CONGENITAL HEART DEFECTS (CHD) -

These are structural defect/s of the

heart; great vessels or both that is
present from birth
- Second to prematurity as a cause of
death in the first year of life
Four Classifications of CHD:
1. Defects with increased pulmonary blood
flow • Patent Ductus Arteriosus (PDA)
• Atrial Septal Defect (ASD)
• Ventricular Septal Defect (VSD)
• Atrioventricular Canal Defect (AV canal)

2. Obstructive Defect
 • Coarctation of the Aorta (CoA)
• Aortic Stenosis (AS)
• Pulmonic Stenosis
3. Defects that decrease pulmonary blood flow
• Tricuspid Atresia (AT)
• Tetralogy of Fallot (TOF)
- VSD, PS, OA, RVH

4. Mixed Defects
• Transposition of Great Vessels (TGV) •
Truncus Arteriosus
• Hypoplastic Left Heart Syndrome
Exact Cause: Unknown
Predisposing Factors:
• Fetal and Maternal Infection during 1st
trimester especially rubella
• Maternal Alcoholism
• Maternal Age over 40 years
• Maternal Dietary Deficiencies
• Maternal Insulin-dependent induced
diabetes
• Siblings with CHD
• Parent with CHD
• Chromosomal Abnormality, e.g., Trisomy 21 •
Teratogenic Drugs: Thalidomide,
Antiviral/Antibiotics, Antiepileptics
TYPES OF CONGENITAL HEART DEFECT
 Acyanotic
- Left to right shunting
- Normal Skin color
- Normal CNS function
- Position: Normal/Orthopneic
- Small stature, FTT
- Possible exercise intolerance
- ↑ frequency of respiratory
infection
Cyanotic
- Right to left shunting
- Cyanosis
- With CNS alterations, e.g.,
seizures, fainting, confusion
(due to cerebral hypoxia)
- Position: squatting
- Small stature, FTT
- Marked exercise intolerance
- Frequent and severe
respiratory infection
- May have hypoxic spells
ACYANOTIC
- Mixing of oxygenated and unoxygenated blood
in the pulmonary circulation resulting to
decreased oxygenated blood in the systemic
circulation

PATENT DUCTUS ARTERISUS

ATRIAL SEPTAL DEFECTS
PULMONIC STENOSIS
AORTIC STENOSIS
COARCTATION OF THE AORTA
ATRIOVENTICULAR CANAL
DEFECT VENTRICULAR SEPTAL
DEFECT
 VENTRICULAR SEPTAL DEFECT
- The most common acyanotic CHD
characterized by an abnormal opening
between the right and left ventricles. The
degree of this defect may vary from a
pinhole to an absent septum
Types of VSD:
• Low Septum VSD
- Defect is small, may spontaneously close, no
treatment is needed

• High Septum VSD

- Large defect, does not close spontaneously,
 requires surgical intervention to close the
defect

A
B
Clinical Manifestations:
• Symptoms vary with the size of the defect,
 age and amount of resistance; usually
asymptomatic
• LOUD, HARSH SYSTOLIC MURMUR at the
left sternal border 3RD to 4TH ICS: dyspnea;
tachypnea
• FTT, excessive sweating, fatigue
• Child may be more susceptible to
respiratory infections
• S/sx of CHF
Diagnostics:
• CXR, ECG
• Echocardiography (2D Echo) – confirmatory test
Medical and Surgical Management:
✓ Some VSD’s may close spontaneously ✓ Others
 are closed with a DACRON PATCH requiring CP
bypass (recommended for children with large
defects, pulmonary artery HPN, CHF, recurrent
infection, FTT
✓ PULMONARY ARTERY BANDING – palliative
procedure for poor surgical candidates
ATRIAL SEPTAL DEFECTS
- An abnormal communication between the two
atria. Results when the atrial septal tissue does
not fuse properly during embryonic
development; severity depends on the size and
location
Clinical Manifestations:
• Most infant tend to be asymptomatic until early
 childhood and many defects close spontaneously
by 5 years of age
• Symptoms vary with the size of the defect; fatigue
and dyspnea on exertion are the most common • Slow
weight gain and frequent respiratory infection •
Systolic ejection murmur may be auscultated, usually
most prominent at the 2nd ICS (pulmonic area)
• Split S2
Medical and Surgical Management:
✓ Surgical closure by SUTURE or DACRON PATCH
which requires CP bypass and is usually
performed during school age. Closure via cardiac
catheterization may be done using a special coil
or umbrella
PATENT DUCTUS ARTERIOSUS
• Results when the fetal ductus arteriosus fails to
close completely after birth
• Most common anomaly with mothers who were
infected by rubella during the 1st trimester
Clinical Manifestations:
 • If the defect is small, the child maybe
asymptomatic
• A loud machine-like murmur accompanied by a
thrill
• Frequent respiratory infections
• Poor feeding, fatigue, hepatosplenomegaly,
poor weight gain, tachypnea, tachycardia,
irritability
• Widened pulse pressure and bounding pulse •
s/sx of CHF
Medical and Surgical Management:
• Administration of Prostaglandin synthetase
Inhibitors (INDOMETHACIN) in premature infants •
SURGICAL LIGATION of PDA via left thoracotomy
without CP bypass or Video-assisted Thoracoscopic
surgery
COARCTATION OF THE AORTA
- Involves a “localized narrowing” of the aorta

Three types of CoA:

❖ Preductal

❖ Postductal

❖ Juxtaductal
Clinical Manifestations:
- Child may be asymptomatic or may experience the
 classic difference in BP and pulse quality between
upper and lower extremities
- Epistaxis, headaches, fainting, lower leg muscle
cramps
- Systolic murmur may be heard over the left anterior
chest and between the scapula posteriorly - Rib
notching may be observed in an older child
Diagnostics:
• ECG
• 2D-Echo
• CXR

Medical and Surgical Management:

✓ Repair includes surgical removal of the stenotic
 area; bypass surgery is not necessary because
repair takes place outside the heart
✓ Nonsurgical repair via Balloon Angioplasty
PULMONIC STENOSIS
- Involves obstruction of blood flow from the
right ventricle into the pulmonary artery due
to narrowing of the pulmonic valve
Clinical Manifestations:
• A child may be asymptomatic or may have a
mild CHF
• A systolic mumur may be heard over the
pulmonic area; a thrill may be palpated if
stenosis is severe
• In severe cases, decreased exercise
 tolerance, dyspnea, precordial pain and
generalized cyanosis may occur
Diagnostics:
• ECH/CXR
• Cardiac Catheterization

Medical and Surgical Management • Balloon

Angioplasty techniques • Surgical
Valvulotomy/Valvolotomy may be performed
AORTIC STENOSIS
- Defect that primarily involves an obstruction
of aortic valve resulting to decreased cardiac
output
- More common in males/boys
 Clinical Manifestations:
• The child with a severe defect may have a faint
pulse, hypotension, tachycardia and a poor
feeding pattern
• The child may experience signs of exercise
intolerance, chest pain and dizziness when
standing for long periods
• A systolic ejection murmur may be heard at the
2nd ICS
Medical and Surgical Management:
• If the child symptoms warrant, surgical aortic
valvulotomy or prosthetic valve replacement is
necessary
• Balloon angioplasty can be used to dilate the
 narrow valve
ATRIOVENTRICULAR CANAL DEFECT
• Incomplete fusion of the endocardial cushion •
Common in trisomy 21
• Shunting: Left to right although blood may flow
among all heart chambers

S/sx: Same with ASD

Tx: PAB, Sx closure and Valve repair/replacement

CYANOTIC
- Poorly oxygenated venous blood mixes with
oxygenated blood in the systemic
circulation
 TETRALOGY OF FALLOT
TRANSPOSITION OF THE GREAT
VESSELS TRUNCUS ARTERIOSUS
TOTAL ANOMALOUS VENOUS
RETURN TRICUSPID ATRESIA
TETRALOGY OF FALLOT
- Most common form of cyanotic CHD
characterized by the presence of 4 defects
namely:
1. Pulmonic Stenosis (PS)
2. Right Ventricular Hypertrophy (RVH)
3. Overriding Aorta (OA)
4. Venticular Septal Defect (VSD)
 Clinical Manifestations:
• Acute episodes of cyanosis (TET SPELLS) and transient
cerebral ischemia
• Cyanosis occurring at rest (as PS worsens) • SQUATTING
(a characteristic posture of older children that serves to
decrease the return of venous blood from the lower
extremities and to increase systemic vascular resistance,
which increases pulmonary blood flow and ease
respiratory effort)
• Slow weight gain
• Clubbing of fingers, exertional dyspnea, fainting or
fatigue
• Pansystolic mumur may be heard at the mid-lower left
sternal border
• Polycythemia
Diagnostics:
• 2D-Echo
• Cardiac catheterization •
Angiography
• CBC
• ABG’s
Medical and Surgical Management: ✓
Elective repair is usually performed during
the infant’s first year of life; but palliative
repairs may be warranted for infants who
cannot undergo primary repair
✓ Total repair involves VSD closure,
infundibular stenosis, resection and
 pericardial patch to enlarge right ventricular
outflow tract
TRANSPOSITION OF THE GREAT VESSELS
(TOGV/TOGA)
- Type of defect wherein the pulmonary artery
leaves the left ventricle and the aorta exits the
right ventricle. There is no communication
between the systemic and pulmonary circulation
- Associated defects may occur such as septal
defects or PDA which sustains the child’s
life
Clinical Manifestations:
• In infants with minimal communication (no
associated defects), severe respiratory depression
 and cyanosis will be evident at birth.
• In infants with associated defects, there is less
cyanosis but the infant may have symptoms of CHF •
No murmur or presence of a murmur that is
characteristic of associated defect
• Easy fatigability; FTT
• Inability to suck
• Hyperpnea
Diagnostics:
• 2D-Echo
• Cardiac catheterization

Medical and Surgical Management

✓ Prostaglandin E is administered to maintain
 a PDA and further blood mixing
✓ An Arterial Switch Procedure known as
Jatene Procedure – within the first week of
life is the surgical procedure of choice
TRUNCUS ARTERIOSUS
- Failure of the normal septation and division
of the embryonic bulbar trunk into
pulmonary artery and aorta. This results in a
single vessel that overrides both ventricles
Clinical Manifestations:
• Neonates with this defect typically appear
normal, however, as pulmonary vascular
resistance decreases after birth, severe
pulmonary edema and CHF develop
 • Marked cyanosis, esp. on exertion
• S/sx of CHF
• LVH, dyspnea, marked activity intolerance and
retarded growth
• Loud systolic murmur best heard at the lower left
sternal border and radiating throughout the
chest
Medical and Surgical Management :
• Surgical Repair

• Palliative Treatment

Pulmonary Artery Banding (Bilateral)

• Corrective Treatment – closing VSD so truncus
originates from the left ventricle, and creating
pathway from the right ventricle
TOTAL ANOMALOUS VENOUS RETURN - Absence
of the direct communication between the
pulmonary veins and the left atrium; pulmonary
veins are attached to the right atrium or to
various veins draining towards the right atrium