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1. CONGENITAL HEART DEFECTS CONGENITAL HEART DISEASE By Sujamol.K.Thomas, RN

2. A congenital heart defect (CHD) is a defect in the structure of the heart and great vessels of a newborn. Most heart defects either obstruct blood flow in the heart or vessels near it or cause blood to flow through the heart in an abnormal pattern. Heart defects are among the most common birth defects and are the leading cause of birth defect-related deaths.

3. A baby's heart begins to develop shortly after conception. During development, structural defects can occur. These defects can involve the walls of the heart, the valves of the heart and the arteries and veins near the heart. Congenital heart defects can disrupt the normal flow of blood through the heart. The blood flow can Slow down Go in the wrong direction or to the wrong place Be blocked completely

4. ETIOLOGY AND RISK FACTORS

5. Maternal factors and CHD It is important to remember that most cases of congenital heart disease have no known cause. However, rarely some types of congenital heart defects are known to occur more often when the mother comes in contact with certain substances during the first few weeks of pregnancy, while the baby's heart is developing. Some maternal illnesses and medications taken for these illnesses have been shown to affect the heart's development. Other illnesses or medications seem to have no impact on the baby's heart. Always consult your physician or obstetrician for more information.

6. Maternal factors and CHD Women who have seizure disorders and need to take anti-seizure medications may have a higher risk for having a child with congenital heart disease, as do women who take lithium to treat depression. Mothers who have phenylketonuria (PKU) who do not adhere to the special diet necessary to manage the disease during pregnancy have a higher risk of having a child with congenital heart disease. Also, women with insulin-dependent diabetes (particularly if the diabetes is not well-controlled) or lupus may have a higher risk of having a child with heart defects. Counseling is important for women with these chronic illnesses before becoming pregnant.

7. Family history and CHD In the general population, about 1 percent of all children are born with congenital heart disease. However, the risk increases when either parent has congenital heart disease (CHD), or when another sibling was born with CHD. Consider the following statistics:

8. Family history and CHD If you have had one child with congenital heart disease, the chance that another child will be born with CHD ranges from 1.5 to 5 percent, depending on the type of CHD in the first child. If you have had two children with CHD, then the risk increases to 5 to 10 percent, to have another child with CHD. If the mother has CHD, the risk for a child to be born with CHD ranges from 2.5 to 18 percent, with an average risk of 6.7 percent. If the father has CHD, the risk for a child to be born with CHD ranges from 1.5 to 3 percent. Congenital heart defects involving obstructions to blood flow in the left side of the heart have a higher rate of recurrence than other heart defects. If another child is born with CHD, it can be a different type of defect than seen in the first child.

9. Chromosome abnormalities and CHD Problems with chromosomes that result in genetic syndromes, such as Down syndrome, often result in a higher incidence of infant heart malformations. Five to 8 percent of all babies with CHD have a chromosome abnormality. Chromosomes are the structures in your cells that contain your genes; genes code for your traits such as eye color and blood type. Usually there are 46 chromosomes in each cell of the body. Having too many or too few chromosomes results in health problems and birth defects. Structural defects of the chromosomes, where a piece of the chromosome is missing or present in duplicate, also causes health problems.

10. Chromosome abnormalities and CHD There are a number of chromosome abnormalities associated with congenital heart defects. Some of these include the following: Down syndrome trisomy 18 and trisomy 13 Turner's syndrome Cri du chat syndrome Wolf-Hirshhorn syndrome Velo-cardio-facial syndrome and/or DiGeorge sequence Williams syndrome

11. Single gene defects There are an estimated 70,000 genes contained on the 46 chromosomes in each cell of the body. Genes come in pairs, one, which is inherited from the mother, the other from the father. Genes not only compose our individual traits, but also can cause health problems when gene alterations (mutations) are present. When a gene is mutated, a number of health problems may occur in a person, due to the single underlying genetic mutation. Several health problems with one genetic cause are often referred to as a syndrome

12. ASSESSMENT GUIDELINES FOR THE CHILD WITH A CARDIAC CONDITION

13. Respiration Inspect the rate,depth,andrespiratiry effort. Is a cough present? Identify the signs of increased respiratory effort Auscultate breath sounds for adventitious sounds.

14. Pulses Assess the pulse rate,rhythm,and quality. Compare the apical,brachial,and radial pulse rates. Compare the brachial and femoral pulses for strength.

15. Blood pressure Compare the blood pressure to expected value for age,sex,and height percentiles. Compare blood pressure values between upper and lower extremities.

16. Colour Observe overall colour Contrast colour in peripheral and central locations. Note whether crying improves or worsens colour.

17. Heart Inspect the anterior chest for bulging or heaving Palpate the chest wall for pulsations,heaves,or vibrations. Locate the point of maximum intensity. Auscultate the heart for the heart sounds and their quality. Are extra heart sounds or murmurs present? Auscultate the heart with the child in sitting and reclining positions to detect defferences in heart sounds.

18. Fluid status Observe for signs of periorbital,facial,or peripheral edema. Observe for abdominal distension. Palpate the liver to detect hepatomagaly. Observe for signs of dehydration with acute illness.

19. Activity and behaviour Is exercise intolerence present? Does the child tire with feeding? Identify changes in activity level or behaviour.

20. General Asses growth Note presence of diaphoresis and when it occurs.

21. DIAGNOSTIC TESTS USED TO EVALUATE CARDIAC CONDITIONS

22. CARDIAC CATHETERIZATION An invasive procedure that passes a radiopaque catheter through a large vein or artery in an arm or leg to the heart. The catheter is threded to the heart chambers or coronary arteries,orboth,guided by fluroscopy,which enables precise measurement of O2 saturation within the heart’s chambers and great arteries and pressure gradients in the pulmonary vessels or heart chambers.this helps identify: Congenital heart defects Cardiac valvular diseases Coronary artery diseases

23. NURSING IMPLICATIONS No food or fluid 6 to 8 hours prior to the test. Obtain history of allergies. Oral anticoagulant therapy is discontinued. An iv line may be started for administering sedation or emergency drugs,as needed. Vital signs and heart rhythm are monitored during the procedure. Once the catheter and guidewires are removed,apply direct pressure on the cath site for 15 mins and then apply a pressure dressing for 6 hours. Monitor the site for bleeding and assess the distal extremity for pulse,capillaryrefill,andtemparature. Maintain bed rest for 6 hours after the procedure and then limit activities for 24 hours. Monitor intake and output as the contrast dye causes diuresis.

24. CHEST RADIOGRAPH STUDY As the common form of imaging, radiographs use irradiation to obtain images and capture them on film for diagnostic and screening puposes. They reveal the size and contour of the heart and characteristics of pulmonary vascular markings.

25. NURSING IMPLICATIONS Explain the procedure to parents and child.Inform them that several images may be taken from different angles. Explain that modern equipment decreases radiation exposure. Have the child practice holding still and holding a breath in preparation for the test.

26. ECHOCARDIOGRAM PURPOSE This is a noninvasive ultrasound study of the heart.An ultrasound probe is held over the chest to produce an ultrasound beam to the tissues.The reflected sound waves or tissues are then transformed into scans,graphs or sounds. It identifies the heart size,structure,pattern of movement,hemodynamics,bloodflow,andbloodflow disturbances.

27. NURSING IMPLICATIONS Explain the procedure to the parents and child.Inform the child of the need to hold still for the procedure. Inform the child that a gel will be applied to the skin and a trasducer will move over the area,but that the test causes no pain.

28. ELECTROCARDIOGRAM This records the electrical impuses of the heart via electrodes and a galvanometer.Electrodes with electropads or pads srtapped to the four extremities,and chest electrodes are applied. Purpose of this procedure include Detection of cardiac arrhythmias Identification of electrolyte imbalances. Monitoring ECG changes during the stress test

29. NURSING IMPLICATIONS Obtain a list of current medications,and when last taken. When applying the chest and extremity leads,explain to the child that the procedure is not painful. Teach methods to relieve anxiety and remain relaxed.

30. EXERCISE TESTING A test performed with a treadmill or a stationary exercise bicycle will evaluate exercicse tolerance. ECG leads,a blood pressure cuff,and sometimes an O2 consumption monitor are attatched and the adolescent begins the exercise. This enables ECG recording with controlled increase in activity to identify significant cardiac compensation or inadequate cardiac output.

31. NURSING IMPLICATIONS Inform the adolescent about the test,what to expect,and that the test can be stopped at any time. Insruct the adolescent to report vertigo,extreme shortness of breath,chestpain,and excessive fatigue. Ensure that the adolescent understands that the test is greater value when the exercise continues to the predetermined stopping level. Take baseline vital sign measurements prior to the exercise.

32. HOLTER MONITOR(AMBULATORY ELECTROCARDIOGRAPHY) PURPOSE ECG leads are attached and a portable recorder is used to enable continuous 24-48 hour recording of the ECG on magnetic tape. It used to detect rhythm disturbances,changes in heart rate with activity or during sleep,as well as responses to antiarrhythmic medications.

33. NURSING IMPLICATIONS The child may not swim or bath in a tube or shower until the electrodes are removed. The child can engage in other ususal activities. A diary of any events or emotional stress that cause symptoms should be kept.A daily schedule of sleep,eating,exercise,and other activities may be requested.

34. HYPEROXITEST PURPOSE Arterial blood is collected before and at least 10 minuter after giving the child 100% O2. It measures differences in arterial blood gas level when an infant has central cyanosis to help distinguish between cardiac disease, pulmonary disease,or central nervous system depression.

35. NURSING IMPLICATIONS Follow guidelines for arterial blood collection from the upper right side of the body. Administer O2 through a plastic hood for at least 10 minutes to replace all alveolar air with O2.

36. MAGNETIC RESONANCE IMAGING PURPOSE MRI produces results similar to those of a CT scan,but it does not use ionizing radiation. provides images of the heart’s myocardium, structure,valvefunction,bloodvessels,and other soft tissues.

37. NURSING IMPLICATIONS Prepare the child for sounds,size of the equipment,and tunnel. Ensure that the child has no metallic implants, and is not connected to metal equipment. Use sedation,ifneeded,to keep the infant or child still.

38. LABORATORY TESTS USED TO EVALUATE CARDIAC CONDITIONS

39. Arterial blood gases Complete blood count Serum digoxin level Anti-streptolysin-O antibody titer Erythrocyte sedimentation rate C-reactive protein Serum lipid panel

40. TYPES OF CHD CYANOTIC NON-CYANOTIC

41. NON-CYANOTIC Ventricular septal defect(VSD) Atrialseptal defect (ASD) Patent ductusarteriosus (PDA) Aortic stenosis Pulmonicstenosis Coarctation of the aorta Atrioventricular canal (endocardial cushion defect)

42. CYANOTIC Tetralogy of fallot Transposition of the great vessels Tricuspid atresia Total anomalous pulmonary venous return Truncusarteriosus Hypoplastic left heart Hypoplastic right heart Ebstein'sanomaly

44. ATRIAL SEPTAL DEFECT In a person with an atrialseptal defect, there's an opening in the wall between the right atrium and the left atrium (this wall is called the atrial septum). As a result, some oxygenated blood from the left atrium flows through the hole in the septum into the right atrium, where it mixes with oxygen-poor blood and increases the total amount of blood that flows toward the lungs. ASD is a type of congenital heart disease, which means that a person is born with it. About 8 out of 1,000 babies born have heart disease, and 6% to 8% of those babies have an ASD

45. PATHOPHYSIOLOGY This opening in the atrial septum permits left to right shunting of blood.Three types of ASDs occur Ostiumprimum Ostiumsecondrum Sinus venosus The opening may be small,as when the foramen ovale fails to close,orlarge,as when the septum may be completely absent.

48. What Causes an AtrialSeptal Defect ASDs occur during a baby's development in the mother's womb and are present at birth. Before birth, the heart develops from a large tube, dividing into sections that will eventually become its walls and chambers. If a problem occurs during this process, a hole in the wall that divides the left atrium from the right may result. For most people with an ASD, no one knows exactly why it happens. In some cases, the tendency to develop an ASD may be inherited, or genetic.

49. CLINICAL MANIFESTATIONS Infants and young children usually have no symptoms.Small and moderate size ASDs may not be diagnosed until preschool years or later. Fatigue and easy tiring and poor growth occur with a large ASD. abnormal heartbeat stroke (this is very rare)

50. Diagnostic procedures A chest radiograph and ECG reveal little information unless the ASD is large,has excessive shunting,and right ventricular hypertrophy is present. An echocardiogram (echo)identifies a dilated right ventricle due to blood overload and the shunt size.

51. CLINICAL THERAPY Spontaneous closure of some ASDs occurs within the first 4 years of life.No activity limitations are needed. Surgery to close or patch the ASD is prformed when significant increased pulmonary blood flow causes CHF,or when spontaneous closure has not occurred by 4 years of age. Secundrum ASDs may be closed by a transcatheter device(septaloccluder)during cardiac cath.

52. PROGNOSIS Many person with uncorrected small-and moderated sized ASDs have lived to middle age without symptoms, But CHF and pulmonary HTN may develop in untreated adults. Atrial arrhythmias may also occur in adults.

53. For 6 months following surgery or catheterization closure, ASD patients will need to take antibiotics before a visit to the dentist or if they need certain other kinds of surgery, such as tonsil removal. This is to prevent bacteria from getting into the bloodstream and causing bacterial endocarditis

54. Ventricular Septal Defect

55. In a person with a ventricular septal defect (VSD), there is an opening in the wall (septum) between the right ventricle and the left ventricle. You might hear this type of problem also referred to as a "hole in the heart.“ As a result, when the heart beats, some of the blood in the left ventricle (which has received oxygen from the lungs already) is able to flow through the hole in the septum into the right ventricle. In the right ventricle, this oxygen-rich blood mixes with the oxygen-poor blood and is directed via the pulmonary artery back to the lungs. This is the most common congenital heart defect,accounting for 40% of all defects.

56. PATHOPHYSIOLOGY An opening in the ventricular septum causes increased pulmonary blood flow. blood is shunted from the left ventricle directly across the open septum into the pulmonary artery.

58. What Causes a VSD? Ventricular septal defects occur during fetal heart development and are present at birth. During the first weeks after conception, the heart develops from a large tube, dividing into sections that will eventually become the walls and chambers. If a problem occurs during this process, a hole in the ventricular septum may result. In some cases, the tendency to develop a VSD may have a genetic basis. There can be genetic syndromes that cause extra or missing pieces of chromosomes that can be associated with VSD. For the vast majority of children with a defect, however, there's no evident reason as to why a VSD develops.

59. CLINICAL MANIFESTATIONS The very small number of teens with moderate and large VSDs that haven't been treated in childhood may notice some symptoms, however These include shortness of breath, a feeling of tiredness or weakness (especially during exercise), poor appetite, and trouble gaining weight,pulmonaryinfections,pulmonary HTN. A systolic murmur is auscultated at the third or fourth left intercostal space at the sternal boarder.

60. Diagnostic procedures A chest radiograph and ECG reveal little when VSDs are small. An enlarged heart and pulmonary vascular markings on chest radiograph may be seen when a large VSD causes shunting. Right and left ventricular hypertrophy may be seen on ECG. echocardiogram establishes the diagnosis if shunting is present. Cardiac catheterization is used only in preparation for surgery.Findings reveal incresed oxygen in the right ventricle and increased systolic pressure in the right ventricle and pulmonary artery.

61. CLINICAL THERAPY Most VSDs close spontaneously witin the first 6 months of life.treatment is conservative when no signs of CHF or PA HTN are present. Surgical patching of VSD during infancy is performed when poor growth is evident. Closure of VSD by transcatheter device during cardiac cath may be attempted for some defects. Prophylaxis for infective endocarditis is required

63. Children respond well to surgery and experience substantial catch-up growth.Tachyarrhythmias and Right Bundle Branch Block are possible complications.

64. ATRIOVENRICULAR CANAL(ENDOCARDIAL CUSHION DEFECT)

67. CLINICAL MANIFESTATIONS Severity of symptoms depend on the amount of mitral regurgitation and the left –to-right shunting of blood across the septum. Infants have CHF,tachycardia,poorgrowth,recurrent respiratory infections,and repeated respiratory failure. A holosystolic murmur is loudest at the left lower sternalboarder,and the intensity reflects the amount of mitral regurgitation

68. DIAGNOSTIC PROCEDURES On chest radiograph,cardiomegaly and pulmonary vascular markings are present. OnECG,atrialenlargement,right ventricular hypertrphy,and an incomplete RBBB are noted. Echocardiogram reveals dilation of the ventricles,septaldefects,and details of valve malformation. Cardiac cath reveals increased oxygen in the right atrium,andincresed right ventricle and PA pressure.

69. CLINICAL THERAPY Surgery is performed during infancy to prevent pulmonary vascular disease. Palliative PA banding may be used to reduce blood flow to the lungs and CHF so the infant can grow before corrective surgery. Patches are placed over septaldefects,and valve tissue is used to form functioning valves. The mitral vave may be replaced. Prophylaxis for infective endocarditis is required.

70. PROGNOSIS Information on long-term survival following successful surgery is lacking. Arrhythmias and mitral valve insufficiency occur postoperatively. There is no difference in short-term survival rates between infants with and without Down syndrome.

71. PATENT DUCTUS ARTERIOSUS(PDA)

72. PATHOPHYSIOLOGY Common congenital heart defect caused by persistent fetal circulaion that accounts for 10% of all congenital heart defects. When pulmonary circulation is established and systemic vascular resistance increases at birth,pressure in the aorta become greater than in the pulmonary arteries. Blood is then shunted from the aorta to the pulmonary arteries,increasing circulation to the pulmonary system. It is a common problem of preterm infants,and is present nearly all preterm infants less than 27 weeks gestation.

74. Clinical manifestations Dyspnea,tachypnea,tachycardia;full,bountingpulses,widened pulse pressure,hypotension may be noted when cardiac output is low. CHF,intercostalretractions,hepatomegaly and growth failure when a large PDA exists. A countinuous machinery murmur during systole and diastole,and a thrill in the pulmonic area. High risk for frequent respiratory infections,pneumonia,and infective endocarditis.

75. DIAGNOSTIC PROCEDURES The chest radiograph and ECG shows left ventricular hypertrophy. The PDA can be visualized,and left to right shunt can be measured on echocardiogram.

76. CLINICAL THERAPY Surgical ligation is the treatment of choice. Intravenous Indomethacin often stimulates closure of the ductusarteriosus in premature infants. Transcatheter closure by obstructive device is sometimes attempted in children over 18 months of age. prophylaxis for infective endocarditis is required until the PDA is closed.

77. PROGNOSIS No long-term sequelae occur if treated before pulmonary vascular disease develops. If PDA is not treated,the child’s life span is shortened because PAHTN and pulmonary vascular obsructive disease develop.

78. PULMONICSTENOSIS

79. PATHOPHYSIOLOGY Stenosis can be above valve,belowvalve,or at the valve. Stenosis obstructs bloodflow into the pulmonary artery,which increases preload and results in right ventricular hypertrophy. This is the second most common congenital heart defect,accounting for 8-12%of all cases. Stenosis may progress in the subvalvular area as the heart muscle grows and develops.

80. CLINICAL MANIFESTATIONS Children with mild stenosis may have no symptoms and grow normally. In moderate stenosisdyspnea and fatigue occur on exertion. Signs of CHF and hepatosplenomegaly are rare but may result from chronic pressure overload. Heart failure and chest pain on exertion occur in severe cases. A loud systolic ejection murmur with a widely split S2 and thrill may be found in the pulmonic listening area.

81. DIAGNOSTIC PROCEDURES The chest radiograph may show an enlarged pulmonary artery with normal heart size and normal pulmanaryvascularity. The ECG may show right atrial enlargement and right ventricular hypertrophy. An echocardiogram provides information about the pressure gradient across the valve and size of valve ring. Cardiac cath findings include increased right ventricular pressure and a normal or slightly lowered pulmonary artery pressure.

82. CLINICAL THERAPY Dilation by balloon valvuloplasty performed during cardiac cath,treats simple pulmonicstenosis. Surgical valvotomy may be used when other defects such as VSD are present. Surgical resection may be needed for narrowing above the valve area.

83. PROGNOSIS Pulmonicstenisis does not typically increase in severity. Lifelong infective endocarditis prophylaxis is necessary.

84. AORTIC STENOSIS

85. PATHOPHYSIOLOGY Narrowing of the aortic valve obstructs bloodflow to the systemic circulation.the valve is often bicuspid rather than tricuspid. The pressure gradient across the valve usually increases as the child grows and cardiac output increases. Aortic stenosis accounts for 3-6% of all cases of congenital heart defects.

87. CLINICAL MANIFESTATIONS Most infants and children are asymptomatic with normal growth and development.life threatening aortic stenosis is detected in some newborns. CHF develops in infants with significant stenosis. The blood pressure is normal,but a narrow pulse pressure is noted.peripheral pulses may be weak. The child may complain chest pain after exercise,but exercise intolerance is uncommon.syncope and dizziness are serious signs that require intervention. A systolisc heart murmur and thrill occur in the aortic or pulmonic areas with transmission to the neck.

88. DIAGNOSTIC PROCEDURES The chest radiograph is usually normal,but may reveal a slight prominence of the left ventricle and aorta with increased severity. The ECG is usually normal in mild cases,but may show mild left ventricular hypertrophy and inverted T waves with increased severity. An echocardiogram reveals the number of the valve cusps,pressure gradient across the valve,and size of aorta. Stress testing may be used in asymptomatic children to determine the amount of obstruction present with exercise.

89. CLINICAL THERAPY Newborns with lifethrearening aortic stenosis need PGE1 to maintain a Patent DuctusArteriosus until the aortic valve can be dilated. The aortic valve may be successfully dilated by balloon valvuloplasty during cardiac cath.Surgicalvalvuloplasty may be performed. Aortic valve replacement is performed when stenosis is severe or if significant regurgitation results from other interventions.

90. PROGNOSIS Chestpain,syncope,and sudden death can occur in symptomatic children,particularly during vigorous exercise. Stenosis is usually progressive during childhood as the valve calcifies. Valve replacement may be necessary once the child reaches adulthood,requiring lifelong anticoagulant therapy.Lifelong infective endocarditis prophylaxis is required.

91. COARCTATION OF THE AORTA

92. PATHOPHYSIOLOGY Narrowing or constriction in the descending aorta,often near the ductusarteriosus or left subclavianartery,obstructs the systemic blood flow. This defect is common,occuring in 5%of all children with congenital heart disease.Up to 30%of girls with Turner Syndrome have COA.

94. CLINICAL MANIFESTATIONS Many children are asymptomatic and grow normaly,but constriction is progressive.up to 30% of infants develop CHF by 3 months of age. Bloodpressure in legs is lower than in the arms.Brachial and radial pulses are typically bounting,but femoral pulses are weak or absent. Oldren children may complain of weaknes and pain in the legs after exercise. A systolic ejection murmur may be heard at the upper right and middle or lower left sternalborder.A thrill may be palpated in the suprasternal notch.

95. DIAGNOSTIC PROCEDURES The chest radiograph may reveal cardiomagaly,pulmonary venous congestion,and indentation of the descending aorta. MRI shows the site of coarctation. ECG shows lt ventricular hypertrophy;rt ventricular hypertrophy may be seen in severe cases. Echocardiogrm shows the size of the aorta,the actual coarctation,and the function of the aortic valve and left ventricle.

96. CLINICAL THERAPY Ballon dilation occurs during cardiac cath for initial relief and re-coarctation. Balloon dilation on infants under 3 months of age may be performed through the umblical artery to avoid injury to the femoral artery. Surgical resection with end-to-end anastomosis or with patching using the subclavian artery may be performed. Repair in the first year of life is preferred to decrease exposure to hypertension.

97. PROGNOSIS If coarctationrecurs,balloonvulvuloplasty is usually performed. Persistent hypertension in adulthood is common. Infective endocarditis prophylaxis is needed.

98. NURSING MANAGEMENT AT THE TIME OF SURGERY The goals of nursing management are to perform assessments,provide supportive care to the family,and meet the child’s nursing care needs before and after the surgery.

99. NURSING ASSESSMENT AND DIAGNOSIS Ineffective breathing pattern related to respiratory muscle fatigue. Acute pain related to surgical incision and expansion of chest with coughing and deep breathing exercises. Risk for imbalanced fluid volume related to impact of surgery on hearts pumping action. Risk for infection related to surgery and chronic disease status.

100. PLANNING AND IMPLEMENTATION Pain management Promote respiratory function Manage fluids and nutrition Activity

101. EVALUATION The child’s pain is effectively managed. Full lung expansion is maintained with incentive spirometry exercises or chest physiotherapy. The childs incision heals without infection. Catch-up growth occurs over the next few months to years.

102. Prevention Avoid alcohol and other drugs during pregnancy. A blood test should be done early in the pregnancy to see if the woman is immune to rubella. If the mother is not immune, she must avoid any possible exposure to rubella and should be immunized immediately following delivery. Poorly controlled blood sugar levels in women who have diabetes during pregnancy are also associated with a high rate of congenital heart defects during pregnancy. Experts believe that some prescription and over-the-counter medications and street drugs used during pregnancy increase the risk of heart defects. There may be some hereditary factors that play a role in congenital heart disease. It is rare but not impossible for more than one child in a family to have a congenital heart defect.

103. TETRALOGY OF FALLOT(TOF)

104. PATHOPHYSIOLOGY Four defects-pulmonicstenosis,RVhypertrophy,VSD,and overriding of aorta-make up the condition Some children have a fifth defect,an opening foramen ovale or atrialseptal defect. About 10%of children with congenital heart defects have TOF Elevated pressures in the rt side of the heart cause a right-to-left shunt.

106. CLINICAL MANIFESTATIONS The infant becomes hypoxic and cyanotic as the ductusarteriosus closes. The degree of pulmonary stenosis determines the severity of symptoms. A systolic murmer is heard in the pulmonic area and transmitted to the suprasternal notch. A thrill may be palpated in the pulmonic area. Polycythemia,hypoxicepisodes,metabolicacidosis,porgrowth,clubbing,and exercise intolerence may develop. Toddlers with uncorrected defects instinctively squat to decrease the return of systemic venous blood to the heart.

108. DIAGNOSTIC PROCEDURES A chest radiograph shows the boot-shaped heart due to the large RT ventricle,decreased pulmonary vascular markings,and a prominent aorta. The ECG shows RT ventricular hypertrophy. The Echocaediogram shows the VSD,obstruction of pulmonary outflow,an overriding aorta,and the size of pulmonary arteries. Cardiac cath provides details about the anatomic defects. Blood tests reveal an elevated hematocrit and hemoglobin and an increased clotting time.

109. CLINICAL THERAPY Management of hypercyanotic episodes includes placing the infant in knee-chest position,calming the child,giving O2and administering Morphine and propanolol intravenously. Monitoring the child for metabolic acidosis or prolonged unconsciousness is critical. A total repair is often performed before 6 months of age when the infant has a hypercyanotic episode. Palliative shunt procedure(e.g,Blalock-Taussing)may be performed to allow the child to grow and improve outcomes with corrective surgery.

110. PROGNOSIS Not all children are cured by surgery,but most have improved quality of life and improved longevity. Arrhythmias may be residual prolems. Lifelong infective endocarditis prophylaxis is required.

111. PULMONARY OR TRICUSPID ATRESIA

112. PATHOPHYSIOLOGY Pulmonary atresia is the absence of communication between the right ventricle and the pulmonary artery,either at the site of the pulmonary valve or in the main pulmonary artery. It occurs in fewer than 1% of children with congenital heart defects. Bloos flows to the left side of the heart through the foramen ovale. The PDA provides the only flow of blood to the pulmonary arteries. A VSD or TGA is also often present.

114. CLINICAL MANIFESTATIONS Cyanosis is present at birth. Tachypnea,CHF,pulmonaryedema,hepatomegaly, acidosis,hypoxicepisodes,clubbing,polycythemia,and growth delays occur. A continuous murmur fromPDA is heard in the pulmonicarea,and a harsh systolic murmur may be heard in the tricuspid area.

115. DIAGNOSTIC PROCEDURES A chest radiograph may reveal a normal size or slightly enlarged sized heart. The ECG may reveal right atrial hypertrophy. The chocardiogram shows a small hypoplasticrt ventricular cavity and tricuspid valve,an absent rt ventricular outflow tract,a dilated rtatrium,andrt-to-lt shunting across the atrial septum.

116. CLINICAL THERAPY Prostaglandin E1 is given immediately to maintain a PDA.Digoxinans diuretics are also used. The Rastelli balloon atrialseptotomy is performed to increase the atrial opening. A rastelli or modified Fontan procedure results in improved survival.

117. PROGNOSIS Outcome depends upon the size of the pulmonary outflow tract developed by surgery and the fibrosis in the right ventricle. The child has increased risk for arrythmia and rt ventricular dysfunction.

118. TRANSPOSITION OF THE GREAT ARTERIES(TGA)

119. PATHOPHYSIOLOGY The pulmonary artery is the outflow tract for the left ventricle,and the aorta is the outflow tract for the right ventricle,creating parallel circulations. The condition is life threatening at birth,and survival initially depends on an open ductusarteriosus and foramen ovale. This condition occurs in about 5% of children with congenital heart disease. An ASD or VSD may also be present with TGA.

122. CLINICAL MANIFESTATIONS Cyanosis,apparent soon after birth,progresses to hypoxia and acidosis.Cyanosis does not improve with O2 administration.Cyanosis may be less apparent when a large VSD is present. CHF may develop immediately or over days or weeks.Tachypnea is often present without retractions or other signs of dyspea. A systolic murmur is present if a VSD is present. Infants take a long time to feed and need frequent rest periods because of rapid respiratory rate and fatigue. Growth failure may be evident as early as 2 weeks of age if corrective surgery is not performed.

123. Diagnostic procedures A chest radiograph may reveal a classic egg shaped heart on a string with enlarged ventricles and increased pulmonary vascular markings. The ECG reveals right ventricular hypertrophy. The echocardiogram often shows the abnormal position of the great arteries rising from the ventricles. A hyperoxitest confirms a cyanotic congenital heart defect. Cardiac cath shows incresed RV pressure,and the catheter can enter the aorta through the right ventricle. Blood tests reveal an incresedhematocrit and hemoglobin or polycythemia.

124. CLINICAL THERAPY prostaglandinE1 is ordered to maintain a patent ductusarteriosus until a palliative procedure can be performed.O2 is administered for severe hypoxemia. Balloon atrialseptostomy may be performed during cardiac cath in new borns as a first stage.this may be corrected surgically.other defects may be repaired in stages as the infant grows. Corrective surgery(arterial switch)is usually performed before 1 week of age.

125. PROGNOSIS Survival without surgery is impossible. The 5 year survival following an arterial switch is greater than 80%. Arrhythmias,decreased RV function,pulmonary vascular disease,and sudden death are long term complications after Mustard and senningprocedures,so follow-up every 6-12 months is needed. Other complications of surgical repair include pulmonary artery or aortic stenosis,coronary artery obstruction,and mitral regurgitation. Infective endocarditis prophylaxis may be necessary.

126. TRUNCUS ARTERIOSUS

127. PATHOPHYSIOLOGY A single large vessel empties both ventricle and provides circulation for the pulmonary,systemic,and coronary circulations. A VSD is usually present. This occurs in less than 1% of congenital heart defects.

129. CLINICAL MANIFESTATIONS Cyanosis develops son after birth;however,this is also a condition of incresed pulmonary blood flow. Severe CHF,dyspnea,retractions,fatigue,poorfeeding,poorgrowth,polycythemia,clubbing,increased pulse pressure,bounting peripheral pulses,a widened pulse pressure,frequent respiratory infections,andcardiomegaly occur. The VSD produces a harsh systolic murmur in the lower sternalborder.a systolic click may be heard in the apex and pulmonic area.

130. DIAGNOSTIC PROCEDURES The chest radiograph shows cardiomegaly ,A large aorta,and increased pulmonary vascular markings. The ECG reveals rt and lt ventricular hypertrophy. The echocardiogram shows the absence of two semilunar valves. Cardiac cath documents a lt-to-rt shunt at the level of the ventricle,equal pressure in the ventricles,thetruncus,and pulmonary arteries.

131. CLINICALTHERAPY A Rastelli procedure is performed to close the VSD and create a passage to pulmonary arteries. Repeated surgery is necessary to enlarge the pulmonary artery conduit. Digoxin and diuretics are given.

132. PROGNOSIS Survival is improved,buttruncal valve stenosis and regurgitation result. The long-term prognisis is unknown.the child should not participate in competitive sports.

133. TOTAL ANOMALOUS PULMONARY VENOUS RETURN

134. PATHOPHYSIOLOGY The pumonary veins empty into the rt atrium or veins leading to the rt atrium rather than into the left atrium. The foramen ovale must remain patent for mixed blood from the rt atrium to pass to the systemic circulation. Any obstruction of the pulmonary veins increses the condition’s severity. It occurs in about 1% of children with a congenital heart defect

136. CLINICAL MANIFESTATIONS Mild cyanosis and frequent respiratory infections occur.Increased cyanosis may occur with feedings as the filled esophagus compresses the common pulmonary vein. The pulmonary veins are obstructed in anyway,cyanosis will be increased.increased pulmonary flow will result in signs of CHF. A precordial bulge may be palpated.An ejection murmur and gallop rhythm may be heard in the pulmonic area.

137. Diagnostic procedures The chest radoigraph shows cardiac enlargement,a large pulmonary artery,and increased pulmonary blood flow. The ECG reveals hypertrophy of the rt atrium and ventricle. The echocardiogram shows enlargement of the rtatrium,a patent foramen ovale,and lack of connections between the pulmonary veins and left atrium. Cardiac cath shows a higher O2 level in the rt atrium and the abnormal circulation.

138. CLINICALTHERAPY prostaglandinE1 is given to maintain patent ductusarteriosus. Hypoxemia and CHF are treated. Balloon atrialseptostomy may be performed to promote better mixing of blood so surgery can be delayed untill the infant can be stabilized. Surgery to reconnect or baffle the pulmonary veins to the left atrium is performed.

139. PROGNOSIS Survivors have lived more than 20 years after correction.

140. HYPOPLASTICLEFT HEART SYNDROME

141. PATHOPHYSIOLOGY This is one of the most severe defects with absence or stenosis of mitral and aortic valves,an abnormally small left ventricle,a small aorta,and aortic or mitral stenosis or atresia. It occurs in 1% of congenital heart defects.

143. CLINICAL MANIFESTATIONS With closure of the ductusarteriosus the newborn has progressive cyanosis, tachycardia, tachypnea,dyspnea,retractions,and decreased peripheral pulses. A systolic murmur may be present or absent Poor peripheral perfision,pulmonaryedema,and CHF lead to shock,acidisis,and death.

144. DIAGNOSTIC PROCEDURES The chest radiograph shows cardiomegaly and increased pulmonary vascularity. The echocardiogram shows the small left ventricle.This condition may diagnosed prenatally. Cardiac cath may be performed in preparation of surgical intervention or to perform an atrialseptostomy to promote blood mixing.

145. CLINICAL THERAPY prostaglandinE1 is given to maintain a petentductusateriosus Three treatment options include comfort or palliative care,theNorwodprocedure,and heart transplantation. Many infants waiting for a heart transplant die because of the scarcity of donor hearts. The Noorwood procedure has become a more common intervention as outcomes have improved.surgery is performed in three stages. The Noorwood procedure is performed in the first week of life,followed by the Glenn procedure at about 3to 8 months of age,and the Fontan procedure at between at 18 months and 3 years of age.

146. PROGNOSIS Without surgery,the median survival time is 3 days.HLHS is the largest contributor of infant deaths due to congenital heart disease. Mortality rates in infants having a first stage Norwod procedure are approximately 10-20% in the first year of life. Some large centres have achieved a 70% 5 year survival rate with the Norwood procedure. The child will have some limitations in physical activity because of a single ventricle. Failure of the single ventricle occurs over time,and these children may require a heart transplant during adolescence or adulthod.

147. NURSING MANAGEMENT AT THE TIME OF SURGERY The goals of nursing management are to perform assessments,provide supportive care to the family,and meet the child’s nursing care needs before and after the surgery.

148. NURSING ASSESSMENT AND DIAGNOSIS Ineffective breathing pattern related to respiratory muscle fatigue. Acute pain related to surgical incision and expansion of chest with coughing and deep breathing exercises. Risk for imbalanced fluid volume related to impact of surgery on hearts pumping action. Risk for infection related to surgery and chronic disease status.

149. PLANNING AND IMPLEMENTATION Pain management Promote respiratory function Manage fluids and nutrition Activity

150. DISCHARGE PLANNING AND HOMECARE TEACHING

151. EVALUATION The child’s pain is effectively managed. Full lung expansion is maintained with incentive spirometry exercises or chest physiotherapy. The childs incision heals without infection. Catch-up growth occurs over the next few months to years.

152. THANK YOU