VENTRICULAR SEPTAL DEFECT.ppt

Heart diseases affecting paediatric population

1. VENTRICULAR
SEPTAL DEFECT
Dominic Vasco
School of Medicine & Health Sciences
Department of Clinical Medicine
Paediatrics and Child
Health III
Kabarak University Department of Clinical
Medicine

2. Learning outcomes
At the end of this lecture series the learner
should;
1. Define malnutrition and classify the
various malnutrition disorders
2. Describe the diagnosis of nutritional
disorders
3. Describe the admission criteria for
malnourished children
4. Outline the management of malnutrition
Kulimankudya Vasco Botso

3. Ventricular septal defect exists as an
opening in the ventricular septum
It 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
They are the most common type of
congenital heart defect occurring in 14 to
17% of babies born each year
Kulimankudya Vasco Botso

4. It follows failure of completion of the
partitioning process in the ventricular
septum
Kulimankudya Vasco Botso

5. Effect
Blood passes through the VSD from the
left ventricle to the right ventricle causing
the right side of the heart to handle a
larger volume of blood
The extra blood then passes through the
pulmonary artery into the lungs
This leads to pulmonary hypertension and
pulmonary congestion
Kulimankudya Vasco Botso

6. Pulmonary arteries become thickened and
obstructed due to the increased pressure
Failure to repair the defect leads to lung
disease with the pressure in the right heart
eventually exceeding the pressure in the
left creating a right to left shunt
Kulimankudya Vasco Botso

7. Due to high pressure there is tissue
damage which may occur in the right
ventricle and bacteria in the blood stream
can easily infect this area causing
bacterial endocarditis
Kulimankudya Vasco Botso

8. Clinical features
Small VSDs are asymptomatic with the
child attaining normal growth and
development
Moderate to large VSDs are associated
with delayed growth and development,
decreased exercise tolerance, recurrent
pulmonary infections and Congestive
Heart Failure during infancy
Kulimankudya Vasco Botso

9. Clinical features…
Longstanding pulmonary hypertension is
accompanied by a history of cyanosis and
decreased level of activity
Kulimankudya Vasco Botso

10. Physical Examination
Infants with small VSDs are well
developed and acyanotic
Infants with large VSDs may have poor
weight gain before 2 or 3 months of age
They may also develop CHF
Cyanosis and clubbing may be present in
patients with Eisenmenger syndrome
Kulimankudya Vasco Botso

11. A systolic thrill may be present at the
lower left sternal border with a large shunt
VSD
A precordial buldge 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
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12. A grade 2 systolic murmur is audile at the
lower left sternal border, it may be
holosystolic or early systolic
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13. Electrocardiography
ECG is normal in small VSD
Moderate VSDs cause left ventricular
hypertrophy and ocassional left atrial
hypertrophy may be seen
Large defects reveal a biventricular
hypertrophy with or without Left Atrial
Hypertrophy
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14. If pulmonary vascular disease develops
the ECG shows Right Ventricular
Hypertrophy only
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15. Chest Xray
Cardiomegally of varying degrees is
present and involves the Left atria, left
ventricle and sometimes the right ventricle
Increased pulmonary vascular markings
The degree of cardiomegaly and the
increase in pulmonary vascular markings
directly relate to the magnitude of the left
to right shunt
Kulimankudya Vasco Botso

16. 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 years 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 resulting right to left shunt usually
does not develop until teenage years
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17. Infective endocarditis rarely occurs
Kulimankudya Vasco Botso

18. Medical management
Treatment of CHF if it develops;
Digoxin
Diuretics
Given for 2 –4 months
Spironolactone may be useful to
minimize potassium loss
Captopril may be given concomitantly as
an afterload reducing agent
Kulimankudya Vasco Botso

19. Frequent feeding on high calorie diet
orally or by NGT
Oral iron therapy is used to correct
anaemia
No exercise restriction is needed in the
absence of pulmonary hypertension
Maintenance of good dental hygiene and
antibiotic prophylaxis against infective
endocarditis are important
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20. Non surgical closure of selected muscular
VSDs is possible using the umbrella
device, this is still in experimental phase
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21. Surgical Management
Indications;
Small infants with large VSDs who
develop CHF and growth retardation -If
growth failure cannot be improved by
medical management the VSD should be
operated on within the first 6 months of life
If the Pulmonary artery pressure is greater
that 50% of systemic pressure, surgical
closure should be done by the end of the
first year
Kulimankudya Vasco Botso

22. After 1 year of age a significant left to right
shunt should be surgically closed
Kulimankudya Vasco Botso

23. Surgical treatment
Palliative;
Pulmonary artery banding involves placing
a band around the pulmonary artery to
decrease the pulmonary blood flow
It increases the resistance to blood flow
through the pulmonary artery
The pressure increases in the right
ventricle and prevents excess shunting
from left to right
Kulimankudya Vasco Botso

24. Complete repair
Small defects are repaired with a purse
string approach
Large defects usually require a knitted
Dacron patch sewn over the opening
Post operative complications include
residual VSD and conduction disturbance
Kulimankudya Vasco Botso

25. 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
Kulimankudya Vasco Botso

26. Post –op follow up
Activity should not be restricted unless
complications have resulted from surgery
Bacterial endocarditis prophylaxis may be
discontinued 6 months after surgery
Patients with post operative history of
transient heart block wit or without a
pacemaker requires long term follow up
Kulimankudya Vasco Botso

27. PATENT DUCTUS
ARTERIOSUS

28. This is 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
Kulimankudya Vasco Botso

29. 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 pulmonary artery
and mixes with the unoxygenated blood
which goes to the lungs
Kulimankudya Vasco Botso

30. This increases the blood volume in the
lungs causing congestion and pulmonary
hypertension
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31. Kulimankudya Vasco Botso

32. Kulimankudya Vasco Botso

33. In many children there is no known reason
for the ductus arteriosus remaining open
PDA is seen more often in the following;
Premature infants
Infants born to a mother who had rubella during
the first trimester of pregnancy
Kulimankudya Vasco Botso

34. Incidence
PDA occurs in 6 –11% of all children with
Congenital Heart Disease
In many children, there is no known
reason for the ductus arteriosus remaining
open
However, PDA is seen more often
Kulimankudya Vasco Botso

35. Clinical features
History
Patients are usually asymptomatic when
the ductus is small
A large shunt PDA may cause a lower
respiratory tract infection, atelectasis and
congestive heart failure accompanied by
tachypnea, and poor weight gain
Kulimankudya Vasco Botso

36. Physical Examination
Tachycardia and tachypnea may be
present in infants with Congestive Heart
Failure
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 murmur continuous
machinery murmur is best heard at the left
infraclavicular area or upper left sternal
border
Kulimankudya Vasco Botso

37. If pulmonary vascular obstructive disease
develops, a right to left ductal shunt
results in cyanosis only in the lower half of
the body
Kulimankudya Vasco Botso

38. Electrocardiography
ECG findings are similar to those of VSD
A normal ECG or Left Ventricular
Hypertrophy is seen with small to
moderate PDA
If pulmonary vascular obstructive disease
develops, Right Ventricular Hypertrophy is
present
Kulimankudya Vasco Botso

39. Chest X ray
The findings may be normal in case of a
small shunt
Cardiomegally of varying degrees occurs
in moderate-to large-shunt PDA with
enlargement of the left atria, left ventricle
and ascending aorta
Pulmonary vascular markings are present
Kulimankudya Vasco Botso

40. Echocardiography
It assesses the size of the ductus
Kulimankudya Vasco Botso

41. Natural history
Spontaneous closure of PDA does not
usually occur in full term infants and
children
This is due to the PDA in infants being as
a result of a structural abnormality of the
ductal smooth muscle
Congestive heart failure or recurrent
pneumonia or both develop if the shunt is
large
Kulimankudya Vasco Botso

42. Pulmonary vascular obstructive disease
may develop if a large PDA with
pulmonary hypertension is left untreated
Infective endocarditis may also occur
An aneurysm of PDA may occur and
rapture later on in life
Kulimankudya Vasco Botso

43. Management
In term neonates;
Indomethacin is ineffective in term infants
with PDA and should be avoided
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 is indicated
Kulimankudya Vasco Botso

44. Non surgical closure
Small ductus less than 4 mm in diameter are
closed by coils
Larger ones are closed by an amplatzer PDA
device
Kulimankudya Vasco Botso

45. Surgical closure
This is reserved for patients in whom non
surgical closure is not considered
applicable
Kulimankudya Vasco Botso

46. Management in preterm
neonates
Clinical evidence of PDA appears in 45%
of infants with birth weight of less than
1750g and about 80% of infants with birth
weight less than 1200 g
Significant PDA with CHF occurs in 15%
of premature infants with birth weight of
less than 1750 g and in 40% to 50% of
those with birth weight less than 1500 g
Kulimankudya Vasco Botso

47. Medical;
Fluid restriction to 120 mls/ day and
diuretic e.g. Furosemide 1mg/kg per day
two to three times a day may be tried for
24 to 48 hours
Pharmacologic closure of the PDA can be
achieved with indomethacin (a
prostaglandin synthetase inhibitor)
Kulimankudya Vasco Botso

48. 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 two times
For those 2 to 7 days old 0.2 mg/kg times
three and for infants older than 7 days 0.2
mg/kg followed by 0.25 mg/kg times 2
Kulimankudya Vasco Botso

49. ARTRIOVENTRICULAR
CANAL DEFECT

50. Also termed endocardial cushion defect
Accounts for about 5% of all congenital
heart diseases
They are most common in infants with
Downs syndrome
15% of infants with Downs syndrome have
ASDs
Kulimankudya Vasco Botso

51. Complete atrioventricular canal
This is a severe defect characterized by a
large communication in the septum which
separates the left and the right sides of
the heart
The hole is in the centre of the heart
where the upper and lower chambers
meet
In a child with a complete atrioventricular
canal there is one valve and it may not
close correctly
Kulimankudya Vasco Botso

52. Partial atrioventricular canal
defects
The communication does not extend
between the lower chambers of the heart
and the valves are better formed
It is also called atrioventricular septal
defect
Kulimankudya Vasco Botso

53. Kulimankudya Vasco Botso

54. Kulimankudya Vasco Botso

55. Pathophysiology
The defect in the septum allows blood to
travel from the left side of the heart to the
right side or the other way around
There is mixture of oxygenatedand
deoxygenatedblood
Extra blood gets pumped into the lungs
demand an increased function of the heart
and lungs and the lungs become
congested
Kulimankudya Vasco Botso

56. Clinical features
Dyspnoea
Mild cyanosis
Newborns show signs of heart failure;
Oedema
Fatigue
Wheezing
Sweating
Irregular heartbeat
Kulimankudya Vasco Botso

57. They present with a characteristic murmur
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58. Management
Palliative pulmonary artery banding
Complete repair;
Patch closure of septal defect
Reconstruction of AV tissue
Kulimankudya Vasco Botso

59. COARCTATION OF THE
AORTA

60. This refers to narrowing of the aorta
It occurs anywhere but is most likely to
occur at the segment just after the aortic
arch
The narrowing restricts the amount of
blood going to the lower segment of the
body
It occurs in 8 –11% of children with
congenital heart disease
Kulimankudya Vasco Botso

61. Effects of coarctation
The left ventricle has to work harder to
overcome the resistance and pump blood
through the narrowing in the aorta
This results in left sided heart failure
The blood pressure is higher above the
narrowing and lower below the narrowing
Kulimankudya Vasco Botso

62. Effects of coarctation…
Older children experience headaches from
too much pressure in the blood vessels in
the head or cramps in the legs or
abdomen due to too little blood in that
region
Over time the walls of the arteries may
become weakened resulting in tears and
bleeding or CVA
There is an increased risk of endocarditis
Kulimankudya Vasco Botso

63. Kulimankudya Vasco Botso

64. Kulimankudya Vasco Botso

65. Clinical features
History in the first 6 weeks of life;
Poor feeding
Dyspnoea
Poor weight gain
Acute circulatory shock
Kulimankudya Vasco Botso

66. Physical Examination;
Infants are pale with varying degrees of
respiratory distress
Oliguria
Anuria
General circulatory shock
Acidaemia
Kulimankudya Vasco Botso

67. Peripheral pulses may be weak and
thread due to congestive heart failure
The S2 is single and loud with a loud S3
gallop often being present
No heart murmur is present in 50% of
patients
A non specific ejection systolic murmur is
audible over the precordium
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68. Electrocardiography
A normal or rightward QRS axis and Right
Heart Failure or RBBB are present
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69. Chest X ray
There is marked cardiomegaly and pulmonary
oedema or pulmonary venous congestion
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70. Echocardiography
This demonstrates the site of the
coarctation
A suprasternal notch view reveals a thin
wedge shaped “posterior shelf” in the
posterolateral aspect of the upper
descending aorta
Kulimankudya Vasco Botso

71. Natural history
About 20% -30% of all patients with
coarctation develop CHF by three months
of age
Undetected or untreated coarctation may
lead to early death due to CHF and renal
shut down in symptomatic patients
Kulimankudya Vasco Botso

72. Management
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
Antifailure regimen with short acting
inotropic agents such as doparmine and
dobutamine, diuretics and oxygen should
be initiated
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73. Baloon valvuloplasty is useful in children
whom standard surgical procedure carries
a great risk
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74. Surgical management
Its indicated if CHF develops early in life
when surgery is performed as an urgent
procedure
The coarctation segment is resected and
the proximal and distal aorta are
anastomosed
Kulimankudya Vasco Botso

75. Coarctation with hypertension in the upper
extremities or with a large systolic
pressure gradient ≥ 20 mmHg between
the arms and legs indicates that an
elective surgical correction is necessary
between the ages of 4 and 6 years
Kulimankudya Vasco Botso

76. Reduction of the aorta by 50% at the level
of Coarctation is also indicative of surgery
Older children should be operated as soon
as the diagnosis is made
In asymptomatic children surgery is
performed by age 4 to 5 as delaying
surgery increases the chances of
developing essential hypertension
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77. In case severe hypertension, CHF or
cardiomegaly are present surgery should
be performed early
Kulimankudya Vasco Botso

78. Surgical procedures
Resection of the aortic segment and end
to end anastomosis
Occasionally a subclavian artery
aortoplasty or circular or patch grafts may
be performed
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79. AORTIC STENOSIS