Congenital Heart Disease

CONGENITAL
HEART
DISEASE
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Congenital Heart Disease: Tetralogy of Fallot Thank You Files
CONGENITAL HEART DISEASE
DEFINITION
It is a heart-related problem that is present since birth
and often as the heart is forming even before birth. It
involves one or more defects in the heart, the heart’s
valves, the veins leading to the heart, the arteries
leaving the heart or the connections among these
various parts of the body.
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Congenital Heart Disease
Incidence
The incidence of CHD in the general population is about

1% or, more precisely, 8 to 12 of 1000 live births.
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What causes congenital heart disease?
In most cases, scientists do not know what makes a baby's
heart develop abnormally.
Both genetic and environmental factors appear to play roles.
Environmental factors: viruses, certain drugs, radiation, living

in high altitudes.
Women who contract rubella (German measles) during the

first three months of pregnancy have a high risk of having a
baby with a heart defect. Other viral infections also may
contribute.
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What causes congenital heart defects?
Certain medications also increase the risk.
Drinking alcohol in pregnancy also can increase the risk of heart

defects—babies with fetal alcohol syndrome (FAS) often have
them.
Studies also suggest that use of cocaine in pregnancy increases
the risk of these birth defects.
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What causes congenital heart defects?
Certain chromosomal defects are associated with

congenital heart disease like Down’s Syndrome
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Classification of Congenital Heart
Diseases
I. Acyanotic Heart Disease
A. Left to Right Shunts

1. Ventricular Septal Defect
2. Atrial Septal Defect
3. Patent Ductus Arteriosus
4. Atrio-Ventricular Canal Defect
5. Partial Anomalous Venous Return
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Classification of Congenital Heart
Diseases
I. Acyanotic Heart Disease (Cont. 2)
B. Obstructive Lesions
1. Pulmonary Stenosis
2. Aortic Stenosis
3. Tricuspid Stenosis
4. Mitral Stenosis
5. Coarctation of the Aorta
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Classification of Congenital Heart Diseases
II. Cyanotic Heart Disease (Right to Left Shunts)
1. Tetralogy of Fallot
2. Transposition of the Great Arteries
3. Tricuspid Valve Atresia
4. Truncus Arteriosus
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Classification of Congenital Heart Diseases
II. Cyanotic Heart Disease (Right to Left Shunts)
5. Pulmonary Valve Atresia

6. Total Anomalous Pulmonary Venous
Return
7. Ebstein Anomaly
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TETRALOGY OF FALLOT
INCIDENCE
8%- 10% of all congenital heart disease
Most common CHD with cyanosis after 1 year of life
Occurs equally in boys and in girls.
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TETRALOGY OF FALLOT
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TETRALOGY OF FALLOT
PATHOLOGY
The four abnormalities shown on the right characterize

this fairly common condition:
1. Ventricular septal defect, large
2. Narrowing of the valve leading to the

pulmonary arteries (pulmonic stenosis)
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TETRALOGY OF FALLOT
PATHOLOGY
The four abnormalities shown on the right

characterize this fairly common condition:
(Cont.)
3. The aorta "overrides" the ventricular

septal defect.
4. There is thickening (hypertrophy) of

the right ventricle.
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TETRALOGY OF FALLOT
Associated Conditions
 Bicuspid Pulmonary Valve (40%)

 Stenosis of Left Pulmonary Artery (40%)
 Right sided Aortic Arc (25%)
 TE Fistula
 Down Syndrome (Trisomy 21%)
 Anomalies of Coronary Arteries (10%)
 Forked ribs, scoliosis
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TETRALOGY OF FALLOT
Clinical Manifestations
HISTORY
1. A systolic ejection murmur

audible at birth
2. Most patients are symptomatic

with cyanosis at birth.
Dyspnea on exertion, squatting,
or hypoxic spells
develop later, even in mildly
cyanotic infants
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TETRALOGY OF FALLOT
HISTORY
3. Infants with acyanotic TOF may be

asymtomatic or may show signs of CHF
from large lefty top right ventricular
shunt
4. Immediately after birth, severe cyanosis

is seen in all
patients with TOF and pulmonary
atresia.
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TETRALOGY OF FALLOT
Physical Examination
1. Varying degrees:
• Cyanosis
• Tachypnea
• Clubbing
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TETRALOGY OF FALLOT
CYANOSIS CLUBBING
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TETRALOGY OF FALLOT
(Cont.)
2. An RV tap along the left sternal

border and systolic thrill at the
upper and middle sternal borders
are commonly present (50%).
3. An ejection click that originates in

the aorta may be audible.
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TETRALOGY OF FALLOT
4. The S2 is usually single (aortic component).
5. A long, loud systolic ejection murmur at the mid,

upper left sternal border.
6. In the acyanotic form, a long systolic murmur,

resulting from VSD and infundibular stenosis, is
audible along the entire left sternal border, and
cyanosis is absent.
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TETRALOGY OF FALLOT
LABORATORY EXAMINATION
Electrocardiography
o Right axis deviation (RAD) (+120 to 150 degrees)

in cyanotic TOF
o Normal QRS axis in acyanotic TOF
o RVH is usually present
o RAH is occasionally present
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TETRALOGY OF FALLOT
LABORATORY EXAMINATION
Chest X-ray studies
 Cyanotic TOF
 Heart size normal
 Decrease pulmonary vascular
markings
 Concave main PA segment with
upturned apex (boot shaped heart
or coeur en sabot)
 RAE (25%)
 Right Aortic Arch (25%)
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TETRALOGY OF FALLOT
KINDS OF BOOT SHAPED HEART

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TETRALOGY OF FALLOT
NATURAL HISTORY
1. 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 atenosis and
polycythemia.
2. Polycythemia or hyperviscosity syndrome

(HCT > 0.65)
3. Relative Iron deficiency
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TETRALOGY OF FALLOT
NATURAL HISTORY
4. Hypoxic spell in infants
5. Growth retardation
6. Brain abscess and CVA
7. Sub-acute Bacterial Endocarditis
8. Aortic Regurgitation
9. Coangulopathy
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TETRALOGY OF FALLOT
MANAGEMENT
Medical
1. Physicians should recognize and treat hypoxic spell

2. Oral propranolol therapy, 0.5 to 1.5 mg/kg every 6
hours
3. Maintenance of good dental hygiene and SBE
antibiotic prophylaxis
4. Relative iron deficiency anemia should be detected
and treated
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TETRALOGY OF FALLOT
MANAGEMENT
Surgical
1. Palliative Shunt Procedures (Blalock-Taussig

Shunt) -Done to increase pulmonary blood flow
Indications:
- Neonates with TOF and pulmonary atresia
- Infants with hypoplastic pulmonary annulus
- Hypoplastic PA’s
- Severely cyanotic infants younger in 3 months of age
- Frequent hypoxic spells in infants
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TETRALOGY OF FALLOT
MANAGEMENT
Surgical
2. Total Correction
• Symptomatic infants with favorable anatomy of

RVOT and PA’s after 3 to 4 months of age
• Asymptomatic and minimally cyanotic children may
have the repair between 3 to 24 months of age
depending on the degree of annular and PA
hypoplasia
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TETRALOGY OF FALLOT
MANAGEMENT
Surgical
2. Total Correction
• Mildly cyanotic infant who have had previous shunts

surgery
• Asymptomatic and acyanotic children have the
operation at 1 to 2 years of age
• Asymptomatic children with coronary artery
anomalies may have a repair in 3 to 4 years
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TETRALOGY OF FALLOT
MANAGEMENT
2. Total Correction
Procedures
 Carried out under cardio pulmonary bypass and

circulatory arrest
 Procedure includes
- patch closure of the VSD
- widening of the RVOT
- placement of a fabric patch
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TETRALOGY OF FALLOT
MANAGEMENT
2. Total Correction
Complications
 Bleeding
 Pulmonary valve regurgitation
 CHF
 Right Bundle Branch Block
 Complete Heart Block
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Congenital Heart Disease

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CONGENITAL

The incidence of CHD in the general population is about

Both genetic and environmental factors appear to play roles.

Environmental factors: viruses, certain drugs, radiation, living

Women who contract rubella (German measles) during the

Drinking alcohol in pregnancy also can increase the risk of heart

Certain chromosomal defects are associated with

I. Acyanotic Heart Disease

A. Left to Right Shunts

I. Acyanotic Heart Disease (Cont. 2)

II. Cyanotic Heart Disease (Right to Left Shunts)

II. Cyanotic Heart Disease (Right to Left Shunts)

5. Pulmonary Valve Atresia

Most common CHD with cyanosis after 1 year of life

Occurs equally in boys and in girls.

The four abnormalities shown on the right characterize

1. Ventricular septal defect, large

2. Narrowing of the valve leading to the

The four abnormalities shown on the right

3. The aorta "overrides" the ventricular

4. There is thickening (hypertrophy) of

 Bicuspid Pulmonary Valve (40%)

1. A systolic ejection murmur

2. Most patients are symptomatic

3. Infants with acyanotic TOF may be

4. Immediately after birth, severe cyanosis

2. An RV tap along the left sternal

3. An ejection click that originates in

4. The S2 is usually single (aortic component).

5. A long, loud systolic ejection murmur at the mid,

6. In the acyanotic form, a long systolic murmur,

o Right axis deviation (RAD) (+120 to 150 degrees)

o Normal QRS axis in acyanotic TOF

o RVH is usually present

o RAH is occasionally present

Chest X-ray studies

KINDS OF BOOT SHAPED HEART

1. Infants with acyanotic TOF gradually become

2. Polycythemia or hyperviscosity syndrome

3. Relative Iron deficiency

4. Hypoxic spell in infants

6. Brain abscess and CVA

7. Sub-acute Bacterial Endocarditis

1. Physicians should recognize and treat hypoxic spell

1. Palliative Shunt Procedures (Blalock-Taussig

• Symptomatic infants with favorable anatomy of

• Mildly cyanotic infant who have had previous shunts

 Carried out under cardio pulmonary bypass and

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