CONGENITAL HEART DISEASES

1. PRESENTED BY DONA MATHEW MSC(N) GOVT.COLLEGE OF NURSING KOTTAYAM CONGENITAL HEART DISEASES 1

2. Congenital heart defect (CHD) Anatomic malformation of the heart or great vessels which occurs during intrauterine development, irrespective of the age at presentation. Congenital heart disease occurs in approximately 0.8% of live births. The incidence is higher in stillborns (3-4%), spontaneous abortuses (10-25%), and premature infants (about 2% excluding patent ductusarteriosus [PDA] 2

3. Causes of congenital heart defects Environmental factors Viral Infections rubella during the first three months of pregnancy Medication lithium (used to manage bipolor disorder), Accutane (acne medication), some anti-seizure medications Alcohol with fetal alcohol syndrome (FAS) Smoking Cocaine Maternal chronic illnesses –diabetes, phenylketonuria (PKU) and a deficiency in the B vitamin folic acid. 3

4. Genetic factors Heredity –occur in siblings or offspring of individuals with heart defects than those without. Mutations –can affect the formation of the heart and lead to congenital heart malformations Linked with other birth defects – More than one-third of children born with Down syndrome have heart defects. About 25% of girls with  Turner syndrome have heart defects 4

5. Classification of congenital heart disease 5

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8. RELATIVE FREQUENCY OF MAJOR CONGENITAL HEART LESIONS LESION % OF ALL LESIONS Ventricular septal defect 35-30 Atrial septal defect ( secundum ) 6-8 Patent ductus arteriosus 6-8 Coarctation of aorta 5-7 Tetralogy of Fallot 5-7 Pulmonary valve stenosis 5-7 Aortic valve stenosis 4-7 d-Transposition of great arteries 3-5 Hypoplastic left ventricle 1-3 Hypoplastic right ventricle 1-3 Truncus arteriosus 1-2 Total anomalous pulmonary venous return 1-2 Tricuspid atresia 1-2 Single ventricle 1-2 Double-outlet right ventricle 1-2 Others 5-10 8

9. ACYANOTIC CONGENITAL HEART DISEASE 9

10. DEFECTS WITH INCREASED PULMONARY BLOOD FLOW 10

11. Atrial Septal Defect (ASD) An opening in the atrial septum An atrial septal defect allows oxygenated (red) blood to pass from the left atrium, through the opening in the septum, and then mix with unoxygenated (blue) blood in the right atrium During fetal heart devt .  The partitioning process does not occur completely, leaving an opening in the atrial septum Occur in 4-10% of all infants w/ CHD Effects : when blood passes through the ASD from the left atrium to the right atrium  a larger volume of blood than normal must be handled by the right side of the heart  e xtra blood then passes through the pulmonary artery into the lungs  pulmonary hypertension and pulmonary congestion 11

12. If the ASD is left uncorrected-->pulmonary hypertension progresses -->pressure in the right side of the heart will become greater than the left side of the heart. This reversal of the pressure gradient across the ASD causes the shunt to reverse --> a  right-to-left shunt  will exist. This phenomenon is known as  Eisenmenger's syndrome Once right-to-left shunting occurs, a portion of the oxygen-poor blood will get shunted to the left side of the heart and ejected to the peripheral vascular system. This will cause signs of cyanosis 12

13. Atrial Septal Defect (ASD) 13

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15. Types of atrial septal defects Ostium secundum atrial septal defect most common type of atrial septal defect comprises 6–10% of all congenital heart diseases. Opening near the center of the septum The secundum atrial septal defect usually arises from an enlarged foramen ovale , inadequate growth of the septum secundum , or excessive absorption of the septum primum . If the ostium secundum ASD is accompanied by an acquired mitral valve stenosis , that is called Lutembacher's syndrome. 15

16. Patent foramen ovale A small channel that has some hemodynamic consequence It is a remnant of the fetal  foramen ovale . On echocardiography, there may not be any shunting of blood noted except when the patient coughs. 16

17. Ostium primum atrial septal defect Opening at the lower end of the septum A defect in the  ostium primum  is occasionally classified as an atrial septal defect but it is more commonly classified an  atrioventricular septal defect .  Ostium primum defects are less common than ostium secundum defects 17

18. Sinus venosus atrial septal defect Opening near the junction of superior vena cava and right atrium, may be associated with partial anomalous pulmonary venous connection Common or single atrium It is a failure of development of the embryologic components that contribute to the atrial septal complex. It is frequently associated with  heterotaxy syndrome. 18

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20. CLINICAL MANIFESTATIONS History. Infants and children with ASDs are usually asymptomatic Physical Examination A relatively slender body build is typical. A widely split and fixed S2 and a systolic ejection murmur are characteristic findings of ASD in older infants and children. . Classic auscultatory findings of ASD are not present unless the shunt is reasonably large 20

21. Electrocardiography Right axis deviation of +90 to +180 degrees and mild right ventricular hypertrophy (RVH) or right bundle branch block (RBBB) with an rsR ' pattern in V1 are typical findings 21

22. X-ray Studies Cardiomegaly with enlargement of the RA and right ventricle (RV) may be present. A prominent pulmonary artery (PA) segment and increased pulmonary vascular markings are seen when the shunt is significant 22

23. Echocardiography A two-dimensional echo study is diagnostic. shows the position as well as the size of the defect, In secundum ASD, a dropout can be seen in the midatrial septum. The primum type shows a defect in the lower atrial septum Indirect signs of a significant left-to-right atrial shunt include RV enlargement and RA enlargement, as well as dilated PA M-mode echo may show increased RV dimension and paradoxical motion of the interventricular septum, which are signs of RV volume overload. 23

24. ASD on 2D echo 24

25. ASD flow by colour Doppler 25

26. Echocardiogram in apical four chamber view of primum ASD 26

27. CONTRAST ECHO If agitated saline is injected into a peripheral vein during echocardiography, small air bubbles can be seen on echocardiographic imaging. It may be possible to see bubbles travel across an ASD either at rest or during a cough. (Bubbles will only flow from right atrium to left atrium if the RA pressure is greater than LA). 27

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29. NATURAL HISTORY OF ASD spontaneous closure of the secundum defect occurs in about 40% of patients in the first 4 years of life Most children with an ASD remain active and asymptomatic. Rarely, congestive heart failure (CHF) can develop in infancy. If a large defect is untreated, CHF and pulmonary hypertension develop in adults who are in their 20s and 30s With or without surgery, atrial arrhythmias (flutter or fibrillation) may occur in adults. 29

30. MANAGEMENT Medical Exercise restriction is unnecessary. Prophylaxis for infective endocarditis indicated in patients with primum ASD. In infants with CHF, medical management is recommended because of its high success rate and the possibility of spontaneous closure of the defect. 30

31. Nonsurgical Closure of ASD catheter-delivered closure device has become a preferred method Devices available for clinical use have included the Sideris buttoned device, the Angel Wings device, the CardioSEAL device, and the Amplatzer ASD Occlusion Device . The amplatzer septal occluder has the widespread use. 31

32. Use of the closure device may be indicated In To close a secundum ASD, measuring 5 mm or more in diameter (but less than 32 mm), A significant left-to-right shunt with clinical evidence of right ventricular volume overload There must be enough rim (4 mm) of septal tissue around the defect for appropriate placement of the device. The timing of the device- because of the possibility of spontaneous closure, it is not used in infancy unless the patient is symptomatic with heart failure. 32

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34. Advantages of nonsurgical closure Complete avoidance of cardiopulmonary bypass Avoidance of pain and residual thoracotomy scars A less than 24-hour hospital stay Rapid recovery All these devices are associated with a higher rate of small residual leak than is operative closure. 34

35. Post-device closure follow-up The patients are administered aspirin 81 mg/day for 6 months. Postprocedure echo studies 35

36. Surgical Closure Indications and Timing A left-to-right shunt with a pulmonary-to-systemic blood flow ratio ( p/ s) of ≥1.5:1 . Surgery is usually delayed until 2 to 4 years of age because the possibility of spontaneous closure. Surgery is performed during infancy-if CHF does not respond to medical management infancy,. 36

37. Infants with associated bronchopulmonary dysplasia and the device closure is not considered appropriate, surgery is performed during infancy. High pulmonary vascular resistance may be a contraindication for surgery 37

38. Procedure. For secundum ASD, the defect is traditionally repairedwith a simple suture or a pericardial or Teflon patch through a midsternal incision under cardiopulmonary bypass by either For sinus venosus defect without associated anomalous pulmonary venous return, the defect is closed using an autologous pericardial patch. 38

39. When it is associated with a pulmonary venous anomaly, a tunnel is created between the anomalous pulmonary vein and the ASD by using a Teflon or pericardial patch For coronary sinus ASD, the ostium of the coronary sinus is closed with an autologous pericardium 39

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41. Mortality. Fewer than 0.5% of patients die Complications. Cerebrovascular accident postoperative arrhythmias Postoperative Follow-up 1. Cardiomegaly on x-ray film and enlarged RV dimension on echo as well as the wide splitting of the S2 may persist for 1 or 2 years postoperatively. The ECG typically demonstrates RBBB (or RV conduction disturbance). 2. Atrial or nodal arrhythmias occur in 7% to 20% of postoperative patients. 3. Rarely, patients with residual shunt may be administered aspirin 81 mg to prevent paradoxical embolization 41

42. Ventricular Septal Defect (VSD) an opening in the ventricular septum allows oxygenated blood to pass from the left ventricle, through the opening in the septum, and then mix with unoxygenated blood in the right ventricle. VSDs are the most commonly occurring type of congenital heart defect, occurring in 14-17 % of babies born each year. occur when the partitioning process does not occur completely, leaving an opening in the ventricular septum. 42

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44. EFFECTS : When blood passes through the VSD from the left ventricle to the right ventricle  a larger volume of blood than normal must be handled by the right side of the heart  e xtra blood then passes through the pulmonary artery into the lungs  pulmonary hypertension and pulmonary congestion  pulmonary arteries become thickened and obstructed due to increased pressure . Ventricular Septal Defect (VSD) 44

45. If VSD is not repaired, and lung disease begins to occur  pressure in the right side of the heart will eventually exceed pressure in the left  R to L shunt  cyanosis Due to high pressure --- tissue damage may eventually occur in the right ventricle  b acteria in the bloodstream can easily infect this injured area  bacterial endocarditis 45

46. CLINICAL MANIFESTATIONS History With a small VSD, the patient is asymptomatic with normal growth and development. With a moderate to large VSD, delayed growth and development, decreased exercise tolerance, repeated pulmonary infections, and CHF are relatively common during infancy. With long-standing pulmonary hypertension, a history of cyanosis and a decreased level of activity may be present . 46

47. Physical Examination Infants with small VSDs are well developed and acyanotic . Before 2 or 3 months of age, infants with large VSDs may have poor weight gain or show signs of CHF. Cyanosis and clubbing may be present in patients with Eisenmenger's syndrome A systolic thrill may be present at the lower left sternal border. Precordial bulge and hyperactivity are present with a large-shunt VSD. The S2 is loud and single in patients with pulmonary hypertension or pulmonary vascular obstructive disease. 47

48. A grade 2 systolic murmur is audible at the lower left sternal border .It may be holosystolic or early systolic. An apical diastolic rumble is present with a moderate to large shunt because of increased flow through the mitral valve during diastole. 48

49. Electrocardiography With a small VSD, the ECG is normal. With a moderate VSD, left ventricular hypertrophy (LVH) and occasional left atrial hypertrophy (LAH) may be seen. With a large defect, the ECG shows biventricular hypertrophy (BVH) with or without LAH If pulmonary vascular obstructive disease develops, the ECG shows RVH only 49

50. VSD (6 month old child)  50

51. X-ray Studies Cardiomegaly of varying degrees is present and involves the LA, left ventricle (LV), and sometimes RV. Pulmonary vascular markings increase. The degree of cardiomegaly and the increase in pulmonary vascular markings directly relate to the magnitude of the left-to-right shunt . 51

52. Echocardiography 52

53. NATURAL HISTORY Spontaneous closure occurs in 30% to 40% of patients with membranous VSDs and muscular VSDs during the first 6 months of life. CHF develops in infants with large VSDs but usually not until 6 to 8 weeks of age. Pulmonary vascular obstructive disease may begin to develop as early as 6 to 12 months of age in patients with large VSDs, but the resulting right-to-left shunt usually does not develop until the teenage years. Infective endocarditis rarely occurs. 53

54. Medical Management Treatment of CHF if it develops, ( digoxin and diuretics for 2 to 4 months ) Addition of spironolactone may be helpful to minimize potassium loss. Concomitant use of an afterload -reducing agent, such as captopril , Frequent feedings of high-calorie formulas, by either nasogastric tube or oral feeding, may help. 54

55. Anemia, if present, should be corrected by oral iron therapy.. No exercise restriction is required in the absence of pulmonary hypertension. Maintenance of good dental hygiene and antibiotic prophylaxis against infective endocarditis are important Nonsurgical closure of selected muscular VSDs is possible using the “umbrella” device, but this is still in the experimental stage. 55

56. Surgical- Indications and Timing Small infants who have large VSDs and develop CHF and growth retardation - If growth failure cannot be improved by medical therapy, the VSD should be operated on within the first 6 months of life. if the PA pressure is greater than 50% of systemic pressure, surgical closure should be done by the end of the first year. After 1 year of age, a significant left-to-right shunt with p/ s of at least 2:1 indicates that surgical closure is needed, regardless of PA pressure. 56

57. Surgical treatment Palliative – pulmonary artery banding Placing a band around the pulmonary artery to decrease the pulmonary blood flow It increases the resistance to blood flow through the pulmonary artery. Pressure increases in the right ventricle and prevents excess shunting from left to right 57

58. Complete repair -small defects are repaired with a purse-string approach. Large defects usually require a Knitted Dacron patch sewn over the opening Both procedures require CPB The repair is generally approached through the right atrium and tricuspid valve Post operative complications include residual VSD and conduction disturbances 58

59. Mortality. Surgical mortality is less than 1%. Mortality is higher for small infants younger than 2 months of age, infants with associated defects, or infants with multiple VSDs 59

60. Postoperative Follow-up Activity should not be restricted unless complications have resulted from surgery. The ECG shows RBBB in 50% to 90% of patients who had VSD repair through right ventriculotomy and up to 40% of the patients who had repair through a right atrial approach. Bacterial endocarditis prophylaxis may be discontinued 6 months after surgery. If a residual shunt is present, endocarditis prophylaxis should be continued indefinitely when the indications arise. A patient with a postoperative history of transient heart block with or without pacemaker therapy requires long-term follow-up. 60

61. Patent Ductus Arteriosus (PDA) characterized by a connection between the aorta and the pulmonary artery All babies are born with a ductus arteriosus . As the baby takes the first breath, the blood vessels in the lungs open up, and blood begins to flow  the ductus arteriosus is not needed to bypass the lungs Most babies have a closed ductus arteriosus by 72 hours after birth. In some babies, however, the ductus arteriosus remains open (patent) . The opening between the aorta and the pulmonary artery allows oxygenated blood to pass back through the blood vessels in the lungs. PDA occurs in 6-11 % of all children with CHD 61

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63. In many children, there is no known reason for the ductus arteriosus remaining open. However, PDA is seen more often in the following: premature infants infants born to a mother who had rubella during the first trimester of pregnancy EFFECTS: PDA  oxygenated blood passes from the aorta to the pulmonary artery & mixes w/ the unoxygenated blood w/c goes to the lungs   blood volume to the lungs  pulmonary hypertension & congestion Further, because blood is pumped at high pressure through the PDA, the lining of the pulmonary artery will become irritated and inflamed. Bacteria in the bloodstream can easily infect this injured area  bacterial endocarditis . Patent Ductus Arteriosus (PDA) 63

64. CLINICAL MANIFESTATIONS History Patients are usually asymptomatic when the ductus is small. A large-shunt PDA may cause a lower respiratory tract infection, atelectasis , and CHF (accompanied by tachypnea and poor weight gain). Exertional dyspnea may be present in children with a large-shunt PDA 64

65. Physical Examination Tachycardia and tachypnea may be present in infants with CHF. Bounding peripheral pulses with wide pulse pressure are characteristic findings. A systolic thrill may be present at the upper left sternal border. A grade 1 to 4/6 continuous (“machinery”) murmur is best audible at the left infraclavicular area or upper left sternal border.. If pulmonary vascular obstructive disease develops, a right-to-left ductal shunt results in cyanosis only in the lower half of the body 65

66. Electrocardiography. The ECG findings in PDA are similar to those in VSD. A normal ECG or LVH is seen with small to moderate PDA. BVH is seen with large PDA. If pulmonary vascular obstructive disease develops, RVH is present. X-ray Studies . X-ray findings are also similar to those of VSD. Chest x-ray films may be normal with a small-shunt PDA. Cardiomegaly of varying degrees occurs in moderate- to large-shunt PDA with enlargement of the LA, LV, and ascending aorta. Pulmonary vascular markings are increased. 66

67. Echocardiography Its size can be assessed by two-dimensional echo in a high parasternal view or in a suprasternal notch view 67

68. NATURAL HISTORY Spontaneous closure of a PDA does not usually occur in full-term infants and children. This is because the PDA in term infants results from a structural abnormality of the ductal smooth muscle CHF or recurrent pneumonia or both develop if the shunt is large. Pulmonary vascular obstructive disease may develop if a large PDA with pulmonary hypertension is left untreated. Infective endocarditis may occur. Although rare, an aneurysm of PDA may develop and possibly rupture in adult life. 68

69. MANAGEMENT- IN TERM NEONATES Medical Indomethacin is ineffective in term infants with PDA and should not be used. Standard anticongestive measures with digoxin and diuretics are indicated when CHF develops. No exercise restriction is needed in the absence of pulmonary hypertension. Prophylaxis for subacute bacterial endocarditis (SBE) is indicated when indications arise . 69

70. Nonsurgical Closure. Small ductus less than 4 mm in diameter are closed by coils Larger ones by an amplatzer PDA device. 70

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73. SURGICAL CLOSURE Surgical closure is reserved for patients in whom a nonsurgical closure technique is not considered applicable Procedure Ligation and division through left posterolateral thoracotomy without cardiopulmonary bypass is the standard procedure. The technique of video-assisted thoracoscopic clip ligation has become the standard of care for surgical management of a ductus with adequate length 73

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75. Patent Ductus Arteriosus in Preterm Neonates Clinical evidence of PDA appears in 45% of infants with birth weight less than 1750 g and in about 80% of infants with birth weight less than 1200 g. Significant PDA with CHF occurs in 15% of premature infants with birth weight less than 1750 g and in 40% to 50% of those with birth weight less than 1500 g 75

76. MANAGEMENT- IN PRE TERM NEONATES Medical Fluid restriction to 120 mL /kg per day and a diuretic (e.g., furosemide , 1 mg/kg, two to three times a day) may be tried for 24 to 48 hours Pharmacologic closure of the PDA can he achieved with indomethacin (a prostaglandin synthetase inhibitor). The dose is given intravenously every 12 hours for a total of three doses. For infants less than 48 hours old, 0.2 mg/kg is followed by 0.1 mg/kg 2 times . 76

77. For those 2 to 7 days old, 0.2 mg/kg times 3, and for infants older than 7 days, 0.2 mg/kg followed by 0.25 mg/kg times 2 Contraindications to the use of indomethacin High blood urea nitrogen (>25 mg/dl) or creatinine (>1.8 mg/dl) levels Low platelet count (<80,000/mm 3 ) Bleeding tendency (including intracranial hemorrhage) Necrotizing enterocolitis , and hyperbilirubinemia . A multicenter prospective study from Europe showed that intravenous ibuprofen (10 mg/kg, followed at 24-hour intervals by two doses of 5 mg/kg) starting on the third day of life was as effective as indomethacin in closing the ductus in preterm newborns.. 77

78. ATRIOVENTRICULAR CANAL DEFECT   Also known as endocardial cushion defects They account for about 5 percent of all congenital heart disease, and are most common in infants with down syndrome. (About 15 percent to 20 percent of newborns with down syndrome have atrioventricular septal defects). 78

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80. Complete atrioventricular canal (CAVC) Complete atrioventricular canal (CAVC) is a severe defect in which there is a large hole in the the septum that separates the left and right sides of the heart. The hole is in the center of the heart, where the upper chambers and the lower chambers meet. In a child with a complete atrioventricular canal defect, there is one large valve, and it may not close correctly. 80

81. Partial atrioventricular canal defects The hole does not extend between the lower chambers of the heart and the valves are better formed. Partial atrioventricular canal is also called atrioventricular septal defect, or AVSD. 81

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83. PATHOPHYSIOLOGY defect in the septum blood to travel from the left side of the heart to the right side of the heart, or the other way around. The oxygenated and unoxygenated blood being mixed up The extra blood being pumped into the lung arteries makes the heart and lungs work harder and the lungs can become congested. 83

84. CLINICAL MANIFESTATION Dyspnea MILD CYANOSIS A newborn baby will show signs of heart failure such as edema, fatigue, wheezing, sweating and irregular heartbeat CHARACTERSTIC MURMUR 84

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86. MANAGEMENT Palliative-pulmonary artery banding Complete repair – Patch closure of septal defect Reconstruction of AV valve tissue 86

87. OBSTRUCTIVE DEFECTS 87

88. Coarctation of the Aorta Narrowing of the aorta can occur anywhere, but is most likely to happen in the segment just after the aortic arch. This narrowing restricts the amount of blood to the lower part of the body occurs in about 8-11 % of all children with CHD 88

89. EFFECTS: The left ventricle has to work harder to try to move blood through the narrowing in the aorta  left-sided heart failure BP is higher above the narrowing, and lower below the narrowing. Older children may have headaches from too much pressure in the vessels in the head, or cramps in the legs or abdomen from too little blood flow in that region. The walls of the arteries may become weakened by high pressure  s pontaneous tears  cause a stroke or uncontrollable bleeding.  risk for bacterial endocarditis . 89

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92. SYMPTOMATIC INFANTS History . Poor feeding, dyspnea , and poor weight gain or signs of acute circulatory shock may develop in the first 6 weeks of life. Physical Examination Infants with COA are pale and experience varying degrees of respiratory distress. Oliguria or anuria , general circulatory shock, and severe acidemia are common.). Peripheral pulses may be weak and thready as a result of CHF. 92

93. A blood pressure differential may become apparent only after improvement of cardiac function with administration of rapidly acting inotropic agents. The S2 is single and loud; a loud S3 gallop is usually present. No heart murmur is present in 50% of sick infants. A nonspecific ejection systolic murmur is audible over the precordium . 93

94. Electrocardiography . A normal or rightward QRS axis and RVH or right bundle branch block (RBBB) are present X-ray Studies . Marked cardiomegaly and pulmonary edema or pulmonary venous congestion are usually present 94

95. The blue arrow to the actual coarctation and the green arrow to the post- stenotic dilation of the descending aorta . 95

96. Echocardiography Two-dimensional echo and color flow Doppler studies usually show the site and extent of the coarctation . In the suprasternal notch view, a thin wedge-shaped “posterior shelf” is imaged in the posterolateral aspect of the upper descending aorta 96

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98. Natural History About 20% to 30% of all patients with COA develop CHF by 3 months of age. If it is undetected or untreated, early death may result from CHF and renal shutdown in symptomatic infants 98

99. Management Medical In symptomatic neonates, PGE 1 infusion should be started to reopen the ductus arteriosus and establish flow to the descending aorta and the kidneys during the first weeks of life. Intensive anticongestive measures with short-acting inotropic agents (e.g., dopamine, dobutamine ), diuretics, and oxygen should be started. Balloon angioplasty can be a useful procedure for sick infants in whom standard surgical management carries a high risk. 99

100. Surgical Indications and Timing If CHF or circulatory shock develops early in life, surgery should be performed on an urgent basis. Procedures Resection and end-to-end anastomosis consists of resecting the coarctation segment and anastomosing the proximal and distal aortas . Subclavian flap aortoplasty consists of dividing the distal subclavian artery and inserting a flap of the proximal portion of this vessel between the two sides of the longitudinally split aorta throughout the coarctation segment. 100

101. With patch aortoplasty , the aorta is opened longitudinally through the coarctation segment and extending to the left subclavian artery, and the fibrous shelf and any existing membrane are excised. An elliptic woven Dacron patch is inserted to expand the diameter of the lumen. 101

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103. ASYMPTOMATIC INFANTS AND CHILDREN Management Medical Children with mild COA should be watched closely for hypertension in the arm or for increasing pressure differences between the arm and leg. Balloon angioplasty A balloon-expandable stainless-steel stent implanted concurrently with balloon angioplasty An absorbable metal stent is in the experimental stage 103

104. Surgical Indications and Timing COA with hypertension in the upper extremities or with a large systolic pressure gradient equal to or greater than 20 mm Hg between the arms and the legs indicates that elective surgical correction is necessary between the ages of 2 and 4 years. Reduction of aortic diameter by 50% at the level of COA is also an indication for surgery. Older children are operated on soon after the diagnosis is made. 104

105. In asymptomatic children, surgery is performed by age 4 to 5; late surgery may increase the risk of developing early essential hypertension. If severe hypertension, CHF, or cardiomegaly is present, surgery is performed at an earlier age. 105

106. Surgical Procedures Resection of the coarctation segment and end-to-end anastomosis Occasionally, subclavian artery aortoplasty or circular or patch grafts may be performed. 106

107. AORTIC STENOSIS Narrowing or stricture o the aortic valve Resistance to blood flow in the left ventricle, decreased cardiac out put, left ventricular hypertrophy and pulmonary vascular congestion Valvular stenosis is the most common type and is usually caused by malformed cusps Sub valvular stenosis is a stricture caused by a fibrous ring below the normal valve Supra valvular stenosis occurs infrequently 107

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109. Pathophysiology A stricture in the aortic outflow tract --> resistance to ejection of blood from left ventricle -> extra work load of the left ventricle -> hypertrophy - > left ventricular failure -> left atrial pressure increases -> increased pressure in the pulmonary veins -> pulmonary edema 109

110. CLINICAL MANIFESTATIONS History Neonates with critical or severe stenosis of the aortic valve may develop signs of hypoperfusion or respiratory distress related to pulmonary edema within days to weeks after birth. Most children with mild to moderate AS are asymptomatic. Occasionally, exercise intolerance may be present. Exertional chest pain, easy fatigability, or syncope may occur in a child with a severe degree of obstruction 110

111. Physical Examination Infants and children with AS are acyanotic and are normally developed. blood pressure is normal in most patients, but a narrow pulse pressure is present in severe AS. A systolic thrill may be palpable at the upper right sternal border, in the suprasternal notch, or over the carotid arteries. An ejection click may be heard with valvular AS. 111

112. Newborns with critical AS may develop signs of reduced peripheral perfusion (with weak and thready pulses, pale cool skin, and slow capillary refill) 112

113. Electrocardiography. In mild cases the ECG is normal. LVH with or without strain pattern may be present in severe cases X-ray Studies The heart size is usually normal in children, but a dilated ascending aorta or a prominent aortic knob may be seen occasionally in valvular AS, resulting from poststenotic dilatation. Significant cardiomegaly does not develop unless CHF occurs later 113

114. Echocardiography 114

115. NATURAL HISTORY Chest pain, syncope, and even sudden death (1% to 2% of cases) may occur in children with severe AS. Heart failure occurs with severe AS during the newborn period or later in adult life. Mild stenosis becomes more severe with time in a significant number of patients 115

116. MANAGEMENT Medical For critically ill newborns with CHF patients are stabilized before surgery balloon valvuloplasty use of rapidly acting inotropic agents and diuretics to treat CHF intravenous infusion of PGE 1 to reopen the ductus . Percutaneous balloon valvuloplasty is now regarded as the first step in the management of symptomatic neonates 116

117. Surgical Valvular AS Closed aortic valvotomy , using calibrated dilators or balloon catheters without cardiopulmonary bypass, may be performed in sick infants if balloon valvuloplasty has been unsuccessful or if it is not available Aortic valve commissurotomy is usually tried if stenosis is the predominant lesion Aortic valve replacement may be necessary if AR is the predominant lesion 117

118. Subvalvular AS- Excision of the membrane is done for discrete subvalvular AS Supravalvular AS- a reconstructive surgery is done using a Y-shaped patch 118

119. Postballoon and Postoperative Follow-up An annual follow-up examination is necessary for all patients who have the aortic valve balloon procedure or surgery in order to detect development of stenosis or regurgitation. Anticoagulation is needed after a prosthetic mechanical valve replacement. The International Normalized Ratio (INR) should be maintained between 2.5 and 3.5 for the first 3 months and 2.0 to 3.0 beyond that time. Low-dose aspirin (75 to 100 mg/day for adolescents) is indicated in addition to warfarin (American College of Cardiology, 2006). After aortic valve replacement with a bioprosthesis and no risk factors, aspirin (75 to 100 mg), but not warfarin , is indicated. Restriction from competitive, strenuous sports may be necessary for children with moderate residual AS or AR, or both 119

120. PULMONARY VALVE STENOSIS Narrowing at the entrance of pulmonary artery Resistance to blood flow causes right ventricular hypertrophy Pulmonary atresia is the extreme form – total fusion of commissures and no blood flows to the lungs rt . Ventricle may be hypoplastic 120

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122. Pathophysiology PS - -> Resistance to blood flow --> RVH If Rt . Ventricular failure develops --> increase in rt , atrial pressure --> re-opening of foramen ovale --> shunting of un oxy. blood in to the Lt.atrium --> systemic cyanosis 122

123. Clinical manifestations May be asymptomatic Some had mild cyanosis Newborn with severe narrowig - cyanotic Characteristic murmur Cardiomegaly on chest radiograph Pt are at risk for infective endocarditis 123

124. Management Surgical In infants trans ventricular valvotomy ( brock procedure) In children pulmonary valvotomy with CPB Non surgical treatment Balloon angioplasty 124

125. CYANOTIC HEART DISEASE 125

126. DEFECTS WITH DECREASED PULMONARY BLOOD FLOW 126

127. Tetralogy of Fallot (TOF) a complex condition of several congenital defects that occur due to abnormal devt . of the fetal heart during the first 8 weeks of pregnancy. These problems include the following: ventricular septal defect (VSD)  Pulmonary valve stenosis overriding aorta -  The aorta sits above both the left and right ventricles over the VSD, rather than just over the left ventricle. As a result, oxygen poor blood from the right ventricle can flow directly into the aorta instead of into the pulmonary artery to the lungs. Right ventricular hypertrophy - The muscle of the right ventricle is thicker than usual because of having to work harder than normal. 127

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129. EFFECTS: If the right ventricle obstruction is severe, or if the pressure in the lungs is high  a large amount of oxygen-poor (blue) blood passes through the VSD, mixes with the oxygen-rich (red) blood in the left ventricle, and is pumped to the body  cyanosis The more blood that goes through the VSD, the less blood that goes through the pulmonary artery to the lungs   oxygenated blood to the left side of the heart. Soon, nearly all the blood in the left ventricle is oxygen-poor (blue). This is an emergency situation, as the body will not have enough oxygen to meet its needs. Tetralogy of Fallot (TOF) 129

130. CLINICAL MANIFESTATIONS History A heart murmur is audible at birth. Most patients are symptomatic with cyanosis at birth or shortly thereafter. Dyspnea on exertion or hypoxic spells develop later, even in mildly cyanotic infants. Immediately after birth, severe cyanosis is seen in patients with TOF and pulmonary atresia . 130

131. Physical Examination Varying degrees of cyanosis, tachypnea , and clubbing (in older infants and children) are present. An RV tap along the left sternal border and a systolic thrill at the upper and mid-left sternal borders are commonly present (50%). An ejection click that originates in the aorta may be audible 131

132. Electrocardiography Right axis deviation (RAD) (+120 to +150 degrees) is present in cyanotic TOF. RVH is usually present BVH may be seen in the acyanotic form. RAH is occasionally present. 132

133. X- ray Decreased pulmonary markings Black lung fields Boot shaped heart 133

134. NATURAL HISTORY Infants with acyanotic TOF gradually become cyanotic. Patients who are already cyanotic become more cyanotic as a result of the worsening condition of the infundibular stenosis and polycythemia . Polycythemia develops secondary to cyanosis. Hypoxic spells may develop in infants. Growth retardation may be present if cyanosis is severe. 134

135. HYPOXIC SPELL Hypoxic spells are characterized by a paroxysm of hyperpnea (i.e., rapid and deep respiration), irritability and prolonged crying, increasing cyanosis, and decreasing intensity of the heart murmur. Hypoxic spells occur in infants, with a peak incidence between 2 and 4 months of age. These spells usually occur in the morning after crying, feeding, or defecation. A severe spell may lead to limpness, convulsion, cerebrovascular accident, or even death. 135

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138. Treatment of the hypoxic spell The infant should be picked up and held in a knee-chest position. Morphine sulfate, 0.2 mg/kg administered subcutaneously or intramuscularly, suppresses the respiratory center and abolishes hyperpnea Oxygen is usually administered, but it has little demonstrable effect on arterial oxygen saturation. Acidosis should be treated with sodium bicarbonate (NaHCO 3 ), 1 mEq /kg administered intravenously. The same dose can be repeated in 10 to 15 minutes.. 138

139. P lacing the child in the knee-chest position either lying supine or over the parent’s shoulder (see below).  This calms the infant, reduces systemic venous return and increases systemic vascular resistance.             139

140. MANAGEMENT OF TOF Medical educate parents to recognize the spell and know what to do. Oral propranolol therapy, 0.5 to 1.5 mg/kg every 6 hours, is occasionally used to prevent hypoxic spells while waiting for an optimal time for corrective surgery Balloon dilatation of the right ventricular outflow tract and pulmonary valve, it is not widely practiced, Maintenance of good dental hygiene and practice of antibiotic prophylaxis against SBE are important . A relative iron deficiency state should be detected and treated 140

141. Surgical Palliative Shunt Procedures Classic Blalock- Taussig shunt , anastomosed between the subclavian artery and the ipsilateral PA, is usually performed for infants older than 3 months because the shunt is often thrombosed in younger infants with smaller arteries 141

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143. Modified Blalock- Taussig (BT) shunt . A Gore-Tex interposition shunt is placed between the subclavian artery and the ipsilateral PA. This is the most popular procedure for any age, especially for small infants younger than 3 months of age 143

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145. The Waterston shunt , anastomosed between the ascending aorta and the right PA, is no longer performed because of a high incidence of surgical complications 145

146. The Potts operation Anastomosed between the descending aorta and the left PA, is no longer performed 146

147. Complete Repair Surgery Usually done in the first year of life Total repair of the defect is carried out under cardiopulmonary bypass The procedure includes patch closure of the VSD, preferably through a transatrial and transpulmonary artery approach widening of the RVOT by division and/or resection of the infundibular tissue; and pulmonary valvotomy ,). The operative mortality for total correction of TOF is 5% 147

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149. Tricuspid Atresia In this condition, there is no tricuspid valve, therefore, no blood flows from the right atrium to the right ventricle. Blood in right atrium  foramen ovale  left atrium and left ventricle  aorta There is complete mixing of the oxy. Blood and un oxy. blood Tricuspid atresia defect is characterized by the following: a small right ventricle a large left ventricle Small VSD and PDA diminished pulmonary circulation cyanosis - bluish color of the skin and mucous membranes caused from a lack of oxygen. 149

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151. CLINICAL MANIFESTATIONS History Cyanosis is usually severe from birth. Tachypnea and poor feeding usually manifest. History of hypoxic spells may be present in infants with this condition. Physical Examination Cyanosis, either with or without clubbing, is always present. A systolic thrill is rarely palpable when associated with PS. A grade 2 to 3/6 holosystolic (or early systolic) murmur of VSD is usually present at the lower left sternal border 151

152. Electrocardiography LVH is usually present; RAH or biatrial hypertrophy (BAH) is common. X-ray Studies . The heart size is normal or slightly increased, with enlargement of the RA and LV Echocardiography . Absence of the tricuspid orifice, marked hypoplasia of the RV, and a large LV can be imaged in the apical four-chamber view. 152

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154. MANAGEMENT Initial Medical Management PGE 1 should be started in neonates with severe cyanosis to maintain the patency of the ductus before planned cardiac catheterization or cardiac surgery. The Rashkind procedure (balloon atrial septostomy ) may be performed as part of the initial catheterization to improve the RA-to-LA shunt, especially when the interatrial communication is considered inadequate by echo studies. 154

155. Surgical. Most infants with tricuspid atresia require one or more palliative procedures before a Fontan -type operation Ideal candidates for a Fontan -type operation are those who have normal LV function and low pulmonary resistance Stage I Blalock- Taussig shunt, when PBF is small This procedure results in the volume load on the LV because the LV supplies blood to both the systemic and pulmonary circulations. 155

156. Damus -Kaye- Stansel and shunt operation   the aorta and pulmonary artery are joined using a patch (pink). The blue tube is known as a Modified Blalock- Taussig Shunt . 156

157. Pulmonary artery banding . PA banding is rarely necessary for infants with CHF resulting from increased PBF. PA banding protects the pulmonary vasculature from developing pulmonary hypertension Medical follow-up after stage I. Watch for: Cyanosis (O 2 saturation <75%)—cardiac catheterization or MRI to find out its cause. Poor weight gain (CHF from too much PBF)—tightening of PA band may be necessary. 157

158. Stage II (at 3 months or by 6 months ). Bidirectional Glenn operation (BDG). also called bidirectional superior cavopulmonary shunt An end-to-side SVC-to-RPA shunt (also called bidirectional superior cavopulmonary shunt) can be performed by 2.5 to 3 months of age This procedure satisfactorily increases oxygen saturation 158

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160. The hemi- Fontan operation . SVC is connected to PA. Blood coming back from the upper body now flows directly to the lungs without going through the heart The Blalock- Taussig (BT) shunt is removed. A patch is placed over the top part of the heart's right upper chamber . This prevents blood from the upper body from entering the heart and blood from the lower body from entering the lungs. It also maintains a connection that is used for the final stage of the repair and greatly simplifies the last operation. 160

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162. Medical follow-up after stage II. Watch for the following : A gradual decrease in O 2 saturation (<75%) may be caused by: . Opening of venous collaterals Pulmonary AV fistula (due to the absence of hepatic inhibitory factor) Transient hypertension—1 to 2 weeks postoperatively—may use ACE inhibitors 162

163. Stage III ( Fontan operation)—within 1 to 2 years after stage II operation The whole premise of the Fontan operation is directing the entire systemic venous blood to the pulmonary arteries without an intervening pumping chamber. The Fontan operation is usually completed when the child is around 2 years of age. This procedure can even be performed on infants. 163

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165. Postoperative Medical Follow-up Patients should maintain a low-salt diet. Medications: Some patients need continued digoxin and diuretic therapy. An angiotensin -converting enzyme (ACE) inhibitor is generally recommended. Aspirin (or even warfarin ) is used to prevent thrombus formation in the RA. Patients should not participate in competitive, strenuous sports. Antibiotic prophylaxis against SBE should be observed when indications arise. 165

166. MIXED BLOOD FLOW 166

167. Transposition of the Great Arteries (TGA) the aorta is connected to the right ventricle, and the pulmonary artery is connected to the left ventricle Oxygen-poor (blue) blood returns to the right atrium from the body  passes through the right atrium and ventricle,  into the misconnected aorta back to the body. Oxygen-rich (red) blood returns to the left atrium from the lungs  passes through the left atrium and ventricle,  into the pulmonary artery and back to the lungs. Other heart defects are often associated with TGA - atrial or ventricular septal defect may be necessary in order for the infant with TGA to survive Allow mixing of blood – providing at least smaller amounts of oxygen to the body 167

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169. CLINICAL MANIFESTATIONS History History of cyanosis from birth is always present. Signs of congestive heart failure (CHF) with dyspnea and feeding difficulties develop during the newborn period. 169

170. Physical Examination Moderate to severe cyanosis is present, The S2 is single and loud.. If CHF supervenes, hepatomegaly and dyspnea develop. 170

171. Electrocardiography There is a rightward QRS axis (i.e., +90 to +200 degrees). Right ventricular hypertrophy (RVH) is usually present Biventricular hypertrophy (BVH) may be present Occasionally right atrial hypertrophy (RAH) is present. 171

172. Echocardiography. In the parasternal short-axis view, the “circle and sausage” appearance of the normal great arteries is not visible. Instead, the great arteries appear as “double circles” 3. In the apical and subcostal five-chamber views, the PA arises from the LV, and the aorta arises from the RV. 172

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174. X-ray Studies Cardiomegaly An egg-shaped cardiac silhouette 174

175. NATURAL HISTORY Without surgical intervention, death occurs in 90% of patients before they reach 6 months of age . 175

176. MANAGEMENT Medical Arterial blood gases and pH should be obtained and metabolic acidosis should be corrected PGE 1 infusion should be started to improve arterial oxygen saturation by reopening the ductus . Oxygen administration A balloon atrial septotomy ( Rashkind procedure) may be performed to increase the mixing by opening the atrial septum 176

177. Surgical An arterial switch procedure- performed in first week of life Transecting the great arteries and anastomosing the main pulmonary artery to the proximal aorta and anastomosing the ascending aorta to the proximal pulmonary artery The coronary arteries are switched from proximal aorta to the proximal pulmonary artery to create a new aorta 177

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179. Intra atrial baffle repairs – An intraatrial baffle is created to divert the venous blood to the mitral valve and pulmonary venous blood to the tricuspid valve using using patients atrial septum or a prosthetic valve. 179

180. Rastelli procedure -operative choice in patients with TGA,VSD, and severe pulmonic stenosis . It involves closure of VSD with baffle->LV blood directed through VSD in to aorta Pulmonic valve is then closed , and a conduit is placed from RV to PA 180

181. Total Anomalous Pulmonary Venous Return No direct communication between the pulmonary veins and the left atrium Drain anomalously into the systemic venous tributeries or into right atrium 4 types Supracardiac :( 50% of TAPVR ). The common pulmonary venous sinus drains into the right SVC Cardiac : (20% of TAPVR) .The common pulmonary venous sinus drains into the coronary sinus 181

182. Infracardiac :( 20% of TAPVR patients) The common pulmonary venous sinus drains to the portal vein, ductus venosus , hepatic vein, or inferior vena cava (IVC). Mixed type : This type, which is a combination of the other types, accounts for 10% of TAPVR patients 182

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184. TOTAL ANOMALOUS PULMONARY VENOUS RETURN – Clinical Manifestations History CHF with gowth retardation Frequent pulmonary infection Mild cyanosis Physical examination Undernourished Signs of CHF Precordial bulge ECG RVH and occational RAH 184

185. TOTAL ANOMALOUS PULMONARY VENOUS RETURN – Clinical Manifestations X- ray studies Cardiomegaly Kerley B lines Snowmans sign/ figure of 8 Echo A large RA and a small LA, with deviation of the atrial septum to the left and dilated PAs, are also present. 185

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187. MANAGEMENT medical Intensive anticongestive measures Metabolic acidosis should be corrected, Infants with severe pulmonary edema-should be intubated and receive ventilator support In some patients with pulmonary hypertension, PGE 1 can increase systemic flow by keeping the ductus open 187

188. Surgical treatment Supracardiac Type . A large, side-to-side anastomosis is made between the common pulmonary venous sinus and the LA. The ASD is closed with a cloth patch. TAPVR to the Right Atrium . The atrial septum is excised and a patch is sewn in such a way that the pulmonary venous return is diverted to the LA . The ASD may have to be enlarged. 188

189. TAPVR to the Coronary Sinus . An incision is made in the anterior wall of the coronary sinus (“ unroofing ”) to make a communication between the coronary sinus and the LA. A single patch closes the original ASD and the ostium of the coronary sinus. This results in the drainage of coronary sinus blood with low oxygen saturation into the LA Infracardiac Type . A large vertical anastomosis is made between the common pulmonary venous sinus and the LA. The common pulmonary vein, which descends vertically to the abdominal cavity, is ligated above the diaphragm 189

190. Truncus Arteriosus The aorta and pulmonary artery start as a single blood vessel, which eventually divides and becomes two separate arteries. Truncus arteriosus occurs when the single great vessel fails to separate completely, leaving a connection between the aorta and pulmonary artery. Usually accompanied by a ventricular septal defect EFFECTS: oxygen-poor (blue) and oxygen-rich (red) blood mix back and forth through the ventricular septal defect. This mixed blood then flows through the common truncal vessel. Some of it will flow to pulmonary artery and on to the lungs, and some of the mixed blood will go into the aortic branch and to the body. The mixed blood that goes to the body does not have as much oxygen as normal, and will cause varying degrees of cyanosis 190

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192. CLINICAL MANIFESTATIONS History Cyanosis may be seen immediately after birth. Signs of CHF develop within several days to weeks after birth. History of dyspnea with feeding, failure to thrive, and frequent respiratory infections is usually present in infants . 192

193. Physical Examination Varying degrees of cyanosis and signs of CHF with tachypnea and dyspnea are usually present. The peripheral pulses are bounding, with a wide pulse pressure. A systolic click is frequently audible at the apex and upper left sternal border. 193

194. Electrocardiography . BVH is present in 70% of cases X-ray Studies . Cardiomegaly is usually present Echocardiography. A large, single great artery arises from the heart .The type of persistent truncus arteriosus can be identified, and the size of the PAs can be determined. 194

195. Medical Vigorous anticongestive measures with digitalis and diuretics surgical Corrective repair Closing the VSD Excising the pul . Arteries from aorta , and attaching them to the RV by means of homograft Prognosis Mortality greater than 10% 195

196. Hypoplastic Left Heart Syndrome HLHS occurs in 1% of all congenital heart defects or 9% of such defects in critically ill newborns Underdevelopment of the left side of the heart resulting in a hypoplastic left ventricle and aortic atresia Most of the blood from LA Flows across PFO to RA RV PA The descending aorta receives blood from pda supplying systemic blood flow 196

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198. Effects PFO allows saturated blood from LA to mix with desaturated blood from RA-->RV -->PA from PA blood flows to lung and then through ductus arteriosus in to the aorta and out f the body The coronary and cerebral vessels receive blood by retrograde flowthrough the hypoplastic ascending aorta 198

199. CLINICAL MANIFESTATIONS A neonate with HLHS becomes critically ill within the first few hours to the first few days of life. Tachycardia, dyspnea , pulmonary crackles, weak peripheral pulses, and vasoconstricted extremities are characteristic. The patient may not have severe cyanosis but has a grayish blue color of the skin with poor perfusion. The S2 is loud and single.. The ECG almost always shows RVH. Chest x-ray films characteristically show pulmonary venous congestion or pulmonary edema Arterial blood gas levels reveal a slightly decreased Po 2 and a normal Pco 2 . 199

200. Therapeutic management PGE1 infusion to maintain ductal patency Surgical procedure Several staged approach 1 st stage- Norwood procedure Anastomosis of main PA to the aorta to create a new aorta, placement of a shunt or inserting a conduit from the RV to PA to provide pulmonary blood flow , and creation of a large ASD. 2 nd stage-Bidirectional Glenn Shunt Done at 6-9 months of age to relieve cyanosis and reduce overload in RV 200

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202. Final repair-Modified Fontan Procedure Transplantation 202

203. Ebstein's Anomaly Ebstein anomaly is a congenital heart defect in which the septal leaflet of the tricuspid valve is displaced towards the apex of theright ventricle of the heart. The valve leaflets, however, are to a varying degree, attached to the walls and septum of the right ventricle. There is subsequent ' atrialization ' of a portion of the morphologic right ventricle 203

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205. Pathophysiology The right ventricle is thus divided into 2 parts by the abnormal tricuspid valve the 1st, a thin-walled “ atrialized ” portion, is continuous with the cavity of the right atrium; the 2nd, often smaller portion consists of normal ventricular myocardium. The right atrium is enlarged as a result of tricuspid valve regurgitation, 205

206. In more severe forms of Ebstein anomaly, the effective output from the right side of the heart is decreased due to a combination of the poorly functioning small right ventricle, tricuspid valve regurgitation, and obstruction of the right ventricular outflow tract produced by the large, sail-like, anterior tricuspid valve leaflet. The increased volume of right atrial blood shunts through the foramen ovale (or through an associated atrial septal defect) to the left atrium and produces cyanosis 206

207. CLINICAL MANIFESTATIONS History In severe cases, cyanosis and CHF develop during the first few days of life. Children with milder cases may complain of dyspnea , fatigue, cyanosis, or palpitation on exertion. 207

208. Physical Examination Mild to severe cyanosis is present, as well as clubbing of the fingers and toes in older infants and children. Characteristic triple or quadruple rhythm is audible. Electrocardiography 1. Characteristic ECG findings of RBBB and RAH are present in most patients with this condition x-ray Studies . . In severe cases, an extreme cardiomegaly with a balloon-shaped heart and decreased pulmonary vascular markings are present 208

209. Echocardiography The tricuspid valve leaflets are elongated, redundant, and dysplastic A large RA, including the atrialized RV, and a small functional RV represent anatomic severity 209

210. MANAGEMENT Medical In severely cyanotic newborns, intensive treatment with mechanical ventilation, PGE 1 infusion inotropic agents correction of metabolic acidosis 210

211. Surgical Palliative procedures Blalock- Taussig shunt Starnes operation- a procedure to reduce the RV or RA Classic Glenn anastomosis or its modification may be considered in severely cyanotic infants 211

212. Definitive procedures Two-ventricular repair Danielson technique: For repair of the tricuspid valve Carpentier technique: This repair also plicates the atrialized portion of the RV and the tricuspid annulus One-ventricular repair : For patients with inadequate size of the RV, a Fontan -type operation is usually performed in stages following the initial palliative procedures such as bidirectional Glenn operation 212

213. Nursing diagnosis Impaired gas exchange related to altered pulmonary blood flow or oxygen deprivation Altered cardiac output related to specific anatomic defect Activity intolerance related to decreased oxygenation in blood and tissues Altered Nutrition: less than body requirements related to the excessive energy demands required by increased cardiac workload Increased potential for infection related to poor nutritional status Anxiety related to diagnostic procedures and hospitalization Developmental delay related to decreased energy, inadequate nutrition, physical limitations and social isolation Alteration in parenting related to parental perception of the child as vulnerable 213

214. NURSING INTERVENTIONS 214

215. Relieve the respiratory distress associated with increased pulmonary blood flow or oxygen deprivation Determine degree of respiratory distress Position child at 45 degree angle to decrease pressure of the viscera on the diaphragm  and increase lung volume Pin diapers loosely and provide loose-fitting pajamas for older children Feed slowly Tilt infant’s head slightly Suction the nose and throat if unable to cough out secretions Provide oxygen therapy as needed Improve oxygenation o that the body functions may be maintained Provide effective oxygen environment Observe response to oxygen therapy Observe response to oxygen weaning therapy Relieve Hypoxic spells associated with cyanotic types of Congenital heart disease Observe for “ tet ” spells Encourage fluid intake Obtain vital signs 215

216. Nursing interventions A. Provide adequate nutritional and fluid intake to maintain the growth and developmental needs of the child Feed in semi-erect position Provide small frequent feedings Provide foods with high nutritional value Determine child’s likes and dislikes Strict input and output Daily weight 216

217. B. Prevent infection Prevent exposure to communicable diseases Immunizations should be up-to-date Handwashing should be observed Be certain that the child receives prophylactic medication for infective endocarditis 217

218. C. Reduce the workload of the heart since decreased activity and expenditure of energy will decrease oxygen requirements Uninterrupted rest Avoid unnecessary activities Prevent excessive crying Provide diversional activities Prevent constipation 218

219. D. Observe child for symptoms of Congestive Heart Failure that occur frequently as a complication of Congenital Heart Disease E. Observe for the development of symptoms of infective endocarditis that may occur as a complication of congenital heart disease F. Observe for the development of thrombosis that may occur as a complication of congenital heart disease G. Prepare the child for diagnostic and treatment procedures H. Explain cardiac problems to child and parents 219

220. Health Education A. Instruct the family in necessary measures to maintain the child’s health B. Teach the family about the defect and its treatment C. Encourage the parents and other persons to treat child in a normal manner as possible 220

221. THANK YOU 221