University of Perpetual Help System Dalta

COLLEGE OF NURSING

Gracila U. Decena, R.N., M.A.N

Clinical Instructor NCM 103 Handouts

Congenital Heart Defects

Introduction
- Congenital means inborn or existing at birth.
- “Congenital heart disease”
- “Congenital cardiovascular disease.”
- The word "defect" is more accurate than "disease."
- A congenital cardiovascular defect occurs when the heart or blood vessels near
the heart don't develop normally before birth
- 8 out of every 1,000 babies born in the United States has a congenital (present at
birth) heart defect.
- Congenital heart defects are the most common birth defects.

Diagnostic tests
- Cardiac Catheterization
- Cardiac Magnetic Resonance Imaging Examination
- Echocardiogram: Sound Imaging of the Heart
- Echocardiography (Ultrasound of the Heart)
- Electrocardiogram (EKG or ECG)
- Exercise Tests
- Fetal Echocardiography
- Pediatric Echocardiography

Treatment
- Atrial Septal Defects: Surgical and Transcatheter
- Heart Transplants
- Patent Foramen Ovale (PFO) Closed Using a Catheter-Based Procedure
- Atrial Septal Defect (ASD) Closed Using a Catheter-Based Procedure

Medical Treatment
- Minimally Invasive Atrial Septal Defect Repair
- Open Heart Surgery
- Pulmonic Stenosis and Balloon Valvuloplasty

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Anatomy of fetal heart

Fetal Heart
- Developed at conception
- Completely formed by eight weeks into the pregnancy.
- Congenital heart defects happen during this crucial first eight weeks of the baby’s
development.

Fetal heart and fetal circulation

- Blood from placenta enters through the umbilical vein (carries oxygemated blood)
- IVC through an accessory structure (ductus venosus)
- Right atrium
- Blood shunted into the LA through foramen ovale
- Left ventricle
- Aorta

- Deoxygenated blood will enter the heart through vena cava

- Right atrium
- Right ventricle
- PA
- Aorta through ductus arteriosus
- Descending aorta
- Umbilical arteries
- Placental villi

Circulatory Event at Birth

- Drying and clamping of the umbilcial cord and stimulation of cold receptors
- Increased PCO2, decreased PO2, and increasing acidosis
- First breath
- Decreased PAP
- Increased PO2 and closure of foramen ovale
- Closure of ductus arteriosus, ductus venosus and umbilical arteries and vein due
to decreased flow

Causes
- no known cause.

Risk factors:
- Familial history- genetic disease
- History of maternal prenatal infection
- High risk maternal factors

Classification
- Acyanotic
Patent Ductus Arteriosus (PDA)
Atrio-septal Defect (ASD)

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 Ventricular-septal defect (VSD)
Atrioventricular septal defect

- Cyanotic
Tetralogy of Fallot
Transposition of the Great Vessel
Truncus Arteriosus

- Obstructive
Coarctation of Aorta
Aortic stenosis
Pulmonic stenosis

Acyanotic Heart defects

- problems that cause too much blood to pass through the lungs
- defects allow oxygen-rich blood that should be traveling to the body to re-
circulate through the lungs, causing increased pressure and stress in the lungs.
- Children are prone to CHF
- Heart conditions that do not cause deoxygenation or low o2 levels
- The skin & the mucus membrane color usually is normal pink.

Complication:
- heart failure
- excessive blood pressure in the lungs (pulmonary hypertension)
- infections of the heart (endocarditis)
- irregular heartbeats (arrhythmias)
- delayed growth

Nursing diagnosis:
- Impaired gas exchange
- Altered peripheral tissue perfusion related to CHF
- Altered cardiopulmonary tissue perfusion
- Altered nutrition, less than body requirements
- Anxiety
- Ineffective family coping: disabling
- Risk for impaired growth & development
- Risk for infection

Planning & intervention

- Occasional spontaneous closure occurs
- Surgical closure
- Prophylactic antibiotics treatment to prevent endocarditis
Pre op care: promote oxygenation

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 a. Patent Ductus Arteriosus
- Accessory fetal vessel that connect the pulmonary artery to the aorta
- Before birth, a temporary blood vessel called the ductus arteriosus connects the
pulmonary artery and the aorta.
- This allows blood to bypass the lungs because oxygen is delivered to the fetus
through the placenta and umbilical cord.
- The temporary vessel normally closes within a few hours or days of birth since
the lungs take over. If it remains open (patent), some blood that should circulate
through the body is misdirected to the lungs.
- This defect can cause heart failure or endocarditis.
- In infants, it can be closed with medications. In older children and adults, plugs,
coils or surgery can be used to close the vessel.
- A ductus that doesn't close is quite common in premature infants but rather rare
in full-term babies.
- If the ductus arteriosus is large, a child may tire quickly, grow slowly, catch
pneumonia easily and breathe rapidly.

Signs and Symptoms

- loud murmur

- Poor weight gain

- Frequent resp inf.

- Tachypnea

- Poor feeding

- Irritability

- fatigue -

- widened pulse

- hepatosplenomegaly

- widened pulse pressure

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Pathophysiology of PDA

Planning & Intervention:

• Surgical intervention or patch of defect (Silastic Patch)
• Trans catheter device closure during catheterization
• Child & family Education:

- Explain, teach parents to support nutrition, reduce stress, promote rest & support
G&D.

- Teach signs of CHF

Therapeutic Management
• Oral or IV Indomethacin
SE: reduced glomerular filtration

 Impared platelet aggregation,

 Diminished GI & cerebral blood flow

b. Atrial Septal Defect

• Left to right shunt
• Opening between the atria
• Foramen ovale fails to close
• Sometimes much septum is absent
• Increased in the pulmonary blood flow

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Complication:
- Arrhythmias
- heart failure
- stroke
- pulmonary hypertension

Signs and Symptoms

- Fatigue
- Slow weight. Gain
- Dyspnea
- Frequent URTI
- Murmur
- CHF

c. Ventricular septal defect

- a hole in the ventricular septum (a dividing wall between the two lower chambers
of the heart – the right and left ventricles) occurs.
- Because of this opening, blood from the left ventricle flows back into the right
ventricle, due to higher pressure in the left ventricle.
- This causes an extra volume of blood to be pumped into the lungs by the right
ventricle, which can create congestion in the lungs.
- Left to right shunt
- Shunting of blood causes an increased load on the right ventricle

d. Atrioventricular Canal (AVC or AV canal)

- atrioventricular canal is a complex heart problem that involves several
abnormalities of structures inside the heart, including atrial septal defect,
ventricular septal defect, and improperly formed mitral and/or tricuspid valves.

Cyanotic Heart Disease

- Cyanotic Heart Defect
- problems that cause too little blood to pass through the lung
- defects allow blood that has not been to the lungs to pick up oxygen (and,
therefore, is oxygen-poor) to travel to the body.
- The body does not receive enough oxygen with these heart problems, and the
baby will be cyanotic.
- In the cyanotic defects, a shunt bypasses the lungs and delivers venous
(deoxygenated) blood from the right side of the heart into the arterial circulation.
- Right to left shunt
- Unoxygenated blood enters the body circulation accounting for the cyanosis

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Nursing Management
- Rest
- Small frequent feedings
- ⇑fowler’s
- Supplement vitamins
- Monitor weight
- O2
- Avoid stress

a. Tetralogy of Fallot
- “A blue baby".
- Comprised of four different components:
Ventricular septal defect (VSD).
Pulmonary stenosis (PS).
Right ventricular hypertrophy
Overriding aorta (aorta lies directly over the ventricular septal defect.)

Signs and symptoms

- Pansystolic murmur may be heard at the mid- lower left sternal border
- hypoxia
- polycytemia
- metabolic acidosis
- poor growth
- RVH
- Irritability
- Pallor
- Blackouts
- Convulsions.
- Cyanosis at rest
- Clubbing
- Squatting
- Dyspnea
- Slow wt. Gain
- Fatigue
- Clubbing of fingers

- Infants and young children with unrepaired tetralogy of Fallot are often blue
(cyanotic).
- The reason is that some oxygen-poor blood is pumped to the body.

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Pathophysiology of TOF

Complications
- "blue baby syndrome,"
- poor eating
- inability to tolerate exercise
- arrhythmias
- delayed growth and development
- stroke.

Management
- PROSTAGLANDIN E1: to maintain patent ductus arteriosus
- Shunting procedures
• Corrective surgery: patching the VSD & relieving the pulmonary stenosis
• VSD closure
• Stenosis resection
• Pericardial patch
• Surgical (2-3 y.o)
• Knee-chest position

Nursing Diagnosis
- Altered pulmonary tissue perfusion
• monitor hgb & hct, keep child calm(-) crying
• hypercyanosis: assist in squatting position
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 • Administer O2
• Morphine as ordered

- High risk for infection:

• limit exposure to individuals with infection
• promote good pulmonary condition
• administer prophylactic antibiotics as prescribed

- Altered Nutrition Less than body requirement:

• SFF
• Soft nipple for infants
• Allow rest

- Risk for impaired gas exchange:

• Limit activity
• Maintain clear airways
• Monitor electrolytes

- Risk for decreased cardiac output:

• Assess Vs
• Monitor for signs of CHF
• Note peripheral edema
• Weigh child q day, strict I&O
• diuretics, O2, digoxin, as ordered

b. Transposition of the Great Vessel

- Aorta originates from the right ventricle
- Pulmonary artery from the left ventricle
- Results in two opposite circulation.
- The arteries are reversed and the aorta carries blood from the right ventricle.
- With this defect, the positions of the aorta and the pulmonary artery (the great arteries)
are reversed (transposed).
- The aorta arises from the right ventricle instead of the left and the pulmonary artery
arises from the left ventricle instead of the right.
- This creates a circulatory pattern that prevents nourishing oxygenated blood from
reaching the body.
- This condition would quickly be fatal to a newborn except it's generally accompanied
by another defect — commonly a septal defect or patent ductus arteriosus — that does
allow oxygen-rich blood to get to the body. Surgical repair is usually necessary shortly
after birth.

Signs and Symptoms

- Severe respiratory depression
- Cyanosis
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- S/s of CHF
- Fatigue
- progressive cyanosis> hypoxia> acidosis
- Tachypnea
- Poor feeding
- Failure to grow

- In order for the infant to survive, they must have some communication between
the right and the left sides of the heart to allow oxygen rich blood to reach the
body.
- Babies with transposition are extremely blue at birth.
- Infants can only survive if there is a shunt between the two sides of the heart,
and an atrial septal defect needs to be actually enlarged to allow adequate
mixing of blood to deliver enough oxygenated blood to the body.
- This is the second most common congenital heart disease encountered in early
infancy.

Treatment
- In order for the infant to survive, open heart surgery is needed in early infancy.
- The surgery involves removal of the pulmonary veins from the right atrium and
anastomosis to the left atrium.
- The ASD is also closed, along with the division of any abnormal connections that
may be present
- The most common surgical procedure :arterial switch operation.
- Other surgical defects may also be needed to correct the communication
between the left and right sides of the heart.

c. Truncus Arteriosus
- The aorta and pulmonary artery start as a single blood vessel, which eventually
divides and becomes two separate arteries.
- Truncus arteriosus occurs when the single great vessel fails to separate completely,
leaving a connection between the aorta and pulmonary artery.

Signs and Symptoms

- Severe pulmonary edema
- CHF
- Cyanosis
- LVH
- Dyspnea
- Fatigue
- Retarded growth
- Murmur

Surgical Treatment

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 - Surgery is required to close the ventricular septal defect and separate blood flow
to the body from blood flow to the lungs.
- Generally done early in infancy to prevent high blood pressure from damaging
the lungs' arteries.
- A patch is used to close the ventricular defect.
- Rastelli repair: The pulmonary arteries are then disconnected from the single
great vessel and a tube (a conduit or tunnel) is placed from the right ventricle to
these pulmonary arteries.

Obstructive Congenital Heart Defect

- problems that cause too little blood to travel to the body
- result of underdeveloped chambers of the heart
- blockages in blood vessels that prevent the proper amount of blood from
traveling to the body to meet its needs.

A. COARCTATION OF AORTA
B. AORTIC STENOSIS
C. PULMONIC STENOSIS

a. Coarctation of Aorta
- This is a narrowing (coarctation), or constriction, in a portion of the aorta.
- Coarctation forces the heart to pump harder to get blood through the aorta and on to
the rest of the body.
- Classic difference in BP & pulse between the upper and lower extremity

Signs and Symptoms

- Epistaxis
- Headaches
- Fainting
- Leg cramps
- Systolic murmur
- Rib notching
- Exercise intolerance
- brachial & radial pulses full, femoral pulse weak
-Dyspnea,
- CVA secondary to hypertension in the upper circulation

Complication
- severe hypertension
- aortic aneurysm
- dissection or rupture
- endocarditis
- brain hemorrhage
- stroke

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 - heart failure
- premature coronary artery disease

Nursing Diagnosis
- Altered tissue perfusion
- Risk for injury
- Activity intolerance
- Knowledge deficit

Therapeutic Management
- Balloon cardiac catheterization
- Surgical resection & patch the coarctation
- Prophylaxis for endocarditis
- Monitor BP

b. Aortic Stenosis (AS)

- This is a defect that narrows or obstructs the aortic valve opening, making it difficult for
the heart to pump blood into the aorta. Mild cases may not have symptoms initially, but
they can worsen over time.

Complication
- heart enlargement
- left-sided heart failure
- arrhythmias
- endocarditis
- fainting

Signs and symptoms

- Faint pulse
- Hypotension
- Tachycardia
- Poor feeding pattern
- Activity intolerance
- Chest pain
- Dizziness
- Systolic murmur

Management
a. Surgical aortic valvulotomy or prosthetic valve replacement
b. Angioplasty

c. Pulmonary Stenosis
- In this condition, the flow of blood from the right ventricle to the pulmonary artery is
obstructed by narrowing at the pulmonary valve.

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- When there's an obstruction (stenosis), the right ventricle must pump harder to get
blood into the pulmonary artery.
- The defect may occur along with other defects, such as thickening of the muscle of the
right ventricle immediately below the valve.
- In many cases, pulmonary stenosis is mild and doesn't require treatment.
Complication
- heart failure
- arrhythmias
- enlargement of the right heart chambers

Signs and Symptoms

- Mild cyanosis or CHF
- Dyspnea
- Systolic murmur
- Pre-cordial pain
- ⇓ activity tolerance
- Generalize cyanosis

Management
- Angioplasty-repair of the stenosis
- Replace the valve.
- Balloon valvuloplasty- Special balloons to widen the valve

Nursing Management
1. Rest
2. Small frequent feedings
3. ⇑fowler’s
4. Supplement vitamins
5. Monitor weight
6. O2
7. Avoid stress

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