congenital diseases of the heart

1. Manikandan.T
R.N.R.M.,M.Sc.,(N),D.C.A.,Ph.D
Research Scholar,
VMRF,Salem.

2. MEANING
Congenital heart disease is a
defect in the structure of the heart
and great vessels that is present at
birth.

3. CONGENITAL HEART DISEASE
ACYANOTIC
CYANOTIC

4. ACYANOTIC
Increased pulmonary Obstruction to blood
blood flow flow from ventricle
1. ASD 1. Coartation of aorta
2. VSD 2. Aortic stenosis
3. PDA 3. Pulmonic stenosis
4. Atrio ventricular
canal

5. CYANOTIC
Increased pulmonary Mixed
blood flow blood flow
1. TOF 1. Transposition of
great arteries
2. Tricuspid 2. Total anomalous
atretia
3. epstien anomaly pulmonary venous return
3. Truncus arteriosus
4. Hypoplastic left heart
syndrome

6. INCIDENCE
.
CHD affects 8 to 12 of every 1,000
neonates
In spontaneously aborted and stillborn
fetus, the incidence is much higher

7. ETIOLOGY
90% of the etiology of congenital cardiac
defects is unknown.
Most are thought to be a result of
multifactorial inheritance.

8. Risk factors
Fetal exposure to drugs such as phenytoin and lithium
and radiation.
Maternal viral infections such as rubella.
Maternal metabolic disorders such as phenylketonuria
and insulin-dependent diabetes mellitus.
Maternal complications of pregnancy such as
increased age and antepartal bleeding.
Maternal dietary deficiencies.
Genetic factors
Chromosomal abnormalities like Turner syndrome,
Down syndrome and Trisomy 13 and 18.

9. ACYANOTIC HEART
DISEASES

10. • Refers to disorders that causes flow of blood
from arterial to venous system. These defects
have normal level of oxyhemoglobin
saturation in systemic circulation.

11. DEFECTS WITH INCREASED
PULMONARY BLOOD FLOW
• Intra cardiac communications
• abnormal connection between the great arteries
• Increased blood to flow from the high-pressure
left side of the heart to the low-pressure right
side of the heart increases pulmonary blood
flow

13. DEFINITION
An atrial septal defect (ASD) is an abnormal
opening between right and left atria resulting
in left-to-right shunt of blood.

15. CAUSES
unknown.
Atrial septal defects occur when the
partitioning process does not occur completely,
leaving an opening in the atrial septum.
defect in a gene
a chromosome abnormality
Drugs – alcohol, lithium
Infections- rubella

16. TYPES
Ostium primum atrial septal defect.
Ostium secundum atrial septal defect.
Sinus venosus atrial septal defect.

17. A heart with ostium
secundum atrial septal
defect
A heart with ostium
primum atrial septal
defect
A heart with sinus
venosus atrial septal
defect.

19. PATHOPHYSIOLOGY
Right ventricular failure
Pulmonary hypertension
Pulmonary vascular occlusive
Increased pulmonary blood flow
Left to right shunt
Atrial septal defect

21. CLINICAL FEATURES
• Asymptomatic
• Dyspnea
• Murmer
• Bulging of chest
• Poor weight gain
Child tired easily when playing
Fatigue
Sweating

22. Cont..
Shortness of breath
Cyanosis may occur in case of sinus venosus
defect because; the defect is close to the
opening of superior venacava.
CHF (usually not until the third or fourth
decade of life).

23. DIAGNOSIS
Physical assessment- murmers
Chest X-ray —heart -enlarged
Electrocardiogram (ECG or EKG) —shows
abnormal rhythms (arrhythmias or
dysrhythmias)
Two-dimensional echocardiogram with
Doppler study and color flow mapping- To
identify the site of the ASD and associated
lesions and document left-to-right flow across
the atrial septum.

24. Cont..
Cardiac Catheterization
Cardiac Magnetic Resonance Imaging
(MRI)

25. MANAGEMENT
Specific treatment for ASD will be determined
by the child's physician based on:
The child's age, overall health and medical
history
Extent of the disease
The child's tolerance for specific medications,
procedures or therapies
How the child's doctor expects the disease to
progress
The opinion or preference .

26. Cont..
Medical Management -- Many children have
no symptoms and require no medications,
however some children may need to take
medications to help the heart work better, since
the right side is under strain from the extra
blood passing through the ASD. Medication
that may be prescribed includes the following:
Digoxin
Diuretics
Infection Control: Children with certain heart defects are
at risk for developing an infection of the inner surfaces of
the heart known as bacterial endocarditis.

27. Cardiac Catheterization

28. SURGICAL REPAIR
Ideal age for surgery is in between 2- 5 yrs. With
the use of hypothermia, patient re being operated
below the age of 2 yrs without any risk.
The surgical closure of an ASD is carried out
through an incision in the middle of the chest. A
heart-lung machine is used to do the work of the
heart while the heart is cooled, stopped, emptied and
opened through the right atrium. The hole in the wall
between the right and left atrium is closed with
stitches if it is small,
if too large, with a patch of thin leather-like
material called pericardium, which makes up the sac
covering the heart, cow (bovine) pericardium, or
rarely, a manmade material, Dacron patch. The
right atrium is then closed and the heart is restarted
as the heart-lung machine is withdrawn.

29. Secundum ASD ASD Closed with Patch

30. COMPLICATIONS
CHF (rare).
Infective endocarditis.
Embolic stroke.
Pulmonary hypertension.
Atrial arrhythmias.

31. PROGNOSIS
Although the prognosis is excellent in
asymptomatic people, it's poor in those
with cyanosis caused by large, untreated
defects.

32. 2. VENTRICULAR SEPTAL
DEFECT (VSD)

33. DEFINITION
Ventricular Septal Defect is a congenital
disorder in which blood moves from left
ventricle to right ventricle through a
defective ventricular septum. It may vary
in size from very small defects (Roger’s
defect) to very large defect.

35. INCIDENCE
It is the most common congenital heart defect,
occurring in approximately 30% to 40% of all
children with congenital heart disease.
Many VSDs (20 – 60%) are thought to close
spontaneously.
Spontaneous closure is most likely to occur
during the first year of life in children having
small or moderate defects.

36. CLASSIFICATION
It may be classified according to location:-
Membranous VSD- the defect is located high in
the membranous portion of the ventricular septum
with a variable extension to the adjoining
muscular part of the septum. Anatomically 90%
of the VSD are located in the membranous part of
the ventricular septum.
Muscular VSD- It is located in the muscular
portion of the septum i.e. toward middle and
lower portion of the septum. It has multiple
apparent channels. It can be at central, apical,
marginal and “Swiss cheese” septum- large
number of muscular defects.

37. Classification based on size:-
Restricted VSD- It is a small VSD or
other words, pinhole VSD.
Non- restricted VSD- Large VSD or
some times, there may be even absence
of septum.

38. PATHOPHYSIOLOGY
Due to etiological factors
Defect in the septum of the ventricle
Due to high pressure in the left ventricle, and more
resistance in systemic arterial circulation than
pulmonary circulation and also due to contraction of the
left ventricle before right ventricle, oxygenated blood
shunts from the left ventricle to the right ventricle.
Declining left ventricular pressure and become lower
than aortic pressure.

39. Cont..
Closure of aortic valve.
Continuation of left to right ventricular shunting.
Blood accumulate in the right ventricle and more
amount of blood flows to the pulmonary artery.
More blood reaches Increased blood volume
the lung. in the right ventricle.

40. More blood reaches the lung.
Pulmonary hypertension.
Pulmonary edema.
Long standing pulmonary over circulation.
Increased pulmonary vascular resistance.
Reverse shunt from right to left, aloes known as
Eisenmenger’s complex (it is a sever condition, in which
there is a combination of pulmonary hypertension with
bidirectional or reversal shunting through a VSD or ASD)

41. Increased blood volume in the right ventricle.
Increased thickness and hypertrophy of muscles
of the right ventricle.
Right ventricular enlargement.
Inadequate emptying of the right atrium.
Pooling of blood in systemic circulation.
Peripheral edema.

42. CLINICAL MANIFESTATIONS
Only 15% of VSDs are large enough to cause
symptoms.
Small VSD usually asymptomatic; high
spontaneous closure rate during the first year of
life.
Large VSDs.
CHF: tachypnea, tachycardia, excessive sweating
associated with feeding, hepatomegaly.
Frequent Upper Respiratory Infections.
Poor weight gain, failure to thrive.
Feeding difficulties.
Decreased exercise tolerance.

43. DIAGNOSTIC EVALUATION
Auscultation: harsh systolic regurgitant murmur heard best at the
lower left sternal border (LLSB); systolic thrill felt at LLSB, narrowly
split S2.
Chest X-ray: varies; normal or cardiomegaly and increased pulmonary
vascular markings.
ECG: varies; normal to biventricular hypertrophy.
Two-dimensional echocardiogram with Doppler study and color flow
mapping to identify the size, number, and sites of the defects, estimate
pulmonary artery pressure, and identify associated lesions.
Cardiac catheterization usually not needed for initial diagnosis; may
be needed to calculate the size of the shunt or to assess PVR. May be
performed if defect can be closed using a ventricular occlusion device
(device can be used only in muscular defects). It is also helps to find
out O2 saturation and pressure in the right ventricle.

44. MANAGEMENT
It has been estimated that, about 70-80% of all
VSD become smaller in size or disappear
entirely, and is known as spontaneous closure.
In almost 90% of the patient with spontaneous
closure occurs by the age of 3yrs, though it
may occur as late as 25 yrs or more.
Treatment is conservative when no signs of
congestive heart disease or pulmonary
hypertension are present.

45. SMALL VSD
Medical management:
Usually no anticongestive therapy is needed.
Infective endocarditis prophylaxis for 6 months
after surgical implantation of a ventricular
occlusion device.
Cardiac catheterization for placement of a
ventricular occlusion device for muscular
defects (for Qp:Qs > 2:1).
Surgical intervention is usually not necessary.

46. MODERATE TO LARGE VSD
Medical Management:
CHF management: digoxin and diuretics (furosemide,
spironolactone) and afterload reduction.
Avoid oxygen; oxygen is a potent pulmonary vasodilator and
will increase blood flow into the PA.
Increase caloric intake:
Infective endocarditis prophylaxis for 6 months after
surgery/ventricular device occluder.
Cardiac catheterization for placement of a ventricular
occlusion device for muscular defects (for Qp:Qs > 2:1).

47. Cont..
Refer for surgical intervention.
Usually repaired before age 1.
One-stage approach: preferred surgical plan; patch closure of
VSD.
Two-stage approach: first surgery Pulmonary artery banding
(placing a band around the main pulmonary artery to decrease
pulmonary blood flow) in infants with severe CHF was
common in the past. It is unusual now because improvements
in surgical techniques and post operative care make complete
repair in infancy; second surgery is to patch close the VSD
with the use of Dacron patch and remove the PA band. Both
procedures are performed via cardiopulmonary bypass. The
repair is generally approached through the right atrium and the
tricuspid valve.
LONG-TERM FOLLOW-UP
Monitor ventricular function.
Monitor for sub aortic membrane and double-chamber RV.

48. PULMONARY ARTERY BINDING

49. POST-OPERATIVE COMPLICATIONS
Complete heart block.
By fascicular block
Reopened or Residual VSD
Conduction disturbances

50. COMPLICATIONS OF VSD
CCF
Recurrent respiratory tract infections
Infective endocarditis
Eisenemenger’s syndrome
Pulmonary stenosis
Pulmonary hypertension
Failure to thrive, poor weight gain

51. PROGNOSIS
Highest risk associated with surgical
repair is in the first few months of life.
Children respond well to surgery and
experience substantial “catch up” growth

52. 3. PATENT DUCTUS
ARTERIOSUS (PDA)

53. MEANING
Patent ductus arteriosus (PDA) is a congenital
disorder in heart wherein a neonate's ductus
arteriosus fails to close after birth. The ductus
arteriosus is a passage between two major
blood vessels, the artery which carries oxygen-
poor blood from the heart to the lungs
(pulmonary artery) and the artery which carries
oxygen-rich blood from the heart to the body
(aorta) (The word "patent" means open.)

56. INCIDENCE
• The female-to-male ratio is 2:1
• PDAs are common in premature neonates
who weigh less than 1,500 g.

57. RISK FACTORS / CAUSES
• High chances in children with Low birth weight
and premature infants
• Congenital rubella syndrome (Becoming infected
with rubella (German measles), The rubella virus
crosses the placenta and spreads through the
baby's circulatory system damaging blood vessels
and organs, including the heart.
• Family history and other genetic conditions.
• Other genetic conditions, such as Down
syndrome, have also been linked to an increased
chance of having a PDA.

58. Cont..
• children with a history of perinatal asphyxia (this
usually only delays the closure of the ductus, and,
over time, the ductus typically closes without
specific therapy)
• Children born at high altitude.
• Poorly controlled diabetes during pregnancy.
Uncontrolled diabetes in the mother in turn affects
her baby's blood sugar, causing various damaging
effects to the developing baby.
• PDA is common in babies with congenital heart
problems, such as hypoplastic left heart
syndrome, transposition of the great vessels, and
pulmonary stenosis

59. PATHOPHYSIOLOGY
A patent ductus arteriosus allows a portion of the
oxygenated blood from the left heart to flow back to the
lungs by flowing from the aorta (which has higher
pressure) to the pulmonary artery.
If this shunt is substantial, the neonate becomes short of
breath: the additional fluid returning to the lungs
increases lung pressure to the point that the neonate has
greater difficulty inflating the lungs.
Usage of more calories than normal and often interferes
with feeding in infancy. If not treated, the blood
pressure in the lungs may increase (pulmonary
hypertension) and the heart may weaken.

60. SIGNS AND SYMPTOMS
• It depends on the size of the ducus and
amountin of shunting. While some cases of
PDA are asymptomatic. Most of the time PDA
is detected through routine examination.
Common symptoms include:
• Tachycardia
• Respiratory problems
• Shortness of breath
• Continuous machine-like murmur
• Enlarged heart
• Left subclavicular thrill

61. Cont..
• Bounding pulse, Widened pulse pressure
• Fast breathing
• Sweating while feeding
• Frequent lung infections
• A bluish or dusky skin tone
• Tiring very easily
• Poor growth
• Decreased exercise tolerance.
• Patients typically present in good health, with normal
respirations and heart rate. If the ductus is moderate or
large, widened pulse pressure and bounding
peripheral pulses are frequently present. Prominent
suprasternal and carotid pulsations may be noted
secondary to increased left ventricular stroke volume.

62. DIAGNOSIS
History
• A history of premature birth, perinatal distress, or
perinatal hypoxia may be present.
• Some series have suggested that children born at
extreme altitude have an increased incidence of a
persistent patent ductus arteriosus.
• Occasionally, a history of feeding difficulties and poor
growth during infancy, described as failure to thrive, is
found.
• History of recurrent chest infection and CHF.
• Babies that have a more severe clinical course of
hyaline membrane disease/RDS (respiratory distress
syndrome) may have a higher prevalence of patent
ductus arteriosus. The exact reason for this is unclear.

63. Cont..
Physical Examination
• As many as one third of children with patent
ductus arteriosus are small for their age. In the
presence of significant pulmonary
overcirculation, tachypnea, tachycardia, and a
widened pulse pressure may be found.
• Findings upon cardiac examination include the
following:
– If the left-to-right shunt is large, precordial activity is
increased
– The apical impulse is laterally displaced. A thrill may
be present in the suprasternal notch or in the left
infraclavicular region.

64. Cont..
• The first heart sound (S1) is typically normal. The second
heart sound (S2) is often obscured by the murmur.
• The peripheral pulses are often referred to as bounding
• In the low birthweight premature infant, the classic signs of a
patent ductus arteriosus are usually absent. The classic
continuous murmur is rarely heard. In that case, typically,
precordial activity is increased and peripheral pulses are
bounding. The increased precordial activity is caused by the
large left ventricular stroke volume. Bounding pulses are
caused by the relatively low systemic arterial blood pressure
due to the continuous runoff of blood from the aorta into the
pulmonary artery.

65. Cont..
Non-Invasive Techniques.
• 2 dimensional Echocardiogram with Doppler
study. This test shows the structure of the child’s
heart and the pattern of blood flow through the
PDA, allowing the doctor to determine the size of
the PDA opening and the amount of blood passing
through it.
• Chest X-ray. To check for an enlarged heart or
changes in child's lungs caused by extra blood
flowing through your child's lungs.
• Electrocardiogram (ECG). This test records the
electrical activity of child's heart, detects abnormal
rhythms (arrhythmias or dysrhythmias) and detects
heart muscle stress.

66. Cont..
Invasive Technique
• Cardiac catheterization. This procedure shows
the structures inside the child’s heart. inserts a
small, thin, flexible tube (catheter) into a blood
vessel in the child's groin and guides it to the
child's heart using X-ray imaging. This test
measures blood pressure and oxygen levels in the
child’s heart and arteries. Doctor may inject a dye
into an artery in the heart to make the arteries
visible under X-ray.
• Angiocardiography
• ABG- to know saturation.

67. Cardiac catheterization.

68. TREATMENT
• A small patent ductus arteriosus may close as
the child grows and generally doesn't need
treatment. Symptomatic PDA can be treated
with both surgical and non-surgical methods.
Sometimes an older child or adult may need
treatment for a PDA.
Treatment plan based on:
• Child’s age, overall health and medical history
• The size of the PDA

69. Cont..
Medications- Diuretics, Digoxin, NSAIDS such as
Indomethacin , Ibuprofen
• Fluid restriction with diuretics.
• Digitalis for Congestive Heart Failure
• Doctor may prescribe for newborns and infants a
medication to tighten (constrict) the muscle in the
wall of the patent ductus arteriosus, which may close
the opening. Because Prostaglandin E2 is
responsible for keeping the ductus patent, NSAIDS
(inhibitors of prostaglandin synthesis) such as
indomethacin or a special form of ibuprofen have
been used to help close a PDA. This is an especially
viable alternative for premature infants.

70. Transcatheter procedure
In this cardiac catheterization procedure, inserts a
long thin tube (catheter) into an artery in child's
groin and guides it to child's heart using X-ray
imaging. child's interventional cardiologist then
uses a small device (plug) or coil that is placed
through the catheter to plug the PDA and stop
blood flowing through the PDA. Doctors most
commonly use the transcatheter method to treat a
PDA. Complications includes,
• Bleeding
• Infection
• Movement of the plug or coil from where it was
placed in the heart.

71. Cont..
Surgery
• Surgical procedure is performed any time
between 6 months to 2yrs. In surgery to repair
the PDA (PDA ligation) or transaction of
ductus, surgeon will make an incision in the
left side of the child's chest. Surgeon then will
close the patent ductus arteriosus by tying or
clipping it, stopping the blood flow across the
PDA. Surgery to repair a PDA generally only
is performed in very small children if the child
requires treatment early in life.

72. PDA ligation

73. COMPLICATIONS
If the patent ductus is not closed, the infant has a risk of
developing
• High blood pressure in the lungs (pulmonary
hypertension). It can cause permanent lung damage.
• Heart failure.
• An infection of the heart (endocarditis). It is an
inflammation of the inner lining of the heart caused
by a bacterial infection.
• Irregular heartbeat (arrhythmia). Enlargement of
the heart due to a patent ductus arteriosus increases
the risk of arrhythmias. This increased risk usually
occurs only with a large patent ductus arteriosus.

74. PROGNOSIS
• Sometimes, a PDA may close on its own. Premature babies have a high
rate of closure within the first 2 years of life. In full-term infants, a
PDA rarely closes on its own after the first few weeks.
• Without treatments, the disease may progress from left-to-right
(noncyanotic heart) shunt to right-to-left shunt (cyanotic heart) called
Eisenmenger syndrome.
• If a small PDA remains open, heart symptoms may or may not
eventually develop. Persons with a moderate or large PDA could
eventually develop heart problems unless the PDA is closed.
• Closure with medications can work very well in some situations, with
few side effects. Early treatment with medications is more likely to be
successful.
• Surgery carries its own significant risks. It may eliminate some of the
problems of a PDA, but it can also introduce a new set of problems.
The potential benefits and risks should be weighed carefully before
choosing surgery.

75. 4. ATRIOVENTRICULAR
CANAL

76. MEANING
Atrioventricular canal refers to a
combination of defects in atrial and ventricular
septa and portions of tricuspid and mitral
valves. This defect is associated with Down’s
syndromes. The most complex AV canal
malformation results in one AV valve and large
septal defects between both atria and
ventricles.

78. CLINICAL MANIFESTATIONS
• Severity of symptoms depends on amount of
mitral regurgitation.
• Congestive heart failure
• Poor growth
• Repeated respiratory failure
• Systolic murmur
• Mild cyanoses

79. DIAGNOSTIC EVALUATION
• Chest x-ray – appears large and pulmonary
vascular markings are present.
• Echocardiogram – presence of septal defects
and details of valvular malformations.
• Cardiac catheterizations – performed to
evaluate pulmonary hypertension and
pulmonary resistance.

80. MEDICAL MANAGEMENT
Surgery is performed during infancy to
prevent pulmonary vascular disease. Oxygen
may be required until surgery.

81. SURGICAL MANAGEMENT
PALLIATIVE
• Pulmonary artery banding for infants with
severe symptoms that are caused by increased
pulmonary blood flow in some centers. Most
centers perform complete repair in infancy.
COMPLETE REPAIR
• Surgical repair consists of patch closure of the
septal defects and reconstruction of the AV
valve tissue. If the mitral valve defect is
severe, a valve replacement may be needed.

82. POSTOPERTAIVE COMPLICATIONS
• Heart block
• Congestive heart failure
• Mitral regurgitation
• Dysrhythmias
• Pulmonary hypertension

83. PROGNOSIS
• Operative mortality is less than 10%.
• Mitral regurgitation, may require valve
replacement.

84. PREVENTION OF CONGENITAL
HEART DISEASES
• Preventing preterm deliveries
• Get early prenatal care, even before becoming
pregnant
• Eat a balanced diet.
• Exercise regularly.
• Avoid risks.
• Avoid infections.
• Keep diabetes under control.

85. LIFESTYLE AND HOME REMEDIES
• Preventing infection. For most people with a patent
ductus arteriosus, regularly brushing and flossing teeth
in combination with getting regular dental checkups is
the best way to help prevent infection. In some cases,
need to take preventive antibiotics before certain dental
and surgical procedures.
• Exercising and play. Parents of children with
congenital heart defects often worry about the risks of
rough play and vigorous activity even after successful
treatment. Although some children may need to limit
the amount or type of exercise, most children with
patent ductus arteriosus will lead normal lives.

86. NURSING DIAGNOSIS
Decreased cardiac output related to structural
defect, myocardial dysfunction.
Ineffective breathing pattern related to pulmonary
congestion.
Fluid volume excess related to fluid accumulation
(edema).
Activity intolerance related to imbalance between
oxygen supply and demand.
Risk for infection related to reduce body defenses,
pulmonary congestion.
Altered family process related to a child with a
life threatening illness.

87. THANK YOU..