Instructional Module in NCM 109-A (Care of Mother

and Child At Risk Or with Problems-(Acute and Chronic)

Instructional Material for Online

Teaching and Learning

CARE OF THE MOTHER AND CHILD AT

RISK OR WITH PROBLEMS
( ACUTE and CHRONIC)
NCM 109

1|P a g e
Lesson 2. Congenital Heart Defects
Chapter 5
Instructional Module in NCM 109-A (Care of Mother
and Child At Risk Or with Problems-(Acute and Chronic)

CHAPTER 5. NURSING MANAGEMENT OF THE HOSPITALIZED CHILD

LESSON 2. CONGENITAL HEART DEFECTS

Definition:

Congenital means inborn or existing at birth. A congenital heart defect is a malformation of the
heart existing at birth. This defect results from the failure of the heart or major blood vessel to
mature normally during gestation.

Incidence
▪ Congenital heart disease affects 25, 000 - 30, 000 children a year. That is eight out of every 1000
birth each year. (American Heart Association)
▪ Major cause of death in the first year (other than prematurity)
▪ Most common congenital malformation in the newborn
▪ Sex affected differently depending on the defect
Male=AS, COA, TGV
Female=PDA, ASD
▪ Children with congenital heart defect are also more likely to have extracardiac defects like
tracheoesophageal fistula, renal agenesis and diaphragmatic hernias

Etiology
▪ Not known but several factors involving genetic and environmental factors are associated with a
higher than normal incidence of the disease
a. Genetic Factors- the influence is multifactorial
1. Sibling/parent with heart defect
2. Chromosomal aberrations (Down’s syndrome)= half of all babies with Down syndrome
have congenital heart defects.
3. Born with other congenital anomalies (as mentioned above)
b. Prenatal factors
1. Maternal viral infection during pregnancy (rubella) especially during the first trim
2. Fetal exposure to drugs (alcohol, lithium, acutane {for acne}, cocaine, antiseizure
medications)
3. Maternal age over 40 years old
4. Maternal metabolic disorders –type 1 DM and PKU {uncontrolled during pregnancy}

PEDIATRIC DIFFERENCES

1. Fetal tissues are accustomed to low oxygen saturation in the utero→newborn with cyanotic
heart disease appear relatively comfortable when the pO2 is 20-25 mmHg (in older children,
death could occur in minutes)
2. RV=LV size; @ 2 months of age, LV twice size of RV
3. Muscle fibers of the heart are less developed and less organized
a. infants have higher risk of heart failure than older children because the immature
heart is more sensitive to volume/pressure overload

2|P a g e
Lesson 2. Congenital Heart Defects
Chapter 5
Instructional Module in NCM 109-A (Care of Mother
and Child At Risk Or with Problems-(Acute and Chronic)

b. limited functional capacity (less compliance=amount of

distention/expansion)→can’t increase stroke volume substantially
 By the age of 5, the heart muscle fibers are developed so that heart function is
comparable to a healthy adult.
4. Cardiac output depends completely on heart rate until muscle fibers are fully developed at 5
y/o.

 Oxygen requirements are high in the first few weeks of life. It is normal in the newborn
to have an increased heart rate to provide adequate oxygen transport.
5. Children respond to severe hypoxemia (respiratory failure and shock) with
bradycardia→cardiac arrest.

DIAGNOSTIC PROCEDURES

1. History
2. Physical Exam
3. Chest X-ray
4. ECG
5. Echocardiography
6. Cardiac Catheterization
7. Exercise Stress Test
8. Cardiac MRI
9. CBC and ABG

1. HISTORY
▪ Possible causes of CHD
▪ Poor weight gain/feeding behavior
▪ Frequent respiratory infections
▪ Prior murmurs
▪ Respiratory difficulties-tachypnea, dyspnea, SOB
▪ Cyanosis
▪ Exercise intolerance/fatigue

2. PHYSICAL EXAM
a. Inspect-nutritional state (failure to thrive, poor weight gain); color(cyanosis, pallor);
distorted chest configuration (enlarged heart); unusual pulsations (visible neck veins;
respiratory difficulties (as mentioned above); clubbing of fingers
b. Palpate-thrills, hepatomegaly, peripheral pulses (rate, regularity and discrepancies)
c. Auscultate-heart rate and rhythm, character of sounds (murmurs)

3. CHEST X-RAY
 Provides information on heart size and pulmonary blood flow markings

4. ECG
 Graphic measurement of electrical activity of the heart
3|P a g e
Lesson 2. Congenital Heart Defects
Chapter 5
Instructional Module in NCM 109-A (Care of Mother
and Child At Risk Or with Problems-(Acute and Chronic)

Holter monitor-24 hour continuous ECG recording to assess dysrhythmias

5. ECHOCARDIOGRAPHY
 Use of high-frequency sound waves obtained by a transducer to produce image of cardiac
structures

a. TRANSTHORACIC- Done with transducer on chest

1. M mode= One dimensional graphic view used to estimate ventricular size and
function
2. Two-dimensional= Real-time, cross sectional views of the heart to identify cardiac
structures and anatomy
3. Doppler= Identifies blood flow patterns and pressure gradients across structures

b. FETAL= Imaging fetal heart in utero

c. TRANSESOPHAGEAL (TEE)= Transducer placed in esophagus behind heart to obtain images
of posterior heart structures or in patients with poor images from chest approach

6. CARDIAC CATHETERIZATION
▪ Imaging study using radiopaque catheters placed in a peripheral blood vessel and advanced
into heart
▪ may be done on OP basis

PURPOSES

1. DIAGNOSTIC- To detect CHD and done before surgical repair

Right Sided Catheterization =more common; via femoral vein access
Left Sided Catheterization= via arteryaortaheart)

a. Hemodynamics- Measures pressures and oxygen saturations in heart chambers

b. Angiography- Use of contrast material to illuminate heart structures and blood
flow patterns
c. Biopsy- Use of a special catheter to remove tiny samples of heart muscle for
microscopic evaluation; used in assessing infection, inflammation, or muscle
dysfunction disorders; also to evaluate for rejection after heart transplant
d. Electrophysiology- Special catheters with electrodes employed to record
electrical activity from within the heart; used to diagnose rhythm disturbances

2. INTERVENTIONAL/THERAPEUTIC-Use of balloon catheter or other devices to alter cardiac

anatomy
a. Dilate stenosed valves or vessels
b. Close abnormal openings

Manner of introduction of catheter:

a. Cutdown
b. Percutaneous-catheter threaded through a large bore needle that is inserted into the
vein
 Guided through the heart with the aid of fluoroscopy
 Once tip is in the heart chamber, contrast medium is injected and films are taken
4|P a g e
Lesson 2. Congenital Heart Defects
Chapter 5
Instructional Module in NCM 109-A (Care of Mother
and Child At Risk Or with Problems-(Acute and Chronic)

Potential Complications: perforation of pulmonary artery, allergic reaction to contrast media,

dysrrhythmias, hypotension, stroke, vascular compromise in the leg (loss of pulse due to clot,
hematoma, intimal tear), bleeding

NURSING CARE

A. BEFORE
1. Monitor height and weight –basis for catheter selection
2. History of allergy-contrast medium is iodine based
3. s/s of infection-ex: severe diaper rash enough reason to cancel procedure if femoral access
required
4. NPO post midnight
5. Void before entering catheter lab/room
6. assess and mark pulses and document –for baseline data
7. IV fluids to prevent dehydration and hypoglycemia especially for patients with polycythemia
8. Sedative IV/oral
9. Prepare the child and family for the procedure-cardiologist explains the procedure, nurse
reinforces and clarifies information (see below for the instructions during procedure)

B. DURING
What happens during the procedure?
1. Groin cleansed with a special brown solution
2. Medications will be given so that the skin will go to sleep (use g30 needle for ID lidocaine
or EMLA cream topically 60 min before procedure)
3. Tube placed in blood vessel and there may be a feeling of little pushing
4. Special medicine (contrast dye) is put in the tubing and there may be a feeling of
warmth
5. When the med is put in, lights will go off and pictures will be taken (child may be fearful
of dark and noise from machine)

C. AFTER
1. Monitor the following
a. vital signs every 15 minutes , with emphasis on HR, count for a full minute
(dysrhytmias/bradycarida)
b. pulses especially below the cath site for symmetry and equality (first few hours, may
be weaker but should gradually increase in strength)
c. temperature and color of the affected extremity (coolness and blanching =arterial
obstruction)
d. BP –hypotension –hemorrhage (cardiac perforation or bleeding at the site of initial
catheterization)
e. Dressing -bleeding or hematoma formation
f. Fluid intake (IV/PO) to ensure adequate hydration ( due to blood loss from
procedure, NPO status and diuretic action of dye used)
g. Hypoglycemia

2. Affected extremity must be maintained straight to facilitate healing of the cannulated

vessel
▪ 4-6 hours after venous catheterization
▪ 6-8 hours for arterial

5|P a g e
Lesson 2. Congenital Heart Defects
Chapter 5
Instructional Module in NCM 109-A (Care of Mother
and Child At Risk Or with Problems-(Acute and Chronic)

3. Diet resumed as tolerated (sips of clear liquids to usual)

4. Encourage to void to clear contrast material
5. Remove pressure dressing the day after catheterization. Cover site with adhesive
bandage strip for several days.
6. Prevent infection-keep site clean and dry; protect from possible contamination; avoid
tub baths for several days but may have a shower; monitor for s/s of infection-redness
swelling, drainage
7. Avoid strenuous exercise for several days

7. EXERCISE STRESS TEST

 Monitoring of heart rate, BP, ECG and oxygen consumption at rest and during progressive
exercise on a treadmill or bicycle

8. CARDIAC MAGNETIC RESONANCE IMAGING (MRI)

 Newest noninvasive imaging technique; used in evaluation of vascular anatomy outside of
heart

9. CBC and ABG

 increased Hgb and Hct due to polycythemia

6|P a g e
Lesson 2. Congenital Heart Defects
Chapter 5
Instructional Module in NCM 109-A (Care of Mother
and Child At Risk Or with Problems-(Acute and Chronic)

TYPES OF CONGENITAL HEART DEFECTS

▪ Sometimes CHD is so mild that there are no outward symptoms. Some heart defects are simply
innocent murmurs which resolve on their own. In other cases, it's so severe that the newborn
becomes ill soon after birth. In still other cases, signs and symptoms occur only in later
childhood. Severe heart defects often require multiple surgeries, a lifetime of medications and
preventative measures to maintain a working heart and a healthy body. For some people, their
only hope is a heart transplant.

▪ The pathophysiology of heart defects is related to hemodynamics.

 As with any fluid, blood flows from an area of high pressure to one of lower pressure and
toward the path of least resistance.
o The higher the pressure gradient, the greater the rate of flow.
o The greater the resistance, the lesser the rate of flow.
o Normal=systemic pressure is greater then ( >) pulmonary pressure causing Left →
Right SHUNT

 So that: abnormal connections between heart chambers, blood will flow from an area of
higher pressure to one of lower pressure

ACYANOTIC HEART DEFECTS= L→R SHUNT= systemic > pulmonary pressure

CYANOTIC HEART DEFECTS= R→L SHUNT= pulmonary > systemic pressure

TYPES OF CONGENITAL HEART DEFECTS

CYANOTIC ACYANOTIC
• Allows unoxygenated blood to flow into the • Interferes with normal flow through the
systemic circulation or conditions that result heart either by slowing it down or by
to obstructive pulmonary blood flow allowing blood to be shunted from the L
(↑pulmonary vascular resistance) to the R side of the heart
 Unoxygenated blood mixes with • Causes the heart to function as an
oxygenated blood-blood pumped to ineffective pump, most commonly leads
tissues has ↓ O2 causing cyanosis to CHF
Types: Magnificent T’s 1. Non-obstructive lesions -increases
1. decreased pulmonary blood flow pulmonary blood flow
• Tetralogy of Fallot (TOF) • VSD-most common
• Tricuspid atresia • Atrial septal defect
2. Mixed blood flow • PDA
• Transposition of great arteries/vessels • Atrioventricular canal
• Truncus arteriosus 2. Obstructive lesions-obstruction to blood
• Total anomalous pulmonary venous flow from ventricles
connection • Pulmonic stenosis
(TAPVC/TAPVR(return)/TAPVD(drainage) • Aortic stenosis
• Hypoplastic left heart syndrome (HLHS) • Coarctation of the aorta (COA)

Assessment
▪ Cyanosis-best recognized in the tongue and ▪ No cyanosis
mucus membrane ▪ Audible murmurs
7|P a g e
Lesson 2. Congenital Heart Defects
Chapter 5
Instructional Module in NCM 109-A (Care of Mother
and Child At Risk Or with Problems-(Acute and Chronic)

▪ Poor, difficult feeding ▪ Retarded physical growth

▪ Retarded physical growth ▪ Frequent URTI
▪ Squatting-a good diagnostic cue for TOF ▪ Tachycardia and tachypnea
▪ Clubbing of fingers and toes ▪ Dyspnea
▪ Polycythemia ▪ Enlargement of liver due to back-up of
▪ Tachycardia, tachypnea blood
▪ Elevated Hct ▪ Easy fatigability, decreased exercise
tolerance
▪ Eisenmenger Complex – clinical situation
where L-R shunt becomes R-L shunt
because of:
MAJOR COMPLICATION: Pulmonary HPN
(irreversible) → reversal of blood flow
Complications Signs of CHF-most common
• Cerebral abscess-most common; bacteria in ❖ RD signs
the blood returning from the systemic ❖ Cough
circulation are usually filtered out by the ❖ diaphoresis
capillaries in the lungs. When unO2 blood
enters the systemic circulation through the R-L
Shunt, bacteria can travel directly to the brain
• Brain infarctions, Blood clots → increased risk
of thromboembolism → chronic hypoxemia
leads to polycythemia in an attempt to
increase the Hgb available to carry O2.
• L-sided heart failure

ACYANOTIC HEART DEFECTS

Non-obstructive lesions - increases pulmonary blood flow

1. VSD-most common
2. Atrial septal defect
3. PDA
4. Atrioventricular canal

Obstructive lesions-obstruction to blood flow from ventricles

1. Pulmonic stenosis
2. Aortic stenosis
3. Coarctation of the aorta

▪ Medical and surgical treatment now offers these children an opportunity to grow and mature
into adult life, an option that once was not available.
▪ Heart defects that are fixed during surgery are grouped into two categories: open and closed.

1. Closed heart surgery implies that the "heart lung machine" or "bypass" machine is not used
and the heart is visualized but not cut open.
2. Open heart surgery implies that the heart needs to be opened in order to repair the defect
and therefore the "bypass" machine is used to oxygenate and circulate the blood without

8|P a g e
Lesson 2. Congenital Heart Defects
Chapter 5
 Instructional Module in NCM 109-A (Care of Mother
and Child At Risk Or with Problems-(Acute and Chronic)

using the heart or lungs. This bypass machine is used so that open heart procedures can be
performed safely.

ACYANOTIC HEART DEFECTS

TYPE STRUCTURAL DEFECT HEMODYNAMICS MANAGEMENT

ASD Abnormal opening between L-R shunting due to incomplete ▪ Sx:(open) DACRON PATCH CLOSURE done at
R & L auricles closure of septum separating preschool age
the 2 auricles
▪ Nonsx: TRANSCATHETER DEVICE CLOSURE

VSD Abnormal opening between L-R shunting may cause R ▪ Palliative: PULMONARY ARTERY BANDING in
R & L ventricles creating ventricular failure (CHF) infants to prevent heart failure (band to narrow
pulmonary vascular the PA↓blood flow and pressure in lungs)
resistance
▪ Open heart surgery when older: removal of
band and complete repair (treatment of
choice)
➢ covered with knitted Dacron patch
➢ purse string approach

PDA Communication between L-R shunting due to incomplete ▪ Med: Indomethacin

PA & aorta due to failure of closure of septum
ductus arteriosus to close ▪ Sx:
after birth ➢ Open: ligation of PDA thru left
thoracotomy
➢ Closed: VATS (visual assisted
thoracoscopic surgery)-use of
thoracoscope and instruments, 3 small
insicion on the L side of chest to place a clip
on the ductus)

▪ Nonsx: COIL OCCLUSION

AVC Incomplete fusion of Alteration in hemodynamics ▪ Palliative: PULMONARY ARTERY BANDING

endocardial cushions
depends on the severity of the
child’s pulmonary vascular
resistance; L→R shunting
occurs and pulmonary blood
flow ↑CHF
▪ Complete repair: patch closure of the septal
defect and reconstruction of the AV valve;
mitral valve replacement if severe

PS Narrowing at the entrance ▪ Resistance to blood flow
of PA causes R ventricular
hypertrophy and decreased
pulmonary blood flow
▪ Nonsx: BALLOON VALVULOPLASTY during
cardiac catheterization to dilate the narrowed
valve/STENT PLACEMENT
▪ Sx:
➢ Infants-TRANSVENTRICULAR
VALVOTOMY/BROCK (closed)
➢ Children-PULMONARY VALVOTOMY
(open)

9|P a g e
Lesson 2. Congenital Heart Defects
Chapter 5
 Instructional Module in NCM 109-A (Care of Mother
and Child At Risk Or with Problems-(Acute and Chronic)

AS Narrowing of the stricture ▪ This causes resistance to ▪ Nonsx: BALLOON VALVULOPLASTY

of the aortic valve blood flow in the left
ventricle, decrease cardiac ▪ Sx:
output, L ventricular ➢ Valvular aortic stenosis: Aortic valvotomy
hypertrophy and pulmonary (palliative); 25% have recurrent stenosis
congestion within 10 years
▪@ risk for bacterial ➢ Subvalvular stenosis: 10% develop
endocarditis, coronary recurrent stenosis
insufficiency and ventricular
dysfunction 1. ROSS PROCEDURE-replacing the child’s
diseased aortic valve with his/her own
pulmonary valve (pulmonary autograft)
and the pulmonary valve replaced with
homograft

2. Aortic homograft with a valve

COA Narrowing of the aortic ▪ L ventricle must generate
lumen higher than normal pressure
to eject adequate stroke
volume
➢ Additional sx for recurrent stenosis: valve
replacement (aritificial) may be required
at the second procedure
▪ Nonsx: BALLOON ANGIOPLASTY for older
children
▪ Sx:
➢ Resection/ removal of narrowed ends and
anastomosis of both ends
➢ Enlargement of the constricted section
using a graft of prosthetic material/portion
of left subclavian artery

Legend:
Surgical=Sx
Nonsurgical=NonSx
Medical=Med

NURSING DIAGNOSIS WITH THE ACYANOTIC HEART DEFECTS AND THEIR COMPLICATIONS

• Fluid volume excess related to pulmonary vasculature overload

• Decreased cardiac output related to an obstructive outflow tract
• Ineffective breathing pattern related to pulmonary vasculature overload
• Altered nutrition (less than body requirement) related to ineffective feeding pattern
• High risk for infection related to altered immune function, surgery/pulmonary overload
• High risk for activity intolerance related to chronic pulmonary vasculature overload
• Altered family processes related to guilt and giving over lack of perfect infant
• High risk for caregiver role strain related to chronic health condition
• Knowledge deficit (parents) related to special care for chronic condition

10 | P a g e
Lesson 2. Congenital Heart Defects
Chapter 5
 Instructional Module in NCM 109-A (Care of Mother
and Child At Risk Or with Problems-(Acute and Chronic)

CYANOTIC HEART DEFECTS

Decreased pulmonary blood flow

1. Tetralogy of Fallot (TOF)
2. Tricuspid atresia

Mixed blood flow

1. Transposition of great arteries/vessels (TGV/TGA)
2. Truncus arteriosus
3. Total anomalous pulmonary venous return(TAPVC/TAPVR(return)/TAPVD(drainage)
4. Hypoplastic left heart syndrome (HLHS)

TYPE STRUCTURAL HEMODYNAMICS MANAGEMENT

DEFECT
Tetralogy of Combination of 4 ▪ R-L shunting impeding ▪ Palliative Shunt (for infants who can’t
Fallot defects flow to lungs with R undergo primary repair): done to increase
1. Pulmonary sided hypertrophy pulmonary blood flow and increase
Stenosis ▪ With acute episodes of oxygen saturation
2. VSD cyanosis and hypoxia
3. Overriding aorta (blue spells/tet spells) ➢ BLALOCK TAUSIG/MODIFIED
4. R Ventricular which usually occur BLALOCK TAUSSIG: provides
hypertrophy when the infant’s blood flow to the pulmonary
oxygen requirement artery from the L or R subclavian
exceed the supply artery

▪ Complete repair in the first few years of

life
➢ BROCK PROCEDURE
(open)=involves closure of the
VSD and resection of the
infindibular stenosis with a
pericardial patch to enlarge the R
ventricular outflow tract

▪ absence of tricuspid  decreased blood flow to ▪ Med: continuous infusion of

Tricuspid or pulmonary valve the lungs PROSTAGLANDIN E1 until surgical
Atresia/ ▪ but often ASD/VSD intervention can be arranged to ensure
Pulmonary and PDA is also  Complete mixing of patency of PDA
Atresia present oxygenated and
unoxygenated blood ▪ Sx: done in 2-3 separate stages to allow the
body time to adjust to the redirected blood
flow and puts less strain on the heart.

First operation: Palliative

➢ shunt placement (pulmonary-to-
systemic artery anastomosis) to
increase blood flow to the lungs

11 | P a g e
Lesson 2. Congenital Heart Defects
Chapter 5
 Instructional Module in NCM 109-A (Care of Mother
and Child At Risk Or with Problems-(Acute and Chronic)

➢ Atrial septostomy (Rashkind

procedure) during cardiac
catheterization if ASD is small

Second operation: BIDIRECTIONAL

GLENN SHUNT/BIDIRECTIONAL CAVO-
PULMONARY SHUNT (BCPS)/PARTIAL
Bidirectional Glenn FONTAN
One And A Half shunt/Bidirectional Cavo- ➢ Preferred in very small babies - below
Ventricle Repair" Pulmonary Shunt (BCPS)/ 2 years of age - in whom the lung
Partial Fontan vessel resistance is still quite high,
and in borderline cases with
abnormal pulmonary arteries.
➢ This is a shunt from SVC to the
pulmonary artery (the first shunt is
removed at the second operation).

Third Operation: FONTAN PROCEDURE

Atriopulmonary Shunt
➢ The IVC is connected to the
pulmonary artery.
➢ Usually done when the patient is
between 2-3 years old
➢ two most common of these
procedures
1. TOTAL CAVOPULMONARY REPAIR (TCR)
▪ Uses an intracardiac right atrial baffle,
directing blood from the inferior and
superior vena cava to the pulmonary
arteries bypassing the right atrium and
right ventricle.
▪ The TCP is used for patients with heart
chambers which allow recirculation
Total Cavopulmonary Repair and stagnation to occur

2. ATRIOPULMONARY SHUNT (AP)

▪ Uses an extracardiac conduit, taking
blood from the inferior vena cava
directly to the pulmonary arteries, thus
bypassing the right ventricle.
▪ This allows the patient to still utilize the
pumping power of the right atrium.

Transposition ▪ Position of aorta ▪ Incompatible with Non-Surgical

Of Great and PA are extrauterine life; a. PROSTAGLANDIN E1 IV
Vessels reversed. The PA venous blood enters R temporarily :increases mixing of blood if
/Arteries leaves the L atrium to R ventricle to systemic and pulmonary mixing is
(TGA/TGV) ventricle and the aorta and systemic inadequate
aorta exists from circulation without
the R ventricle oxygenation b. BALLOON ATRIAL SEPTOSTOMY
(RASHKINDPROCEDURE)
▪ performed during cardiac
catheterization to increase mixing

12 | P a g e
Lesson 2. Congenital Heart Defects
Chapter 5
 Instructional Module in NCM 109-A (Care of Mother
and Child At Risk Or with Problems-(Acute and Chronic)

and maintain cardiac output over

longer period of time

SURGICAL TREATMENT (CORRECTIVE)

a. ARTERIAL SWITCH PROCEDURE

▪ procedure of choice in the 1st weeks
of life
▪ reestablishes normal circulation with
the L ventricle acting out as the
systemic pump
▪ transecting great arteries and
anastomosing the main pulmonary
artery just above the aortic valve and
anastomosing the ascending aorta to
the proximal pulmonary artery
▪ coronary arteries switched from
proximal aorta to the proximal
pulmonary artery

b. INTRAATRIAL BAFFLE REPAIR

▪ Rarely done
▪ Created to divert venous blood to the
mitral valve and pulmonary blood to
the tricuspid valve
→ Senning-use of the patient’s
atrial septum; Mustard –if
prosthetic material
▪ Continuing role of the right ventricle
as the systemic pump and the late
development of right ventricular
failure

c. RASTELLI PROCEDURE
▪ Operative choice in infants with TGA,
VSD and severe PS
▪ involves (1)closure of the VSD with a
baffle directing L ventricular blood
through the VSD into the aorta and
(2)closure of the pulmonic valve and
placement of a conduit from the R
ventricle to the pulmonary artery
▪ requires multiple conduit
replacements as the child grows

Truncus Failure of normal ▪ Mixing of oxygenated ▪ Sx: Corrective; done in the first few
Arteriosus septation and and unoxygenated months of life
division of the blood; increased
pulmonary blood flow MODIFIED RASTELLI PROCEDURE
embryonic bulbar
trunk into the  Closure of VSD and excision of
pulmonary artery from aorta and
pulmonary artery
and the aorta,

13 | P a g e
Lesson 2. Congenital Heart Defects
Chapter 5
 Instructional Module in NCM 109-A (Care of Mother
and Child At Risk Or with Problems-(Acute and Chronic)

resulting in a single attaching it to right ventricle via

vessel that overrides homograft
both ventricles  Requires additional procedures to
replace conduit as its size becomes
inadequate in relation to child growth.

Total ▪ pulmonary veins are ▪ Mixing of oxygenated ▪ Corrective repair in early infancy;
Anomalous abnormally and unoxygenated blood common pulmonary vein anastomosed to
Pulmonary connected to left atrium, ASD closed and the
venous systemic venous anomalous pulmonary venous connection
connection circuitR is ligated
ventricular
hypertrophy and L
atrium remains small
▪ Associated ASD
allows venous blood
to be shunted from
the R atrium to left
ventricle

Hypoplastic ▪ Underdevelopment ▪ Mixing of oxygenated Nonsx: Prostaglandin E1 infusion to ensure

left heart of the left side of the and unoxygenated adequate systemic blood flow
syndrome heart resulting in blood
hypoplastic left
ventricle and aortic Surgical: several staged approach
atresia 1. Norwood
▪ Associated defects: ➢ performed soon after birth
ASD, PDA ➢ anastomosis of the main pulmonary
artery to the aorta, to create a new
aorta, shunting to provide pulmonary
blood flow and the creation of a large
ASD
2. Bidirectional Glenn shunt
3. Fontan procedure
4. Heart transplantation

BROCK PROCEDURE

A palliative operation to increase pulmonary blood flow and reduce right to left shunting in tetralogy
of Fallot. It involved resection of part of the RV infundibulum using a punch or biopsy-like instrument
introduced through the right ventricle so as to reduce RV outflow tract obstruction, without VSD
closure

Rastelli procedure (operation)

An operation for repair of complete transposition of the great arteries in association with a
large VSD and pulmonic stenosis, wherein a communication is established between the LV and
the aorta via the VSD using a baffle within the RV. The RV is connected to the pulmonary artery
using a valved conduit, and the LV-PA connection is obliterated. As a consequence, the left
ventricle supports the systemic circulation. (Rastelli GC et al. Anatomic correction of
transposition of the great arteries with ventricular septal defect and subpulmonary stenosis.

14 | P a g e
Lesson 2. Congenital Heart Defects
Chapter 5
Instructional Module in NCM 109-A (Care of Mother
and Child At Risk Or with Problems-(Acute and Chronic)

NURSING DIAGNOSES FOR CYANOTIC HEART DEFECTS

 Altered cardiopulmonary tissue perfusion related to reduced pulmonary blood flow

 Decreased CO related to developing CHF
 High risk for infection related to unfiltered bacteria in the blood and sites of blood shunting that
promote bacterial growth
 Altered nutrition (less then body requirement) related to dyspnea and fatigue
 High risk for caregiver role strain related to care of a child with chronic illness
 Activity intolerance related to cyanosis and dyspnea on exertion
 Altered growth and development related to hypoxemia
 High risk for ineffective management of therapeutic regimen related to prophylactic antibiotics for
dental care procedures
 Knowledge deficit (parents) related to assessment and management of cyanotic spells with which
are unpredictable events

NURSING CARE

1. Improve tissue oxygenation and decrease oxygen consumption

➢ Humidified oxygen
➢ Monitor oxygen saturation
➢ Position:
ACYANOTIC-ORTHOPNEIC POSITION
CYANOTIC-SQUATTING/KNEE-CHEST POSITION

TET/HYPERCYANOTIC/BLUE SPELLS
 Sudden infindibular spasm↓pulmonary blood flow and ↑RL shunting
 Usually occur in the morning (on rising, there may be ↓pulmonary blood flow + increase
in CO and venous return from conditions that follow→), may be preceded by crying,
feeding, defecation and stressful procedures (IV insertion or blood drawing)
a. place in knee chest position for infants and squat for older children to relieve
chronic hypoxia
 The knee-chest position reduces the cardiac output by decreasing the venous
return from the lower extremities and by increasing the systemic vascular
resistance
b. Oxygen
c. Morphine sulfate SC or IV as prescribed-reduces infindibular spasm
(infindibulum-outflow portion of the right ventricle)
d. IV fluids as prescribed –to keep hematocrit and blood viscosity within acceptable
limits to reduce risk of CVA
e. Calm, comforting approach
f. Prevent respiratory infection
 Any condition that reduces pulmonary function can worsen hypoxemia
in the cyanotic child
1. Accurate assessment - report promptly febrile states
2. Preventive measures

15 | P a g e
Lesson 2. Congenital Heart Defects
Chapter 5
Instructional Module in NCM 109-A (Care of Mother
and Child At Risk Or with Problems-(Acute and Chronic)

a. Good handwashing, avoid persons with respiratory

infections, aggressive pulmonary hygiene
b. antibiotic prophylaxis for infective endocarditis before and
after surgical procedure for all cyanotic conditions

2. Decrease cardiac demands

➢ Modify/Organize/cluster nursing activities for uninterrupted periods of rest
➢ Anticipate needs to prevent crying especially cyanotic heart defects (the stress of crying
exhausts limited energy)
➢ Small frequent feedings, small prime nipple-decreases energy expenditure
➢ Avoid unnecessary stress-explain procedure, provide adequate rest periods and quiet
activities
➢ Prevent infection-report promptly febrile states, good handwashing, avoid persons with
respiratory infection, prevent skin breakdown
➢ Keep warm

3. Improve cardiac function

➢ Digitalis as ordered

4. Remove accumulated fluid and Na

➢ Diuretics and POSSIBLE fluid and sodium restriction

5. Maintain hydration and nutrition

6. Support child and family

a. Help child and family adjust and cope with effects of the disorder
➢ Allow time to grieve before assimilation of the meaning of the defect
➢ Effort must be made to foster attachment
 encourage to hold, rock and look at their child.
➢ Mother may feel inadequate in mothering ability because of giving birth to a child with a
defect and are unable to keep child well; father and siblings may feel neglected and
resentful.
➢ Parents feel not confident leaving the child in another’s care
 Minimized by gradually teaching someone how to care for the child.
➢ Discipline and consistent limits while in infancy (before child learns to control the family)
to prevent later problems
 Use of behavior modification techniques (rewards or social reinforcement) to
teach them how to tolerate frustration and delayed gratification
➢ Overdependency of the child because of parental fear that the child may die
 Assist parents in learning ways to foster optimum development-show parents
what activities the child can do so that they do not focus only on physical
limitations and encourage dependency.
➢ Encourage opportunities for social development.
 These children do not need to be prevented from playing with other children
(because of concern of overexertion). Children usually limit their activities if
allowed to set their own pace.
➢ Introduce to other families with similarly affected children to help them to adjust to the
daily stresses

16 | P a g e
Lesson 2. Congenital Heart Defects
Chapter 5
Instructional Module in NCM 109-A (Care of Mother
and Child At Risk Or with Problems-(Acute and Chronic)

b. Educate about the disorder

c. Prepare for surgery
1. Introduce child and family to the environment
2. Familiarize child and family with equipment and procedures
d. Provide postoperative care
1. Observe v/s
2. Maintain respiratory status
3. Monitor fluids
4. Provide rest and progressive activity – decrease cardiac workload and promote
healing; ambulation usually initiated 2nd day post-op when contraptions are
removed
5. Provide comfort and emotional support
e. Plan for discharge and home care
1. Medications
2. Activity restrictions
3. Diet and nutrition
4. Wound care (dressings, suture removal, bathing)
5. Bacterial endocarditis prophylaxis
6. Follow-up appointments
7. When to call physician: s/s of postoperative problems

References:

Murray, S and McKinney, E. (2014). Foundations of Maternal-Newborn and Women’s Health Nursing. 6th ed.
Singapore: Elsevier

Pillitteri, A. (2018). Maternal & child health nursing, care of the child bearing & childrearing family (7th ed.).
Lippincott: Williams, &Wilkins.

17 | P a g e
Lesson 2. Congenital Heart Defects
Chapter 5