CONGENITAL HEART DISEASE

Congenital Heart Disease: Tetralogy of Fallot

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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.

Congenital Heart Disease: Tetralogy of Fallot

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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.

Congenital Heart Disease: Tetralogy of Fallot

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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.
Congenital Heart Disease: Tetralogy of Fallot
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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.

Congenital Heart Disease: Tetralogy of Fallot

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What causes congenital heart defects?
Certain chromosomal defects are associated with congenital heart disease like Down’s Syndrome

Congenital Heart Disease: Tetralogy of Fallot

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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

Congenital Heart Disease: Tetralogy of Fallot

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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

Congenital Heart Disease: Tetralogy of Fallot

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Classification of Congenital Heart Diseases
II. Cyanotic Heart Disease (Right to Left Shunts) 1. 2. 3. 4. Tetralogy of Fallot Transposition of the Great Arteries Tricuspid Valve Atresia Truncus Arteriosus

Congenital Heart Disease: Tetralogy of Fallot

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Classification of Congenital Heart Diseases
II. Cyanotic Heart Disease (Right to Left Shunts) 5. 6. 7. Pulmonary Valve Atresia Total Anomalous Pulmonary Venous Return Ebstein Anomaly

Congenital Heart Disease: Tetralogy of Fallot

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Congenital Heart Disease: Tetralogy of Fallot

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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.

Congenital Heart Disease: Tetralogy of Fallot

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TETRALOGY OF FALLOT

Congenital Heart Disease: 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)

Congenital Heart Disease: Tetralogy of Fallot

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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.
Congenital Heart Disease: Tetralogy of Fallot
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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

Congenital Heart Disease: Tetralogy of Fallot

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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
Congenital Heart Disease: Tetralogy of Fallot
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TETRALOGY OF FALLOT
Clinical Manifestations 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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Congenital Heart Disease: Tetralogy of Fallot

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TETRALOGY OF FALLOT
Clinical Manifestations Physical Examination 1. Varying degrees: • Cyanosis • Tachypnea • Clubbing

Congenital Heart Disease: Tetralogy of Fallot

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TETRALOGY OF FALLOT

CYANOSIS
Congenital Heart Disease: Tetralogy of Fallot

CLUBBING
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Congenital Heart Disease: Tetralogy of Fallot

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TETRALOGY OF FALLOT
Clinical Manifestations Physical Examination (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.

Congenital Heart Disease: Tetralogy of Fallot

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TETRALOGY OF FALLOT
Clinical Manifestations Physical Examination
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.

Congenital Heart Disease: Tetralogy of Fallot

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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

Congenital Heart Disease: Tetralogy of Fallot

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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%)
Congenital Heart Disease: Tetralogy of Fallot
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TETRALOGY OF FALLOT

KINDS OF BOOT SHAPED HEART
Congenital Heart Disease: Tetralogy of Fallot
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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

Congenital Heart Disease: Tetralogy of Fallot

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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
Congenital Heart Disease: Tetralogy of Fallot
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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

Congenital Heart Disease: Tetralogy of Fallot

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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
Congenital Heart Disease: Tetralogy of Fallot
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Congenital Heart Disease: Tetralogy of Fallot

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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
Congenital Heart Disease: Tetralogy of Fallot
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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

Congenital Heart Disease: Tetralogy of Fallot

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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

Congenital Heart Disease: Tetralogy of Fallot

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TETRALOGY OF FALLOT
MANAGEMENT
2. Total Correction

Complications
     Bleeding Pulmonary valve regurgitation CHF Right Bundle Branch Block Complete Heart Block

Congenital Heart Disease: Tetralogy of Fallot

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Congenital Heart Disease: Tetralogy of Fallot

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Congenital Heart Disease: Tetralogy of Fallot

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