CONGENITAL HEART DISEASES

PRESENTED BY
DONA MATHEW
MSC(N)
GOVT.COLLEGE OF NURSING
KOTTAYAM
1

Congenital heart defect
(CHD)
 Anatomic malformation of the heart or great
vessels which occurs during intrauterine
development, irrespective of the age at
presentation.
 Congenital heart disease occurs in
approximately 0.8% of live births.
 The incidence is higher in stillborns (3-4%),
spontaneous abortuses (10-25%), and
premature infants (about 2% excluding
patent ductusarteriosus [PDA]
2

Causes of congenital heart
defects
 Environmental factors
 Viral Infections
 rubella during the first three months of pregnancy
 Medication
 lithium (used to manage bipolor disorder), Accutane
(acne medication), some anti-seizure medications
 Alcohol
 with fetal alcohol syndrome (FAS)
 Smoking
 Cocaine
 Maternal chronic illnesses –diabetes, phenylketonuria
(PKU) and a deficiency in the B vitamin folic acid.
3

 Genetic factors
 Heredity –occur in siblings or offspring of
individuals with heart defects than those
without.
 Mutations –can affect the formation of the
heart and lead to congenital heart
malformations
 Linked with other birth defects – More than
one-third of children born with Down
syndrome have heart defects. About 25% of
girls with Turner syndrome have heart defects
4

Classification of congenital
heart disease
• Acyanotic
• Cyanotic
5

ACYANOTIC
INCREASED
PULMONARY
BLOOD FLOW
ASD,VSD,PDA,
ATRIOVENTRICU
LAR
CANAL
MALFORMATOIO
N
OBSTRUCTION
TO BLOOD
FLOW FROM
VENTRICLES
COARCTATION OF
AORTA,AORTIC
STENOSIS,
PULMONARY
STENOSIS
6

CYANOTIC
DECRESED
PULMONARY
BLODD FLOW
TETRALOGY OF FALLOT.
TRICUSPID ATRESIA
MIXED BLOOD
FLOW
TRANS POSITION OF
GREAT
ARTERIES,TOTAL
ANOMOLOUS
PULMONARYVENOUS
RETURN,TRUNCUS
ARTERIOSUS,
HYPOLPLASTIC LEFT
HEART SYNDROME
7

RELATIVE FREQUENCY OF MAJOR CONGENITAL HEART
LESIONS
LESION % OF ALL LESIONS
Ventricular septal defect 35-30
Atrial septal defect (secundum) 6-8
Patent ductus arteriosus 6-8
Coarctation of aorta 5-7
Tetralogy of Fallot 5-7
Pulmonary valve stenosis 5-7
Aortic valve stenosis 4-7
d-Transposition of great arteries 3-5
Hypoplastic left ventricle 1-3
Hypoplastic right ventricle 1-3
Truncus arteriosus 1-2
Total anomalous pulmonary venous
return
1-2
Tricuspid atresia 1-2
Single ventricle 1-2
Double-outlet right ventricle 1-2
Others 5-10 8

ACYANOTIC CONGENITAL
HEART DISEASE
9

DEFECTS WITH INCREASED
PULMONARY BLOOD FLOW
10

Atrial Septal Defect (ASD)
 An opening in the atrial septum
 An atrial septal defect allows oxygenated (red) blood to pass
from the left atrium, through the opening in the septum, and
then mix with unoxygenated (blue) blood in the right atrium
 During fetal heart devt. The partitioning process does not
occur completely, leaving an opening in the atrial septum
 Occur in 4-10% of all infants w/ CHD
 Effects: when blood passes through the ASD from the left
atrium to the right atrium  a larger volume of blood than
normal must be handled by the right side of the heart  extra
blood then passes through the pulmonary artery into the lungs
 pulmonary hypertension and pulmonary congestion
11

 If the ASD is left uncorrected-->pulmonary
hypertension progresses -->pressure in the right
side of the heart will become greater than the
left side of the heart.
 This reversal of the pressure gradient across the
ASD causes the shunt to reverse --> a right-to-
left shunt will exist. This phenomenon is known
as Eisenmenger's syndrome
 Once right-to-left shunting occurs, a portion of
the oxygen-poor blood will get shunted to the
left side of the heart and ejected to the
peripheral vascular system.
 This will cause signs of cyanosis
12

Types of atrial septal
defects
Ostium secundum atrial septal defect
 most common type of atrial septal defect
 comprises 6–10% of all congenital heart
diseases.
 Opening near the center of the septum
 The secundum atrial septal defect usually arises
from an enlarged foramen ovale, inadequate
growth of the septum secundum, or excessive
absorption of the septum primum.
 If the ostium secundum ASD is accompanied by
an acquired mitral valve stenosis, that is called
Lutembacher's syndrome.
15

Patent foramen ovale
 A small channel that has some hemodynamic
consequence
 It is a remnant of the fetal foramen ovale.
 On echocardiography, there may not be any
shunting of blood noted except when the
patient coughs.
16

Ostium primum atrial septal defect
 Opening at the lower end of the septum
 A defect in the ostium primum is occasionally
classified as an atrial septal defect but it is
more commonly classified an atrioventricular
septal defect. Ostium primum defects are less
common than ostium secundum defects
17

Sinus venosus atrial septal defect
 Opening near the junction of superior vena
cava and right atrium, may be associated
with partial anomalous pulmonary venous
connection
Common or single atrium
 It is a failure of development of the
embryologic components that contribute to
the atrial septal complex. It is frequently
associated with heterotaxy syndrome.
18

CLINICAL MANIFESTATIONS
 History.
 Infants and children with ASDs are usually
asymptomatic
 Physical Examination
 A relatively slender body build is typical.
 A widely split and fixed S2 and a systolic ejection
murmur are characteristic findings of ASD in older
infants and children. .
 Classic auscultatory findings of ASD are not
present unless the shunt is reasonably large
20

Electrocardiography
Right axis deviation of +90 to +180 degrees
and mild right ventricular hypertrophy (RVH)
or right bundle branch block (RBBB) with an
rsR' pattern inV1 are typical findings
21

 X-ray Studies
 Cardiomegaly with enlargement of the RA and
right ventricle (RV) may be present.
 A prominent pulmonary artery (PA) segment and
increased pulmonary vascular markings are seen
when the shunt is significant
22

 Echocardiography
 A two-dimensional echo study is diagnostic.
 shows the position as well as the size of the
defect,
 In secundum ASD, a dropout can be seen in the
midatrial septum.
 The primum type shows a defect in the lower atrial
septum
 Indirect signs of a significant left-to-right atrial
shunt include RV enlargement and RA
enlargement, as well as dilated PA
 M-mode echo may show increased RV dimension
and paradoxical motion of the interventricular
septum, which are signs of RV volume overload.
23

Echocardiogram in apical
four chamber view of primum
ASD
26

CONTRAST ECHO
 If agitated saline is injected into a
peripheral vein during echocardiography,
small air bubbles can be seen on
echocardiographic imaging.
 It may be possible to see bubbles travel
across an ASD either at rest or during a
cough. (Bubbles will only flow from right
atrium to left atrium if the RA pressure is
greater than LA).
27

NATURAL HISTORY OF ASD
 spontaneous closure of the secundum defect
occurs in about 40% of patients in the first 4
years of life
 Most children with an ASD remain active and
asymptomatic.
 Rarely, congestive heart failure (CHF) can
develop in infancy.
 If a large defect is untreated, CHF and
pulmonary hypertension develop in adults who
are in their 20s and 30s
 With or without surgery, atrial arrhythmias
(flutter or fibrillation) may occur in adults.
29

MANAGEMENT
 Medical
 Exercise restriction is unnecessary.
 Prophylaxis for infective endocarditis indicated
in patients with primum ASD.
 In infants with CHF, medical management is
recommended because of its high success rate
and the possibility of spontaneous closure of
the defect.
30

Nonsurgical Closure of ASD
 catheter-delivered closure device has become
a preferred method
 Devices available for clinical use have
included the Sideris buttoned device, the
Angel Wings device, the CardioSEAL device,
and the Amplatzer ASD Occlusion Device.
 The amplatzer septal occluder has the
widespread use.
31

Use of the closure device
may be indicated In
 To close a secundum ASD, measuring 5 mm
or more in diameter (but less than 32 mm),
 A significant left-to-right shunt with clinical
evidence of right ventricular volume overload
 There must be enough rim (4 mm) of septal
tissue around the defect for appropriate
placement of the device.
 The timing of the device- because of the
possibility of spontaneous closure, it is not
used in infancy unless the patient is
symptomatic with heart failure. 32

Advantages of nonsurgical
closure
 Complete avoidance of cardiopulmonary
bypass
 Avoidance of pain and residual thoracotomy
scars
 A less than 24-hour hospital stay
 Rapid recovery
 All these devices are associated with a higher
rate of small residual leak than is operative
closure.
34

Post-device closure follow-
up
 The patients are administered aspirin 81
mg/day for 6 months.
 Postprocedure echo studies
35

Surgical Closure
Indications andTiming
 A left-to-right shunt with a pulmonary-to-
systemic blood flow ratio ( p/ s) of ≥1.5:1 .
 Surgery is usually delayed until 2 to 4 years of
age because the possibility of spontaneous
closure.
 Surgery is performed during infancy-if CHF
does not respond to medical management
infancy,.
36

 Infants with associated bronchopulmonary
dysplasia and the device closure is not
considered appropriate, surgery is performed
during infancy.
 High pulmonary vascular resistance may be a
contraindication for surgery
37

Procedure.
 For secundum ASD, the defect is traditionally
repairedwith a simple suture or a pericardial
or Teflon patch through a midsternal incision
under cardiopulmonary bypass by either
 For sinus venosus defect without associated
anomalous pulmonary venous return, the
defect is closed using an autologous
pericardial patch.
38

 When it is associated with a pulmonary
venous anomaly, a tunnel is created between
the anomalous pulmonary vein and the ASD
by using aTeflon or pericardial patch
 For coronary sinus ASD, the ostium of the
coronary sinus is closed with an autologous
pericardium
39

Mortality.
 Fewer than 0.5% of patients die
Complications.
 Cerebrovascular accident
 postoperative arrhythmias
Postoperative Follow-up
 1. Cardiomegaly on x-ray film and enlarged RV
dimension on echo as well as the wide splitting of
the S2 may persist for 1 or 2 years postoperatively.
 The ECG typically demonstrates RBBB (or RV
conduction disturbance).
 2. Atrial or nodal arrhythmias occur in 7% to 20%
of postoperative patients.
 3. Rarely, patients with residual shunt may be
administered aspirin 81 mg to prevent paradoxical
embolization 41

Ventricular Septal Defect (VSD)
 an opening in the ventricular septum
 allows oxygenated blood to pass from the left
ventricle, through the opening in the septum, and
then mix with unoxygenated blood in the right
ventricle.
 VSDs are the most commonly occurring type of
congenital heart defect, occurring in 14-17 % of
babies born each year.
 occur when the partitioning process does not occur
completely, leaving an opening in the ventricular
septum.
42

 EFFECTS:
 When blood passes through the VSD from the
left ventricle to the right ventricle  a larger
volume of blood than normal must be handled
by the right side of the heart  extra blood then
passes through the pulmonary artery into the
lungs  pulmonary hypertension and pulmonary
congestion  pulmonary arteries become
thickened and obstructed due to increased
pressure
 .
Ventricular Septal Defect (VSD)
44

 IfVSD is not repaired, and lung disease
begins to occur  pressure in the right
side of the heart will eventually exceed
pressure in the left  R to L shunt 
cyanosis
 Due to high pressure --- tissue damage
may eventually occur in the right ventricle
 bacteria in the bloodstream can easily
infect this injured area  bacterial
endocarditis
45

 History
 With a small VSD, the patient is asymptomatic
with normal growth and development.
 With a moderate to large VSD, delayed
growth and development, decreased exercise
tolerance, repeated pulmonary infections, and
CHF are relatively common during infancy.
 With long-standing pulmonary hypertension,
a history of cyanosis and a decreased level of
activity may be present.
46

 Infants with small VSDs are well developed and
acyanotic.
 Before 2 or 3 months of age, infants with large
VSDs may have poor weight gain or show signs
of CHF.
 Cyanosis and clubbing may be present in
patients with Eisenmenger's syndrome
 A systolic thrill may be present at the lower left
sternal border.
 Precordial bulge and hyperactivity are present
with a large-shuntVSD.
 The S2 is loud and single in patients with
pulmonary hypertension or pulmonary vascular
obstructive disease.
47

 A grade 2 systolic murmur is audible at the
lower left sternal border .It may be
holosystolic or early systolic.
 An apical diastolic rumble is present with a
moderate to large shunt because of increased
flow through the mitral valve during diastole.
48

Electrocardiography
 With a smallVSD, the ECG is normal.
 With a moderate VSD, left ventricular
hypertrophy (LVH) and occasional left atrial
hypertrophy (LAH) may be seen.
 With a large defect, the ECG shows
biventricular hypertrophy (BVH) with or
without LAH
 If pulmonary vascular obstructive disease
develops, the ECG shows RVH only
49

 X-ray Studies
 Cardiomegaly of varying degrees is present and
involves the LA, left ventricle (LV), and sometimes RV.
 Pulmonary vascular markings increase.
 The degree of cardiomegaly and the increase in
pulmonary vascular markings directly relate to the
magnitude of the left-to-right shunt.
51

NATURAL HISTORY
 Spontaneous closure occurs in 30% to 40% of
patients with membranous VSDs and muscular
VSDs during the first 6 months of life.
 CHF develops in infants with large VSDs but
usually not until 6 to 8 weeks of age.
 Pulmonary vascular obstructive disease may
begin to develop as early as 6 to 12 months of
age in patients with large VSDs, but the resulting
right-to-left shunt usually does not develop until
the teenage years.
 Infective endocarditis rarely occurs.
53

Medical Management
 Treatment of CHF if it develops, (digoxin
and diuretics for 2 to 4 months )
 Addition of spironolactone may be helpful
to minimize potassium loss.
 Concomitant use of an afterload-reducing
agent, such as captopril,
 Frequent feedings of high-calorie
formulas, by either nasogastric tube or
oral feeding, may help.
54

 Anemia, if present, should be corrected by oral
iron therapy..
 No exercise restriction is required in the absence
of pulmonary hypertension.
 Maintenance of good dental hygiene and
antibiotic prophylaxis against infective
endocarditis are important
 Nonsurgical closure of selected muscular VSDs is
possible using the “umbrella” device, but this is
still in the experimental stage.
55

Surgical-Indications and Timing
 Small infants who have large VSDs and develop
CHF and growth retardation - If growth failure
cannot be improved by medical therapy, the VSD
should be operated on within the first 6 months
of life.
 if the PA pressure is greater than 50% of
systemic pressure, surgical closure should be
done by the end of the first year.
 After 1 year of age, a significant left-to-right
shunt with p/ s of at least 2:1 indicates that
surgical closure is needed, regardless of PA
pressure.
56

Surgical treatment
 Palliative – pulmonary artery banding
 Placing a band around the pulmonary artery
to decrease the pulmonary blood flow
 It increases the resistance to blood flow
through the pulmonary artery. Pressure
increases in the right ventricle and prevents
excess shunting from left to right
57

 Complete repair-small defects are repaired
with a purse-string approach.
 Large defects usually require a Knitted
Dacron patch sewn over the opening
 Both procedures require CPB
 The repair is generally approached through
the right atrium and tricuspid valve
 Post operative complications include residual
VSD and conduction disturbances
58

Mortality.
 Surgical mortality is less than 1%. Mortality is
higher for small infants younger than 2
months of age, infants with associated
defects, or infants with multipleVSDs
59

Postoperative Follow-up
 Activity should not be restricted unless complications
have resulted from surgery.
 The ECG shows RBBB in 50% to 90% of patients who had
VSD repair through right ventriculotomy and up to 40%
of the patients who had repair through a right atrial
approach.
 Bacterial endocarditis prophylaxis may be discontinued
6 months after surgery. If a residual shunt is present,
endocarditis prophylaxis should be continued indefinitely
when the indications arise.
 A patient with a postoperative history of transient heart
block with or without pacemaker therapy requires long-
term follow-up.
60

Patent Ductus Arteriosus (PDA)
 characterized by a connection between the aorta and the
pulmonary artery
 All babies are born with a ductus arteriosus.
 As the baby takes the first breath, the blood vessels in
the lungs open up, and blood begins to flow  the
ductus arteriosus is not needed to bypass the lungs
 Most babies have a closed ductus arteriosus by 72 hours
after birth.
 In some babies, however, the ductus arteriosus remains
open (patent) .
 The opening between the aorta and the pulmonary
artery allows oxygenated blood to pass back through the
blood vessels in the lungs.
 PDA occurs in 6-11 % of all children with CHD
61

 In many children, there is no known reason for the ductus
arteriosus remaining open. However, PDA is seen more
often in the following:
 premature infants
 infants born to a mother who had rubella during the first
trimester of pregnancy
EFFECTS:
 PDA  oxygenated blood passes from the aorta to the
pulmonary artery & mixes w/ the unoxygenated blood w/c
goes to the lungs   blood volume to the lungs 
pulmonary hypertension & congestion
 Further, because blood is pumped at high pressure through
the PDA, the lining of the pulmonary artery will become
irritated and inflamed. Bacteria in the bloodstream can
easily infect this injured area  bacterial endocarditis.
Patent Ductus Arteriosus (PDA)
63

 History
 Patients are usually asymptomatic when the
ductus is small.
 A large-shunt PDA may cause a lower
respiratory tract infection, atelectasis, and
CHF (accompanied by tachypnea and poor
weight gain).
 Exertional dyspnea may be present in
children with a large-shunt PDA
64

 Tachycardia and tachypnea may be present in
infants with CHF.
 Bounding peripheral pulses with wide pulse
pressure are characteristic findings.
 A systolic thrill may be present at the upper left
sternal border.
 A grade 1 to 4/6 continuous (“machinery”)
murmur is best audible at the left
infraclavicular area or upper left sternal
border..
develops, a right-to-left ductal shunt results in
cyanosis only in the lower half of the body
65

Electrocardiography.
 The ECG findings in PDA are similar to those in
VSD.
 A normal ECG or LVH is seen with small to
moderate PDA. BVH is seen with large PDA.
develops, RVH is present.
X-ray Studies.
 X-ray findings are also similar to those ofVSD.
 Chest x-ray films may be normal with a small-
shunt PDA.
 Cardiomegaly of varying degrees occurs in
moderate- to large-shunt PDA with enlargement
of the LA, LV, and ascending aorta. Pulmonary
vascular markings are increased.
66

 Its size can be assessed by two-dimensional
echo in a high parasternal view or in a
suprasternal notch view
67

NATURAL HISTORY
 Spontaneous closure of a PDA does not
usually occur in full-term infants and
children. This is because the PDA in term
infants results from a structural abnormality
of the ductal smooth muscle
 CHF or recurrent pneumonia or both
develop if the shunt is large.
 Pulmonary vascular obstructive disease may
develop if a large PDA with pulmonary
hypertension is left untreated.
 Infective endocarditis may occur.
 Although rare, an aneurysm of PDA may
develop and possibly rupture in adult life.
68

MANAGEMENT- IN TERM NEONATES
 Medical
 Indomethacin is ineffective in term infants
with PDA and should not be used.
 Standard anticongestive measures with
digoxin and diuretics are indicated when CHF
develops.
 No exercise restriction is needed in the
absence of pulmonary hypertension.
 Prophylaxis for subacute bacterial
endocarditis (SBE) is indicated when
indications arise.
69

Nonsurgical Closure.
 Small ductus less than 4 mm in diameter are
closed by coils
 Larger ones by an amplatzer PDA device.
70

SURGICAL CLOSURE
 Surgical closure is reserved for patients in whom
a nonsurgical closure technique is not considered
applicable
Procedure
 Ligation and division through left posterolateral
thoracotomy without cardiopulmonary bypass is
the standard procedure.
 The technique of video-assisted thoracoscopic
clip ligation has become the standard of care for
surgical management of a ductus with adequate
length

73

Patent Ductus Arteriosus in
Preterm Neonates
 Clinical evidence of PDA appears in 45% of
infants with birth weight less than 1750 g and
in about 80% of infants with birth weight less
than 1200 g.
 Significant PDA with CHF occurs in 15% of
premature infants with birth weight less than
1750 g and in 40% to 50% of those with birth
weight less than 1500 g
75

MANAGEMENT- IN PRE TERM
NEONATES
 Medical
 Fluid restriction to 120 mL/kg per day and a
diuretic (e.g., furosemide, 1 mg/kg, two to three
times a day) may be tried for 24 to 48 hours
 Pharmacologic closure of the PDA can he
achieved with indomethacin (a prostaglandin
synthetase inhibitor).
 The dose is given intravenously every 12 hours
for a total of three doses. For infants less than 48
hours old, 0.2 mg/kg is followed by 0.1 mg/kg
2 times .
76

 For those 2 to 7 days old, 0.2 mg/kg times 3, and
for infants older than 7 days, 0.2 mg/kg followed
by 0.25 mg/kg times 2
 Contraindications to the use of indomethacin
 High blood urea nitrogen (>25 mg/dl) or creatinine
(>1.8 mg/dl) levels
 Low platelet count (<80,000/mm3)
 Bleeding tendency (including intracranial hemorrhage)
 Necrotizing enterocolitis, and hyperbilirubinemia.
 A multicenter prospective study from Europe
showed that intravenous ibuprofen (10 mg/kg,
followed at 24-hour intervals by two doses of 5
mg/kg) starting on the third day of life was as
effective as indomethacin in closing the ductus in
preterm newborns..
77

ATRIOVENTRICULAR CANAL
DEFECT
 Also known as endocardial cushion defects
 They account for about 5 percent of all
congenital heart disease, and are most
common in infants with down syndrome.
 (About 15 percent to 20 percent of newborns
with down syndrome have atrioventricular
septal defects).
78

Complete atrioventricular
canal (CAVC)
 Complete atrioventricular canal (CAVC) is a
severe defect in which there is a large hole in
the the septum that separates the left and
right sides of the heart.
 The hole is in the center of the heart, where
the upper chambers and the lower chambers
meet.
 In a child with a complete atrioventricular
canal defect, there is one large valve, and it
may not close correctly.
80

Partial atrioventricular
canal defects
 The hole does not extend between the lower
chambers of the heart and the valves are
better formed.
 Partial atrioventricular canal is also called
atrioventricular septal defect, or AVSD.
81

PATHOPHYSIOLOGY
 defect in the septum
 blood to travel from the left side of the heart
to the right side of the heart, or the other way
around.
 The oxygenated and unoxygenated blood
being mixed up
 The extra blood being pumped into the lung
arteries makes the heart and lungs work
harder and the lungs can become congested.
83

CLINICAL MANIFESTATION
 Dyspnea
 MILD CYANOSIS
 A newborn baby will show signs of heart
failure such as edema, fatigue, wheezing,
sweating and irregular heartbeat
 CHARACTERSTIC MURMUR
84

MANAGEMENT
 Palliative-pulmonary artery banding
 Complete repair –
 Patch closure of septal defect
 Reconstruction of AV valve tissue
86

Coarctation of the Aorta
 Narrowing of the aorta
 can occur anywhere, but is most likely to happen in the
segment just after the aortic arch.
 This narrowing restricts the amount of blood to the
lower part of the body
 occurs in about 8-11 % of all children with CHD
88

EFFECTS:
 The left ventricle has to work harder to try to move
blood through the narrowing in the aorta  left-
sided heart failure
 BP is higher above the narrowing, and lower below
the narrowing.
 Older children may have headaches from too
much pressure in the vessels in the head, or
cramps in the legs or abdomen from too little
blood flow in that region.
 The walls of the arteries may become weakened
by high pressure  spontaneous tears  cause a
stroke or uncontrollable bleeding.
  risk for bacterial endocarditis.
89

SYMPTOMATIC INFANTS
 History.
 Poor feeding, dyspnea, and poor weight gain
or signs of acute circulatory shock may
develop in the first 6 weeks of life.
 Infants with COA are pale and experience
varying degrees of respiratory distress.
 Oliguria or anuria, general circulatory shock,
and severe acidemia are common.).
 Peripheral pulses may be weak and thready
as a result of CHF.
92

 A blood pressure differential may become
apparent only after improvement of cardiac
function with administration of rapidly acting
inotropic agents.
 The S2 is single and loud; a loud S3 gallop is
usually present.
 No heart murmur is present in 50% of sick
infants.
 A nonspecific ejection systolic murmur is
audible over the precordium.
93

 Electrocardiography.
 A normal or rightward QRS axis and RVH or
right bundle branch block (RBBB) are present
X-ray Studies.
 Marked cardiomegaly and pulmonary edema
or pulmonary venous congestion are usually
present
94

The blue arrow to the actual coarctation
and the green arrow to the post-stenotic dilation of the
descending aorta.
95

Echocardiography
 Two-dimensional echo and color flow
Doppler studies usually show the site and
extent of the coarctation.
 In the suprasternal notch view, a thin wedge-
shaped “posterior shelf” is imaged in the
posterolateral aspect of the upper
descending aorta
96

Natural History
 About 20% to 30% of all patients with COA
develop CHF by 3 months of age.
 If it is undetected or untreated, early death
may result from CHF and renal shutdown in
symptomatic infants
98

Management
Medical
 In symptomatic neonates, PGE1 infusion should
be started to reopen the ductus arteriosus and
establish flow to the descending aorta and the
kidneys during the first weeks of life.
 Intensive anticongestive measures with short-
acting inotropic agents (e.g., dopamine,
dobutamine), diuretics, and oxygen should be
started.
 Balloon angioplasty can be a useful procedure
for sick infants in whom standard surgical
management carries a high risk.
99

Surgical
 Indications andTiming
 If CHF or circulatory shock develops early in life,
surgery should be performed on an urgent basis.
Procedures
 Resection and end-to-end anastomosis consists
of resecting the coarctation segment and
anastomosing the proximal and distal aortas .
 Subclavian flap aortoplasty consists of dividing
the distal subclavian artery and inserting a flap of
the proximal portion of this vessel between the
two sides of the longitudinally split aorta
throughout the coarctation segment.
100

 With patch aortoplasty, the aorta is opened
longitudinally through the coarctation
segment and extending to the left subclavian
artery, and the fibrous shelf and any existing
membrane are excised. An elliptic woven
Dacron patch is inserted to expand the
diameter of the lumen.
101

ASYMPTOMATIC INFANTS AND CHILDREN
 Management
 Medical
 Children with mild COA should be watched
closely for hypertension in the arm or for
increasing pressure differences between the
arm and leg.
 Balloon angioplasty
 A balloon-expandable stainless-steel stent
implanted concurrently with balloon
angioplasty
 An absorbable metal stent is in the
experimental stage
103

 Surgical
 Indications andTiming
 COA with hypertension in the upper
extremities or with a large systolic pressure
gradient equal to or greater than 20 mm Hg
between the arms and the legs indicates that
elective surgical correction is necessary
between the ages of 2 and 4 years.
 Reduction of aortic diameter by 50% at the
level of COA is also an indication for surgery.
 Older children are operated on soon after the
diagnosis is made.
104

 In asymptomatic children, surgery is
performed by age 4 to 5; late surgery may
increase the risk of developing early essential
hypertension.
 If severe hypertension, CHF, or cardiomegaly
is present, surgery is performed at an earlier
age.
105

Surgical Procedures
 Resection of the coarctation segment and
end-to-end anastomosis
 Occasionally, subclavian artery aortoplasty or
circular or patch grafts may be performed.
106

AORTIC STENOSIS
 Narrowing or stricture o the aortic valve
 Resistance to blood flow in the left ventricle,
decreased cardiac out put, left ventricular
hypertrophy and pulmonary vascular
congestion
 Valvular stenosis is the most common type
and is usually caused by malformed cusps
 Sub valvular stenosis is a stricture caused by
a fibrous ring below the normal valve
 Supra valvular stenosis occurs infrequently
107

Pathophysiology
 A stricture in the aortic outflow tract -->
resistance to ejection of blood from left
ventricle -> extra work load of the left
ventricle -> hypertrophy -> left ventricular
failure -> left atrial pressure increases ->
increased pressure in the pulmonary veins ->
pulmonary edema
109

 History
 Neonates with critical or severe stenosis of the
aortic valve may develop signs of hypoperfusion
or respiratory distress related to pulmonary
edema within days to weeks after birth.
 Most children with mild to moderate AS are
asymptomatic. Occasionally, exercise
intolerance may be present.
 Exertional chest pain, easy fatigability, or
syncope may occur in a child with a severe
degree of obstruction
110

 Infants and children with AS are acyanotic
and are normally developed.
 blood pressure is normal in most patients, but
a narrow pulse pressure is present in severe
AS.
 A systolic thrill may be palpable at the upper
right sternal border, in the suprasternal
notch, or over the carotid arteries.
 An ejection click may be heard with valvular
AS.
111

 Newborns with critical AS may develop signs
of reduced peripheral perfusion (with weak
and thready pulses, pale cool skin, and slow
capillary refill)
112

 In mild cases the ECG is normal. LVH with or
without strain pattern may be present in
severe cases
 X-ray Studies
 The heart size is usually normal in children,
but a dilated ascending aorta or a prominent
aortic knob may be seen occasionally in
valvular AS, resulting from poststenotic
dilatation.
 Significant cardiomegaly does not develop
unless CHF occurs later
113

NATURAL HISTORY
 Chest pain, syncope, and even sudden death
(1% to 2% of cases) may occur in children
with severe AS.
 Heart failure occurs with severe AS during the
newborn period or later in adult life.
 Mild stenosis becomes more severe with time
in a significant number of patients
115

MANAGEMENT
 Medical
 For critically ill newborns with CHF
 patients are stabilized before surgery
 balloon valvuloplasty
 use of rapidly acting inotropic agents and diuretics
to treat CHF
 intravenous infusion of PGE1 to reopen the
ductus.
 Percutaneous balloon valvuloplasty is now
regarded as the first step in the management
of symptomatic neonates
116

 Surgical
 Valvular AS
 Closed aortic valvotomy, using calibrated
dilators or balloon catheters without
cardiopulmonary bypass, may be performed
in sick infants if balloon valvuloplasty has
been unsuccessful or if it is not available
 Aortic valve commissurotomy is usually tried
if stenosis is the predominant lesion
 Aortic valve replacement may be necessary if
AR is the predominant lesion
117

 Subvalvular AS- Excision of the membrane is
done for discrete subvalvular AS
 Supravalvular AS- a reconstructive surgery is
done using aY-shaped patch
118

 Postballoon and Postoperative Follow-up
 An annual follow-up examination is necessary for all
patients who have the aortic valve balloon procedure or
surgery in order to detect development of stenosis or
regurgitation.
 Anticoagulation is needed after a prosthetic mechanical
valve replacement.
 The International Normalized Ratio (INR) should be
maintained between 2.5 and 3.5 for the first 3 months
and 2.0 to 3.0 beyond that time.
 Low-dose aspirin (75 to 100 mg/day for adolescents) is
indicated in addition to warfarin (American College of
Cardiology, 2006).
 After aortic valve replacement with a bioprosthesis and
no risk factors, aspirin (75 to 100 mg), but not warfarin, is
indicated.
 Restriction from competitive, strenuous sports may be
necessary for children with moderate residual AS or AR,
or both 119

PULMONARY VALVE STENOSIS
 Narrowing at the entrance of pulmonary
artery
 Resistance to blood flow causes right
ventricular hypertrophy
 Pulmonary atresia is the extreme form – total
fusion of commissures and no blood flows to
the lungs
 rt .Ventricle may be hypoplastic
120

Pathophysiology
 PS--> Resistance to blood flow-->RVH
 If Rt . Ventricular failure develops
-->increase in rt, atrial pressure -->re-opening
of foramen ovale-->shunting of un oxy. blood
in to the Lt.atrium--> systemic cyanosis
122

Clinical manifestations
 May be asymptomatic
 Some had mild cyanosis
 Newborn with severe narrowig- cyanotic
 Characteristic murmur
 Cardiomegaly on chest radiograph
 Pt are at risk for infective endocarditis
123

Management
 Surgical
 In infants trans ventricular valvotomy (brock
procedure)
 In children pulmonary valvotomy with CPB
 Non surgical treatment
 Balloon angioplasty
124

DEFECTS WITH DECREASED
PULMONARY BLOOD FLOW
126

Tetralogy of Fallot (TOF)
 a complex condition of several congenital defects that occur
due to abnormal devt. of the fetal heart during the first 8
weeks of pregnancy.These problems include the following:
1. ventricular septal defect (VSD)
2. Pulmonary valve stenosis
3. overriding aorta - The aorta sits above both the left and
right ventricles over the VSD, rather than just over the left
ventricle. As a result, oxygen poor blood from the right
ventricle can flow directly into the aorta instead of into the
pulmonary artery to the lungs.
4. Right ventricular hypertrophy - The muscle of the right
ventricle is thicker than usual because of having to work
harder than normal.
127

 EFFECTS:
 If the right ventricle obstruction is severe, or if the
pressure in the lungs is high  a large amount of
oxygen-poor (blue) blood passes through the VSD, mixes
with the oxygen-rich (red) blood in the left ventricle, and
is pumped to the body  cyanosis
 The more blood that goes through the VSD, the less
blood that goes through the pulmonary artery to the
lungs   oxygenated blood to the left side of the
heart.
 Soon, nearly all the blood in the left ventricle is oxygen-
poor (blue). This is an emergency situation, as the body
will not have enough oxygen to meet its needs.
Tetralogy of Fallot (TOF)
129

 History
 A heart murmur is audible at birth.
 Most patients are symptomatic with cyanosis
at birth or shortly thereafter. Dyspnea on
exertion or hypoxic spells develop later, even
in mildly cyanotic infants.
 Immediately after birth, severe cyanosis is
seen in patients with TOF and pulmonary
atresia.
130

 Varying degrees of cyanosis, tachypnea, and
clubbing (in older infants and children) are
present.
 An RV tap along the left sternal border and a
systolic thrill at the upper and mid-left sternal
borders are commonly present (50%).
 An ejection click that originates in the aorta
may be audible
131

 Electrocardiography
 Right axis deviation (RAD) (+120 to +150
degrees) is present in cyanoticTOF.
 RVH is usually present
 BVH may be seen in the acyanotic form. RAH
is occasionally present.
132

X- ray
 Decreased pulmonary markings
 Black lung fields
 Boot shaped heart
133

NATURAL HISTORY
 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 stenosis and polycythemia.
 Polycythemia develops secondary to cyanosis.
 Hypoxic spells may develop in infants.
 Growth retardation may be present if cyanosis is
severe.
134

 HYPOXIC SPELL
 Hypoxic spells are characterized by a
paroxysm of hyperpnea (i.e., rapid and deep
respiration), irritability and prolonged crying,
increasing cyanosis, and decreasing intensity
of the heart murmur.
 Hypoxic spells occur in infants, with a peak
incidence between 2 and 4 months of age.
 These spells usually occur in the morning
after crying, feeding, or defecation.
 A severe spell may lead to limpness,
convulsion, cerebrovascular accident, or even
death.
135

Treatment of the hypoxic
spell
 The infant should be picked up and held in a
knee-chest position.
 Morphine sulfate, 0.2 mg/kg administered
subcutaneously or intramuscularly, suppresses
the respiratory center and abolishes hyperpnea
 Oxygen is usually administered, but it has little
demonstrable effect on arterial oxygen
saturation.
 Acidosis should be treated with sodium
bicarbonate (NaHCO3), 1 mEq/kg administered
intravenously. The same dose can be repeated in
10 to 15 minutes..
138

 MANAGEMENT OFTOF
 Medical
 educate parents to recognize the spell and know
what to do.
 Oral propranolol therapy, 0.5 to 1.5 mg/kg every
6 hours, is occasionally used to prevent hypoxic
spells while waiting for an optimal time for
corrective surgery
 Balloon dilatation of the right ventricular outflow
tract and pulmonary valve, it is not widely
practiced,
 Maintenance of good dental hygiene and
practice of antibiotic prophylaxis against SBE are
important .
 A relative iron deficiency state should be
detected and treated
140

 Surgical
 Palliative Shunt Procedures
 Classic Blalock-Taussig shunt, anastomosed
between the subclavian artery and the ipsilateral
PA, is usually performed for infants older than 3
months because the shunt is often thrombosed
in younger infants with smaller arteries
141

 Modified Blalock-Taussig (BT) shunt. A
Gore-Tex interposition shunt is placed
between the subclavian artery and the
ipsilateral PA. This is the most popular
procedure for any age, especially for small
infants younger than 3 months of age
143

 TheWaterston shunt, anastomosed between
the ascending aorta and the right PA, is no
longer performed because of a high incidence
of surgical complications
145

 The Potts operation
Anastomosed between the descending aorta
and the left PA, is no longer performed
146

 Complete Repair Surgery
 Usually done in the first year of life
 Total repair of the defect is carried out under
cardiopulmonary bypass
 The procedure includes patch closure of the
VSD, preferably through a transatrial and
transpulmonary artery approach
 widening of the RVOT by division and/or
resection of the infundibular tissue; and
pulmonary valvotomy,).
 The operative mortality for total correction of
TOF is 5%
147

Tricuspid Atresia
 In this condition, there is no tricuspid valve, therefore, no
blood flows from the right atrium to the right ventricle.
 Blood in right atrium  foramen ovale  left atrium and
left ventricle  aorta
 There is complete mixing of the oxy. Blood and un oxy.
blood
 Tricuspid atresia defect is characterized by the following:
 a small right ventricle
 a large left ventricle
 SmallVSD and PDA
 diminished pulmonary circulation
 cyanosis - bluish color of the skin and mucous
membranes caused from a lack of oxygen.
149

History
 Cyanosis is usually severe from birth.
 Tachypnea and poor feeding usually manifest.
 History of hypoxic spells may be present in infants
with this condition.
Physical Examination
 Cyanosis, either with or without clubbing, is always
present.
 A systolic thrill is rarely palpable when associated
with PS.
 A grade 2 to 3/6 holosystolic (or early systolic)
murmur ofVSD is usually present at the lower left
sternal border
151

Electrocardiography
 LVH is usually present;
 RAH or biatrial hypertrophy (BAH) is
common.
X-ray Studies.
 The heart size is normal or slightly increased,
with enlargement of the RA and LV
Echocardiography.
 Absence of the tricuspid orifice, marked
hypoplasia of the RV, and a large LV can be
imaged in the apical four-chamber view.
152

MANAGEMENT
 Initial Medical Management
 PGE1 should be started in neonates with severe
cyanosis to maintain the patency of the ductus
before planned cardiac catheterization or
cardiac surgery.
 The Rashkind procedure (balloon atrial
septostomy) may be performed as part of the
initial catheterization to improve the RA-to-LA
shunt, especially when the interatrial
communication is considered inadequate by
echo studies.
154

 Surgical.
 Most infants with tricuspid atresia require
one or more palliative procedures before a
Fontan-type operation
 Ideal candidates for a Fontan-type operation
are those who have normal LV function and
low pulmonary resistance
 Stage I
 Blalock-Taussig shunt, when PBF is small
 This procedure results in the volume load on
the LV because the LV supplies blood to both
the systemic and pulmonary circulations.
155

 Damus-Kaye-Stansel and shunt operation
the aorta and pulmonary artery are joined using a
patch (pink).The blue tube is known as a Modified
Blalock-Taussig Shunt.
156

 Pulmonary artery banding. PA banding is
rarely necessary for infants with CHF resulting
from increased PBF. PA banding protects the
pulmonary vasculature from developing
pulmonary hypertension
 Medical follow-up after stage I.Watch for:
 Cyanosis (O2 saturation <75%)—cardiac
catheterization or MRI to find out its cause.
 Poor weight gain (CHF from too much PBF)—
tightening of PA band may be necessary.
157

 Stage II (at 3 months or by 6 months).
 Bidirectional Glenn operation (BDG).
 also called bidirectional superior
cavopulmonary shunt
 An end-to-side SVC-to-RPA shunt (also
called bidirectional superior cavopulmonary
shunt) can be performed by 2.5 to 3 months
of age
 This procedure satisfactorily increases
oxygen saturation
158

 The hemi-Fontan operation.
 SVC is connected to PA.
 Blood coming back from the upper body now
flows directly to the lungs without going through
the heart
 The Blalock-Taussig (BT) shunt is removed.
 A patch is placed over the top part of the heart's
right upper chamber . This prevents blood from
the upper body from entering the heart and
blood from the lower body from entering the
lungs. It also maintains a connection that is used
for the final stage of the repair and greatly
simplifies the last operation.
160

 Medical follow-up after stage II.Watch for
the following:
 A gradual decrease in O2 saturation (<75%)
may be caused by: .
 Opening of venous collaterals
 Pulmonary AV fistula (due to the absence of
hepatic inhibitory factor)
 Transient hypertension—1 to 2 weeks
postoperatively—may use ACE inhibitors
162

 Stage III ( Fontan operation)—within 1 to 2
years after stage II operation
 The whole premise of the Fontan operation is
directing the entire systemic venous blood to
the pulmonary arteries without an
intervening pumping chamber.
 The Fontan operation is usually completed
when the child is around 2 years of age. This
procedure can even be performed on infants.
163

Postoperative Medical
Follow-up
 Patients should maintain a low-salt diet.
 Medications:
 Some patients need continued digoxin and diuretic
therapy.
 An angiotensin-converting enzyme (ACE) inhibitor is
generally recommended.
 Aspirin (or even warfarin) is used to prevent
thrombus formation in the RA.
 Patients should not participate in competitive,
strenuous sports.
 Antibiotic prophylaxis against SBE should be
observed when indications arise.
165

MIXED BLOOD FLOW
Difference in the PA pressure and aortic pressure--> pulmonary
congestion
Volume load in ventricles -->decresed cardiac out put
Relative desaturation of systemic blood,
Mixing of blood from pulmonary and systemic circulation within
the heart chambers
166

Transposition of the Great Arteries
(TGA)
 the aorta is connected to the right ventricle, and the pulmonary artery is
connected to the left ventricle
 Oxygen-poor (blue) blood returns to the right atrium from the body 
passes through the right atrium and ventricle,  into the misconnected
aorta back to the body.
 Oxygen-rich (red) blood returns to the left atrium from the lungs 
passes through the left atrium and ventricle,  into the pulmonary artery
and back to the lungs.
 Other heart defects are often associated withTGA
- atrial or ventricular septal defect
- may be necessary in order for the infant withTGA to survive
- Allow mixing of blood – providing at least smaller amounts of oxygen
to the body
167

 History
 History of cyanosis from birth is always
present.
 Signs of congestive heart failure (CHF) with
dyspnea and feeding difficulties develop
during the newborn period.
169

 Moderate to severe cyanosis is present,
 The S2 is single and loud..
 If CHF supervenes, hepatomegaly and
dyspnea develop.
170

Electrocardiography
 There is a rightward QRS axis (i.e., +90 to
+200 degrees).
 Right ventricular hypertrophy (RVH) is
usually present
 Biventricular hypertrophy (BVH) may be
present
 Occasionally right atrial hypertrophy (RAH) is
present.
171

Echocardiography.
 In the parasternal short-axis view, the “circle
and sausage” appearance of the normal great
arteries is not visible. Instead, the great
arteries appear as “double circles”
 3. In the apical and subcostal five-chamber
views, the PA arises from the LV, and the
aorta arises from the RV.
172

 X-ray Studies
 Cardiomegaly
 An egg-shaped cardiac silhouette
174

NATURAL HISTORY
 Without surgical intervention, death occurs in
90% of patients before they reach 6 months
of age.
175

MANAGEMENT
 Medical
 Arterial blood gases and pH should be
obtained and metabolic acidosis should be
corrected
 PGE1 infusion should be started to improve
arterial oxygen saturation by reopening the
ductus.
 Oxygen administration
 A balloon atrial septotomy (Rashkind
procedure) may be performed to increase the
mixing by opening the atrial septum
176

Surgical
 An arterial switch procedure- performed in
first week of life
 Transecting the great arteries and
anastomosing the main pulmonary artery to
the proximal aorta and anastomosing the
ascending aorta to the proximal pulmonary
artery
 The coronary arteries are switched from
proximal aorta to the proximal pulmonary
artery to create a new aorta
177

 Intra atrial baffle repairs –An intraatrial baffle
is created to divert the venous blood to the
mitral valve and pulmonary venous blood to
the tricuspid valve using using patients atrial
septum or a prosthetic valve.
179

 Rastelli procedure-operative choice in
patients withTGA,VSD, and severe pulmonic
stenosis.
 It involves closure ofVSD with baffle->LV
blood directed throughVSD in to aorta
 Pulmonic valve is then closed , and a conduit
is placed from RV to PA
180

Total Anomalous Pulmonary
Venous Return
 No direct communication between the pulmonary
veins and the left atrium
 Drain anomalously into the systemic venous
tributeries or into right atrium
4 types
 Supracardiac:( 50% of TAPVR ). The common
pulmonary venous sinus drains into the right SVC
 Cardiac: (20% of TAPVR) .The common
pulmonary venous sinus drains into the coronary
sinus
181

 Infracardiac:( 20% of TAPVR patients) The
common pulmonary venous sinus drains to
the portal vein, ductus venosus, hepatic vein,
or inferior vena cava (IVC).
 Mixed type: This type, which is a
combination of the other types, accounts for
10% ofTAPVR patients
182

TOTALANOMALOUS PULMONARYVENOUS
RETURN – Clinical Manifestations
History
 CHF with gowth retardation
 Frequent pulmonary infection
 Mild cyanosis
Physical examination
 Undernourished
 Signs of CHF
 Precordial bulge
ECG
 RVH and occational RAH
184

TOTALANOMALOUS PULMONARYVENOUS
RETURN – Clinical Manifestations
X- ray studies
 Cardiomegaly
 Kerley B lines
 Snowmans sign/ figure of 8
Echo
A large RA and a small LA, with deviation of
the atrial septum to the left and dilated
PAs, are also present.
185

MANAGEMENT
 medical
 Intensive anticongestive measures
 Metabolic acidosis should be corrected,
 Infants with severe pulmonary edema-should
be intubated and receive ventilator support
 In some patients with pulmonary
hypertension, PGE1 can increase systemic
flow by keeping the ductus open
187

Surgical treatment
Supracardiac Type.
 A large, side-to-side anastomosis is made
between the common pulmonary venous
sinus and the LA. The ASD is closed with a
cloth patch.
TAPVR to the Right Atrium.
 The atrial septum is excised and a patch is
sewn in such a way that the pulmonary
venous return is diverted to the LA . The
ASD may have to be enlarged.
188

TAPVR to the Coronary Sinus.
 An incision is made in the anterior wall of the
coronary sinus (“unroofing”) to make a
communication between the coronary sinus
and the LA. A single patch closes the original
ASD and the ostium of the coronary sinus. This
results in the drainage of coronary sinus blood
with low oxygen saturation into the LA
 InfracardiacType.
 A large vertical anastomosis is made between
the common pulmonary venous sinus and the
LA. The common pulmonary vein, which
descends vertically to the abdominal cavity, is
ligated above the diaphragm
189

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.
 Usually accompanied by a ventricular septal defect
EFFECTS:
 oxygen-poor (blue) and oxygen-rich (red) blood mix back and forth through
the ventricular septal defect.
 This mixed blood then flows through the common truncal vessel. Some of it
will flow to pulmonary artery and on to the lungs, and some of the mixed
blood will go into the aortic branch and to the body.
 The mixed blood that goes to the body does not have as much oxygen as
normal, and will cause varying degrees of cyanosis
190

 History
 Cyanosis may be seen immediately after
birth.
 Signs of CHF develop within several days to
weeks after birth.
 History of dyspnea with feeding, failure to
thrive, and frequent respiratory infections is
usually present in infants.
192

 Varying degrees of cyanosis and signs of CHF
with tachypnea and dyspnea are usually
present.
 The peripheral pulses are bounding, with a
wide pulse pressure.
 A systolic click is frequently audible at the
apex and upper left sternal border.
193

 BVH is present in 70% of cases
 X-ray Studies.
 Cardiomegaly is usually present
 Echocardiography.
 A large, single great artery arises from the
heart .The type of persistent truncus
arteriosus can be identified, and the size of
the PAs can be determined.
194

Medical
 Vigorous anticongestive measures with
digitalis and diuretics
surgical
 Corrective repair
 Closing theVSD
 Excising the pul. Arteries from aorta , and
attaching them to the RV by means of
homograft
Prognosis
Mortality greater than 10%
195

Hypoplastic Left Heart
Syndrome
 HLHS occurs in 1% of all congenital heart
defects or 9% of such defects in critically ill
newborns
 Underdevelopment of the left side of the heart
resulting in a hypoplastic left ventricle and
aortic atresia
 Most of the blood from LA Flows across PFO
to RA RV PA
 The descending aorta receives blood from pda
supplying systemic blood flow
196

Effects
 PFO allows saturated blood from LA to mix
with desaturated blood from RA-->RV -->PA
 from PA blood flows to lung and then
through ductus arteriosus in to the aorta and
out f the body
 The coronary and cerebral vessels receive
blood by retrograde flowthrough the
hypoplastic ascending aorta
198

 A neonate with HLHS becomes critically ill within
the first few hours to the first few days of life.
 Tachycardia, dyspnea, pulmonary crackles, weak
peripheral pulses, and vasoconstricted
extremities are characteristic.
 The patient may not have severe cyanosis but
has a grayish blue color of the skin with poor
perfusion.
 The S2 is loud and single..
 The ECG almost always shows RVH.
 Chest x-ray films characteristically show
pulmonary venous congestion or pulmonary
edema
 Arterial blood gas levels reveal a slightly
decreased Po2 and a normal Pco2. 199

Therapeutic management
 PGE1 infusion to maintain ductal patency
 Surgical procedure
 Several staged approach
 1st stage- Norwood procedure
 Anastomosis of main PA to the aorta to create a
new aorta, placement of a shunt or inserting a
conduit from the RV to PA to provide pulmonary
blood flow , and creation of a large ASD.
 2nd stage-Bidirectional Glenn Shunt
 Done at 6-9 months of age to relieve cyanosis
and reduce overload in RV
200

 Final repair-Modified Fontan Procedure
 Transplantation
202

Ebstein's Anomaly
 Ebstein anomaly is a congenital heart
defect in which the septal leaflet of
the tricuspid valve is displaced towards the
apex of theright ventricle of the heart.
 The valve leaflets, however, are to a varying
degree, attached to the walls and septum of
the right ventricle.
 There is subsequent 'atrialization' of a portion
of the morphologic right ventricle
203

Pathophysiology
 The right ventricle is thus divided into 2 parts
by the abnormal tricuspid valve
 the 1st, a thin-walled “atrialized” portion, is
continuous with the cavity of the right
atrium;
 the 2nd, often smaller portion consists of
normal ventricular myocardium.
 The right atrium is enlarged as a result of
tricuspid valve regurgitation,

205

 In more severe forms of Ebstein anomaly, the
effective output from the right side of the
heart is decreased due to a combination of
the poorly functioning small right ventricle,
tricuspid valve regurgitation, and obstruction
of the right ventricular outflow tract
produced by the large, sail-like, anterior
tricuspid valve leaflet.
 The increased volume of right atrial blood
shunts through the foramen ovale (or
through an associated atrial septal defect) to
the left atrium and produces cyanosis
206

 History
 In severe cases, cyanosis and CHF develop
during the first few days of life.
 Children with milder cases may complain of
dyspnea, fatigue, cyanosis, or palpitation on
exertion.
207

 Mild to severe cyanosis is present, as well as
clubbing of the fingers and toes in older infants
and children.
 Characteristic triple or quadruple rhythm is
audible.
 Electrocardiography
 1. Characteristic ECG findings of RBBB and RAH
are present in most patients with this condition
 x-ray Studies.
 . In severe cases, an extreme cardiomegaly with
a balloon-shaped heart and decreased
pulmonary vascular markings are present
208

 The tricuspid valve leaflets are elongated,
redundant, and dysplastic
 A large RA, including the atrialized RV, and a
small functional RV represent anatomic
severity
209

MANAGEMENT
 Medical
 In severely cyanotic newborns, intensive
treatment with mechanical ventilation,
 PGE1 infusion
 inotropic agents
 correction of metabolic acidosis
210

Surgical
 Palliative procedures
 Blalock-Taussig shunt
 Starnes operation- a procedure to reduce the
RV or RA
 Classic Glenn anastomosis or its modification
may be considered in severely cyanotic infants
211

 Definitive procedures
 Two-ventricular repair
 Danielson technique: For repair of the
tricuspid valve
 Carpentier technique: This repair also
plicates the atrialized portion of the RV and
the tricuspid annulus
 One-ventricular repair:
 For patients with inadequate size of the RV,
a Fontan-type operation is usually
performed in stages following the initial
palliative procedures such as bidirectional
Glenn operation
212

Nursing diagnosis
 Impaired gas exchange related to altered pulmonary blood flow
or oxygen deprivation
 Altered cardiac output related to specific anatomic defect
 Activity intolerance related to decreased oxygenation in blood
and tissues
 Altered Nutrition: less than body requirements related to the
excessive energy demands required by increased cardiac
workload
 Increased potential for infection related to poor nutritional status
 Anxiety related to diagnostic procedures and hospitalization
 Developmental delay related to decreased energy, inadequate
nutrition, physical limitations and social isolation
 Alteration in parenting related to parental perception of the child
as vulnerable
213

 Relieve the respiratory distress associated with increased pulmonary
blood flow or oxygen deprivation
 Determine degree of respiratory distress
 Position child at 45 degree angle to decrease pressure of the
viscera on the diaphragm and increase lung volume
 Pin diapers loosely and provide loose-fitting pajamas for older
children
 Feed slowly
 Tilt infant’s head slightly
 Suction the nose and throat if unable to cough out secretions
 Provide oxygen therapy as needed
 Improve oxygenation o that the body functions may be
maintained
 Provide effective oxygen environment
 Observe response to oxygen therapy
 Observe response to oxygen weaning therapy
 Relieve Hypoxic spells associated with cyanotic types of
Congenital heart disease
 Observe for “tet” spells
 Encourage fluid intake
 Obtain vital signs
215

Nursing interventions
 A. Provide adequate nutritional and fluid
intake to maintain the growth and
developmental needs of the child
 Feed in semi-erect position
 Provide small frequent feedings
 Provide foods with high nutritional value
 Determine child’s likes and dislikes
 Strict input and output
 Daily weight
216

 B. Prevent infection
 Prevent exposure to communicable diseases
 Immunizations should be up-to-date
 Handwashing should be observed
 Be certain that the child receives prophylactic
medication for infective endocarditis
217

 C. Reduce the workload of the heart since
decreased activity and expenditure of energy
will decrease oxygen requirements
 Uninterrupted rest
 Avoid unnecessary activities
 Prevent excessive crying
 Provide diversional activities
 Prevent constipation
218

 D. Observe child for symptoms of Congestive
Heart Failure that occur frequently as a
complication of Congenital Heart Disease
 E. Observe for the development of symptoms of
infective endocarditis that may occur as a
complication of congenital heart disease
 F. Observe for the development of thrombosis
that may occur as a complication of congenital
heart disease
 G. Prepare the child for diagnostic and treatment
procedures
 H. Explain cardiac problems to child and parents
219

Health Education
 A. Instruct the family in necessary measures
to maintain the child’s health
 B.Teach the family about the defect and its
treatment
 C. Encourage the parents and other persons
to treat child in a normal manner as possible
220

CONGENITAL HEART DISEASES

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