenosis (narrowing of the pulmonary valve). This narrowing decreases the amount of oxygen-poor blood from the right ventricle that can squeeze through the narrowed opening and travel through the pulmonary artery to the lungs. Thus, there is a decreased blood flow to the lungs. Over-riding aorta (displacement of aorta over ventricular septal defect). The aorta is receiving both oxygen-poor blood from the right ventricle and oxygen-rich blood from the left ventricle, and carries this mixture to the rest of the body. Right ventricular hypertrophy (thickening of the wall of the right ventricle). An enlargement of the muscle tissue of the right ventricle due to overexertion, as a result of increased blood flow to the right side of the heart (due to the VSD) and pumping against a narrowed pulmonary artery.
The two that are most important are the narrowing in the pulmonary artery and the VSD. What causes the most trouble out of the four defects is the degree of the pulmonic stenosis that they are having and sometimes it is really significant. The degree of cyanosis is dependent on the severity of the narrowing. If pulmonic stenosis is mild, there is little or no right-to-left shunting.
X-ray: heart is “boot-shaped” due to under-developed pulmonary artery.
Hypercyanotic episode—the infant or child becomes cyanotic, tachypneic, diaphoretic, limp, and as purple as a grape. These “Tet” spells can become life threatening if not treated immediately. The child becomes progressively more hypoxic and limp, loses consciousness, is likely to have a seizure or stroke, and may die. The child will be placed in the knee-chest position and given oxygen. For severe cases, the child will be given both morphine and propanolol intravenously, along with intravenous fluids. Monitor for metabolic acidosis and prolonged unconsciousness. Often the episode is preceded by crying, feeding, or having a bowel movement. Hypoxic spells are more likely to happen when the baby is a little &quot;dry&quot; or dehydrated and may be prevented by careful attention to hydration particularly if the baby is having problems with vomiting or diarrhea. Spells are also more likely if the baby is anemic (low H & H) so if this is noted the doctor may order an iron supplement or packed red blood cells to improve oxygen delivery. Oral propanolol (Inderol) is administered to decreased the frequency of “Tet” spells, because it helps open up the pulmonary artery by reducing spasms.
These spells are probably due to sudden, increased pulmonary stenosis. Hypercyanotic episode—put the infant in a knee-chest (knee tuck) position. Have an older child squat. Squatting is a spontaneous compensatory mechanism by older children to relieve tet spells. The squatting or knee chest position decreases the amount of blood returning from the heart and allows the child time to compensate. Squatting and a knee-tuck position increase systemic resistance while decreasing venous return to the heart from the inferior vena cava. This increases pulmonary blood flow and eases respiratory effort. Hypercyanotic episodes are seen most frequently in the first year of life and occur mainly in the morning. The child needs careful observation for signs of increased cyanosis in the morning. A total surgical repair is usually performed before 4 months of age when the infant has a hypercyanotic spell. Prevention: avoid stress, dehydration, iron supplements, fiber and stool softeners.
“D” is correct.
D. Bowel movement
Untreated, this condition tends to lead to death before the age of 20. Fortunately, surgery is available to correct the problem, giving the child a more than a 94 percent chance of surviving the next 25 years. Surgery to correct Tetralogy of Fallot may be done any time from infancy to three to five years old, depending on how severe the problem is. The sooner the surgery is done, the better, to prevent permanent damage. Surgery is being performed on increasingly younger infants. Currently, surgery is scheduled at 2 to 4 months of age for asymptomatic infants and earlier for symptomatic infants. Once a hypercyanotic spell has occurred, immediate palliative or corrective surgery is scheduled. Sometimes two surgeries are done: one to relieve symptoms by increasing the blood flow to the lungs (palliative), and one to correct the underlying defects (corrective).
Palliative surgery: Blalock-Taussig Shunt: palliative surgery for children with tetralogy of fallot. A vascular connection (shunt) between the systemic circulation and the pulmonic circulation is created. A subclavian artery to pulmonary artery anastamosis.This surgery does not correct the heart defect, but directly increases the blood flow to the lungs. If the baby has other health concerns or there is an unusual location of one of the heart arteries the surgeon may choose to delay the total repair and do a shunt operation. This shunt procedure allows for growth of the pulmonary arteries and the child. This operation will give the baby adequate blood flow to the lungs and provide protection from the dangers of hypoxic spells until other concerns can be fixed or the baby is older. A shunt operation is not an open-heart surgery and does not require the heart lung bypass machine. It involves an incision in the left chest, under the arm between the ribs. A tube is placed between the left pulmonary artery and a blood vessel branching off the aorta, the left subclavian artery. Some blood in the aorta will go through the shunt into the pulmonary artery and to the lungs to get oxygen. This protects blood flow to the lungs even if the narrowing out the right ventricle is really severe. The shunt will be taken out when the full repair is done.
“B” is correct.
Corrective surgery (open heart sugery with sternotomy; on cardiopulmonary bypass): Generally, repair is performed on babies with tetralogy of Fallot around 2 to 4 months of life or sooner if spells occur. If the baby has a spell, repair is then done at that time no matter the baby&apos;s age. The age of the child at operation and the kind of operation will depend on the child’s symptoms and the precise anatomy of the defect. In a corrective surgery, the pulmonary valve is widened to increase blood flow, and the ventricular septal defect is closed or patched. Neither the displaced aorta nor the enlarged right ventricle requires treatment. Before and after surgery, the patient is at risk for an infection of the heart&apos;s walls or valves (endocarditis). Also, patients will most likely be instructed to use antibiotics before dental work and surgeries for the rest of their lives.
“D” is correct.
The heart’s aorta and the pulmonary artery are reversed from their normal connections. The two &quot;great&quot; arteries of the body are the aorta and pulmonary artery. The aorta normally comes out of the left ventricle, and carries oxygenated blood to the rest of the body. The pulmonary artery arises from the right ventricle and carries deoxygenated blood from the veins into the lungs for oxygenation. Transposition of the Great Arteries - means the two arteries are &quot;transposed, “ or reversed. The aorta arises from the RIGHT ventricle, and the pulmonary artery from the LEFT ventricle. The two arteries routing blood out of the heart were switched during development, severing the connection between the pulmonary and systemic circulations. Oxygenated blood cycles uselessly between the heart and the lungs while the body receives only oxygen-poor blood. This results in two totally separate and parallel circulations. The right heart manages systemic circulation and the left heart pulmonary circulation. In other words, the heart is sending oxygenated blood back and forth between the heart and the lungs, depriving the body of oxygen-rich blood. No oxygenated blood gets to the rest of the body.
Transposition of the great arteries, occurs in about 40 of every 100,000 live births, about 5% of all congenital heart disease. Survival of a newborn with transposition of the great arteries depends on mixing of these two separate circulations through the fetal structures—continued patency of the ductus arteriosus and foramen ovale. The newborn must have one or more connections that will allow the oxygenated blood to go to the body. Even with the mixing of these two circulations through the fetal connections, babies with TGA are very blue after birth because these connections do not allow enough oxygenated blood to pass to the body. They quickly develop congestive heart failure. Prompt diagnosis and treatment is essential for survival. The child will need corrective surgery within three to five days of birth.
Prostaglandins are hormone-like substances that dilate blood vessels, to help with circulation and blood pressure regulation. The ductus arteriosus is a tube connecting the aorta and pulmonary artery. It normally closes within minutes or hours after birth. Prostaglandins cause relaxation of smooth muscle within the ductus arteriosus—will cause the ductus to stay artificially open. Used in infants with severe cyanotic heart defects, where the open ductus is the only route of blood flow into the lungs or the rest of the body. This is called a DUCTAL DEPENDENT circulation. For the baby to survive, the ductus arteriosus has to REMAIN PATENT, until repair of the associated condition is performed. Prostaglandin is the mainstay in treatment of TGA patients. Prostaglandin infusion is started as soon as the diagnosis of TGA is made, and continued until surgery is possible, usually within a few hours or days. A possible side effect of the prostaglandin is apnea, so the infant will often be placed on a ventilator.
To improve the oxygen supply of the body, a special procedure called &quot;Balloon Atrial Septostomy&quot;, is used during heart catheterization to enlarge the atrial opening. This procedure improves the baby’s condition by reducing the degree of cyanosis. Corrective surgery—arterial switch procedure: aorta and pulmonary artery returned to their normal positions. Must be performed within 3 to 5 days of birth. After surgery and recovery, lifelong medical management will be necessary for all patients that were born with TGA because they are at risk for congestive heart failure and certain rhythm disturbances.
Surgeons at Stanford University&apos;s Lucile Packard Children&apos;s Hospital believe that Jerrick De Leon, born more than 13 weeks early, is the smallest baby ever to survive an open-heart procedure called an arterial switch. At the time of his operation, on February 6, 2005, Jerrick weighed just over 1.5 pounds (700 grams), said his surgeon. Surgeons believe Jerrick is the smallest baby to survive this type of open-heart surgery. His heart was the size of a grape. In diameter, the arteries were the size of the tip of a pencil, and the aorta was 3 millimeters, or about one-eighth of an inch, long. His chest was the length of the doctor&apos;s finger.
Hypoplastic left heart syndrome (HLHS) is a very serious congenital heart disease. Basically, the entire left ventricle is missing or very small (hypoplastic). The chambers, valves and related vessels of the left side of the heart are malformed and can not efficiently pump blood to the rest of the body. So you see the left ventricle, the aortic valve, mitral valve, and ascending aorta all may be small or hypoplastic. Basically you are left with only one effective ventricle (the right ventricle).
In every fetus, the open ductus arteriosus creates a normal situation in which both the left and right ventricles pump blood to the rest of the body. In an HLHS fetus, this in-utero system allows blood to circulate fairly normally, despite the fact that the left ventricle is not helping the right ventricle to pump blood to the rest of the body. However, the ductus arterious normally closes soon after the baby takes its first breath, so that the right ventricle pumps blood only to the lungs via the pulmonary artery, and the defective left ventricle becomes fully responsible for pumping blood to rest of the body via the aorta. As a result, the signs of HLHS quickly begin to appear. Most infants present within the first few days of life with tachypnea and early CHF from increased pulmonary blood flow.The child will exhibit difficulty breathing (dyspnea) as a result of the congestive heart failure. As the ductus arteriosus begins to close, you see systemic hypoperfusion and shock. The infant appears grayish blue in color, with dyspnea and hypotension.
In the past, there used to be only one choice; to let the baby pass away. But in the past 20 years, new procedures have been developed and the survival rate has increased dramatically.
Corrective surgery: heart transplant. This child is pictured at a year and a half old, doing well. Received donor heart at 11 days of age. The scarcity of neonatal donor hearts greatly limits the number of infants who may receive transplants. The child will also require immunosuppressive medications for life, and these have serious side effects.
Palliative surgery for HLHS involves a three-stage surgical approach to improve the flow of blood throughout the body. Stage 1 (Norwood Procedure): A new pathway to bypass the left side of the heart is created. The base of the pulmonary artery is connected with the aorta, and a shunt is placed between the aorta and the other part of the pulmonary artery. Blood moves from the right ventricle through the pulmonary artery to the aorta and out to the body. Some blood moves through the shunt to the lungs for oxygenation. This effectively allows the right ventricle to act as the systemic ventricle and the pulmonary artery to act as the aorta (“neoarta”).
Stage 2—a graft is used to route blood from the superior vena cava to the pulmonary artery instead of to the right atrium. Increases the amount of blood being oxygenated. Stage 3—a graft and internal baffle are used to route blood flow from the inferior vena cava to the pulmonary artery instead of to the right atrium. The Fontan procedure is generally performed between 18 months and 2 years of age.
Survival following a Norwood operation is around 80%, following a bidirectional Glenn operation around 100%, and following a Fontan operation around 95%. Overall survival at 5 years of age is around 70 - 75%. 4-year survival rate is greater than 50%. As more and more patients are surviving into adulthood, several unique problems have arisen, which we classify as those of advanced disease. Especially in the patient with a single right ventricle, clinical conditions such as loss of serum proteins into the gastrointestinal tract (low albumin), chronic pleural effusions and ascites, refractory dysrhythmias, along with ventricular and valvular dysfunction are becoming more prevalent and difficult to treat.
Trend: to intervene at an early age. Open-heart surgery means an operation in which the heart-lung machine is used to support the patient’s circulation while the surgeon opens and makes changes to the heart or the arteries on the surface of the heart. A sternotomy is performed, the child is put on cardiopulmonary bypass, and the heart is stopped (for repair). Potential complications: bleeding, stroke, myocardial infarction, dysrhythmias, fluid and electrolyte imbalance, and death.
Correct answer is “A”
Acquired heart disease—any heart disease that was not present at birth.
Rheumatic fever is an inflammatory autoimmune response to an untreated strep throat infection. As the body tries to fight off the strep infection, it can sometimes produce antibodies that attack both the strep bacteria and healthy cells. This disease can cause chronic progressive damage to the entire heart and its membranes, especially the mitral and aortic valves. In fact, rheumatic fever is the most common cause of severe valve malfunction. Until 1960, it was a leading cause of death in children and a common cause of structural heart disease. The incidence of rheumatic fever has been greatly reduced by widespread use of antibiotics effective against the streptococcal bacterium that causes rheumatic fever. The only definitive test for strep is a throat culture, in which the nurse rubs the lining of the throat with a cotton swab and tests the sample overnight for presence of strep bacteria. Once an infection is confirmed, strep is usually treated with a ten-day course of penicillin.
Carditis is the most dangerous manifestation of rheumatic fever. It is the major cause of morbidity and mortality. May result in diseased valves, especially the mitral valve—The pumping ability of the heart decreases. First manifestation of valve damage will be a new murmur. Cardiac valvular disease is the major long-term consequence of rheumatic fever.
Monitor for congestive heart failure, murmurs, seizures.
For the child with rheumatic fever without cardiac complication: Prophylaxis with penicillin for 5 years or through age 21 to 25 years (whichever is longer). For the child with rheumatic heart disease: Prophylaxis for at least 10 years and until age 40.
When the heart&apos;s valve or valves become diseased, the pumping ability decreases. The valve most frequently affected is the mitral valve. It may become leaky (regurgitant) or stenoic (too tight). Regurgitant valves allow some blood to flow backwards into the heart. Stenotic valves hinder the forward flow of blood. You will hear a murmur in the client. Most common complication with rheumatic fever: mitral stenosis. Either problem can seriously interfere with the heart&apos;s ability to pump blood. If the problem is severe the diseased valve may be replaced to restore normal heart function.
“B” is correct.
Affecting children under the age of 5, diagnostic criteria include at least 5 days of fever, conjunctival eye redness without discharge, red-fissured lips, strawberry tongue, red rash, redness and/or swelling of palms and soles, and lymph nodes in the neck.
Fever, diffuse rash, strawberry tongue, and erythematous palms of KD. Nursing interventions: comfort measures and adequate hydration; encourage fluid intake; keep the child’s skin clean and dry and avoid soap irritation; decrease stimuli and keep the child calm and comfortable.
Strawberry tongue is a swollen, red tongue. The surface looks like a strawberry. Children with Kawasaki disease also tend to have bright red, cracked, sometimes swollen lips.
High doses of aspirin are given while the fever is high. Aspirin therapy is ordered 80 to 100 mg/kg/day until fever drops. Then aspirin is continued at 10 mg/kg/day until platelet count drops. Aspirin is used as an anti-pyretic and anti agglutination drug.
Hypertension through rare in children when compared with adults occurs in 1 – 2 % of school-age children; the incidence is rising at an alarming rate. Hypertension is a major health problem, especially because it often has no symptoms. If left untreated, hypertension can lead to heart attack, stroke, enlarged heart, or kidney damage. It is defined in children as an average systolic and/or diastolic blood pressure that exceeds or is equal to the 95th percentile for age and gender. Cause of hypertension in 80 – 85% of children are due to kidney diseases. Treatment should address the underlying cause. Hypertension in older children and adolescents is strongly associated with overweight or obesity. Weight loss reduction plays an important role in lowering blood pressure. Weight loss requires a program of diet, exercise, and lifestyle changes. Pharmacologic treatment: beta-blockers, diuretics, and ACA inhibitors.
Obesity epidemic: More than half of the children in the US are obese. Prevention of adult cardiovascular disease is becoming a national priority. Children and adolescents need to develop heart-healthy habits during these years to reduce their risk for coronary artery disease and other cardiovascular problems during adulthood. Children older than 2 years: sensible low-fat dietary program. This includes using nonfat or low-fat dairy products, limiting red meat intake, and decreasing the amount of saturated fat. The total daily fat intake should be no more than 30% of total calories with saturated fat being no more than 10% of daily caloric intake. Cholesterol-limiting medications generally are not used in the pediatric population.
Alterations in Cardiovascular
Function in Children
Joy A. Shepard, PhD(c), RN-C, CNE
Joyce Buck, MSN, RN-C, CNE
Describe anatomy and physiology of cardiovascular
system focusing on blood flow and action of heart
Contrast pathophysiology associated with congenital
heart defects with increased pulmonary circulation,
decreased pulmonary circulation, and obstructed
systemic blood flow
Create a nursing care plan for the infant with a
congenital heart defect cared for at home prior to
Learning Outcomes (cont’d)
Plan the nursing care for a child undergoing open
Recognize signs of congestive heart failure
Develop a nursing care plan for child with
congestive heart failure
Differentiate between heart diseases acquired
during childhood and congenital heart defects
Distinguish between hypovolemic shock,
distributive shock, and cardiogenic shock
Function of the Heart
Pumps blood to the
lungs for oxygenation
and removal of carbon
blood to the body
wastes taken away
Anatomy of the Heart
Muscular pump with
Valves, veins, and
arteries connect the
The Heart is Like a House….
Powers the pump
repolarization of the
Sinoatrial node (SA) —>
Bundle of His —>
Left and right bundle
Two pumps: left and
Pressures in the left
much higher than right
pumping action keeps
the body supplied with
Valves are essential to the
heart's pumping function
Keeps blood flowing
forward through the
One-way valve at the
exit of each of the
Physiology of the Heart
CO = HR X SV
Until late school-age and adolescence, cardiac output in the
child is RATE DEPENDENT, not stroke volume dependent,
making heart rate more rapid.
differs from neonatal
circulation in three
Gas Exchange—Placenta; Umbilical
removal of carbon
place in the placenta
oxygenated blood to
inferior vena cava
Oxygenated blood flow (from the
placenta) is from right to left
Oxygenated blood enters right side of
Blood pressure on the right side of the
heart > blood pressure on the left side
of the heart
High resistance in fetal lungs (arterioles
are constricted, alveoli are filled with
Resistance to blood flow in the lungs
(pulmonary vascular resistance) >
Resistance to blood flow in the body
(systemic vascular resistance)
Fetal lungs don’t receive much blood
vena cava to
Most of fetal liver
shunted away from
Fetal Circulation— 2nd
Inferior vena cava→
Left atrium (by way
of the foramen
Most of lungs
Left ventricle→ aorta
(supplying heart and
Deoxygenated blood from
the upper body mixes with
oxygenated blood from
the placenta @ the right
(bypassing the lungs)→
Blood returns to the
placenta (by way of the
two umbilical arteries)
Circulation After Birth—
Umbilical cord clamped→
↓ Blood to right heart
Blood pressure (left side of the heart) >
blood pressure (right side of heart)
Systemic vascular resistance >
pulmonary vascular resistance
Circulation After Birth—
Lungs inflate with
air, allowing gas
Resistance to blood
flow in the lungs
Much more blood
flows into the lungs
First breath: alveoli
expand and blood
vessels in the the
lungs open up
Circulation After Birth—
Circulation After Birth—Shunts
close in response
changes, ↑ Pa02
levels, in the
Normal fetal circulation (lungs and liver bypassed)
Normal circulation after birth (systemic AND
pulmonary circulations; high systemic pressure)
Table 5-10, p. 141
Assessment Guidelines for the Child with a Cardiac
Condition, p. 604
Congenital heart disease in family
Maternal illness, infections, medications taken
Child’s behavior patterns (playfulness, irritability)
Feeding problems (fatigue or
diaphoresis during feeding; poor weight
Respiratory difficulties (tachypnea,
shortness of breath, cyanosis; frequent
Chronic fatigue, exercise intolerance
An infant who has a congenital heart defect comes into
the clinic with complaints of irritability, pallor, and
increased cyanosis that began quickly over the last 30
minutes. As the nurse assesses the infant, the parent asks
why the child’s color is bluish. The best response by the
nurse is, “Skin color is:
A. Related to the time of day.”
B. Related to brain function.”
C. Related to hemoglobin level and oxygen saturation.”
D. Unrelated to your child’s condition.”
Normal areas of
ICS infants; 4th
ICS < 7; 5th
ICS > 7
S3 —normal finding in
S4 —cardiac failure
Murmurs, clicks, friction
40Auscultate apical pulse with stethoscope for a full 60 seconds
Normal Heart Rates for Children of Different Ages
An infant born at 39 weeks gestation is sent
to the intensive care nursery. The nurse
suspects a possible cardiac anomaly when
the admission assessment reveals:
A. Projectile vomiting
B. An irregular respiratory rhythm
C. Hyperreflexia of the extremities
D. Unequal peripheral blood pressures
The pediatric nurse practitioner orders a
complete blood workup for a 5-month-old
infant with a congenital heart defect. Because
of the infant’s heart disease, the nurse would
expect the report to show:
D. A decreased hematocrit level
Evaluates degree of
oxygen saturation in the
blood using a small
infrared light probe that
is placed on:
Finger, toe, earlobe,
bridge of the nose
Norm: 95 – 100%
Cyanosis not visible
until sat < 85%
X-ray picture of
heart, organs in
Size of the heart and
Blood flow to lungs
Thickness of heart walls
Size of cardiac chambers
Motion of valves and septa
(walls) within the heart
Great vessels and various
Defects in structure or
Oxygen levels in cardiac
chambers and great
Cardiac output / stroke
performed by use of
open narrowed valves
extra vessels, “holes” in
Nursing Care (p. 605)
Child must lay still, supine, with affected leg straight
for 4 – 6 hours
Vital signs, insertion site observed, distal pulses
checked q15 minutes X 1st
hour, then q30 minutes
Observe for bleeding at site; pallor, loss of pulses,
coolness in extremity distal to site
Push fluids to help flush the dye out of the body
Observe for reactions to dye (vomiting, rash,
increased creatinine, decreased urinary output)
Strict I & O
A 3-1/2-year-old child returns to the room
after a cardiac catheterization. Post-
procedure nursing care for the child should
A. Encouraging early ambulation
B. Monitoring the insertion site for bleeding
C. Restricting fluids until blood pressure is
D. Comparing blood pressure in affected and
Difficulties, Stunted Growth
Difficulty and fatigue
Frequent emesis, at
risk for NEC
Failure to thrive
Provide periods of uninterrupted rest
Neutral thermal environment
Feed child slowly and more often (q3°
Frequent, small feedings may be less tiring
Concentrating formula 24-27 kcal/oz may
increase caloric intake without increasing
Hold infant in upright
Place infant on right
side after feeding,
HOB↑ 30 - 45º
Limit bottle feedings to 20-30 minutes
If child unable to consume appropriate
amount during 30-minute feeding every
3 hours, consider nasogastric feeding
Monitor for increased tachypnea,
diaphoresis, or feeding intolerance
Infants with cardiac
feedings to provide
for growth and
When caring for a 4-month-old infant
with congestive heart failure, the nurse
A. Force nutritional fluids
B. Provide small, frequent feedings
C. Measure the head circumference daily
D. Position the infant flat on the abdomen
Manifestation: Pallor and
Bluish discoloration of
skin, nail beds, mucous
deprived of adequate
amounts of oxygen (02
sat < 85%)
Hgb must be at least 5
Often appears when
child is feeding
to chronic hypoxia
Fingers or toes
Base of nails
Ends of digits
increase in size
Angle b/n nail and
nailbed ≥ 180º
When attempting to identify the presence of a
congenital heart defect in an infant, the nurse
should understand that:
A. In the absence of cyanosis, poor sucking is
B. Many infants retain mucous that may interfere
C. Feeding problems are fairly common in infants
during the first year
D. Poor sucking and swallowing may be early
indications of heart defects
Pediatric Congestive Heart
Failure (p. 622)
Pump is insufficient to meet the metabolic
demands of the body
Heart becomes overloaded and unable to
deliver adequate cardiac output
Most commonly caused by congenital heart
Acquired conditions: rheumatic heart disease,
endocarditis, myocarditis, cardiomyopathies,
Pediatric Congestive Heart
Infants have a
greater risk of heart
failure than older
the immature heart
is more sensitive to
volume or pressure
overload (Figure 21-8, p. 623)
Pediatric Congestive Heart
Right-sided heart failure:
Periorbital and facial
edema, enlarged liver or
spleen, ascites, wheezing,
neck vein distension
↑ CVP (pooling of venous
shunts (VSD, ASD)
Pediatric Congestive Heart
Most children with CHF have a combination
of left and right
Both: Tachycardia, cardiomegaly, decreased
CO, gallop rhythm (S4), decreased peripheral
perfusion, excessive diaphoresis, weight
Blood diverted to vital organs and away from
GI tract—at risk for necrotizing enterocolitis
Review CP, pp. 626-629
Decreased Cardiac Output r/t decreased
Ineffective Tissue Perfusion r/t increased
Excess Fluid Volume r/t left and right
ventricular overload and ineffective
Review CP, pp. 626-629
Ineffective Breathing Pattern r/t pulmonary
Imbalanced Nutrition: Less than Body
Requirements r/t increased energy
Deficient Knowledge r/t unfamiliarity with
disease process, treatment, interventions,
and home care
Three early signs of CHF in
A 4-month-old who has a congenital heart
defect develops congestive heart failure and
is exhibiting marked dyspnea at rest. This
finding is attributed to:
C. Pulmonary edema
D. Metabolic acidosis
Medications for Pediatric
Aim of medications:
See “Medications Used to Treat Congestive Heart Failure,” p. 624
agent (Increases the
contractility of the
Monitor: Dig level, K+,
Signs of toxicity:
Administering Digoxin, p. 627
Count apical heart rate for one full minute
Call for physician’s advice before giving if HR
< 100 BPM infant/toddler, < 70 children
Give at same time each day, with doses
equally spaced apart (morning, evening)
If the dosage is spit up or vomited, do not
Don’t use teaspoon, eye dropper—draw up in
syringe or calibrated dropper
Always remove the cap before administering
the medication into the child's mouth
Notify physician: nausea, vomiting,
Keep medications out of reach of children
Refill medicine 1 week before it runs out and
ask for new prescription at doctor’s visit
IV / IM / PO
Loop diuretic that blocks sodium
reabsorption in the ascending loop of
Decreases preload by increasing water
Side effects: Electrolyte imbalances (↓ K+,
↓ Mg++); metabolic alkalosis; hypotension
Monitor: Electrolytes, urinary output, BP,
Side effects: Hyperkalemia,
hypovolemia, contraindicated in renal
Monitor: I & O, electrolytes, BP
Captopril (ACE Inhibitors)
Decreases angiotensin II
Dilates blood vessels, making it easier for the
heart to pump blood forward into the body
Reduces afterload (blood pressure)
Less strain on heart muscle and valves,
decreased myocardial oxygen use
Monitor blood pressure carefully; monitor
A toddler is hospitalized with CHF and
is receiving digoxin and Lasix. She has
vomited twice in the past 4 hours. The
nurse’s best action is to:
A. Increase the child’s fluid intake.
B. Omit the next dose of Lasix.
C. Check the child’s blood pressure prior to
the next dose of digoxin.
D. Get an order to draw a digoxin level.
The mother of a 5-month-old infant with
congestive heart failure questions the
necessity of weighing the infant every
morning. The nurse’s response should be
based on the fact that this daily information is
important in determining:
A. Renal failure
B. Fluid retention
C. Nutritional status
D. Medication dosage
Heart Disease in Children
One or more heart structure abnormalities
that develop before birth or persistence of
a fetal structure after birth
Any cardiac condition that was not present
Congenital Heart Disease is
Family history of
Fetal exposure to drugs
such as dilantin and
Maternal viral infections
such as rubella
Trisomy 21 (Down
Acyanotic Heart Lesions—
Systemic pressure >
backwashing to right side
Venous blood does NOT
enter systemic circulation
ALL of the blood returning
to the right side of the
heart passes through the
Ventricular Septal Defect
↑ Pulmonary blood flow
symptomatic at birth
Loud, harsh systolic murmur
Large defects: CHF,
poor feeding, failure to
Children with congenital heart defects at
risk for bacterial endocarditis and damage
to heart valves
Must use antibiotics:
Before and after dental, oral, and invasive
procedures / surgeries
Any unexplained fever or malaise within 2
months of procedure may be a sign of
The father of a child with a congenital heart
defect asks the nurse why his daughter has
to take penicillin before she gets her teeth
cleaned by the dentist. The nurse explains
that this is necessary to prevent:
A. Bacterial endocarditis
B. Congestive heart failure
C. Rheumatic fever
D. Infected gums
VSD—Pulmonary Artery (PA)
Closed heart surgery—
does not require
Band placed around
Used when VSD cannot be
closed (multiple VSD’s, very
small infants with severe
Less frequently done than
“Patch” over large
Open heart surgery
A cardiac catheterization is scheduled for a 5-
year-old with a ventricular septal defect to:
A. Identify the degree of cardiomegaly present
B. Demonstrate the exact location of the defect
C. Confirm the presence of a pansystolic murmur
D. Establish the presence of ventricular
Patent Ductus Arteriosus
(PDA) (p. 608)
Opening between aorta
and pulmonary artery
↑ Pulmonary blood flow
Back to left atrium/left
CHF (left side)
pulmonary artery and aorta
NSAID—inhibits the production of prostaglandins
Promotes closure of PDA; onset of action usually
Closure of PDA in 80% of patients
Monitor renal function
Side effects: bleeding (intraventricular
hemorrhages, GI bleeds)
Atrial Septal Defect (ASD)
Hole or defect in
atrial septum (wall)
Atrial Septal Defect
Large defect: CHF,
disease (often 1st
appearing as an
Atrioventricular Septal Defect (AVSD)
(Endocardial Cushion, AV Canal) (p.
LARGE hole in
center of heart
ASD, VSD, and
combined mitral and
opening or “canal”)
Common with Down
Atrioventricular Septal Defect
Backward flow of
through both ASD and
VSD to right side of
Right-sided heart failure
Atrioventricular Septal Defect
Medical: digoxin, diuretics, and afterload
Treatment: surgical repair early in infancy (3-
4 mos) before the lungs become damaged
and permanent hemodynamic changes occur
“Patches” over both septal defects and
A toddler has been diagnosed with an
acyanotic cardiac defect. Which
assessment data would most likely
indicate congestive heart failure?
A. Heart murmur.
B. Cardiac volume overload.
Obstructive/ Stenotic Lesions
Coarctation of the aorta
Pulmonary Stenosis (p. 614)
Area where blood
exits the heart's
lower right chamber
is too narrow (right
Harsh systolic murmur
Severe or critical:
Aortic Stenosis (p. 621)
Obstruction of blood
flow from heart to
body (obstruction to
blood flow leaving left
↓ Cardiac output
↓ Systemic blood flow
Harsh systolic murmur
Severe or critical:
CHF, left ventricular
Mild: continue to
Severe or critical: aortic
May need aortic valve
Coarctation of the Aorta
(COA) (p. 621)
Restricts flow of blood
out of the left ventricle
Aorta is pinched at
some point along its
length, limiting the
amount of oxygen-rich
blood that can reach the
rest of the body
Coarctation of the Aorta
↓ Cardiac output
↓ Systemic blood flow
↑ Pulmonary congestion
(pooling of blood in left
side of heart)
Enlarged left ventricle;
Decreased pulses and
BP in the lower
Cold feet, legs
Epistaxis (nose bleeds),
BP higher in arms
Symptomatic CHF: digoxin,
infusion to keep ductus
arteriosus patent (improve
perfusion to lower body)
Surgical repair: left
Antibiotic prophylaxis for
An infant with a left-to-right shunt was
admitted to the hospital in congestive heart
failure. She weighed 3.6 kg yesterday. A
finding that indicates a worsening of her
condition today is:
A. Weight 3.66 kg.
B. Urine output of 40 ml in past 8 hours.
C. Crackles in the lower lobes.
D. All of the above.
Cyanotic Heart Lesions
(Decrease Pulmonary Blood Flow, p. 613)
Tetralogy of Fallot
Transposition of the
Great Arteries (TGA)
Cyanotic Heart Lesions—
Pulmonic pressure >
circulation and goes
directly to the left
side of the heart to
be pumped into the
A child diagnosed with tetralogy of Fallot becomes
upset, crying and thrashing around when a blood
specimen is obtained. The child’s color becomes blue
and the respiratory rate increases to 44
breaths/minute. Which of the following actions would
the nurse do first?
A. Obtain an order for sedation for the child.
B. Assess for an irregular heart rate and rhythm.
C. Explain to the child that it will only hurt for a
D. Place the child in a knee-to-chest position.
A newborn with TOF is fed in the semi-Fowler’s
position. After the nurse feeds and burps the infant
and changes the infant’s position, the infant has a
bowel movement and almost immediately becomes
cyanotic, diaphoretic, and limp. These symptoms are
most likely caused by the:
C. Position change
D. Bowel movement
TOF Surgical Repair
Palliative—Relieves symptoms by
increasing the blood flow to the
Corrective—Repairs the underlying
Shunt created from aorta
to pulmonary artery
↑ Blood flow to lungs
“Buys” time for infant to
↑ Success of corrective
surgery when done later
The nurse is aware that the aim of
palliative surgery for children with
tetralogy of Fallot is to directly increase
the blood flow to the:
D. Right ventricle
2 to 4 months
No treatment: Displaced
aorta, enlarged rt.
Antibiotic prophylaxis for
Parents of a toddler with tetralogy of Fallot
explain that they do not want him to overexert
himself; so they always keep him in his
playpen or crib to limit his mobility. Based on
this information, the most appropriate nursing
A. Activity Intolerance
B. Risk for Impaired Parenting
C. Caregiver Role Strain
D. Risk for Delayed Development
Transposition of the Great Arteri
(TGA) (p. 616)
Reversal of aorta and pulmonary artery
Transposition of the Great
Babies born with
TGA can only
survive if they have
one or more
oxygenated blood to
go to the body
Prostaglandin E1 (PGE1)
Causes the ductus
arteriosus to stay
artificially open (patent)
For infants with ductal-
HLHS, severe TOF)
Side effects: Apnea,
flushing, bleeding, third-
Transposition of the Great
hole in the atrial septum
Corrective surgery is
performed in the early
days following birth:
and pulmonary artery
returned to their
Hypoplastic Left Heart Syndrome
(HLHS) (p. 622)
development of the
left side of the heart
Only one functional
Aortic valve, mitral
may also be small or
Without surgery, fatal within the first 2 weeks of
Medications: PGE1 infusion to keep the
Surgery: 3-staged palliative surgery (ages
1 week, 6 months and 2 years of age)
Heart transplant—infrequent due to
scarcity of neonatal donor hearts
Child With HLHS Who
Received Heart Transplant
approach to improve
flow of blood throughout
1 week of age
Provides blood flow
from the right ventricle
directly to the aorta
6 months of age
The Fontan Procedure
2 years of age
Re-routes blood flow
around the defective
areas of the heart by
creating new pathways
for blood circulation to
and from the lungs
4-year survival following
staged repair up to 85%
remains to be seen
ventricular and valvular
Parents of children with congenital heart
problems often experience loss of control
when the child is hospitalized. The nurse who
understands this will:
A. Encourage parents to participate in their child’s
B. Explain procedures prior to performing them.
C. Answer questions honestly.
D. Do all of the above.
Cardiac Surgery (p. 610)
repair to the
structures around the
heart but not the heart
muscle itself; done
Read pp. 610-613
Trend: to intervene
at an early age
Post Cardiac Surgery
Chest tube, NGT,
monitor, pulse oximetry
Encourage cough, IS
Monitor VS, cardiac,
respiratory, I & O,
electrolytes, chest tube
Very painful surgery:
Adequate pain control
A 5-year-old returns from the surgical recovery room
following cardiac surgery. The child has a left chest
tube attached to water-seal drainage, an IV of
D51/2NS at 40 ml/hr, and a nasogastric tube to
gravity. The child is attached to a cardiac monitor and
has a left chest dressing. Upon admission to the unit,
the nurse should first:
A. Take the vital signs
B. Check the identification bracelet
C. Measure the chest and gastric drainage
D. Check the suction pressure of the water seal
Rheumatic Fever (RF) (p.
Children ages 5 – 15
Result of “strep throat” that
has not been treated with
Manifests 1 – 3 weeks
Affects connective tissue of
heart, joints, subcutaneous
tissues, blood vessels
Most serious: Cardiac
valvular disease (esp.
scarring of mitral valve)
Prevention: refer all children
with sore throats for throat
Carditis—inflammation of all
parts of the heart (mitral, aortic
movements of legs, arm, and
Erythema marginatum—red skin
nontender lumps located over
RF—Diagnostics / Treatment
O titer (+ ASO)
↑ SED (erythrocyte
Monitor for cardiac
Streptococcal Prophylaxis for the
Child with Rheumatic Fever
Damaged valves can become further
damaged with repeated infections
Streptococcal prophylaxis is lifelong if
there is actual valve involvement
Intramuscular penicillin, administered
monthly, is the drug of choice
Alternatives include oral penicillin twice
daily or oral sulfadiazine once a day
When examining the laboratory work
of a child with the diagnosis of
rheumatic fever, the nurse would
expect the findings to demonstrate:
A. A negative C-reactive protein
B. A positive antistreptolysin titer
C. An elevated reticulocyte count
D. A decreased erythrocyte
Kawasaki Disease (KD)
Children < 4 years (peak incidence 18 – 24 months)
Mucocutaneous lymph node syndrome
Inflammatory autoimmune disease—principle area of
involvement is the heart
If caught and treated early the prognosis is usually
If left untreated or caught too late, it can be fatal or
leave the child with irreversible heart and/or organ
Kawasaki Disease (KD)
May damage the coronary arteries (vasculitis)
and heart muscle
Most common cause of acquired heart
disease in U.S. children
Can lead to coronary aneurysms and
myocardial infarctions later in life
Cause: ??? noncontagious infection;
associated with rug shampooing, dust mites,
or proximity to stagnant water
Seasonal: ↑ Late winter, early spring
KD—Signs and Symptoms
Affecting children under
the age of 5, diagnostic
criteria include at least:
5 days of fever (> 39 ° C,
102.2° F), unresponsive to
Conjunctival eye redness
swelling of palms and
soles, and lymph nodes
in the neck
KD—Phases and Treatment
Acute phase: 1 to 2 weeks, with high fever >
Nurse needs to closely monitor cardiac
status, VS, observe for CHF
Echocardiogram, ECG, ↑ SED rate
Early treatment can dramatically reduce the
likelihood of cardiovascular damage
High-dose IV Gamma-globulin
High-dose PO ASA therapy
PO, administer with milk or food
Antiplatelet agent (inhibit platelet function by
making them less sticky)
High dose therapy (KD): 80-100 mg/kg/day
for the first two weeks to reduce fever,
swelling and inflammation; and to decrease
the chance of blood clots
Don’t give to children <16 y with chickenpox
or flu-like symptoms (Reye syndrome)
Side effects: Bleeding
A toddler with Kawasaki’s disease is ordered to
receive aspirin therapy. Typical administration of
aspirin for Kawasaki’s disease would include which of
the following principles?
A. High doses of aspirin should be given while fever is
B. Aspirin therapy is given to reduce fever.
C. Aspirin dose increases after fever is gone.
D. Aspirin dosage is unrelated to platelet count.
Gamma Globulin (IVIG)
One time dose 2 g/kg
Administered through an intravenous
(I.V.) line for 10 hours
If given first 10 days, will dramatically
reduce the risk of damage to the
Side effects: Hypotension; facial
flushing; tightness in the chest
Monitor BP closely
Benadryl and acetaminophen: control
Epinephrine: anaphylactic reactions
Vaccines must be delayed for 5 months
months after treatment