congenital heart defect

1. Congenital Heart disease
• CHD is the structural malformation of the heart or great vessels,
present at birth.
• It is the most common congenital malformation
• The exact number of prevalence is not known
Incidence
• 5-8 / 1000 live birth

5. Classification of CHD

6. • CLINICAL FEATURES OF CONGENITAL HEART DISEASES
(1) Cyanosis : there is a mixture of oxygenated blood with deoxy-
genated blood resulting in cyanosis.
(2) Clubbing : pronounced in children with cyanosis
(3) Squatting ( especially in TOF)
(4) exertional dyspnoea, fatigue, cyanosis , tachypneaetc.
(5) Sub-optimal development
(6) Recurrent respiratory infections
(7) Focal sepsis :- Focal sepsis of the teeth, tonsils and ears lead to
increased complications and morbidity.
(8) Bacterial endocarditis
(9) Poor feeding

7. ATRIAL SEPTAL DEFECT
Definition
Atrial septal defect (ASD) is an congenital heart defect in which
abnormal opening is between the right and left atrium, which allowing
the blood to flow from left to right atrium

8. Types
The opening can be three types
• Ostium primum : When opening at lower end of septum, it can be
associated with mitral valve abnormalities.
• Ostium secundum (ASD 2): When opening near center of septum.
• Sinus venosus defect : When opening is top of atrial septum ,near junction of
superior vena cava and right atrium.
Causes
• genetically oriented, this may occur due to abnormal chromosome, defective
gene.
• Environmental exposure also leads congenital heart defects.
• Associated with other system congenital defects

9. Pathophysiology
 Because left atrial pressure slightly exceeds right atrial pressure, blood flows
from the left to the right atrium, causing an increased flow of oxygenated blood
into the right side of the heart.
 Despite the low pressure difference, a high rate of flow can still occur because
of low pulmonary vascular resistançe and the greater distensibility of the right
atrium, which further reduces flow resistance .
 This volume is well tolerated by the right ventricle because it is delivered un-
der much lower pressure than in a ventricular septal defect
 Although there is right atrial and ventricular enlargement, cardiac failure is
unusual in an uncomplicated ASD. Pulmonary congestion is common ue to
increased blood flow to lungs via pulmonary artery
 Pulmonary vascular changes usually occur only after several decades if the
defect is unrepaired.

10. Clinical Manifestations :
• The child may be asymptomatic.
• Congestive heart failure( later)
• Heart murmur
• Risk of dysrhythmias
• Fatigue
• Poor growth and weight gain
• Cardiac enlargement
• Decreased exercise tolerance and Dyspnea
• Sweating
• Pallor
• edema

11. Diagnostic evaluations :
• Closed physical examination : Systolic ejection murmer heard in the upper
left sternal border ( increased blood flow across the defect) and diastolic
murmur in large left to right shunt ( increased blood flow through mitral
valve). First heart sound is louder
• Chest-x-ray : It shows right atrium and right ventricle dilation because of
more amount of blood from left side.
• Electrocardiogram (ECG) : shows right ventricular overload and
dysrhythmias
• Echocardiogram : total structure of heart including septal defect size,
position, flow of blood through the opening etc.can be visualized
• Cardiac catheterization : A catheter is passed in heart through the blood
vessel in groin and contrast dye is injected through the catheter to visualize
the heart defects and picture is taken to view the result.

12. Management
Medical Management
• Congestive heart failure and arrhythmias or dysarhythmias should be treated
with medications such as digoxin which strengthen the heart muscles for regular
pumping function.
• Antibiotics to prevent infections and
• diuretics for remove the excess fluid by the kidney from the body.
Surgical Management
• Small ASD will close spontaneously requiring no interventions
• Surgical management include - patch closure (pericardial patch or dacron patch)
of moderate to large defects similar to closure of ventricular septal defects.
• Open repair is done through a median sternotomy and requires cardio pulmonary
bypass . Usually performed at 2-4 yrs
• If the defect is small , purse string closure is done by stitching around the
opening and pulling it closed
• The ASD 1 (ostium primum) may require mitral valve repair of replacement of
mitral valve (rarely).

13. • The ASD 2 (ostium secundum) may also be closed using devices
during cardiac catheterization.

14. Complications :
• Pulmonary arterial hypertension.
• Infective endocarditis.
• Retardation of growth.
• CCF
• Post-operative complication( cardiac enlargement, dysrhythmias)

15. Ventricular septal Defect (VSD)
• A ventricular septal defect is an opening in the ventricular septum, or
dividing wall between the two lower chambers of the heart known as
the right and left ventricles.
• VSD is a congenital (present at birth) heart defect.
• It is most common acyanotic congenital heart disease.
• It is found approximately 25% of all CHD.
• The size of defect can be small & large.

17. Etiology
• In most children, the cause isn't known
• Heredity
• occurs with other genetic problems, such as Down syndrome
• Rubella infection during pregnancy
• Poorly controlled diabetes in pregnancy
• Drug or alcohol use or exposure to certain radiation

18. Types of VSD
 Perimembraneous VSD : The opening is at the upper part of the
septum and near the valves
 Muscular VSD: Opening occurs in the lower part of the septum

19. Pathophysiology
 Because of the higher pressure within the left ventricle and because the
systemic arterial circulation offers more resistance than the pulmonary
circulation, blood flows through the defect into the pulmonary artery
 The increased blood volume is pumped into the lungs, which may eventually
result in increased pulmonary vascular resistance
 Increased pressure in the right ventricle as a result of left-to-right shunting
and pulmonary resistance causes the muscle to hypertrophy
 If the right ventricle is unable to accommodate the increased workload, the
right atrium may also enlarge as it attempts to overcome the resistance
offered by incomplete right ventricular emptying
 In severe defects Eisenmenger syndrome may develop due to reverse of the
shunt as a result of increased pulmonary resistance

20. Clinical features
• The size of the ventricular septal opening will affect the type of symptoms noted, the
severity of symptoms, and the age at which they first occur.
• The following are the most common symptoms
– fatigue
– sweating
– rapid and heavy breathing
-- exercise/ activity intolerance
-- Dyspnea
--Pulmonary hypertension
--congested breathing
-- Pulmonary infection
-- Tachypnea
--pallor
--edema
– disinterest in feeding, or tiring while feeding
– poor weight gain
--Failure to thrive

21. Diagnosis
• Cardiac examination : systolic heart murmurs ,due to which the first heart
sound intensity is reduced .
• chest x-ray – Heart size and pulmonary vascular markings are increased,
pulmonary edema
• electrocardiogram (ECG) – normal in small VSD . In moderate to large VSD,
ECG shows left ventricular hypertrophy
• echocardiogram (echo) – Helps to determine size and location of VSD. The
degree of left to right shunting and PVR also can be assessed
• cardiac catheterization- to find out the size of VSD, location and other
associated lesions
• cardiac magnetic resonance imaging (MRI)

22. Management
• In small VSD usually no medical management is required.
• Surgical repair may be indicated in some cases.
• Spontaneous closure of VSD occurs in 30 to 50% of cases with small defect.
• In large VSD, initial management of associated problem like CCF
(digoxin,diuretics, fluid restriction) and endocarditis, should be done with
appropriate treatment.
• Surgery is done as one- stage or two-stage operation.
• One stage operation with Dacron patch closure of VSD by Open-heart
method can be performed.
• In two stage approach, first, pulmonary artery banding done to restrict
pulmonary blood flow by closed- heart method.
• Second stage surgery is done to patch close the VSD and remove the PA
band by open heart surgery
• Device closure during cardiac catheterization is under trial for muscular
VSD

24. Complications
• CCF
• recurrent respiratory tract infection
• Infective endocarditis
• pulmonary stenosis
• Pulmonary hypertension.
• pulmonary edema
• Residual VSD and conduction disturbances ( post operative)

25. PATENT DUCTUS ARTERIOSUS (PDA)
Definition :
• It is abnormal communication or opening between the pulmonary artery and the
aorta.
• In fetal circulation, there is a normal communication in between the aorta and
pulmonary artery, known as ductus arteriosus. After the birth, ductus arteriosus is
closed with in first 10-15 hrs of life, if it fails to close and remain open or patent ,then
condition is known as patent ductus arteriosus.

26. Pathophysiology:
 The hemodynamic consequences of PDA depend on the size of the ductus and
the pulmonary vascular resistance.
 At birth the resistance in the pulmonary and systemic circulations is almost
identical, thus equalizing the resistance in the aorta and pulmonary artery.
 As the systemic pressure exceeds the pulmonary pressure, blood begins to
shunt from the aorta, across the duct, to the pulmonary artery (left-to-right
shunt).
 The additional blood is recirculated through the lungs and returned to the left
atrium and left ventricle.
 The effect of this altered circulation is increased workload on the left side of
the heart, increased pulmonary vascular congestion and possibly resistance,
and potentially increased right ventricular pressure and hypertrophy.

27. Clinical manifestation
• Some children may be asymptomatic
• Machinery like murmur
• Strong pulse
• Dyspnea
• Widened pulse pressure (Increased systolic pressure and low diastolic pressure)
• Difficulty in feeding
• Hoarseness of voice(the vocal muscles can be affected due to interruption with
laryngeal nerve)
• Tachypnea
• Tachycardia
• Growth failure
• Slow in weight gain
• Easy fatigability
• Congestive heart failure

28. Diagnostic Evaluation
• History collection
• Cardiac examination: Systolic murmur or continuous murmur may be
present, which is best heard in second to third left intercostal space.
Pulse is usually bounding in these children.
• Electrocardiogram: The ECG is usually normal, it may show left
ventricular hypertrophy and left atrial dilatation in older children.
• Chest radiograph: The chest radiograph shows increased pulmonary
vascularity with normal or increased heart size.
• Echocardiogram: helps in visualization of Patent Ductus Arteriosus.
With a Doppler, the amount of blood flow across the PDA can be
estimated.
• Cardiac catheterization : helps in detecting the defect

29. Management
• Medical management : It include administration of indomethacin ( prostaglandin
inhibitor) 0.1 to 0.25 mg/kg/ dose intra venously slowly. It can be repeated upto
three times at an interval of 12-24 hours
• it is useful medication in closing of patent ductus in premature infants and some
newborn babies. It should be administered within 10 days of birth
• Do the management of CCF with proper medications.
• PDA in small size may not require treatment or it may close with out treatment
spontaneously.
• Surgical Management : It include surgical division or ligation of the patent vessel
via a left thoracotomy ( closed heart surgery).
• A newer technique, visual assisted thoracoscopic surgery (VATS), uses a
thoracoscope and instruments placed through three small incisions on the left
side of the chest to place a clip on the ductus. It speeds the post-operative
recovery.
• In some centers, PDA can be closed by using the coils ( cook’s coil) in
catheterization laboratory.

31. Complication :
• pulmonary hypertension
• endocarditis,
• congestive cardiac failure
• rheumatic heart disease

32. COARCTATION OF AORTA
Definition:
Coarctation of aorta is defined as narrowing of the aortic
arch near to the ductus arteriosus which produce more pressure to the
head and upper extremities (proximal areas) and lower pressure at
body and lower extremities (distal areas).It is often seen along with
PDA and VSD. It constitutes 7% of all CHD
Causes :
• genetic factors- heredity,
• chromosomal abnormalities
• environmental factors
• gene defects

34. Types
 Preductal COA : constriction is between left subclavian artery and ductus
arteriosus
 Post ductal COA : constriction of aorta distal to ductus arteriosus

35. Pathophysiology:
 The effect of a narrowing within the aorta is increased pressure proximal to the
defect and decreased pressure distal to it.
 In the preductal type of COA the lower half of the body is supplied with blood
by the right ventricle through the ductus arteriosus.
 In the postductal type, right ventricular outflow cannot maintain blood flow to
the descending aorta. Therefore collateral circulation develops during fetal life
to maintain flow from the ascending to the descending aorta.
 In the presence of VSD and post ductal type, pulmonary blood flow will be
increased and can later develop left ventricular hypertyrophy

36. Clinical Manifestation :
• Symptoms depends on severity of narrowing.
• High blood pressure and bounding pulse in arms.
• Weak or absent femoral pulses.
• Low blood pressure and cool extremities.
• stroke
• Infants can develop congestive heart failure, acidosis, tachypnea etc.
• In older children
• headache,
• epistaxis,
• fainting,
• dizziness,
• exertional dyspnea and fatigue.
• weakness,
• Poor weight gain.
• Poor feeding.
• Pain and weakness of the leg
• Sweating etc.

37. Diagnostic evaluation
• Physical examination : systolic murmur along the left mid to upper sternal
border
• Electro cardiogram (ECG): indicative of left ventricular hypertrophy
• Echo cardiogram: Helps to determine presence of COA and degree of
narrowing as well as presence of other cardiac defects
• Cardiac catheterization and MRI : for clearly defining the area and extent of
narrowing

38. Management
• Medical management-
• Prostaglandin administration – to maintain PDA
• hypertension – Anti hypertensives ( ACE inhibitors, Ca channel blockers, beta blockers)
• CHF management- Digoxin, diuretics, Potassium , fluid restrictions
• Infection prevention – antibiotics
• Surgical management
• Surgical management is performed within 2 -3years.
• End-to-end anastomosis :Resection of coarcted portion with an end to end anastomosis of the aorta
• Subclavian flap aortoplasty: Enlargement of the constricted section using a portion of the left subclavian
artery.
• Patch aortoplasty: In this procedure, coarcted area is excised and a prosthetic patch graft is placed on aorta
to widen it
• This defect is out side the heart and pericardium, so cardio pulmonary bypass is not required and a left
thoracotomy incision is used.
• Balloon aortoplasty : It is a procedure in which a balloon catheter is introduced into the aorta during cardiac
catheterization and inflated at the site of coarctation to relieve the obstruction
• Stent implantation: A stent is placed at the narrowed portion via cardiac catheterization procedure
• Post-operative hypertension is treated with intra venous sodium nitroprusside followed by oral medications
such as captopril, hydralazine or propranolol.

40. Complications
• Aortic aneurysm
• Systemic aortic hypertension
• aortic rupture
• stroke
• aortic valve stenosis
• mitral valve regurgitation
• CCF
• Bacterial endocarditis
• Organ dysfunction especially kidney, liver (due to decreased blood supply)
• Premature coronary artery disease ( due to narrowing of the blood vessels that supply
the heart)
• Post operative hypertension
• Post operative recurrence of COA
• Post operative aneurysm
• decreased vascular supply ( to left arm)

41. AORTIC STENOSIS
Definition :
Aortic stenosis is defined as narrowing or stricture of the aortic valve,
which produce obstruction to blood flow from the left ventricle,
decreased cardiac output, left ventricular hypertrophy and pulmonary
vascular congestion. It constitutes about 5% of all CHD
Types :
• Valvular stenosis : This is most common type, it is usually caused by
malformed cuspus resulting in a bicuspid rather than tricuspid valve
or fusion of the cusps.
• Subvalvular stenosis : It is a stricture caused by a fibrous ring below a
normal valve or narrowing of the left ventricular outflow tract.
• Supravalvular stenosis : Stenosis above the aortic valve

43. Pathophysiology
 A stricture in the aortic outflow tract causes resistance to ejection of
blood from the left ventricle.
 The extra workload on the left ventricle causes hypertrophy. This also
interferes with coronary artery perfusion and may result in
myocardial infarction
 If left ventricular pressure fail, left atrial pressure will increase, this
causes increased pressure in the pulmonary veins, resulting in
pulmonary vascular congestion and pulmonary edema
 The increased pulmonary congestion will put a pressure in the
pulmonary artery leading to pulmonary artery hypertension , finally
leading to rt. Ventricular failure

44. • Causes :
• Environmental factors.
• Defective gene.
• Chromosomal abnormalities etc.
Clinical manifestations :
• Hypotension
• Tachycardia
• Tacchypnea
• Paleness
• irritable
• Poor feeding
• Dizziness
• Chest pain( due to ischemia)
• Murmur
• Shortness of breath
• Palpitations
• Fatigue
• Exertional dyspnea

45. Diagnosis
• Cardiac examination: Systolic ejection murmur heard best at right
upper sternal border
• Chest x-ray: Increased pulmonary vascular markings, pulmonary
edema
• Echocardiogram: Helps to visualize type of aortic stenosis as well as
presence of other defects. Also helps to evaluate left ventricular wall
thickness and left ventricular function
• Electrocardiogram (ECG): Demonstrate left ventricular hypertrophy.
There may be ST segment depression , which indicates myocardial
ischemia
• Cardiac catheterization: Helps to determine the site and size of
stenosis

46. Management
Valvular aortic stenosis
• Aortic valvotomy : In this surgical method, there is removal of adhesions
which obstructs the valve leaflets from opening properly ( via median
sternotomy).
• Aortic valve replacement : Aortic valve is replaced with different
mechanisms and consist of 3 types of valve replacements.
(i) Tissue or biological valve - it consists of animal valve.
(ii) Mechanical valve, which consists of metal, plastic and other artificial
valves.
(iii) Aortic homograft : In this, a section of aorta from donor person with
the valve intact is used to replace the aortic valve and section of the
ascending aorta.
• Aortic balloon valvuloplasty: It is completed with cardiac catheterization. In
this a small flexible catheter is inserted in blood vessel of the groin and
send to the inside of the heart. This tube consists of a balloon at the tip.
When the tube is reached in the narrowed valve area, balloon is inflated
and stretch the narrowed area and make it in normal opening. Then,
deflate the balloon and remove the catheter.

47. • Ross procedure : In this, first a section of the child’s own pulmonary artery with the valve
intact is used to replace the aortic valve and a section of aorta. After this a section of
pulmonary artery from the donor with its valve intact is used to replace the transferred
pulmonary artery.
Subvalvular aortic steosis
• Konno procedure : Mainly done in subvalvular aortic stenosis. It involves the incising a
membrane or cutting the fibromuscular ring below the aortic valve. The narrowed left
ventricular out flow tract and annulus is widened with a patch ( annuloplasty), and the
aortic valve is replaced with artificial valve.
Supravalvular aortic stenosis : It is repaired by incising the narrowed segment of aorta and
widening the area with a patch graft
Complications
 Bacterial endocarditis
 Ventricular dysfunction
 Myocardial ischemia
 Stroke
 Pulmonary edema
 Arrhythmia : the heart stretch out of shape, it changes the electrical system of the heart.
And this change can lead to atrial fibrillation.

48. • Ross procedure

49. PULMONARY STENOSIS
Definition :
Pulmonary stenosis may be defined as narrowing of entrance to the
pulmonary artery, which obstruct blood flow to pulmonary artery and
causes right ventricular hypertrophy. An associated defect like PDA is
common. Pulmonary atresia is the extreme form of pulmonic stenosis
Types
(a) Valvar pulmonary stenosis : In this, valve leaflets are become
thickened and/or become narrowed which cause obstruction in blood
flow.
(b) Supravalvar pulmonary stenosis : In this, just above the pulmonary
valve, the pulmonary artery is narrowed.
(c) Subvalvar (infundibular) pulmonary stenosis : In this, below the
valve area, the pulmonary artery muscles become thickened and cause
narrowing of the out flow from the right ventricle.

50. Clinical Manifestations :
• Some children may be asymptomatic.
• Short breath
• Poor exercise tolerance
• Mild cyanosis
• Murmur
• Rapid heart rate
• Rapid breathing
• Fatigue
• Poor feeding
• Exercise intolerance
• Swelling may occur at face, eyelids, ankles, abdomen and feet
Diagnostic evaluation
• Complete physical examination- Systolic murmur at second left intercoastal space, split second heart sound
• Electro cardiogram (ECG)- indicative of right ventricular and right atrial hypertrophy
• Echocardiogram: help demonstrate size of right ventricle , right ventricular outflow tract and the level of
obstruction
• X-ray of chest : shows right ventricular hypertrophy and post stenotic dilation of the pulmonary artery

51. Pathophysiology
 When pulmonic stenosis (PS) is present, resistance to blood flow causes right
ventricular hypertrophy.
 If right ventricular failure develops, right atrial pressure will increase and may
result in reopening of the foramen ovale, shunting of deoxygenated blood into
the left atrium and systemic cyanosis.
 If PS is severe, CHF occurs, and systemic venous congestion will be noted.
 An associated defect, such as PDA partially compensates for the obstruction
by shunting blood from the aorta to the pulmonary artery and into lungs.

52. Management
• Children with mild pulmonary stenosis does not require any treatment.
• In newborns, prostaglandin infusion is given to maintain PDA to improve pulmonary circulation
• Brock procedure (infants): It is also called transventricular valvotomy. This approach entails an
incision into the right ventricular chamber and the cutting of the stenosed pulmonary valve or the
removal of obstructing tissue in the outflow tract of the right ventricle. Done as a closed procedure
• Pulmonary Valvotomy (older children): In this, adhesions are released from the valve and make
valve leaflet to work freely for opening and closed. Done with cardiopulmonary bypass
• Balloon angioplasty : This procedure is based by cardiac catheterization. In this, a small flexible
tube (catheter) is inserted into a blood vessels of groin and inserted into the heart. When this tube
is inserted into the heart at the narrowed valve, the balloon is inflated and stretch the narrowed
area and make it open to flow the blood. This method is mostly used for the valvular and
supravalvular types of pulmonary stenosis.
• Annuloplasty : If the valve annulus (the ring of tissue to which the leaflets of pulmonary valve are
attached) is small, an incision into the annulus is made and a patch is used to enlarge the annulus
• For supravalvular stenosis, the area of obstruction is incised and a patch graft is inserted to widen
the area
• Pulmonary valve replacement : replacement of the pulmonary valve is done later if there is damage
to the valve or leaking in the valve

54. Complications
 Bacterial endocarditis
 Cardiomegaly
 Heart failure
 Arrhythmia
 Stroke
 seizures
 Post operative
 Restenosis
 Aneurysm ( especially at site of graft)

55. TETRALOGY OF FALLOT
Definition :
Tetralogy of fallot is a complex congenital defect with decreased pulmonary
blood flow. It includes a combination of 4 defects
1) Ventricular septal defect : It is an opening between the right and left
ventricle.
2) Pulmonary stenosis : It is narrowing of the pulmonary artery which
obstruct blood flow from the right ventricle.
3) Over-riding of aorta : Actual origination of aorta is from the left ventricle
but in this condition aorta origination is shifted above the VSD or above
both the ventricles.
4) Right ventricular hypertrophy : It occurs because of obstruction in the
pulmonary artery and increased workload of right ventricle

57. Pathophysiology
 The altered hemodynamics varies widely depending primarily on the degree of
PS, but also on the size of the VSD and the pulmonary and systemic resistance
to flow.
 Because the VSD is usually large, pressure may be equal in the right and left
ventricles. Therefore, the shunt direction depends on the difference between
pulmonary and systemic vascular resistance.
 If pulmonary vascular resistance is higher than the systemic resistance, the
shunt is from right to left. If systemic resistance is higher than the pulmonary
resistance, the shunt is from left to right.
 PS decreases blood flow to the lungs and consequently the amount of
oxygenated blood that returns to the left heart. Depending on the position of
the aorta, blood from the both ventricles may be distributed systemically.

58. Clinical manifestation
• The clinical manifestations depends on the size of ventricular septal defects,
and pulmonary obstruction.
• Blue colour of skin, lips and nail buds occurs while crying and feeding.
• Dyspnea with exertion- child often assumes squatting position as it relieves
dyspnea
• Acute episodes of cyanosis and hypoxia, which known as blue spells or ‘tett
spells’. Lasts from few minutes to few hours and can result in acidosis,
unconsciousness, convulsions or even death
• Child may have cool, clammy skin.
• Some time loss of consciousness can occur.
• child may develop clubbing.
• Child at risk of emboli( compensatory mechanism to hypoxia– polycythemia-
increased viscosity- emboli) and seizure

59. Diagnostic evaluation
• History collection.
• Physical examination : systolic ejection murmur is heard along left sternal
border.( mainly due to PS)
• Echocardiogram : The VSD, overriding of aorta and pulmonary stenosis can
be visualized
• Chest-x-ray : boot shaped heart (due to right ventricular hypertrophy, right
atrial enlargement, apex is slightly turned upward and the pulmonary artery
segment is concave because the pulmonary artery is small)
• Electrocardiogram (ECG) : It shows right ventricular hypertrophy
• Cardiac catheterization : It gives information about the structural
abnormalities, their degree of obstruction in blood flow and helps in finding
out the location of defect.

60. Management
1) Medical Management :
• The child should be treated for cyanosis and hypoxic spells.
• Treat dehydration, anemia.
• Oxygen therapy.
• Place the baby in knee chest position to treat hypoxic spells.
• Sodium bicarbonate to treat acidosis
• Provide I.V. fluids.
• Provide intravenous administration of vasopressors such as methoxamine ( increase
SVR).
• Prostaglamdin E, intravenously for severe tetralogy of fallot which produce
dilatation of the ductus and increase pulmonary blood flow.
• Propanolol is administered in a dose of 1mg/kg body weight , upto 4 times a day to
reduce the pulmonary artery and valve spasm( tett spell )and also it increases SVR

61. 2) Surgical Management
Palliative shunt : This is used for infants who cannot undergo primary repair (too
young). In this, a palliative procedure to increase pulmonary blood flow and increase
oxygen saturation may be performed. The preferred procedure is –
• Blalock Taussig or Modified Blalock Taussig Shunt : In older infants and children, an
artificial ductus is created by connecting right or left subclavian artery (branch of
aorta) to the pulmonary artery of same side. This allows increased blood flow to
the lungs. The procedure is performed through a lateral thoracotomy
incision.
• Pott's Procedure: The upper descending aorta is anastomosed with left pulmonary
artery.
• Waterston Shunt: It involves side to side anastomosis of ascending aorta with right
pulmonary artery.
• Brock's procedure: In this surgery, pulmonary valvotomy is done to correct
pulmonary stenosis. The surgery increases pulmonary blood flow but does not
correct VSD.

62. Complete repair (elective surgery) :
• It is usually performed in the first year of life.
• Indications for repair include increasing cyanosis and the development of
hypercyanotic spells.
• Complete repair involves closure of the VSD and resection of the
infundibular stenosis, with a pericardial patch to enlarge the right ventricular
out flow tract. The procedure requires a median sternotomy and the use of
cardio-pulmonary bypass.
pericardial patch

64. Complications
 bacterial endocarditis
 thrombus formation
 stroke
 heart failure
 Arrhythmias
Post operative complications
 Infections
 Pulmonary insufficiency/regurgitation
 Pulmonary restenosis
 Right ventricular hypertrophy
 Conduction abnormalities

65. TRICUSPID ATRESIA.
Definition :
It is defined as failure of tricuspid valve to develop, so that there is no
communication from the right atrium to the right ventricle. ASD or patent
foramen ovale , VSD and PS is common in this condition

66. Pathophysiology
• In this lesion, tricuspid valve fails to develop so there is no communication
between the right atrium and right ventricle.
• The right ventricle is usually hypoplastic (small) and an Atrial Septal Defect
or Patent Foramen Ovale is present. A Ventricular Septal Defect is
commonly present, the pulmonary arteries may be small or normal in size
• In presence of Tricuspid Atresia, unoxygenated blood returning to the right
atrium cannot pass into the right ventricle.
• It passes through Atrial Septal Defect or Patent Foramen Ovale into the left
atrium and left ventricle.
• From left ventricle, a portion of blood flows through Ventricular Septal
Defect (if present) into the small right ventricle and pulmonary circulation.
• If no Ventricular Septal Defect is present, then blood flow to lungs is solely
through the Patent Ductus Arteriosus.

67. Clinical manifestation
• Cyanosis, blue colour of skin, lips and nail.
• tachycardia.
• Dyspnea
• Hypoxic spells
• Rapid breathing.
• Cool, clammy skin.
• Chronic hypoxemia with clubbing.
• Acidosis
• Delayed growth
• Congestive cardiac failure

68. Diagnostic evaluation :
 History collection.
 Physical examination heart and lungs: Systolic murmurs associated with ASD and VSD
 Chest-x-ray : The heart size can be normal or increased. The pulmonary vascularity is decreased
 Echo cardiogram: Absence of tricuspid valve, size of right ventricle and presence of other cardiac defect can
be identified
 Electro cardiogram (ECG): Reveals left and right atrial enlargement, decreased or absent right ventricular
pressure and left ventricular hypertrophy
 Cardiac catheterization
Management
Medical management :
• In the neonate whose pulmonary blood flow depends on the patency of the ductus arteriosus, a
continuous infusion of prostaglandin E, is started at 0.1 mg/kg of body weight/min until surgical treatment
can be followed.
• Antibiotic – for endocarditis
• Sodium bicarbonate – for acidosis
• Mechanical ventilation

69. Surgical management :
1) Palliative treatment - is the placement of a shunt (pulmonary to
systemic artery anastomosis) to increase blood flow to the lungs.
• Blalock- Taussig shunt: In this procedure, the right or left subclavian artery is
connected to the pulmonary artery of same side( in absence or small VSD)
• Balloon Atrial Septostomy: If inadequate atrial communication exists, atrial
septostomy is done during cardiac catheterization to ensure the interatrial
communication
2) Corrective surgery
• Fontan procedure: In this procedure systemic venous return is directed to the
lungs through surgical connection between the right atrium and pulmonary
artery or the right ventricle. Also any ASD or VSD or any previous shunt , if
present are closed. It is done at 4-5 years of age by open heart surgery

70. Complications :
• Dysrhythmia
• Systemic venous hypertension.
• Pericardial and pleural effusion
• Ventricular dysfunctions.
• Chronic kidney disease
• Infective endocarditis
• CHF

71. TRANSPOSITION OF GREAT ARTERIES/VESSELS
( TGA/TGV)
Definition :
Transposition of great arteries may be defined as origination of
pulmonary artery from the left ventricle and the aorta originate from
the right ventricle with no communication between the systemic and
pulmonary circulation.
Mixing of oxygenated and deoxygenated blood occurs in presence of
associated lesions( Patent foramen ovale, ASD, VSD, PDA)
Incidence
It accounts for about 9% of CHD. It occurs more in males.

73. Pathophysiology
 With great arteries transposed, the pulmonary artery arising from left
ventricle carries blood to the lungs for oxygenation. This oxygenated blood
returns to the left atrium and then to left ventricle. From left ventricle
through pulmonary artery the blood is recycled to the pulmonary circulation.
 The aorta arising from the right side of heart delivers unoxygenated blood to
the systemic circulation. This blood then returns to the right atrium and
ventricle and without being oxygenated, circulated back to the systemic
circulation via aorta with progressive hypoxemia.
 Mixing of oxygenated and unoxygenated blood occurs in presence of
associated lesions (Patent Foramen Ovale, ASD, VSD, PDA).
If none of these lesions is present death may occur.

74. Clinical manifestations :
• These depends on size and associated defects.
• Cyanosis at birth
• Metabolic acidosis
• Dyspnoea
• Severe hypoxia
• Symptoms of congestive heart failure
• Cardiomegaly
• Growth failure
• Clubbing secondary to cyanosis may occur in few months
• Tachycardia
• Cool and clammy skin.
• Edema
• Poor feeding

75. Diagnosis :
 History collection.
 Physical examination - cyanosis during examination. Systolic murmurs associated
with ASD and VSD. Single heart sound is heard ( aorta emerging from right
ventricle)
 Chest-X-ray - shows cardiomegaly. Pulmonary vascularity may be normal,
increased or decreased depending on the defect.
 Echocardiogram : helps identify the defects
 Cardiac catheterization: Helps to determine the pulmonary artery pressure and
resistance
 Electrocardiogram (ECG) : indicates right ventricular hypertrophy
Management
Medical management :
• Administration of intravenous prostaglandin E, which may be initiated to
temporarily increase blood mixing of systemic and pulmonary circulation by
keeping the ductus arteriosus open
• Oxygen therapy ,in profoundly cyanotic neonates may be harmful, as it enhances
closure of PDA

76. Surgical management :
1) Palliative surgery
• Rashkind procedure :During cardiac catheterization a atrial balloon
septostomy may also be performed to increase interatrial communication. It
helps in adequate mixing of oxygenated and unoxygenated blood. In this
procedure, a balloon catheter is passed through the foramen ovale, it is
inflated and then pulled back to tear and stretch open foramen ovale thereby
enlarging opening between the two atria
• Blalock Hanon procedure: Creation of an ASD or enlargement of foramen
ovale surgically is termed as Blalock Hanlon procedure

77. 2) Corrective surgery
• Arterial switch procedure:
This is the procedure of choice performed in first week of life. It involves
transecting the great arteries and anastomosing the main pulmonary artery
to the proximal aorta (just above the aortic valve) and anastomosing the
ascending aorta to the proximal pulmonary artery.
The coronary arteries are switched from the proximal aorta to the proximal
pulmonary artery, creating a new aorta.
Reimplantation of the coronary arteries is critical to the infant's survival, and
they must be reattached without torsion or kinking to provide the heart with
its supply of oxygen.
Potential complication of the arterial switch include narrowing at the great
artery anastomoses or coronary artery insufficiency.

78. • Mustard procedure:
 A new atrial septum is created by using pericardium to make a baffle. This
baffle alters blood flow by redirecting unoxygenated blood from the right
atrium to left ventricle and out to lungs via pulmonary artery. Oxygenated
blood from left atrium is redirected to right ventricle and then to systemic
circulation via aorta
• Senning procedure :
 This procedure creates same redirection of blood flow but without the use of
pericardial patch. The atrial septum and a portion of the atrial wall are used to
reroute the blood flow
All these procedures are performed via median sternotomy requiring an
cardiopulmonary bypass

79. Arterial switch procedure Mustard procedure

80. Complications
 Arrhythmias
 acidosis
 embolism
 Valve regurgitation
 IVC and SVC obstruction( post operative)
 Myocardial ischemia
 Post operative stenosis

81. TRUNCUS ARTERIOSUS
Definition
Failure of normal septation and division of the embryonic bulbar trunk into the
pulmonary artery and the aorta, results in single vessel that overrides the both ventricles.
Blood from both ventricles mixes in the common great artery, causing desaturation and
hypoxemia. Blood ejected from the heart flows low pressure pulmonary artery causing
increased pulmonary blood flow and decreased systemic blood flow.

82. Pathophysiology
 Blood ejected from the left and right ventricles enters the common trunk,
mixing pulmonary and systemic circulations.
 Blood flow is distributed to the pulmonary and systemic circulations according
to the relative resistance of each system.
 The amount of pulmonary blood flow depends on the size of pulmonary and
pulmonary vascular resistance.
 Generally, resistance to pulmonary blood flow is less than systemic vascular
resistance, resulting in preferential blood flow to the lungs.

83. Clinical manifestations
 Variable cyanosis
 poor growth
 activity intolerance
 exertional dyspnea
 tachypnea
 tachycardia
 pulmonary edema
 diaphoresis
 other features of CCF

84. Diagnosis
 ECG : May show left or right ventricular hypertrophy or both
 Chest radiography : heart is large, pulmonary vasculature is increased
 Echo : shows the overriding truncal root, VSD
 Cardiac catheterization
Management
 Palliative : Pulmonary artery banding to reduce the pulmonary blood flow
and increase the systemic blood flow
 Corrective surgery : Usually done within the first few months. It involves
losing the VSD so that the truncus arteriosus receives the outflow from the
left ventricle, excising the pulmonary arteries from the aorta and attaching
them to the right ventricle by means of a homograft conduit
Post operative complications include persistent heart failure,
bleeding, pulmonary artery hypertension, dysrhythmias and residual VSD

85. NURSING MANAGEMENT
Nursing Assessment
 Obtain a thorough history of the infant from parents.
 Perform a head to-toe physical assessment with focus on following:
 Vital signs and oxygen saturation.
 Skin colour -pink or cyanosed
 Extremities are checked for peripheral pulse, edema, colour and temperature.
 Presence of clubbing.
 Heart sounds to determine rate and rhythm and identify any murmur.
 Signs of respiratory distress.
 Child's level of activity tolerance.
 Child's growth and developmental level.

86. Nursing Diagnosis
a) Impaired gas exchange related to altered pulmonary blood flow or pulmonary
hypertension.
 Position the child in semi upright position as this position facilitates breathing.
 Suction oral and nasal secretions, if required.
 Monitor oxygen saturation and administer oxygen if required.
 Administer the prescribed medication like diuretics and bronchodilators.
 Arterial blood gas analysis may be done.
 ET intubation and mechanical ventilation
b) Decreased cardiac output related to reduced myocardial functioning.
 Provide bed rest to the child.
 Administer IV fluids as prescribed
 The child should not be allowed to do strenuous work or activity.
 Organize medication schedule and nursing care to provide periods of uninterrupted rest.
 Administer the prescribed medications like Digoxin and diuretics which improve cardiac
output.
 Check the BP before administering these medications.

87. c) Impaired physical mobility and fatigue related to activity intolerance secondary to pulmonary congestion and
hypoxia.
 Ensure uninterrupted period of rest and sleep.
 Administer oxygen, if ordered.
 Monitor oxygen saturation through pulse oxymeter.
 Advice parents to assist activities of daily living for the child so that the child's energy can be conserved.
 Provide feeding, dry diapers and tactile stimulation before the infants cries, as crying increases energy
expenditure.
d) Nursing Diagnosis: Altered nutrition less than body requirement related to anorexia and decreased energy
available for sucking and chewing.
 Nasogastric feeding may be started if the child is unable to take oral feeds or get cyanosed while feeding.
 Provide small frequent feeds to the child.
 Limit the oral feeding time to 15 – 20 mts
 Administer 24kcal/oz formula to the baby.
 Monitor the child's weight daily .Steady weight gain indicates adequacy of feeds.
 Maintain intake and output chart.
 Determine the child’s like and dislike and plan meals accordingly
 Calorie rich food to be given as these children are usually anorectic

88. e) High risk for impaired growth and development related to inadequate tissue
perfusion.
 Assess the child for developmental milestones.
 Encourage attainment of age appropriate emotional, social and cognitive task.
 Provide visual, tactile and auditory stimulation, appropriate for the child's age.
 Encourage the parents to provide tender loving care to the child.
 Assure the parents that most of the child's emotional, social and cognitive tasks can
be met; despite of the defect and motor tasks will develop quickly once physical
health improves.
f) Risk of infection related to hospitalization.
 Observe the child for fever and early signs of infection.
 Strict aseptic techniques should be used while providing care to the child.
 Prevent child's exposure to people with respiratory infections or communicable
diseases.
 Provide routine immunization to the child.
 Administer prophylactic antibiotics, if prescribed.

89. Post-Operative Care
The goal of post-operative nursing management is to assist in restoring optimal functioning of
the cardiopulmonary, gastrointestinal, renal and central nervous system.
The child is shifted from recovery to ICU, 24-48 hours after surgery. Within this duration the
vital signs and all body systems are stabilized.
 Receive the baby in warm bed to prevent hypothermia.
 Check the vital signs.
 Observe the cardiac rhythm and auscultate the breath sounds at least hourly
 Provide semi fowlers positions to the baby.
 Administer digoxin when indicated and monitor BP and heart rate.
 Provide adequate rest to the child, in order to decrease the demands of oxygen on heart to
promote healing of operative area.
 Plan nursing care in such a way that many activities are done at one time, to avoid
disturbing the child repeatedly, Plan care according to the child's natural rhythm of activity
and sleep.
 Administer the prescribed antibiotics prophylactically.
 Provide ventilator care as the child require mechanical ventilation in the immediate post
operative period

90.  suctioning is performed when needed and carefully to avoid vagal stimulation
which can trigger dysrhythmias and laryngospasm
 Suctioning should not take more than 5 seconds
 During suctioning, observe for signs and symptoms of respiratory distress(
tachypnea, use of accessory muscles for breathing, restlessness)
 Frequently( hourly) check the chest tube drainage for quantity and presence of
hemorrhage, which is evident by blood in chest tube.
 Chest tube drainage greater than 3ml/kg/hr for more than three consecutive
hours or 5-10 ml/kg in any one hour indicate post operative hemorrhage
 Also monitor the patient for increased pulse rate and hypotension which
indicates hemorrhage.
 Chest tube is removed by third post operative day after which a petroleum
covered guaze dressing is immediately applied over the wound and securely
dressed
 The dressing is checked for drainage

91.  Administer narcotic analgesics.
 Monitor fluid and electrolyte balance and prevent hypervolemia and hypovolemia.
 Strict intake and output charting is to be done.
 Initially IV fluids are given based on intake and output chart
 Start oral fluids, when condition of the child permits. Administer iron rich diet to the
child and discourage salt intake.
 Daily weight is checked
 Feed the child slowly. Allow for frequent rest between feeding.
 Provide adequate rest post operatively to decrease the work load of the heart
 Ambulation is initiated early , once the chest tubes, arterial lines and assisted
ventilatory equipments are removed
 Activity progresses from sitting on the edge of the bed and dangling the legs to
standing up and to sitting in a chair. Heart rate and respiratory rate are monitored
during activity to assess the degree of cardiac demand
 Help the child to select activities, appropriate for age, condition and ability of the
child.

92.  Provide emotional support to the child and parents.
 Provide adequate information about the treatment plan , medications
,procedures and behaviour of the child
 Explain the parents , child may be angry and uncooperative after the
surgery ( response to pain and restrictions)
 Plan for discharge- provide both verbal and written instructions on
medications, nutrition, activity restrictions, wound care, signs and symptoms
of infection or complications. Also explain the importance of follow up
 Observe the child for any post operative complications like -Infection,
Respiratory distress, Digitalis toxicity, Cardiovascular collapse, Cyanosis,
Cerebral thrombosis , CCF, dysrhythmias, atelectasis, pulmonary edema,
pleural effusion,pneumothorax, bleeding