CONGENITAL HEART DEFECTS
Prof: Leonardo R. Sanchez IV, RN
Adapted from: PowerPoint/Lecture
Transcribed by: Julia Rae Delos Santos (3BSN-12)
CONGENITAL HEART DEFECTS
- 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
• 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 the Great Vessels (TGV)
• Truncus Arteriosus
• Hypoplastic Left Heart Syndrome
Exact Cause: Unknown
Predisposing Factors:
▪ Fetal and Maternal Infection during 1st
trimester esp rubella
▪ Maternal Alcoholism
▪ Maternal Age over 40
▪ 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 (Sulfonamides
Tetracyclines, Antiepileptics
PHINMA-UPANG
CHS Batch 2024
NUR 155 (MS LEC)
August 28, 2022
TYPES OF CHD
Acyanotic – Mixing of oxygenated and
unoxygenated blood in the pulmonary circulation
resulting to decreased oxygenated blood in the
systemic circulation.
- Left to right shunting
- Normal skin color
- Normal CNS function
- Position: Normal/Orthopneic
- Small stature, FTT
- Possible exercise intolerance
- ↑ frequency of respiratory infection
Patent Ductus Arteriosus
Atrial Septal Defects
Pulmonic Stenosis
Aortic Stenosis
Coarctation of the Aorta
Atrioventricular Canal Defect
Ventricular Septal Defect
Cyanotic – Poorly oxygenated venous blood mixes
with oxygenated blood in the systemic circulation
- Right to left shunting
- Cyanosis
- With CNS alterations (e.g., seizures, fainting,
confusion due to cerebral hypoxia)
- Small stature, FTT
- Marked exercise intolerance
- Frequent and severe respiratory infection
- May have hypoxic spells
Tetralogy of Fallot
Transposition of the Great
Vessels
& Truncus Arteriosus
Total Anomalous Venous
Return
 CONGENITAL HEART DEFECTS PHINMA-UPANG
Prof: Leonardo R. Sanchez IV, RN CHS Batch 2024
Adapted from: PowerPoint/Lecture NUR 155 (MS LEC)
Transcribed by: Julia Rae Delos Santos (3BSN-12) August 28, 2022

ACYANOTIC CHDs Medical and Surgical Mngt:

Ventricular Septal Defect ➢ Some VSDs may close spaontaneously

➢ Other are closed with a DACRON PATCH
- The most common acyanotic CHD
requiring CP bypass (recommend for children
characterized by an abnormal opening
with large defects, pulmonary artery HPN, CHF,
between the right and left ventricles. The
recurrent infection, FTT
degree of this defect may vary from a pinhole
➢ Pulmonary Artery Banding – palliative
to an absent septum.
procedure for poor surgical candidates

Atrial Septal Defect

- 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.

Type 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. Clinical Manifestations:

Clinical Manifestations: ➢ Most infant tend to be asymptomatic until

early childhood and many defects close
➢ Symptoms vary with the size of the defect, age
spontaneously by 5 years of age
and amount of resistance; usually
➢ Symptoms vary with the size of the defect;
asymptomatic
fatigue and dyspnea on exertion are the most
➢ LOUD, HARSH SYSTOLIC MURMUR at the left
common
sternal border 3rd to 4th ICS: dyspnea;
➢ Slow weight gain and frequent respiratory
tachypnea
infection
➢ FTT, excessive sweating, fatigue
➢ Systolic ejection murmur may b e auscultated,
➢ Child may be more susceptible to respiratory
usually most prominent at the 2nd ICS
infections
(pulmonic area)
➢ S/sx of CHF
➢ Plit S₂
Diagnostics:
Medical and Surgical Mngt:
➢ CXR, ECG
➢ Surgical closure by SUTURE or DARCON PATCH
➢ Echocardiography (2D Echo) – confirmatory
which requires CP bypass and is usually
test
performed during school age. Closure via
 CONGENITAL HEART DEFECTS PHINMA-UPANG
Prof: Leonardo R. Sanchez IV, RN CHS Batch 2024
Adapted from: PowerPoint/Lecture NUR 155 (MS LEC)
Transcribed by: Julia Rae Delos Santos (3BSN-12) August 28, 2022

cardiac catheterization may be done using a • Postductal

special coil or umbrella • Juxtaductal
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:

➢ 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
Clinical Manifestations:
anterior chest and between the scapula
➢ If the defect is small, the child maybe posteriorly
asymptomatic ➢ Rib notching may be observed in an older child
➢ A loud machine-like murmur accompanied by
Diagnostics:
a thrill
➢ Frequent respiratory infections ➢ ECG
➢ Poor feeding fatigue, hepatosplenomegaly, ➢ 2D-Echo
poor weight gain, tachypnea, tachycardia, ➢ CXR
irritability
➢ Widened pulse pressure and bounding pulse Medical and Surgical Mngt:
➢ S/sx of CHN ➢ Repair includes surgical removal of the stenotic
Medical and Surgical Mngt: area; bypass surgery is not necessary because
repair takes place outside the heart
➢ Administration of Prostaglandin synthetase ➢ Nonsurgical repair via Balloon Angioplasty
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
 CONGENITAL HEART DEFECTS PHINMA-UPANG
Prof: Leonardo R. Sanchez IV, RN CHS Batch 2024
Adapted from: PowerPoint/Lecture NUR 155 (MS LEC)
Transcribed by: Julia Rae Delos Santos (3BSN-12) August 28, 2022

Pulmonic Stenosis

- Involves obstruction of blood flow from the

right ventricle into the pulmonary artery due to
Clinical Manifestations:
narrowing of the pulmonic valve.
➢ 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 Mngt:

➢ If the child symptoms warrant, surgical aortic

Clinical Manifestations: valvulotomy or prosthetic valve replacement is
necessary
➢ A child may be asymptomatic or may have a ➢ Balloon angioplasty can be used to dilate the
mild CHF narrow valve
➢ A systolic murmur may have been heard over
the pulmonic area; a thrill may be palpated if Atrioventricular Canal Defect
stenosis is severe - Incomplete fusion of the endocardial cushion
➢ In severe cases, decreased exercise tolerance, - Common in trisomy 21
dyspnea, precordial pain and generalized - Shunting: left to right although blood may flow
cyanosis may occur among all heart chambers
Diagnostics:

➢ ECH/CXR
➢ Cardiac Catheterization

Medical and Surgical Mngt:

➢ Balloon Angioplasty techniques

➢ Surgical Valvulotomy may be performed

Aortic Stenosis

- Defect that primarily involves an obstruction of

aortic valve resulting to decreased cardiac
output
- More common in males/boys

S/sx: same with ASD

Tx: PAB, Surgical closure and Valve

repair/replacement
 CONGENITAL HEART DEFECTS PHINMA-UPANG
Prof: Leonardo R. Sanchez IV, RN CHS Batch 2024
Adapted from: PowerPoint/Lecture NUR 155 (MS LEC)
Transcribed by: Julia Rae Delos Santos (3BSN-12) August 28, 2022

CYANOTIC CHDs Medical and Surgical Mngt:

Tetralogy of Fallot ➢ Elective repair is usually performed during the

infant’s first year of life; but palliative repairs
- Most common form of cyanotic CHD
may be warranted for infants who cannot
characterized by the presence of 4 defects
undergo primary repair
namely:
➢ Total repair involves VSD closure, infundibular
1. Pulmonic Stenosis
stenosis, resection and pericardial patch to
2. Right Ventricular Hypertrophy (RVH)
enlarge right ventricular outflow tract
3. Overriding Aorta (OA)
4. Ventricular Septal Defect (VSD) Modified Blalock-Taussig (“BT Shunt”) – this
procedure diverts blood from an aortic branch to
the pulmonary artery, allowing blood to flow to the
lungs to receive oxygen.

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
Clinical Manifestations: defects or PDA which sustains the child’s life

➢ 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, exertion dyspnea, fainting
Clinical Manifestations:
or fatigue
➢ Pansystolic murmur may be heard at the mid- ➢ In infants with minimal communication (no
lower left sternal border associated defects), severe respiratory
➢ Polycythemia depression and cyanosis will be evident at
birth.
Diagnostics:
➢ In infants with associated defects, there is less
➢ 2D-Echo cyanosis but the infant may have symptoms of
➢ Cardiac catheterization CHF.
➢ Angiography ➢ No murmur or presence of a murmur that is
➢ CBC characteristic of associated defect
➢ ABGs ➢ Easy fatigability, FTT
➢ Inability to suck
➢ Hyperpnea
 CONGENITAL HEART DEFECTS
Prof: Leonardo R. Sanchez IV, RN
Adapted from: PowerPoint/Lecture
Transcribed by: Julia Rae Delos Santos (3BSN-12)
Diagnostics:
➢ 2D-Echo
➢ Cardiac catheterization
Medical and Surgical Mngt:
➢ 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
Total Anomalous Venous Return
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 a 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.
Tricuspid Atresia
PHINMA-UPANG
CHS Batch 2024
NUR 155 (MS LEC)
August 28, 2022
Medical and Surgical Mngt:
➢ 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.
- 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.
Clinical Manifestations:
➢ Cyanosis, pulmonary congestion and heart
failure
➢ Murmur audible
Management:
➢ Surgical correction involves restoring the
normal pulmonary venous circulation
➢ Cut the pulmonary veins from its attachments
and connect it to the left atrium via
homografts.
- Failure of the tricuspid valve to develop during
embryonic stage
- There is no communication between the right
atrium and right ventricle
- Associated defects like ASD or patent foramen
ovale and VSD or PDA is present
- Characterized by small RV; large LV and ↓
pulmonary circulation
Clinical Manifestations:
➢ Cyanosis is usually noted in the newborn
➢ Tachycardia and dyspnea
➢ Older children have signs of chronic hypoxia
(e.g., clubbing of fingers)
 CONGENITAL HEART DEFECTS PHINMA-UPANG
Prof: Leonardo R. Sanchez IV, RN CHS Batch 2024
Adapted from: PowerPoint/Lecture NUR 155 (MS LEC)
Transcribed by: Julia Rae Delos Santos (3BSN-12) August 28, 2022

Medical and Surgical Mngt: cardiac workload → Respiratory

➢ Palliative procedures such as a Pulmonary to
Systemic artery anastomosis are performed on
children with CHF
➢ Corrective surgery may be performed using the
FONTAN or HEMI-FONTAN procedure
Hypoplastic Left Heart Syndrome
- Underdeveloped/non-functional left ventricle
- + mitral/aortic valve atresia
Management: Heart transplantation
GENERAL PRINCIPLES OF CARE FOR CHD:
PREVENT CHD
1. Optimal maternal nutrition, preanatal
care, avoidance of drugs or alcohol
2. Immunization against rubella and
avoidance of infections that are considered
as teratogenic
Maintain respiration
1. Positioning: First action upon admission
a. Orthopneic position for acyanotic type
b. Squatting: for older children with
cyanotic CHD
c. Knee-chest: for younger children
(infants and toddlers) with cyanotic
CHD
2. Suction and Oxygen prn
3. Keep warm
a. Cold stress → Increased metabolism →
More oxygen utilization → Increased
Distress
b. Avoid extremes of temperature
4. Promote Rest
a. Well-planned nursing care
b. Anticipate needs
c. Small frequent feedings
d. Gavage feeding as needed primarily to
promote rest
e. Enlarged nipple hole/soft nipples for
bottled/formula milk
5. Prevention of Infection
a. Handwashing – most important
b. Immunization
c. Be alert for early s/sx of infection
6. Maintain Nutrition and Hydration
a. High protein, vitamins and minerals
especially iron
b. Small frequent feedings
c. Non-constipating foods
d. Gavage feeding
e. Monitor I&O, daily weight as necessary
7. Administer ordered drugs
a. Antibiotics: to prevent/treat infection
b. Diuretics: for CHF especially edema
c. Digitalis: for CHF
• Check CR before giving Digitalis, if
bradycardic, withhold and refer to
the physician
• If dose is missed and more than 4
hours has lapsed, withhold the
dose and give it at regular time
• If child vomits within 15 minutes,
give same dose
• If more than 2 consecutive doses,
notify the physician
• Check serum digoxin level
regularly (Normal = 0.5-2.0 ng/mL)
Prepare the child and family for various
diagnostic and treatment procedures including
surgery
a. Encourage verbalizations and concerns
b. Accept family or children reactions
c. Provide psychological support